50 Cases in Cardiology

50 Cases in
Clinical Cardiology
50 Cases in
Clinical Cardiology
A Problem Solving Approach
Atul Luthra MBBS MD DNB

Diplomate
National Board of Medicine
Physician and Cardiologist
New Delhi, India
www.atulluthra.in
atulluthra@sify.com
Foreword
JPS Sawhney
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50 Cases in Clinical Cardiology: A Problem Solving Approach
First Edition: 2014
ISBN 978-93-5152-110-5
Printed at
Dedicated to
My Parents
Ms Prem Lata Luthra
and
Mr Prem Prakash Luthra
Who guide and bless me
from heaven
Foreword
With the widespread availability of sophisticated cutting-edge technology, the

clinician’s approach towards the diagnosis of heart disease has undergone a
paradigm shift. These days, it is not uncommon for the patient to be wheeled into
the ECHO-room or even the cath-lab, without anyone taking a medical history
or even caring to place a stethoscope over the patient’s precordium. This is not a
good sign since history-taking along with clinical examination should continue
to occupy their rightful place in the practice of bedside cardiology. Moreover, a
wealth of information is available in simple diagnostic modalities such as the
ECG and X-ray chest, which should be interpreted in the light of clinical data.
I must compliment Atul Luthra for this brilliant compilation of a wide variety
of real-world clinical situations, encountered during the practice of cardiology.
He has elegantly discussed each case and solved the clinical problem in a
meticulous way. The section on discussion incorporates a bewildering array of
high-quality ECG strips, X-ray films and ECHO images. Students preparing for
their examinations, resident doctors working in cardiac units and clinicians
involved in heart-care are bound to benefit from this book. I wish Atul and his
excellent book, all the best.
JPS Sawhney
Chief of Clinical Cardiology
Chairman, Department of Cardiology
Sir Ganga Ram Hospital, New Delhi, India
www.preventivecardiology.in
Preface
Present-day cardiology is replete with a bewildering array of sophisticated

investigative techniques, that have eclipsed the art of arriving at a diagnosis
on the bedside of the patient. Yet, a relevant medical history and a meticulous
physical examination are indispensable tools to mentally construct a plausible
clinical diagnosis. Further, simple but informative investigations such as
electrocardiography (ECG), chest radiography (X-ray) and echocardiography
(ECHO), have withstood the test of time in clinical cardiology. Moreover, they are
cost-effective in resource-sensitive settings and can be performed at the patient’s
bedside.
It gives me immense pleasure to proudly present 50 Cases in Clinical
Cardiology: A Problem Solving Approach, a compilation of real-world situations
in clinical cardiology. Each case is introduced with a brief history and findings
on physical examination. The clinical problem is then discussed analytically
and ultimately solved with the aid of one or more simple bedside investigations.
The case concludes with pertinent management issues along with some recent
advances in diagnostics and therapeutics pertaining to that clinical entity. The
text is suitably complemented by impressive illustrations of ECG strips, chest
X-rays and ECHO images.
I have tried to incorporate most clinical situations encountered in heart clinics
and cardiology ward-rounds, but there might be some omissions. Nevertheless,
I sincerely hope that the wealth of clinical material on cardiac symptoms, physical
signs and auscultatory findings, will rekindle the romance between the clinician
and clinical cardiology. This book should be most useful for cardiology students
preparing for examinations, resident doctors working in cardiac units as well as
for physicians involved in the care of heart patients.
Atul Luthra
Acknowledgments
I am extremely grateful to:

• My teachers in school, who helped me to acquire good command over spoken
and written English language.
• My lecturers and professors in medical college, who taught me the science
and art of bedside cardiology.
• My heart patients, whose findings on clinical examination and results of
investigations made me wiser.
• Learned authors of textbooks on clinical cardiology to which I referred
liberally, while preparing the manuscript.
• My esteemed readers of earlier books, whose generous appreciation and
constructive criticism keep me going.
• M/s Jaypee Brothers Medical Publishers (P) Ltd., New Delhi, India, who
repose their unflinching faith in me and provide excellent editorial support.
Contents
Section 1: Congenital Heart Diseases

Case 1 : Ventricular Septal Defect 3
Case 2 : Atrial Septal Defect 7
Case 3 : Fallot’s Tetralogy 11
Case 4 : Ebstein’s Anomaly 15
Case 5 : Patent Ductus Arteriosus 19
Section 2: Mitral Valve Diseases

Case 6 : Mitral Stenosis 25
Case 7 : Mitral Regurgitation 29
Case 8 : Mitral Valve Prolapse 33
Section 3: Aortic Valve Diseases

Case 9 : Aortic Stenosis 39
Case 10 : Aortic Regurgitation 43
Case 11 : Aortic Sclerosis 48
Section 4: The Cardiomyopathies

Case 12 : Dilated Cardiomyopathy 55
Case 13 : Restrictive Cardiomyopathy 59
Case 14 : Hypertrophic Cardiomyopathy 63
Case 15 : Takotsubo Cardiomyopathy 67
Section 5: Aortic Diseases

Case 16 : Aneurysm of Aorta 73
Case 17 : Dissection of Aorta 77
Case 18 : Coarctation of Aorta 81
Case 19 : Sinus of Valsalva Aneurysm 85
xiv 50 Cases in Clinical Cardiology: A Problem Solving Approach
Section 6: Pulmonary Diseases

Case 20 : Pulmonary Stenosis 91
Case 21 : Pulmonary Hypertension 95
Case 22 : Pulmonary Embolism 99
Case 23 : Obstructive Pulmonary Disease 103
Section 7: Pericardial Infections

Case 24 : Acute Pericarditis 109
Case 25 : Pericardial Effusion 113
Case 26 : Constrictive Pericarditis 117
Section 8: Myocardial Infections

Case 27 : Rheumatic Fever 123
Case 28 : Acute Myocarditis 127
Section 9: Endocardial Infections

Case 29 : Aortic Valve Endocarditis 133
Case 30 : Tricuspid Valve Endocarditis 137
Section 10: Intracardiac Masses

Case 31 : Atrial Myxoma 143
Case 32 : Atrial Thrombus 147
Case 33 : Ventricular Thrombus 151
Section 11: Typical ECG Abnormalities

Case 34 : Left Ventricular Hypertrophy 157
Case 35 : Left Bundle Branch Block 161
Case 36 : Features of Hypokalemia 165
Case 37 : Features of Hyperkalemia 169
Section 12: Electrocardiac Syndromes

Case 38 : Prolonged Q-T Syndrome 175
Case 39 : Sick Sinus Syndrome 179
Case 40 : Early Repolarization Syndrome 183
Case 41 : Brugada Syndrome 186
Case 42 : WPW Syndrome 189
Contents xv
Section 13: Cardiac Arrhythmias

Case 43 : Supraventricular Tachycardia 195
Case 44 : Atrial Fibrillation 199
Case 45 : Ventricular Premature Beats 203
Case 46 : Ventricular Tachycardia 207
Section 14: Coronary Artery Diseases

Case 47 : Chronic Stable Angina 213
Case 48 : Acute Coronary Syndrome 217
Case 49 : Papillary Muscle Rupture 222
Case 50 : Left Ventricular Aneurysm 226
Index 231
SECTION
1
Congenital Heart
Diseases
C A S E
1
Ventricular
Septal Defect
Case Presentation
A 31-year old man was referred to the cardiologist by a general physician, for
evaluation of a heart murmur. This young man had been denied a life insurance
policy because the physician, empanelled by the insurance company, had
incidentally noticed the murmur during medical examination. The man was normally
very active and denied complaints of chest pain, breathlessness, palpitations or
syncope. There was no history of cyanotic spells, joint pains or repeated chest
infections during childhood and he regularly played cricket and football in school.
However, the patient recollected that the doctor in the school medical room had
noticed the murmur and made a note of it in his medical report.
On examination, the man was of average built and height and looked healthy.
The pulse was 84 beats/min. and regular with no special character. The BP was
134/76 mm Hg in the right arm while sitting. There was no anemia, cyanosis or
clinical sign of congestive heart failure. The apex beat was ill-sustained, heaving in
nature and slightly displaced towards the axilla. There was a pansystolic murmur over
the middle of the left sternal border with a S3 sound in early diastole. The murmur
did not radiate towards the axilla. There was no parasternal heave and the lower
border of the liver was not palpable. The lung fields were clear.
CLINICAL DISCUSSION
From the history and physical examination, this asymptomatic young man
had a parasternal pansystolic murmur. Typical causes of a pansystolic murmur
are mitral regurgitation, ventricular septal defect and tricuspid regurgitation.
Sometimes, tight coarctation of aorta or a patent ductus arteriosus with pulmonary
hypertension can also produce a pansystolic murmur but these murmurs are
usually located at the upper left sternal edge. The murmur of mitral regurgitation
radiates towards the axilla while the murmur of tricuspid regurgitation is usually
associated with engorged neck veins and an enlarged pulsatile liver.
ECG of the patient showed biphasic RS complexes in the mid-precordial
leads. X-ray chest showed mild cardiomegaly with minimal signs of pulmonary
congestion. On ECHO, the left ventricle was normal in size with normal ejection
fraction. A signal drop-out was noticed in the mid-portion of the interventricular
4 Section 1 Congenital Heart Diseases
Figure 1.1: C
olor flow map extending from
left ventricle to right ventricle
septum. There was no abnormality of the cardiac valves and the estimated
pulmonary artery pressure was normal. On color Doppler, an abnormal flow map
was observed extending from the left ventricle to the right ventricle (Fig. 1.1), with
a high velocity jet on continuous wave Doppler. Therefore, the definite diagnosis
in this case is ventricular septal defect (VSD).
Figure 1.2: Ventricular septal defect
In VSD, a breach in the continuity of the interventricular septum creates a

left-to-right shunt between the ventricles (Fig. 1.2). This congenital cardiac defect
occurs due to complexity of embryological development of the septum, which has
a membranous and a muscular portion. Most (80%) VSDs occur at the junction
of these sections and are termed as perimembranous VSD (Fig. 1.3). Some VSDs
occur in the muscular section (muscular VSD) and may be multiple (sieve-like).
Rare varieties of VSD are endocardial cushion defects (supracristal VSD) and
outlet septal defect (subpulmonic VSD) (Table 1.1).
Case 1 Ventricular Septal Defect 5
Figure 1.3: Various locations of ventricular septal defect (VSD)

RA: Right atrium; RV: Right ventricle
Table 1.1: Types of ventricular septal defect

• Perimembranous VSD
• Subpulmonic VSD
• Supracristal VSD
• Muscular VSD
A small VSD (Maladie de Roger) generates a loud pansystolic murmur in a

localized area on the precordium. The murmur is located in the upper parasternal
area in outlet VSD and in the mid-portion in perimembranous VSD. A muscular
VSD produces a short systolic murmur since the defect shuts off during muscle
contraction in later systole. This murmur is located over the lower parasternal
area. A large VSD with elevated right ventricular pressure that equals left
ventricular pressure (bidirectional shunt) is also associated with an early systolic
murmur. Therefore, there is no correlation between the length or intensity of the
murmur and the size of the VSD.
A large shunt may be accompanied by a diastolic flow murmur and a S3
sound, due to torrential flow across the mitral valve. The S2 is widely split due to
early aortic valve closure. On ECHO, signal drop-out is not observed if the VSD is
too small (<3 mm size) or muscular in location. The width of the colour flow map
approximates the VSD size. On Doppler, high flow velocity indicates a small VSD.
The flow velocity is low if the VSD is large and the shunt is bidirectional.
VSD is the commonest form of congenital acyanotic heart disease and accounts
for 25% of all cardiac malformations. VSD may occur in isolation or as part of a
complex constellation of congenital cardiac abnormalities. Aortic regurgitation
may be associated due to lack of support to the aortic valve in perimembranous
VSD. Complications of VSD in childhood are growth retardation and repeated

chest infections. Reversal of shunt can occur later in life when pulmonary
pressure exceeds the systemic pressure. Endocarditis can follow any non-cardiac
surgical procedure.
MANAGEMENT ISSUES
Large sized VSDs allow large volumes of left-to-right shunt and usually present
in childhood with failure to thrive, breathlessness and recurrent respiratory
infections. They can lead to pulmonary hypertension, right heart failure and
ultimately reversal of shunt (right-to-left). This is designated as the Eisenmenger’s
syndrome. Such VSDs are usually closed in childhood to avoid complications and
before the Eisenmenger’s syndrome has developed.
Medium sized VSDs are associated with a moderate sized shunt. The shunt
is large enough to cause breathlessness, but not enough to cause pulmonary
hypertension and shunt reversal. Such patients do reasonably well during
childhood, but may become progressively symptomatic as left ventricular
compliance declines with age and pulmonary venous congestion develops. Such
VSDs are usually closed in adulthood, to avoid the development of heart failure.
Small sized VSDs do not cause significant shunting and are often asympto
matic. Some of them may close as the child grows older. Those that do not close
spontaneously are closed by intervention for reasons other than the shunt.
These reasons are development of endocarditis or associated significant aortic
regurgitation (Table 1.2).
Table 1.2: Indications for surgical closure of VSD

• L arge-sized VSD with volume overload
(pulmonary to systemic flow ratio >2:1)
• Medium-sized VSD with congestive symptoms
without pulmonary hypertension
• Small-sized VSD without congestive symptoms
with endocarditis or aortic regurgitation
RECENT ADVANCES
The last decade or two have witnessed remarkable progress in the percutaneous
techniques for closure of ventricular septal defects, thus avoiding the risks
associated with open heart surgery. Although transesophageal echocardiography
(TEE) generally suffices to guide the deployment of the closure device, intracardiac
ultrasound provides more accurate assessment. Sonography can provide vital
information pertaining to the location and size of the defect and the rim around
it, so as to facilitate proper device selection and placement.
C A S E
2
Atrial
Septal Defect
Case Presentation
A 36-year old woman was referred to a physician by a gynecologist, for preoperative
assessment prior to elective hysterectomy. The patient had multiple uterine fibroids
on ultrasonography and complained of excessive bleeding during menstruation. For
the past 6 months, she had been complaining of exertional dyspnea and fatigue,
which were attributed to anemia as a result of blood loss. She denied complaints
of chest pain, palpitations or dizziness. There was no history of cyanotic spells, joint
pains or recurrent respiratory infections during her childhood. The patient was
married, had 2 sons aged 11 and 9 years and she had never been hospitalized for any
major illness or surgical procedure.
On examination there was mild anemia but no cyanosis, icterus or sign of
congestive heart failure. The pulse was 90 beats/min. regular, with a BP of 136/80
mm Hg in the right arm. The apex beat was normal in location with a sustained
left parasternal heave on palpation. The S1 was normal with a loud P2; no S3 or S4
sound was heard. The S2 components namely A2 and P2 were widely spaced and the
time gap between them did not increase further during inspiration. A short systolic
murmur was heard over the upper left sternal border. The murmur was not preceded
by an ejection click or accompanied by a palpable thrill and did not radiate to the
neck. The lung fields were clear on auscultation.
CLINICAL DISCUSSION
From the history and physical examination, this young woman had effort
intolerance with an ejection murmur in the pulmonary area. Typical causes of
such a murmur are innocent hemic murmur (Still’s murmur), pulmonary valve
stenosis, pulmonary hypertension and atrial septal defect. The murmur of
pulmonary stenosis may be preceded by an ejection click and accompanied by
a palpable thrill. The P2 component of S2 is muffled and the splitting between A2
and P2 is wide, but widens further during inspiration. An innocent hemic murmur
is not associated with a loud P2 or wide splitting of S2. Pulmonary hypertension of
any etiology can produce a systolic murmur with loud P2 but wide fixed splitting
of S2 is only a feature of atrial septal defect.
ECG of the patient showed sinus rhythm with incomplete right bundle
branch block and a rightward QRS axis. X-ray chest showed enlarged right-sided
Figure 2.1: Color flow map extending from

left atrium to right atrium
chambers with dilated main pulmonary artery, prominent hila and pulmonary
plethora. On ECHO, the right atrium and right ventricle were dilated and a signal
drop-out was noticed in the interatrial septum. On colour Doppler, an abnormal
flow map was observed extending across the area of echo drop-out, from the left
atrium to the right atrium (Fig. 2.1). There were no abnormalities of the cardiac
valves and the estimated pulmonary artery pressure was normal. Therefore, the
definite diagnosis in this case is atrial septal defect (ASD).
Figure 2.2: Atrial septal defect
In ASD, breach in the continuity of the interatrial septum creates a left-to-

right shunt between the atria (Fig. 2.2). The septal defect occurs due to complexity
of its embryological development. Most (75%) ASDs occur in the mid-portion
of the septum, in the region of the foramen ovale and are termed as ostium
secundum ASD. Some ASDs occur lower down the inter-atrial septum and are
termed as ostium primum ASD (Fig. 2.3). Ostium primum ASDs are associated
with cleft leaflets, regurgitation of the atrioventricular valves and are also known
as endocardial cushion defect. An uncommon variety of ASD in the upper portion
Case 2 Atrial Septal Defect 9
Figure 2.3: Various locations of atrial septal defect (ASD)

SVC: Superior vena cava; IVC: Inferior vena cava
Table 2.1: Types of atrial septal defect

• Ostium secundum ASD
• Ostium primum ASD
• Sinus venosus defect
• Vena caval defect
is sinus venosus defect, which is accompanied by anomalous pulmonary venous

connections (Table 2.1). Inferior vena caval defects are very rare. An ASD may
be associated with trisomy 21 (Down’s syndrome) or abnormalities of the hand
(Holt Oram syndrome).
The systolic murmur of ASD is due to increased flow across the pulmonary
valve and not due to the shunt. The intensity of murmur does not correlate with
the size of the ASD. However, a large ASD is associated with a diastolic flow
murmur and a right-sided S3, due to torrential flow across the tricuspid valve. An
accompanying pansystolic murmur due to mitral and/or tricuspid regurgitation
is a feature of ostium primum ASD. In ASD, the splitting of S2 is wide and fixed. It
is wide because of increased pulmonary ejection time, which delays the P2.
Other reasons for wide splitting of S2 are right bundle branch block or
pulmonary stenosis (delayed P2) and mitral regurgitation or ventricular septal
defect (premature A2). The splitting of S2 is also wide in WPW syndrome Type A, in
which there is pre-excitation of the left ventricle. The splitting of S2 is fixed in ASD
because the shunt equalizes atrial pressures throughout the respiratory cycle and
there is no inspiratory augmentation of right ventricular filling.
On ECHO, since the signal from the interatrial septum is weak, false echo
drop-out may be seen even in normal persons. The subcostal window may be a
better option to diagnose an ASD but transesophageal echocardiography (TEE)
provides excellent visualization particularly in endocardial cushion defects and
sinus venosus ASD. Sometimes, contrast echo is needed to visualize the shunt
using agitated saline, which contains air bubbles that cross over the septal defect.
ASD is the commonest congenital heart disease diagnosed in adulthood,
with either absent or mild symptoms. It is 7 times more common in females than
in males. Complications of ASD in adults are effort intolerance and pulmonary

hypertension. Reversal of shunt and right heart failure are rare compared to
ventricular septal defect. Atrial tachyarrhythmias including atrial fibrillation
are common. Typically, sinus arrhythmia is never observed, because the shunt
negates the effect of inspiration on venous return. Systemic thrombo-embolism
can occur due to emboli from peripheral or pelvic veins, passing across the septal
defect (paradoxical embolism).
MANAGEMENT ISSUES
Most ostium secundum atrial septal defects are amenable to percutaneous
device closure. Large ASDs allow large volumes of left-to-right shunt and usually
persent with exertional breathlessness and fatigue. They can lead to pulmonary
hypertension and right heart failure, although reversal of shunt (right-to-left) is
less common than in case of ventricular septal defect. Therefore, ASDs larger than
10 mm in size should ideally be closed before significant pulmonary hypertension
develops.
Smaller ASDs with small volumes of shunt may become progressively more
symptomatic as left ventricular compliance declines with age and the degree of
shunting increases. Such ASDs that lead to right ventricular dilatation, should
be closed during adulthood. Patients with ASD may develop paradoxical
emboli which arise in the venous system and cross the septal defect to reach the
systemic circulation. ASDs with history of thrombo-embolism should be closed,
irrespective of their size (Table 2.2).
Table 2.2: Indications for surgical closure of ASD

• Large-sized ASD more than 10 mm in size
with pulmonary to systemic flow ratio > 1.5:1
• Medium-sized ASD with dilated right ventricle
without significant pulmonary hypertension
• Small-sized ASD without dilated right ventricle
with history of systemic thrombo-embolism
Ostium primum atrial septal defects with atrioventricular valvular abnor

malities and sinus venosus defects with anomalous pulmonary venous drainage
are not amenable to percutaneous device closure because of their complexity.
They require a definitive surgical procedure for their correction.
RECENT ADVANCES
Percutaneous deployment of a closure device for atrial septal defect has been
standard practice for several decades. Transesophageal echocardiography (TEE)
is widely used to guide the deployment. Recently, intracardiac ultrasound has
been used to accurately assess the anatomy, for better selection and placement
of the device. Vital anatomical information includes size of the defect, the rim
around the defect and proximity to the mitral and tricuspid valves.
C A S E
3
Fallot’s
Tetralogy
CASE PRESENTATION
A 14-year old girl was admitted to a tertiary-care hospital of a metropolitan city, with
the complaint of progressive shortness of breath since 2 months, more so for the last
5 days. There was no history of fever, productive cough, chest pain or hemoptysis.
The girl’s mother also noticed an increase in the child’s abdominal girth and swelling
around both her ankles. The patient was born after a Caesarian section and was
noticed to be cyanosed at birth. At the age of 3 years, the girl had undergone a
surgical operation for congenital heart disease, in this very hospital. There was no
history of repeated chest infection or childhood asthma, but the patient’s growth
milestones of early childhood were delayed.
On examination, the patient was slightly breathless at rest but was not distressed.
The pulse rate was 84 beats/min., with a BP of 96/66 mm Hg. There was cyanosis
over the tongue and lips and the finger-tips and toes were clubbed. Pitting ankle
edema was present and the neck veins were engorged. Per abdomen findings were a
5 cm hepatomegaly with mild ascites. The breath sounds were vesicular in character
without any rhonchi or crepts. On precordial examination, the apex beat was normal
in location with a left parasternal heave. Auscultation revealed a normal S1 and
S2 with an early-diastolic murmur over the pulmonary area and a soft pansystolic
murmur over the lower left parasternal area.
CLINICAL DISCUSSION
From the history and physical examination, this young girl had congenital cyanotic
heart disease that was operated upon during her early childhood. At present she
was in right heart failure with pulmonary and tricuspid valve regurgitation and
had right ventricular enlargement. Right ventricular enlargement is associated
with a palpable left parasternal heave. Common causes of right ventricular
enlargement are pulmonary valve stenosis and pulmonary arterial hypertension.
The commonest cause of pulmonary regurgitation is pulmonary hypertension,
but it can also follow a surgical procedure on the pulmonary valve. Rare causes
of pulmonary regurgitation are subvalvular pulmonary stenosis and carcinoid
syndrome (Table 3.1). Functional tricuspid regurgitation (dilated annulus)
is a consequence of right ventricular dilatation due to any cause. Tricuspid
regurgitation is associated with raised JVP with prominent v waves and rapid
Table 3.1: Causes of pulmonary regurgitation

• Primary: Carcinoid syndrome
• Congenital: Subvalvular stenosis
• Iatrogenic: Pulmonary valvotomy
• Secondary: Pulmonary hypertension
y descent. An enlarged pulsatile liver with a pansystolic murmur over the lower
left parasternal area are also observed.
ECG showed sinus rhythm with tall R waves in right precordial leads and T
wave inversion, suggestive of right ventricular hypertrophy with strain (Fig. 3.1).
P. pulmonale and right axis deviation of the QRS were also seen. X-ray chest
findings were increased cardio-thoracic ratio with reduced pulmonary vascular
markings and a right-sided aortic arch. On ECHO, the left ventricle was normal
in size with an ejection fraction of 55% but the right ventricle was significantly
dilated. The mitral and aortic valves were normal in structure but there was
moderate pulmonary regurgitation and significant tricuspid regurgitation.
Figure 3.1: ECG showing tall R waves in leads V1 to V3.
This patient was operated upon for a cardiac defect, when she was 3 years
old. The most common congenital cyanotic heart disease that is associated
with survival until adolescence and sometimes even into adulthood is tetralogy
of Fallot. Therefore, in all probability, this patient was operated upon for
Fallot’s tetralogy and had now developed pulmonary regurgitation (Fig. 3.2),
as a complication of the surgical procedure on the pulmonary valve. The right
ventricular enlargement that ensued, led to secondary tricuspid regurgitation
because of annular dilatation.
Tetralogy of Fallot is the most common congenital cyanotic heart disease that
survives into adolescence. It is associated with a right-to-left shunt since birth
unlike isolated septal defects which are left-to-right shunts and undergo reversal
only after the development of pulmonary hypertension. The four components
(Fig. 3.3) of Fallot’s tetralogy are:
• Pulmonary stenosis (PS)
• Overriding of aorta (OA)
• Ventricular septal defect (VSD)
• Right ventricular hypertrophy (RVH)
Case 3 Fallot’s Tetralogy 13
Figure 3.2: Pulmonary regurgitation
The primary developmental abnormality is of the pulmonary subvalvular or

infundibular area leading to pulmonary stenosis (PS) and right ventricular outflow
tract (RVOT) obstruction. Rarely, the pulmonary valve is absent (pulmonary
atresia). The ventricular septal defect (VSD) is membranous in location. The
aorta is displaced rightward and overrides the septum, the overriding aorta (OA).
Therefore, the septum is not in line with the anterior aortic wall but with the
aortic valve closure point. The right ventricular hypertrophy (RVH) is secondary
to RVOT obstruction. Rarely, an atrial septal defect (ASD) may be associated, in
which case the constellation is designated as pentalogy of Fallot.
Figure 3.3: The four components of Fallot’s tetralogy. AO: Aorta; OA: Overriding aorta;
RA: Right atrium; LA: Left atrium; RV: Right ventricle; LV: Left ventricle PS: Pulmonary stenosis;
PA: Pulmonary artery; VSD: Ventricular septal defect; RVH: Right ventricular hypertrophy
In a typical unrepaired case of Fallot’s tetralogy, auscultatory findings are a

loud, single S2 and a parasternal systolic murmur. The S2 is single because the P2
is muffled and the A2 is loud because the aorta is anteriorly placed. The systolic
murmur originates from the subvalvular pulmonary stenosis and not from the
ventricular septal defect. The classical clinical features of Fallot’s tetralogy are
central cyanosis, finger clubbing, anoxic spells, growth retardation and exercise
intolerance. Congestive heart failure is rare because the septal defect balances the
right and left ventricular pressures. If left unrepaired, catastrophic complications
in adolescence are arterial thrombo-embolism and cerebral abscess.
MANAGEMENT ISSUES
Until the 1970s and even early-1980s, surgical interventions in very early
childhood were largely palliative. These shunt procedures were performed
to bypass the RVOT obstruction and to enhance pulmonary blood flow. These
shunts were Blalock-Taussig shunt (subclavian artery to pulmonary artery) and
Waterston shunt (ascending aorta to right pulmonary artery). However, even after
these procedures, patients remained symptomatic and complications occurred
unabated. Nowadays, total surgical correction is undertaken to close the shunt
and to enhance pulmonary blood flow. This includes patch closure of the VSD
with pulmonary subvalvular muscle resection and valvotomy.
Cardiac surgeons are increasingly encountering complications of prior
surgical correction, as these children survive into their teens. Complications after
surgery include residual shunt, residual stenosis or, post-valvotomy pulmonary
regurgitation and right ventricular enlargement as in our case. Pulmonary valve
replacement with tricuspid annular repair would be the best course of action in
this case.
RECENT ADVANCES
Prior cardiac surgery often distorts the anatomy of the heart to an extent that the
information obtained from transthoracic echocardiography is generally skewed
and inconclusive. Modern cardiac imaging techniques of computed tomography
(CT) and magnetic resonance imaging (MRI) are particularly useful to evaluate
postoperative patients.
Percutaneous techniques are currently being evaluated in the management of
Fallot’s tetralogy. Pulmonary balloon angioplasty and artificial valve deployment
by non-surgical intervention have been recently described.
C A S E
4
Ebstein’s
Anomaly
CASE PRESENTATION
A 26-year old married woman visited a cardiologist’s chamber, with the complaint
of occasional fluttering sensation in the chest of 2 years duration. The episodes of
palpitation were associated with some light-headedness, but she had never fainted.
Her palpitation was at times related to some emotional upset or undue physical
exercise, but there was no history of exertional fatigue, chest pain or breathlessness.
There was also no history of tremor of the hands or weight loss. Her childhood
had been uneventful with normal growth milestones and there was no history of
cyanotic spells during sports activities. She also denied having had recurrent sore-
throat, joint pains or any prolonged febrile illness during her school days.
On examination, the patient was comfortable, relaxed and not dyspneic. There
was no tremor of the fingers, visible goiter or eye-signs of Grave’s disease. The JVP
was raised 5 cm above the angle of Louis and showed large v waves with a prominent
y descent. The pulse was regular, fair in volume, at a rate of 84 beats/min. and the
BP was 130/80 mm Hg. On examining the abdomen, there were visible epigastric
pulsations, with the liver edge 6 cm below the right costal margin and pulsatile; no
ascites was demonstrable. The apex beat was normal in nature and location but a left
parasternal heave was palpated. The S1 and S2 were both split with wide splitting of
S2 appreciated during inspiration. No S3 or S4 gallop sound was heard. A pansystolic
murmur was audible at the lower end of the left sternal edge. Breathing was vesicular
and no rhonchi or crepts were heard over the lung fields.
CLINICAL DISCUSSION
From the history and physical examination, this patient had paroxysmal
tachycardia with clinical signs of tricuspid valve regurgitation. ECG showed tall
P waves (P. pulmonale) with normal P-R interval and right bundle branch block
(RBBB). X-ray chest finding was an enlarged cardiac silhouette, more so towards
the right of the midline. ECHO revealed normal sized left ventricle with normal
ejection fraction. The mitral and aortic valves were normal and the left atrium was
not dilated. There was no echo drop-out in the region of either septum. However,
the right atrium was markedly enlarged and the right ventricle was dilated as
well as hyperkinetic. The tricuspid valve was displaced downwards into the right
ventricle, with distal attachment of the septal tricuspid leaflet which showed
exaggerated excursion. On colour flow mapping, a regurgitant jet was seen in

the right atrium. These findings are consistent with the diagnosis of Ebstein’s
anomaly.
In a young woman, history of episodic palpitation raises several clinical
possibilities. Anxiety neurosis, panic attacks and paroxysmal supraventricular
tachycardia are usual causes but in these, the heart is structurally normal.
Perimenopausal symptoms in women include palpitation but our patient was
young. Thyrotoxicosis is a possibility but our patient had no goiter, tremor or
eye-signs of Grave’s disease. Pre-excitation syndrome (WPW syndrome) may be
responsible for paroxysmal tachyarrhythmia but the ECG did not show short P-R
interval or delta waves on the QRS complex. Mitral valve prolapse (MVP) and
atrial septal defect (ASD) are structural cardiac abnormalities that are responsible
for tachyarrhythmias. However, in our case the mitral valve was normal and there
was no septal defect.
Ebstein’s anomaly is an uncommon congenital acyanotic heart disease
characterized by abnormal tricuspid valve architecture, tricuspid regurgitation
and association with paroxysmal supraventricular tachyarrhythmias. The physical
examination of the patient and interpretation of simple cardiac investigations is a
good exercise in bed-side clinical cardiology. A raised JVP with large v waves and
prominent y descent are characteristic of tricuspid regurgitation into the right
atrium (Fig. 4.1). So is an enlarged and pulsatile liver on abdominal examination.
A sustained left parasternal heave is indicative of right ventricular volume
overload.
Figure 4.1: Tricuspid regurgitation
The S1 is split because of delayed tricuspid valve closure (T1) due to right
bundle branch block (RBBB) as well as the wide excursion of the septal tricuspid
leaflet. The S2 is widely split due to delayed pulmonary closure (P2) because of the
RBBB. Sometimes, the S2 is single because of soft P2 due to low pulmonary ejection
volume. Rarely, the S2 is paradoxically split because of pre-excitation of the right
ventricle caused by WPW syndrome Type B. The pansystolic murmur of tricuspid
regurgitation is best audible over the lower left parasternal area and does not
radiate towards the axilla or the base of the heart. Like all right-sided murmurs, it
Case 4 Ebstein’s Anomaly 17
Figure 4.2: X-ray showing cardiomegaly due

to enlargement of right atrium
increases in intensity during inspiration, provided the right ventricular function

is normal.
In sinus rhythm, the ECG shows tall P waves (P. pulmonale) due to right atrial
enlargement and wide QRS complexes due to right bundle branch block (RBBB).
Sometimes, wide QRS complexes are due to WPW syndrome, in which case there
is a short P-R interval (pre-excitation). At times, the rhythm is atrial fibrillation.
On X-ray chest, the cardiac silhouette is enlarged towards the right of the midline,
due to the large right atrium (Fig. 4.2). Superficially, this resembles a pericardial
effusion. The differentiating feature is that the right lower portion of the cardiac
silhouette curves inwards towards the center of the chest and not outwards, as it
would in case of pericardial effusion.
On ECHO apical view, there is downward displacement of the tricuspid
valve into the body of the right ventricle, towards the apex. The septal tricuspid
Figure 4.3: ECHO showing enlarged right atrium

with displaced tricuspid valve
leaflet is attached to the IV septum, 10 mm or more distal to the anterior mitral

leaflet. The tricuspid leaflet is large and shows wide excursion, often with a
whip-like motion. The right ventricle is dilated and hyperkinetic due to volume
overload. The right atrium is enlarged because of tricuspid regurgitation as well
as due to “atrialization” of the upper portion of the right ventricle (Fig. 4.3). On
long-axis view, because of downward displacement of the tricuspid valve, there
is simultaneous recording of the mitral and tricuspid valves (MV and TV). On
short-axis view, the tricuspid valve is shifted clockwise, from the normal 9 0’clock
position to the 11 0’clock position.
The commonest reason for tricuspid regurgitation is dilatation of the tricuspid
valve annulus secondary to right ventricular dilatation. Reason for annular
dilatation is usually pulmonary hypertension due to congenital left-to-right
shunt, rheumatic mitral valve disease or chronic cor pulmonale. Sometimes,
dilated cardiomyopathy causes annular dilatation (Table 4.1). Primary tricuspid
valvular regurgitation has several causes except coronary artery disease and
systemic hypertension, the commonest forms of heart disease. Usual causes of
tricuspid valve regurgitation are tricuspid leaflet prolapse, Ebstein’s anomaly,
rheumatic heart disease, carcinoid syndrome and right-sided endocarditis.
Uncommon causes include endocardial cushion defects, endomyocardial fibro
sis and connective tissue disorders.
Table 4.1: Causes of tricuspid regurgitation

Valvular regurgitation
• Tricuspid valve prolapse
• Right-sided endocarditis
• Ebstein’s anomaly
• Carcinoid syndrome
• Endocardial cushion defect
• Endomyocardial fibrosis
• Connective tissue disease
Annular dilatation
• Pulmonary hypertension
• Pulmonary regurgitation
• Dilated cardiomyopathy
MANAGEMENT ISSUES
The management of Ebstein’s anomaly includes the treatment of supraventricular
tachyarrhythmias and the control of tricuspid regurgitation. Drugs that block the
atrioventricular (AV) node to reduce the heart rate, such as betablockers and
verapamil, are the agents of choice. In the presence of WPW syndrome, amiodarone
is preferable. Digoxin may be used to treat atrial fibrillation in which case, an
anticoagulant is also prescribed to reduce the risk of thrombo-embolism. If the
tachyarrhythmias are refractory to drug treatment or an accessory bypass tract is
present, radiofrequency ablation can be offered. In the presence of moderate to
severe tricuspid regurgitation, valve repair with restrictive annuloplasty or even
valve replacement may be considered.
C A S E
5
Patent Ductus
Arteriosus
CASE PRESENTATION
A 6-year old boy was taken to the pediatrician by his mother, for treatment of fever
with coryza and cough. While auscultating the chest of the child, the pediatrician
incidentally heard a loud murmur over the upper precordium. Although the child had
been taken to several doctors in the past for consultation and vaccination, nobody
had noticed the murmur. The boy was born after normal vaginal delivery, without
any intervention and was not cyanosed at birth. His mother had experienced no
difficulty in nursing him. The boy’s growth milestones of early childhood were not
delayed.
On examination, the child was irritable because of his respiratory catarrhe but
not tachypneic. He was febrile but not anemic or icteric and there was no cyanosis
or clubbing of the fingers or toes. The extremities were warm but not sweaty and his
radial pulse was bounding in nature at a rate of 110 beats/min. The thyroid gland was
not enlarged and there was no sign of congestive heart failure. The BP over the right
arm in the supine position was 160/60 mm Hg and similar in the left arm. The child’s
mother was quite sure that his blood pressure had never been checked earlier.
On examination of the precordium, the apex beat was hyperkinetic but there
was no palpable parasternal heave. The loud murmur over the precordium was
wide-spread but maximally audible in the 2nd left intercostal space, just below the
middle of the left clavicle. On careful auscultation, the murmur was pansystolic
but extended upto and through S2, well into diastole. No S3 or S4 sound could be
appreciated because of the long murmur. The lung fields were clear on auscultation
without any rhonchi or crepitations.
CLINICAL DISCUSSION
From the history and physical examination, this boy had a bounding pulse and
an incidentally detected systolo-diastolic murmur. A good volume radial pulse,
which can be appreciated even with the arm elevated above the head, is known as
collapsing pulse. A collapsing pulse is indicative of a wide pulse pressure. Causes
of a wide pulse pressure are:
• Severe anemia
• Thyrotoxicosis
• Paget’s disease
• Beri-beri disease
• Aortic regurgitation
• Arterio-venous fistula
• Patent ductus arteriosus.
A long murmur that extends throughout systole and crosses S2 to spill over
into diastole, is known as a continuous murmur. Causes of a continuous murmur
are as follows:
• Venous hum
• Mammary souffle
• Coarctation of aorta
• Patent ductus arteriosus
• Aortopulmonary window
• Coronary arterio-venous fistula
• Ruptured aneurysm sinus of Valsalva
Figure 5.1: X-ray showing cardiomegaly with

prominent pulmonary artery
ECG of the patient showed tall R waves in left precordial leads and deep S
waves in right precordial leads. X-ray chest findings were cardiomegaly with left
ventricular contour and a prominent main pulmonary artery with pulmonary
plethora ( Fig. 5.1). On ECHO, the left ventricle was dilated with normal systolic
function. The left atrium was also dilated and all cardiac valves were structurally
normal. On colour flow mapping, there was a retrograde mosaic jet extending
from the left pulmonary artery to the dilated main pulmonary artery (Fig. 5.2).
Figure 5.2: ECHO showing retrograde jet from left

branch to main pulmonary artery
Case 5 Patent Ductus Arteriosus 21
On pulsed-wave Doppler, with the sample volume moving distally from the right
ventricular outflow tract and across the pulmonary valve, increased velocity was
detected in the pulmonary artery. The estimated pulmonary artery pressure was
normal. Therefore, the definite diagnosis in this case is patent ductus arteriosus.
The ductus arteriosus is a channel that connects the descending aorta distal
to the origin of left subclavian artery, to the left pulmonary artery just distal to
the bifurcation of main pulmonary artery. The ductus remains open during
intrauterine life and closes soon after birth when its purpose is fulfilled. When the
ductus fails to close physiologically within 24 hours after birth and anatomically
within a week, it provides a communication between the aortic and pulmonary
circulations. Flow from the aorta (at higher pressure) to the pulmonary artery (at
lower pressure) creates a left-to-right shunt across the PDA (Fig. 5.3). Persistence
of the ductus is sometimes associated with maternal rubella syndrome and
premature delivery.
Figure 5.3: Patent ductus arteriosus
The continuous murmur of PDA is classically described as a “machinery”

murmur and is referred to as Gibson’s murmur. It is maximally audible below
the middle of the left clavicle, just before and just after the S2. The continuous
murmur may be accompanied by a mid-diastolic murmur over the mitral area,
due to torrential flow across the mitral valve. There may also be reverse splitting
of S2, due to delayed closure of the aortic valve. When pulmonary hypertension
develops, the murmur of PDA is confined to systole.
As already mentioned, there are several causes of a continuous murmur. In
aorto-pulmonary window, there is a proximal communication between the aorta
and the pulmonary artery. Although the murmur of aortic coarctation is typically
systolic, in tight stenosis the murmur may extend into diastole. Venous hum is a
low-pitched continuous murmur which is loudest over the supraclavicular fossa,
but sometimes also heard over the precordium. It is accentuated by looking over
the shoulder while sitting and abolished by compression of the jugular vein.
A mammary soufflé is heard widely over the precordium in pregnant women. It

is better appreciated while lying down and during systole. Coronary fistula and
ruptured aneurysm of sinus of Valsalva are rare arterio-venous communications
that can also produce a continuous murmur.
Ventricular septal defect (VSD) produces a pansystolic murmur while aortic
regurgitation causes an early diastolic murmur. When the two are associated
as in perimembranous VSD, the murmur is systolo-diastolic. A similar systolo-
diastolic murmur occurs when an atrial septal defect (ASD) is associated with
mitral stenosis, the Lutembacher syndrome. In contrast to a continuous murmur,
the two components of a systolo-diastolic murmur have a different character.
PDA is the commonest cause of cardiomegaly and heart failure in infancy and
childhood. Conversely, heart failure is the commonest cause of morbidity and
mortality in PDA, at any age. Causes of heart failure in childhood are:
• Congenital cardiomyopathy
• Anomalous left coronary artery arising from pulmonary artery (ALCAPA).
When pulmonary hypertension develops in PDA, reversal of the shunt from
pulmonary artery to aorta may occur. In that case, the continuous murmur gets
shorter and quieter. The toes get more cyanosed and clubbed than the fingers.
The reason for this differential cyanosis is that the ductus is distal to the left
subclavian artery and predominantly the lower limbs get deoxygenated blood.
Other complication of PDA are endarteritis, aneurysm formation and rarely
rupture of the ductus.
MANAGEMENT ISSUES
Percutaneous device closure of patent ductus arteriosus was one of the earliest
non-surgical interventions in cardiology. All PDAs of significant size and shunt
should be ligated, except in ductus dependent complex congenital cyanotic heart
diseases of infancy.
SECTION
2
Mitral Valve
Diseases
C A S E
6
Mitral
Stenosis
CASE PRESENTATION
A 23-year old woman of low socio-economic status came to a general hospital with
shortness of breath, fatigue and palpitations since 2 years and fever with productive
cough for the last 1 week. She denied any chest pain, wheezing or hemoptysis. At the
age of 12, she had a prolonged febrile illness with joint pains and ever since, she had
been receiving monthly injections of penicillin.
On examination she was tachypneic with pallor but there was no cyanosis
or icterus. Pulse rate was 96 beats/min. regular, with a BP of 100/70 mm Hg. Her
temperature was 1000 F. The JVP was not raised, thyroid gland was not enlarged and
there were no palpable lymph nodes. There was no evidence of pharyngo-tonsillitis,
swollen joints or petechial spots over the skin, eyes or finger-tips. The apex beat
was tapping in nature with a left parasternal heave. The S1 was loud and the P2 was
also accentuated. A low-pitched mid-diastolic rumbling murmur was heard over the
cardiac apex. The murmur was preceded by an opening snap and accentuated just
before systole. There were scattered rhonchi and crepts over the lung fields.
CLINICAL DISCUSSION
From the history and physical examination, this young woman in all probability
had rheumatic heart disease with mitral valve stenosis (Fig. 6.1). The S1 is loud
Figure 6.1: Mitral valve stenosis

26 Section 2 Mitral Valve Diseases
since the mitral valve leaflets are distant from each other at the end of diastole
and snap together loudly. Other reasons for a loud S1 are sinus tachycardia and a
short P-R interval, where the diastole is short. The mid-diastolic murmur of mitral
stenosis (MS) is best heard with the patient in the left lateral decubitus position,
using the stethoscope bell.
The length of the murmur correlates with the severity of stenosis. The murmur
undergoes presystolic accentuation due to atrial contribution to ventricular
filling. In mild MS, the murmur may be only presystolic. If MS is associated
with atrial fibrillation the S1 is variable in intensity due to variable duration of
diastole. Also, presystolic accentuation is lost due to lack of atrial contribution to
ventricular filling. Presystolic accentuation is also absent in a calcified valve and
after commissurotomy. Severe mitral stenosis may be silent due to low cardiac
output and the fact that the right ventricle underlies most of the precordium
because of clockwise cardiac rotation.
The opening snap heralds the onset of ventricular diastolic filling and the
end of isovolumic relaxation. It indicates pliability of the valve, suitability for
valvotomy and is absent in a heavily calcified valve or after commissurotomy.
An early opening snap after A2 (short 2A-OS interval), indicates higher left atrial
pressure and more severe MS. Besides mitral stenosis, other reasons for a mid-
diastolic murmur are acute rheumatic valvulitis (Carey-Coomb murmur), aortic
regurgitation (Austin-Flint murmur), left atrial myxoma (prolapse into mitral
orifice) and left-to-right shunt (increased transmitral inflow).
Figure 6.2: M-mode ECHO showing reduced excursion

of AML with paradoxical motion of PML
ECG showed sinus rhythm, P mitrale, right ventricular hypertrophy with a

rightward QRS axis. X-ray chest findings were straightening of the left heart border
with pulmonary congestion. On M-mode ECHO, excursion of the anterior leaflet
was reduced with paradoxical excursion of the posterior leaflet (Fig. 6.2). On 2-D
ECHO, the left atrium and right ventricle were dilated. The mitral valve leaflets
were thickened with limited excursion and restricted valve opening. The anterior
mitral leaflet showed diastolic doming (Fig. 6.3). There was no abnormality of the
aortic valve and the estimated pulmonary artery pressure was elevated.
The severity of mitral stenosis can be gauged and classified, according
to several calculated indices from echocardiography. These are the pressure
gradient across the valve (PG), the time taken for the PG to fall to half (P½t;
pressure half-time), the pulmonary artery pressure (PAP) estimated from the
peak tricuspid velocity (TR Vmax) and the mitral valve area (MVA) calculated by
planimetry (Table 6.1). However, there are some fallacies associated with these
Case 6 Mitral Stenosis 27
Figure 6.3: 2-D ECHO showing restricted opening with

diastolic doming and dilated left atrium
Table 6.1: Assessment of the severity of mitral stenosis

Mild Moderate Severe
PG (mm Hg) <5 5-10 >10
P½ t (msec) <150 150-220 >220
TRVmax (m/sec) <2.7 2.7-3.0 >3.0
PAP (mm Hg) <30 30-50 >50
2
MVA (cm ) >1.5 1.0-1.5 <1.0
calculations. The peak velocity and pressure gradient depend on the heart rate
and stroke volume. Measurement of mitral valve area may be fallacious due to
heavily calcified leaflets, subvalvular pathology and prior commissurotomy.
Rheumatic heart disease is the predominant cause of mitral stenosis. 80% of
cases are women in the 2nd or 3rd decade of life, from the developing countries. Rare
causes are congenital MS (parachute valve), mucopolysaccharidosis (Hurler’s
syndrome), severe annular calcification and connective tissue disorders (Table
6.2). Complications of MS are pulmonary congestion, respiratory infections,
hemoptysis, right heart failure and systemic thrombo-embolism due to atrial
fibrillation associated with left atrial thrombus.
Table 6.2: Causes of mitral stenosis

• Rheumatic heart disease (commonest)
• Congenital parachute valve
• Mitral annular calcification
• Connective tissue disorder
• Hurler’s mucopolysaccharidosis
PERTINENT INVESTIGATIONS
Since rheumatic heart disease is the predominant cause of mitral stenosis, all
patients who are febrile should be investigated for rheumatic fever, infective
endocarditis and respiratory tract infection. Relevant investigations are Hb, TLC,
DLC, ESR, urine R/E, CRP value and ASLO titre. Throat-swab culture for beta-
hemolytic Streptococcus and bacterial blood cultures may be included.
Transthoracic ECHO is central to the diagnosis and assessment of mitral
stenosis. Besides determining the severity of MS, it can assess left atrial size, left
ventricular function and estimate pulmonary artery pressure. Transesophageal
echocardiography (TEE) allows better assessment of the subvalvular and
commissural architecture and improves the detection of a left atrial thrombus.
MANAGEMENT ISSUES
All patients of mitral stenosis should be offered penicillin prophylaxis against
rheumatic fever, until they attain the age of 40 years. A broad-spectrum antimicro
bial agent should be administered prior to any invasive dental or surgical proce
dure, to guard against infective endocarditis. Diuretics reduce pulmonary
congestion, especially in patients with associated mitral or aortic regurgitation.
Rate control with digoxin, beta-blocker or verapamil improves left ventricular
diastolic filling, in patients who are in sinus rhythm and controls the ventricular
response, in whom the rhythm is atrial fibrillation. Since atrial fibrillation in
patients with mitral stenosis is associated with a high risk of thrombo-embolism,
these patients should also be on a long-term oral anticoagulant agent like warfarin.
Percutaneous balloon mitral valvuloplasty (BMV) is the procedure of choice
in symptomatic severe mitral stenosis, provided a transesophageal echocardio
graphy (TEE) does not reveal subvalvular fusion, commissural calcification,
atrial thrombus or more than mild MR (Table 6.3). Recurrence of symptoms
after valvotomy is more often due to induced mitral regurgitation rather than
restenosis. If valvotomy cannot be performed because of the aforementioned
reasons, mitral valve replacement (MVR) is undertaken. During MVR, if atrial
fibrillation is present, left atrial radiofrequency ablation (RFA) and appendage
ligation are also performed.
Table 6.3: Indications for valvotomy in MS

The absence of concomitant:
• Subvalvular fusion
• Left atrial thrombus
• Immobility of leaflets
• Commissural calcification
• Moderate/severe regurgitation
RECENT ADVANCES
Although transesophageal echocardiography (TEE) has vastly improved acquisi
tion of detailed information over the transthoracic approach, multi-slice
computed tomography (CT) is being increasingly used to accurately assess the
area and the precise nature of the mitral valve.
C A S E
7
Mitral
Regurgitation
CASE PRESENTATION
A 46-year old woman of stocky built, was asked to see a cardiologist by her family
doctor. She complained of exertional shortness of breath and had a cardiac murmur.
She was a known case of hypertension, adequately controlled by medication. Her
breathlessness had been felt since the last 4 months but had significantly worsened
over the preceding 2 weeks. There was no history of exertional chest pain or of
palpitation and fainting. She did not smoke or consume alcohol but she seldom
exercised and did not follow the diet-plan suggested by her physician.
On examination, she was overweight without any clinical signs of cortisol excess
or hypothyroidism. The pulse was regular, good in volume at a rate of 92 beats/min.
The BP was 136/88 mm Hg in the right arm. There was no sign of heart failure. The
apex beat was hyperdynamic, ill-sustained heaving in nature and displaced towards
the left axilla. The S1 was soft. A2 was loud and a soft S3 was audible in early diastole.
A soft blowing pansystolic murmur was audible over the mitral area, that radiated
towards the axilla. Also, a short diastolic rumble was heard over the cardiac apex.
Few basilar rales were auscultated over the lower lung fields.
Figure 7.1: Mitral regurgitation
CLINICAL DISCUSSION
From the history and physical examination, this woman had mitral regurgitation
(Fig. 7.1) with possibly associated mitral stenosis and evidence of left ventricular
dilatation. The S1 was soft since the mitral leaflets are close to each other at the end
of diastole and snap together softly. The A2 was loud due to associated systemic
hypertension. The displacement of the apex beat and audible S3 sound are
indicative of left ventricular diastolic overload. The S3 is a low-pitched sound that
follows the A2. It indicates abrupt halting of left ventricular filling. Physiological
S3 is appreciated in mitral regurgitation, left-to-right shunt and high cardiac
output states. Pathological S3 is audible in aortic regurgitation and left ventricular
systolic dysfunction.
The pansystolic murmur of mitral regurgitation typically radiates to the
left axilla (and sometimes even to the left scapula), if the anterior mitral leaflet
is diseased. It radiates to the base of the heart, if the posterior mitral leaflet
is involved. This differentiates it from the pansystolic murmur of tricuspid
regurgitation or a ventricular septal defect. The accompanying diastolic rumble
does not necessarily indicate concomitant mitral stenosis and is due to torrential
flow across the valve, which is the sum of normal atrial blood volume and the
regurgitant volume. It is worth mentioning that the murmur of acute or severe
MR is not pansystolic but early systolic, because less turbulence is generated
by the large valve orifice. Moreover, the rapid rise of left atrial pressure impedes
regurgitation during later systole. The murmur of mitral annular calcification is
also typically early systolic.
Figure 7.2: X-ray showing cardiac enlargement

straightening of left heart border
ECG of the patient showed tall R waves in left precordial leads with upright
T waves, indicating left ventricular diastolic overload. X-ray chest findings
were moderate cardiomegaly, pulmonary congestion and straightening of the
left heart border (Fig. 7.2). The “4-bump” left heart border is due to a large left
atrial appendage, which is damaged during acute carditis and bulges due to
high left atrial pressure in rheumatic mitral regurgitation (MR). On ECHO, the
left ventricle was dilated and hyperkinetic with an ejection fraction of 55% and
left atrial enlargement. Importantly, in functional MR due to annular dilatation
secondary to cardiomyopathy, there is global hypokinesia of the left ventricle
Case 7 Mitral Regurgitation 31
or a regional wall motion abnormality. In acute MR due to ruptured chordae

tendinae, there is minimal left ventricular dilatation. The mitral valve leaflets were
thickened and fibrotic indicating rheumatic MR. Other potential abnormalities of
the mitral valve in case of MR are mitral leaflet redundancy and prolapse, flail
leaflet, annular calcification or vegetations in case of endocarditis.
Figure 7.3: ECHO showing a color-flow map

in the left atrium
On colour flow mapping, a regurgitant jet was seen entering the left atrium
(Fig. 7.3). The volume of MR (ml/beat), the MR jet area (cm2) and the percentage of
left atrial area (%) filled by the jet are used to gauge the severity of MR (Table 7.1).
However, there are some fallacies associated with these calculations. The spatial
profile of the MR jet does not truly reflect the regurgitant volume. It depends upon
the shape of the valve orifice, the angle of the jet, left ventricular filling pressure
and the size of the left atrium. The MR jet may be “eccentric” or “off-centre” in
case of leaflet prolapse, papillary muscle rupture and paraprosthetic leak.
Table 7.1: Assessment of the severity of mitral regurgitation

MR jet area (cm2) <4 4-8 >8
Percent of LA area (%) <25 25-50 >50
MR Volume (ml/beat) <30 30-59 >60
LV dysfunction Mild-to-moderate Severe
Ejection fraction (%) 30-60 <30
LV diameter (mm) 40-55 >55
There are several causes of mitral regurgitation (MR). Usual causes of

primary MR are rheumatic heart disease, mitral leaflet prolapse, papillary muscle
dysfunction, infective endocarditis and mitral annular calcification. Uncommon
causs include congenital endocardial cushion defects, endomyocardial fibrosis
Table 7.2: Causes of mitral regurgitation

Chronic MR
• Mitral valve prolapse
• Endomyocardial fibrosis
• Rheumatic heart disease
• Mitral annular calcification
• Papillary muscle dysfunction
Acute MR
• Acute myocardial infarction
• Infective endocarditis
• Blunt chest trauma.
and connective tissue disorders. Occasionally, secondary MR is due to mitral

annular dilatation, as a result of dilated or ischemic cardiomyopathy. Acute
MR can result from rupture of papillary muscle or chordae tendinae in case of
myocardial infarction, infective endocarditis or after blunt trauma to the chest
(Table 7.2).
MANAGEMENT ISSUES
Patients of rheumatic MR below the age of 40 should receive penicillin prophylaxis
to protect them from acute rheumatic fever. Those undergoing a dental or
surgical procedure, need antibiotic prophylaxis against infective endocarditis.
Vasodilators such as ACE inhibitors or ARBs combined with a diuretic relieve
pulmonary congestion, especially if systemic hypertension is present. Digoxin
and an anticoagulant are used if there is concomitant atrial fibrillation. Secondary
MR is reduced by lowering preload and afterload, which decrease the diameter
of the mitral annulus.
Surgical operations for MR are mitral valve repair and valve replacement.
Repair of the valve or annuloplasty is preferable if the valvular anatomy is
suitable, since it preserves left ventricular geometry and spares the patient from
the problems of anticoagulation. Replacement of the valve is indicated if the
valvular anatomy is severely distorted.
RECENT ADVANCES
Mitral regurgitation in the context of ischemic heart disease is a clinical
challenge. The MR is not due to an intrinsic valvular abnormality, but the result
of ventricular dilatation with annular enlargement, papillary muscle dysfunction
and dysfunctional ventricular remodeling with increased sphericity. These
patients can be offered restrictive annuloplasty, which involves insertion of an
undersized annular ring to improve leaflet apposition. Therefore, patients who
are to undergo coronary artery bypass graft (CABG) surgery should be adequately
assessed preoperatively for mitral regurgitation.
C A S E
8
Mitral Valve
Prolapse
CASE PRESENTATION
A 26-year old married woman visited a renowned cardiologist for coronary
angiography, with the complaints of episodic chest pain and palpitation. The chest
pain was precordial in location and described as sharp and pricking. There was no
feeling of tightness in the chest or suffocation and the pain did not radiate to the
left arm or lower jaw. The palpitation was accompanied by restlessness, dryness of
mouth and dizziness and sometimes caused “black-out” with fainting. The episodes
of chest pain and palpitation were unrelated to physical exercise, taking meals or
change in body posture, but usually related to some emotional upheaval. She had
first experienced these symptoms during her final high-school exams at the age
of 18, but the frequency of episodes had significantly increased, ever since her
arranged marriage 3 months back. There was history of repeated sore-throat during
childhood, but she never had a prolonged febrile illness with painful joints.
On examination, the patient had a slender body habitus with an anxious look
on her face. The extremities were warm with sweaty palms and a fine distal tremor.
There was no anemia, cyanosis or edema and the JVP was not raised. The thyroid
gland was not enlarged and there were no eye-signs of Grave’s disease. The pulse
was rapid, good in volume at a rate of 96 beats/min with a BP of 140/84 mm Hg.
The precordium was hyperdynamic with a pectus excavatum sternal deformity.
The apex beat was normal in location and there was no parasternal heave. The S1
was loud, S2 was normally split and no S3 or S4 was heard in diastole. A high-pitched
systolic murmur was audible between the cardiac apex and the left sternal border.
The murmur started well after the S1 and had a typical honking character. It was
associated with a sharp clicking sound in mid-systole. The lung fields were clear on
auscultation.
CLINICAL DISCUSSION
From the history and physical examination, this young anxious lady had atypical
cardiac symptoms with a mid-systolic click and a mid-systolic murmur. The most
likely diagnosis in this case is mitral valve prolapse (MVP). The mid-systolic click
is a high-pitched sharp sound produced by sudden tensing of the redundant
mitral leaflet. At times, multiple clicks are appreciated. This is often followed by a
mid- or late-systolic murmur that typically has a whooping or honking character.
Another cause of a mid-systolic murmur is papillary muscle dysfunction. The

click and murmur can vary with alteration of left ventricular (LV) volume, by
change in patient posture. During standing or Valsalva manoeuvre, when the LV
volume is reduced, the click moves closer to S1 and the murmur becomes louder.
Conversely, during squatting when the LV volume increases, the click moves
closer to S2 and the murmur becomes softer.
In mitral valve prolapse syndrome, the SI is loud for several reasons. The
high adrenergic activity increases the heart rate and shortens diastole. The wide
excursion of the myxomatous, redundant valve leaflet increases the force of mitral
valve closure. Finally, merger of the non-ejection click with the S1 , increases the
intensity of the latter.
Figure 8.1: ECHO showing buckling of mitral leaflets,

above the plane of mitral annulus
ECG of the patient showed sinus rhythm at the rate of 92 with few atrial
ectopic beats and T wave inversion in leads LIII, aVF, V5, V6. The X-ray chest
was unremarkable. On ECHO, the left ventricle was normal in size and systolic
function, but the left atrium was mildly dilated. The anterior mitral leaflet was
thick and redundant, with systolic buckling above the plane of the mitral annulus
into the left atrium (Fig. 8.1). On M-Mode scan, there was abrupt posterior
displacement of both leaflets in systole, giving a “hammock-like” appearance
(Fig. 8.2). On colour flow mapping, an eccentric regurgitant jet was seen entering
the left atrium.
Figure 8.2: M-mode scan showing posterior motion of

mitral leaflets (“hammock” appearance)
Case 8 Mitral Valve Prolapse 35
According to the extent of motion, mitral valve prolapse (MVP) can be

classified into 3 types (Table 8.1). In type 1, the anterior leaflet only moves
upto the annulus while in type 2, it bows into the left atrium. In type 3, both the
leaflets enter the left atrium. Strict echocardiographic criteria must be used to
diagnose MVP because needless anxiety may be created by over-reporting this
abnormality. Minor “technical” MVP may be observed in normal women due to
high transducer position and caudal angulation. Conversely, true MVP may be
missed due to low transducer position and cranial angulation.
Table 8.1: Classification of mitral valve prolapse

Type 1: AML and PML move upto the annulus
Type 2: Large AML bows into the left atrium
Type 3: Both AML and PML enter left atrium
Mitral valve prolapse is known as “floppy valve” or “myxomatous valve” or

“billowing valve” and the condition is also designated as “Barlow’s syndrome”.
MVP is far more commonly seen in females and occurs in 7% of middle-aged
women. Often these women have a type A personality with history of panic
attacks and migranous headaches. They may have a slender body habitus with
thoracic skeletal deformities including pectus excavatum, straight back and
scoliosis. The valvular abnormality is due to myxomatous degeneration leading
to thickening, nodularity or redundancy of one or both mitral leaflets. Sometimes
MVP is associated with other cardiac conditions such as ostium seundum ASD,
Marfan syndrome and WPW syndrome (Table 8.2). Mitral regurgitation may be
present due to faulty coaptation of leaflets and predisposes to endocarditis.
Table 8.2: Conditions associated with MVP

• Myxomatous degeneration
• Ostium secundum ASD
• WPW syndrome
• Marfan syndrome
• Turner syndrome
Atypical chest pain, palpitation, fatigue, orthostatic symptoms and neuro

psychiatric complaints have all been well-described in patients with mitral valve
prolapse. Whether these non-specific symptoms are directly attributable to MVP
or due to autonomic dysfunction, continues to be widely debated and their cause-
effect relationship remains unproven. Besides the classical symptoms, focal
neurologic findings, such as transient ischemic attacks, amaurosis fugax, retinal
artery occlusion and rarely hemiparesis have all been reported in patients with
MVP. These neurologic findings probably occur as a result of thrombo-embolism
from the prolapsing valve.
The ECG at rest frequently shows T-wave inversion in the inferolateral leads
(LIII, aVF, V5, V6). False-positive ECG stress tests occur in up to 50% of patients with
MVP. Premature beats are most common, although practically any arrhythmia
can occur. The cause of the arrhythmia is not known but may be related to
autonomic dysfunction or mechanical effects of the floppy valve. Incidence of
syncope correlates poorly with the presence of arrhythmias.
It is not unusual for patients of mitral valve prolapse to undergo a battery of
sophisticated cardiac investigations, in the search for the diagnosis of a serious
heart disease. Besides ECG and ECHO which do show some typical abnormalities,
exercise stress test is done which is more often false-positive. Ambulatory
24-hour Holter monitoring frequently shows supraventricular and sometimes
ventricular ectopic beats and rarely if ever reveals life-threatening arrhythmias.
Myocardial perfusion imaging and coronary angiography expectedly do not show
any significant abnormality. Hormonal assays are sometimes performed to rule
out thyrotoxicosis and likewise, urinary catecholamines are rarely measured, to
rule out the possibility of phaeochromocytoma.
MANAGEMENT ISSUES
Most women with mitral valve prolapse need reassurance that their cardiac
condition is not serious or life-threatening. Anxiolytic drugs during the day with a
mild tranquilizer at night are useful for those having overt anxiety and disturbed
sleep. A beta-blocker such as propranolol has multiple benefits in these patients.
It controls tachycardia and ectopic beats, reduces the degree of leaflet prolapse,
treats the associated tremor and serves as a prophylactic drug against migraine.
Low-dose aspirin is prescribed to prevent thrombo-embolism. Patients of MVP
with mitral regurgitation (MR) and not those without demonstrable MR, require
antibiotic prophylaxis against infective endocarditis, prior to a dental, endoscopic
or surgical procedure.
SECTION
3
Aortic Valve
Diseases
C A S E
9
Aortic
Stenosis
CASE PRESENTATION
A 52-year old man presented to the out-patient cardiology clinic with easy
fatiguability and breathlessness on exertion for the last 1 year. In the preceding
month, he had experienced three distinct episodes of dizziness, followed by
fainting. The syncopal episodes were unrelated to exercise and were not preceded
by palpitation or chest pain. There was no history of prolonged febrile illness or joint
pains during childhood.
On examination, the pulse was of low volume with a slow upstroke, at a rate of 84
beats/min. with a BP of 96/72 mm Hg. There was no anemia, cyanosis or icterus and
the thyroid gland was not enlarged. The JVP was not raised and there was no ankle
edema. The apex beat was normal in position and heaving in nature. The S1 was
normal, S2 appeared single and S4 was audible in pre-systole. An ejection systolic
murmur was audible over the aortic area, that was preceded by an ejection click and
associated with a palpable thrill. The murmur and thrill typically radiated towards
the carotid arteries.
CLINICAL DISCUSSION
From the history and physical examination, this man had low cardiac output,
possibly due to left ventricular outflow tract (LVOT) obstruction. The most likely
diagnosis in this case is aortic valve stenosis (Fig. 9.1). A pulse of low volume
(pulsus parvus) with slow upstroke (pulsus tardus) is a typical feature of aortic
valve stenosis. A heaving apex beat indicates left ventricular hypertrophy and is
also observed in uncontrolled systemic hypertension and in coarctation of aorta.
The low-pitched S4 sound in pre-systole, indicates forceful atrial contraction
over a non-compliant left ventricle. It coincides with the a wave of the jugular
vein. The S4 is always pathological in aortic stenosis, systemic hypertension and
restrictive or hypertrophic cardiomyopathy. An acute rise in left ventricular end-
diastolic pressure (LVEDP) as in acute coronary syndrome or acute valvular
regurgitation causes acute onset of S4.
The S2 appears single because the A2 is muffled. If A2 is audible, the splitting
of S2 is paradoxical or reverse, with the A2 following the P2 due to prolonged left
ventricular ejection time. The ejection click heralds the onset of systole and the
end of isovolumic relaxation. It indicates that the stenosis is valvular in nature
40 Section 3 Aortic Valve Diseases
Figure 9.1: Aortic valve stenosis
and not subvalvular (infundibular) in location. The click may be absent if the
valve is heavily calcified. The ejection systolic murmur of aortic stenosis indicates
turbulent flow across the narrow aortic valve orifice. It is described as a diamond-
shaped murmur, since it builds up and declines gradually, with maximal intensity
in mid-systole. This pattern coincides with the temporal profile of the pressure
gradient across the valve. This murmur is also described as a “crescendo-
decrescendo murmur”. Length and loudness of the murmur does not correlate
with the severity of aortic stenosis.
Figure 9.2: ECG showing tall R waves in lateral chest leads
ECG showed tall R waves in left precordial leads with T wave inversion,
indicative of left ventricular hypertrophy with strain (Fig. 9.2). X-ray chest findings
were a boot-shaped heart with a prominent ascending aorta, suggestive of post-
stenotic dilatation. On ECHO, the left ventricular cavity was small in size, with a
good ejection fraction. There was concentric thickening of the interventricular
septum (IVS) and the left ventricular posterior wall (LVPW). The aortic valve
leaflets were thickened and calcific with restricted excursion and reduced
opening of the valve. Due to fusion at the leaflet tips, there was systolic doming of
leaflets. On colour flow mapping, a mosaic jet was observed in the proximal aorta
(Fig. 9.3) with an increased systolic velocity across the valve on CW Doppler.
Case 9 Aortic Stenosis 41
Figure 9.3: ECHO showing a mosaic colored jet

in the proximal aorta
Table 9.1: Assessment of the severity of aortic stenosis

Vmax (m/sec) <2.5 2.5-4 >4
PG (mm Hg) <2.5 25-60 >60
AVa (cm2) >1.5 1.0-1.5 <1.0
Vmax : Peak velocity
PG : Pressure gradient
AVa : Aortic valve area
Transthoracic echocardiography (TEE) generally suffices for the diagnosis

of aortic stenosis. However, the severity of stenosis is determined from the
peak velocity and pressure gradient across the valve on Doppler (Table 9.1).
Measurements from various echo views are taken to obtain the peak aortic flow
velocity. In rheumatic aortic stenosis, assessment of concomitant mitral valve
abnormalities is crucial as the mitral valve is almost invariably involved. Finally,
left ventricular wall thickness, end-diastolic diameter and ejection fraction
are to be measured. There are some fallacies associated with the calculation of
aortic stenosis severity. Reverberation artifacts in a heavily calcified valve may
overestimate AS severity. The peak velocity and pressure gradient depend on the
heart rate and stroke volume. They also depend upon the degree of parallelism
obtained between the Doppler beam and aortic flow direction.
Table 9.2: Causes of aortic stenosis

• Congenital bicuspid valve
• Senile calcific degeneration
The main causes of AS are congenital bicuspid aortic valve, rheumatic heart
disease and senile calcific degeneration (Table 9.2). Rheumatic aortic stenosis
usually presents in the 2nd or 3rd decade of life and is almost always associated
with mitral valve disease. Congenital bicuspid aortic valve leads to aortic stenosis
because of increased mechanical and shear stress on the valve and usually
presents in the 4th of 5th decade. Calcific aortic stenosis is akin to atherosclerosis
and accompanied by multiple cardiovascular risk factors. It typically presents
in the 6th or 7th decade of life. Rarely, a discrete membrane or ring may cause
subvalvular or supravalvular AS. Complications of AS are left ventricular
hypertrophy with diastolic dysfunction earlier on and systolic heart failure in the
later stages. Syncopal episodes are due to tachyarrhythmias or LVOT obstruction.
Angina may occur because of coronary ostial stenosis or the increased oxygen
demand of the hypertrophied myocardium.
Exercise ECG testing and Dobutamine stress ECHO may be undertaken in
asymptomatic patients of aortic stenosis, to assess their functional capacity.
The characteristic response is a drop in blood pressure during the test due to
vasodilatation and failure of the cardiac output to rise. Patients with a positive
test may be taken up for valve replacement. Coronary angiography is indicated to
assess the coronary circulation and need by bypass graft surgery (CABG), at the
time of aortic valve replacement. Besides showing occlusive coronary disease,
angiography may reveal coronary ostial stenosis due to calcification of the aortic
valve.
MANAGEMENT ISSUES
There is no medical treatment for aortic stenosis barring perhaps low-does
aspirin with a statin in those who have associated coronary risk factors. Aortic
valve replacement (AVR) is the procedure of choice in symptomatic severe aortic
stenosis or in asymptomatic stenosis with left ventricular dysfunction or a positive
stress test. AVR may also be undertaken in moderate AS at the time of CABG
surgery or in those engaged in high profile jobs such as aircraft pilots or military
personnel (Table 9.3). Complications of aortic valve replacement are prosthetic
valve malfunction, thrombo-embolism or bleeding and infective endocarditis.
Table 9.3: Indications for surgery in aortic stenosis

• Moderate to severe AS with symptoms
• Moderate AS with a positive stress test
• Moderate to severe AS with LV dysfunction
• Moderate AS with high profile job (e.g. pilot)
• Moderate AS with planned surgery (e.g. CABG)
RECENT ADVANCES
The technique of percutaneous aortic valve replacement (AVR) has been recently
refined and its feasibility is now clearly established. It is particular suitable for
candidates at high risk for a surgical procedure under general anesthesia. The
role of percutaneous aortic balloon valvuloplasty is currently limited.
C A S E
10
Aortic
Regurgitation
CASE PRESENTATION
A 54-year old man presented with breathlessness on exertion and throbbing
headache. His exertional dyspnea started about 8 months back and progressed to
the extent that he found it difficult to climb even one flight of stairs. His throbbing
headache was a daily feature and he also felt some pulsations over the neck. There
was no history of exertional chest pain, palpitation or syncopal episodes. The patient
had visited an orthopedician for a still back with difficulty in bending forward. On
X-ray of the lumbo-sacral spine, it was diagnosed as ankylosing spondylitis. He was
taking oral steroids prescribed by an ophthalmologist for iridocyclitis, in addition to
his antihypertensive medication.
On examination, the radial pulse was of good volume and bounding in nature
at a rate of 84 beats/min. The BP was 160/64 mm Hg in the right arm. Pulsations
were visible over the carotid arteries and the femoral pulses were also bounding
in nature. There was no clinical sign of congestive heart failure. The apex beat was
diffuse and hyperkinetic in nature and significantly displaced towards the axilla. The
S1 was normal and the S2 was soft, with a S3 gallop sound in early diastole. A high-
pitched blowing murmur was heard in diastole soon after the S3, on either side of the
upper sternum. A short diastolic rumble was also heard over the cardiac apex. Few
scattered crepts were audible over the lower lung fields.
CLINICAL DISCUSSION
From the history and physical examination, this man had aortic regurgitation
(Fig. 10.1) with possibly mitral stenosis and evidence of left ventricular dilatation.
Aortic root dilatation with AR is a known complication of ankylosing spondylitis.
The early-diastolic decrescendo murmur of AR is soft and blowing in character,
best heard with the patient sitting up and leaning forward. Interestingly, when
AR is due to aortic root dilatation, the murmur is louder along the right sternal
border. When AR is due to aortic valve pathology, the murmur is louder along
the left sternal border. In acute AR, for instance due to dissection of aorta, the
murmur is very short and soft and associated with a S4 and not S3.
The diastolic rumble at the apex does not necessary indicate concomitant
mitral stenosis. It is due to fluttering of the anterior mitral leaflet between the
AR jet and mitral valve inflow and is known as Austin-Flint murmur. A bounding
Figure 10.1: Aortic regurgitation
(“water-hammer”) pulse that can be appreciated even with the arm elevated
above the head is known as a collapsing pulse and is indicative of a wide
pulse-pressure. Besides aortic regurgitation, causes of wide pulse pressure are
severe anemia, thyrotoxicosis, beri-beri disease, patent ductus arteriosus and
arteriovenous fistula. Certain classical clinical signs of wide pulse-pressure, have
been described in aortic regurgitation (Table 10.1).
Table 10.1: Clinical signs of aortic regurgitation

• Corrigan sign: Vigorous pulsations in the carotid vessels
• De Musset sign: Nodding of the head with each heart beat
• Quincke sign: Visible capillary pulsations in the nail bed
• Traube sign: Pistol-shot sounds over femoral arteries
• Duroziez sign: Diastolic murmur over femoral arteries
ECG of the patient showed tall R waves in left precordial leads with upright
T waves indicating left ventricular diastolic overload. X-ray chest findings were
cardiomegaly and pulmonary congestion, giving it a “bat-wing” appearance
(Fig. 10.2). On ECHO, the left ventricle was dilated with an ejection fraction of
45%. There was also dilatation of the aortic root but the aortic valve leaflets were
not thickened or calcific and had no vegetations. The mitral valve was struc
turally normal and the left atrium was normal in size. On colour flow mapping, a
regurgitant jet was seen entering the left ventricular outflow tract (Fig. 10.3). The
volume of AR (ml/beat), the width of the AR jet as percentage of LVOT diameter
(%) and the depth of the jet entry into the left ventricle are used to gauge the
severity of AR (Table 10.2). However, there are some fallacies associated with
these calculations. The narrow jet of mild AR may extend deep into the left
ventricle while the broad jet of severe AR may not extend that far if it is “eccentric”
or “off-centre”.
Case 10 Aortic Regurgitation 45
Figure 10.2: X-ray showing cardiomegaly

with pulmonary congestion
Figure 10.3: ECHO showing regurgitant jet

in the outflow tract
Table 10.2: Assessment of the severity of aortic regurgitation

AR jet depth upto LVOT Mid–LV LV apex
AR jet width (% of LVOT) <25 25-65 >65
AR volume (ml/beat) <30 30-59 >60
AR: Aortic regurgitation on Doppler
LVOT: Left ventricular outflow tract
The causes of aortic regurgitation can be classified into the causes of aortic
valve disease and the causes of aortic root dilatation. Valvular diseases are
congenital bicuspid aortic valve, rheumatic heart disease and connective tissue
disorders. Aortic root dilatation occurs in uncontrolled hypertension, Marfan’s
syndrome and ankylosing spondylitis. Acute AR can result from aortic dissection,
annulo–aortic ectasia, infective endocarditis or after blunt trauma to the chest
(Table 10.3).
Table 10.3: Causes of aortic regurgitation

Valvular AR
• Bicuspid aortic valve
Root dilatation
• Marfan syndrome
• Ankylosing spondylitis
• Systemic hypertension
Acute AR
• Chest wall trauma
• Dissection of aorta
In addition to echocardiography, cardiac computed tomography (CT) or
magnetic resonance imaging (MRI) may be required for the detailed assessment
of the aortic valve as well as the proximal aorta. Radionuclide ventriculography
may be required for the detailed assessment of left ventricular volume, geometry
and systolic function. Exercise testing may be considered in an asymptomatic
person to assess his functional capacity. Coronary angiography is indicated to
assess the coronary circulation and the need for bypass surgery at the time of
aortic valve replacement. At the time of angiography, a root aortogram should
also be performed to assess the proximal aorta.
MANAGEMENT ISSUES
As far as the medical management of AR is concerned, diuretics and vasodilators
are the mainstay of therapy. The diuretics reduce blood pressure, left ventricular
volume and the symptoms of heart failure. Vasodilators reduce afterload,
increase forward flow and decrease the regurgitant volume. Additionally, they
control systolic blood pressure which is nearly always an issue in these patients.
Aortic valve replacement (AVR) is the surgical procedure of choice in patients of
AR who are symptomatic or have left ventricular dilatation (Table 10.4). Aortic
root repair or replacement may be combined with AVR, if there is a significant
degree of aortic dilatation. AVR may be combined with coronary artery bypass
graft (CABG) surgery, if the latter is indicated.
Table 10.4: Indications for surgery in aortic regurgitation

• Symptomatic acute moderate to severe AR
• Symptomatic chronic moderate to severe AR
• A
symptomatic chronic AR of any degree with
left ventricular dysfunction and dilatation
LV dilatation : end-systolic diameter >55 mm
end-diastolic diameter >75 mm
LV dysfunction : LV ejection fraction <50%
Case 10 Aortic Regurgitation 47
RECENT ADVANCES
Aortic valve replacement with a mechanical or a bioprosthetic valve is associated
with either the need for a long-term anticoagulant (requires monitoring and
carries risk of bleeding) or the late failure of a bioprosthetic valve. Therefore, there
is now growing interest in aortic valve repair, if the valvular anatomy is suitable.
However, the long-term feasability of this technique needs further evaluation.
C A S E
11
Aortic
Sclerosis
CASE PRESENTATION
A 77-year old elderly gentleman was brought by his son to his regular physician,
for a routine periodic medical check-up. The patient had been hypertensive for the
last 35 years and presently he was taking amlodipine 5 mg and metoprolol 50 mg
daily. About 3 years back, he was also prescribed isosorbide mononitrate 40 mg and
aspirin 75mg daily, because his ECG showed T wave inversion in the lateral leads. The
patient did complain of breathlessness on climbing stairs but there was no history
of angina, orthopnea or paroxysmal nocturnal dyspnea. Over the last three months,
he also felt dizzy and light-headed on standing up from the lying position, but there
was no history of palpitation or syncope.
On examination, the pulse was of good volume with few missed beats and the
BP was 154/76 mm Hg, with a heart rate of 66 beats/min. The patient was conscious,
cooperative and in no distress. The JVP was not raised but there was minimal pitting
edema over both his ankles. The apex beat was slightly displaced towards the axilla
and heaving in character. Systolic pulsations were observed over the aortic area
and in the suprasternal notch. The S1 was normal, A2 was loud but no gallop was
audible. A harsh systolic murmur was heard over the upper left sternal edge that
radiated towards the neck. The murmur was not preceded by an ejection click or
accompanied by a palpable thrill. A different soft systolic murmur was heard over
the cardiac apex that radiated towards the left axilla. The lung fields were clear on
auscultation.
CLINICAL DISCUSSION
From the history and physical examination, this elderly hypertensive gentleman
had aortic root dilatation, with probably left ventricular outflow tract (LVOT)
obstruction and also mitral regurgitation. ECG showed tall R waves with inverted
T waves in the lateral precordial leads (Fig. 11.1). There were no significant Q
waves or S-T segment shift, but few unifocal ventricular premature beats were
observed. X-ray chest findings were mild cardiomegaly, dilated ascending aorta
and a prominent aortic knuckle (Fig. 11.2).
ECHO revealed normal sized left ventricular cavity, with an ejection fraction
of 45%. There was mild concentric hypertrophy, but no wall motion abnormality
of any ventricular segment. The aortic valve annulus and leaflets showed bright
Case 11â•‡ Aortic Sclerosis 49
Figure 11.1: ECG showing tall R waves with

inverted T waves in lateral leads
Figure 11.2: X-ray showing dilated ascending aorta

with prominent aortic knuckle
echo-reflectivity (Fig. 11.3) with some restriction of leaflet excursion, but no

systolic doming. The posterior segment of the mitral valve annulus and the base
of the posterior leaflet also showed high echogenicity. On colour flow mapping,
a mosaic jet was seen entering the left atrium across the mitral valve. On Doppler
study, a peak systolic velocity (Vmax) of 2.5 m/sec was seen across the aortic
valve with a calculated pressure gradient (PG) of 25 mm Hg.
Figure 11.3: ECHO showing calcification of aortic valve leaflets (A) and annulus (B)
Table 11.1: Causes of aortic root dilatation

• Post-stenotic Aortic stenosis
• Medial necrosis Marfan’s syndrome
• Aorto-arteritis Takayasu’s disease
• Atherosclerotic Elderly hypertensive
• Collagen disorder Ankylosing spondylitis
Dilatation of the proximal aorta or aortic root is not uncommon in elderly

hypertensive subjects due to atherosclerosis. The aortic valve annulus however,
is normal in diameter. Aortic dilatation also occurs in aortic valve stenosis due to
the high velocity jet through the narrow, distorted valve impinging on the aortic
wall. This is known as post-stenotic dilatation. Sometimes, aortic root dilatation is
due to medial necrosis (Marfan’s syndrome), aorto-arteritis (Takayasu’s disease)
or a connective tissue disorder (Ankylosing spondylitis). In these cases, the
aortic valve annulus is also dilated and aortic regurgitation is often associated
(Table 11.1).
In elderly hypertensive patients, besides aortic root dilatation, calcification
of the aortic valve with or without stenosis is sometimes observed. This entity
is referred to as aortic valve sclerosis. Aortic valve calcification may also be
accompanied by mitral annular calcification (MAC). The calcified mitral annulus
sometimes causes mild to moderate mitral regurgitation and rarely even mitral
stenosis. Aortic sclerosis occurs in 25% subjects above the age of 65 years. It
leads to aortic stenosis more often in those about 80 years of age with a severely
calcified valve and in the presence of chronic kidney disease. There is a 50%
risk of myocardial infarction, stroke or death within 5 years, due to multiple
cardiovascular risk factors and severe atherosclerosis.
The systolic murmur of aortic stenosis is sometimes preceded by an ejection
click, accompanied by a palpable thrill and followed by a muffled A2 due to reduced
ejection volume. On the other hand, with the murmur of aortic sclerosis, there is
no click or thrill and the A2 is loud because of associated systemic hypertension.
A history of dizziness or syncope should always altert us to the possibility of left
ventricular outflow tract (LVOT) obstruction. Alternatively, syncope can occur
due to a tachyarrhythmia or because of sinus node dysfunction. In a patient
of aortic sclerosis, angina pectoris may be multifactorial. One, there may be
coronary artery stenosis. Two, the calcification of the valve may cause coronary
ostial occlusion. Three, angina may be due to the increased myocardial oxygen
demand, as a consequence of left ventricular hypertrophy.
MANAGEMENT ISSUES
The medical management of aortic valve sclerosis includes adequate control
of hypertension and other cardiovascular risk factors. This patient was already
taking amlodipine and metoprolol. Ramipril may be added since it is known to
facilitate regression of left ventricular hypertrophy. Diuretics should be avoided if
there is history of syncope. A statin can be added to the ongoing aspirin since both
Case 11 Aortic Sclerosis 51
these agents are proven to prevent cardiovascular events. Elderly hypertensive

patients often have associated orthostatic symptoms and they should be advised
adequate fluid intake and gradual change of posture.
Aortic valve replacement (AVR) may be considered in symptomatic moderate
to severe aortic stenosis especially with left ventricular dysfunction. At the time
of AVR, coronary angiography should be performed to assess the status of the
coronary vasculature. Conversely, all patients to be taken up for coronary artery
bypass graft (CABG) surgery should be assessed for aortic stenosis and AVR can
be performed at the time of operation.
RECENT ADVANCES
The technique of percutaneous aortic valve replacement (AVR) has been recently
refined and it is now clearly feasible in candidates at high risk of a surgical
procedure under general anesthesia.
SECTION
4
The
Cardiomyopathies
C A S E
12
Dilated
Cardiomyopathy
CASE PRESENTATION
A 48-year old man presented to the emergency room with recent worsening of
breathlessness, over the past few days. His dyspnea was worse at night and he
required two or more pillows under his head to catch some sleep. Yet his nocturnal
breathlessness woke him up frequently. For the past 4 months he had experienced
increasing fatigue on walking and on climbing stairs. He was not a diabetic or
hypertensive and denied any chest pain, palpitation or syncope. He did not smoke
but took 5-6 large pegs of alcohol on most days of the week, for the last 30 years.
None of his family members had history of heart disease.
On examination, he was tachypneic and diaphoretic. Pulse was rapid and feeble
with a changing volume in alternate beats and the extremities were cold. His heart
rate was 120 beats/min with a BP of 106/74 mm Hg. The JVP was clearly elevated
and there was also pitting edema over his ankles. The liver edge was palpable 6 cm
below the right costal margin. The apex beat was diffuse and displaced towards the
axilla. The S1 and S2 were normal and there was a soft pansystolic murmur over the
mitral area with an audible gallop sound in early diastole. There were bilateral basilar
rales over the lower one-third of both lung fields.
CLINICAL DISCUSSION
Form the history and physical examination, this patient was obviously in
congestive cardiac failure. The heart failure was systolic in nature, of the low-
output variety and biventricular in origin. ECG showed sinus tachycardia, narrow
QRS complexes and non-specific T-wave inversion, without any S-T segment shift
or Q-waves. X-ray chest findings were moderate cardiomegaly, Kerley B lines,
hilar congestion, and cephalized vessels, with pulmonary edema and a small
right-sided pleural effusion (Fig. 12.1). ECHO revealed a dilated left ventricle
(ejection fraction 25-30%) with reduced septal and posterior wall motion (global
hypokinesia). Excursion of the mitral valve leaflets was decreased and the E-point
septal separation was increased (Fig. 12.2). There were no regional wall motion
abnormalities or abnormal architecture of the cardiac valves. There was mild
mitral regurgitation and mild right ventricular dilatation. There are three main
diagnostic possibilities in this case. These are:
56 Section 4 The Cardiomyopathies
Figure 12.1: X-ray showing the classical findings of

left ventricular systolic dysfunction
Figure 12.2: ECHO showing reduced excursion

of the septum and mitral leaflets
• Organic mitral regurgitation

• Ischemic cardiomyopathy
• Dilated cardiomyopathy.
Organic mitral regurgitation with volume overload leading to severe
left ventricular dysfunction is unlikely in this case, because the mitral valve
architecture was normal and valvular regurgitation was only mild in severity.
Ischemic cardiomyopathy is a diagnostic possibility, but there are several reasons
against it. The patient had no significant coronary risk factors or family history
of heart disease. ECG did not show any S-T segment shift or the presence of
Q-waves. On ECHO, there was global hypokinesia instead of regional wall motion
abnormality. Moreover, there were no dyskinetic segments in the left ventricular
Case 12 Dilated Cardiomyopathy 57
Table 12.1: Differences between dilated and ischemic cardiomyopathy

Dilated CMP Ischemic CMP
Hypokinesia Global Regional
RWMA and the coronary territory Do not conform Conform
Dyskinesia Not seen Seen
RV involvement Often Rare
wall while right ventricular dilatation was present (Table 12.1). Therefore, the
most probable diagnosis in this case is dilated cardiomyopathy.
There are several causes of dilated cardiomyopathy (DCMP). The idiopathic
variety is believed to follow an acute viral myocarditis. Prominent definite causes
of DCMP are heavy alcoholism, diabetes mellitus and the peri-partum period.
Familial DCMP is seen in Duchenne’s muscular dystrophy and Friedrich’s
ataxia. Deficiency of thiamine (vitamin B1), selenium and carnitine can cause
a reversible form of DCMP. Drugs implicated in the causation of DCMP are
doxorubicin, imatinib, cyclophosphamide and trastuzumab. Uncommonly,
a persistent tachycardia due to any cause, can lead to permanent DCMP
(Table 12.2).
Table 12.2: Causes of dilated cardiomyopathy

• Idiopathic
• Alcoholism
• Diabetes
• Peripartum
• Nutritional
• Drug induced
• Tachycardia related
While echocardiography is a dependable diagnostic modality for dilated
cardiomyopathy, a battery of tests can be performed to ascertain the etiological
diagnosis. This is because there are several causes of DCMP and a vigorous
search is warranted to identify a treatable cause, with the optimism of preventing
relentless progression of the disease.
Inflammatory markers (ESR and CRP) are elevated in the presence of
myocarditis while levels of biomarkers (CPK, LDH) are increased in myocardial
necrosis. Patients are screened for a chronic infection (HIV, Hepatitis C) or
nutritional deficiency (thiamine, selenium, carnitine). Serum iron, ferritin and
transferrin are ordered if anemia or hemochromatosis are suspected and T3, T4
and TSH if thyrotoxicosis is to be ruled out. Renal and liver function tests are
ordered to assess additional causes of fluid overload.
Cardiac magnetic resonance imaging (MRI) is useful to assess left ventricular

size and geometry, LV remodelling and for tissue characterization with
gadolinium enhancement. Coronary angiography is performed in the presence of
atherosclerotic risk factors. Endomyocardial biopsy is rarely performed because
of its low diagnostic yield.
MANAGEMENT ISSUES
The first step in the management of DCMP is to identify and treat reversible
factors. Measures include withdrawal of the offending drug and correction of
any nutritional deficiency. Abstinence from alcohol and control of diabetes are
equally important. The heart rate has to be controlled in tachycardia related
cardiomyopathy.
Treatment of most of these patients is on the lines of standard heart failure
therapy. Diuretics are prescribed to treat fluid overload and modulators of renin-
angiotensin system (ACE-inhibitors or ARBs) to reduce cardiac afterload. Beta-
blockers are used particularly if extreme tachycardia or atrial fibrillation are
present. If the patient has persistent atrial fibrillation, rate control with digoxin
is required. Additionally, the patients are initiated on an oral anticoagulant to
reduce the risk of thrombo-embolic complications.
RECENT ADVANCES
A significant proportion of patients with dilated cardiomyopathy succumb to
malignant ventricular arrhythmias and not to heart failure per se. Antiarrhythmic
drugs reduce ejection fraction and have proarrhythmic potential, besides their
systemic side-effects. The use of automatic implantable cardioverter defibrilla
tors (AICDs) is now advocated as a primary preventive strategy against sudden
cardiac death (SCD) in such patients. Anticoagulant therapy is widely prescribed
to patients who are in atrial fibrillation and in those where a mural thrombus
has been documented. Currently, trials are underway to evaluate the role of
anticoagulants against thromboembolic complications in patients who are in
sinus rhythm.
In patients with moderate to severe symptoms (NYHA Class III or IV) with
severe left ventricular dysfunction (LVEF below 30%) and wide QRS complexes
(QRS duration more than 120 msec), cardiac resynchronization therapy (CRT)
may be offered. A biventricular pacemaker is placed to synchronize the ejection
of right and left ventricles if there is left bundle branch block (LBBB) or an
intraventricular conduction defect (IVCD). Finally, a ventricular assist device
may be implanted to improve left ventricular function in those with significant
hemodynamic deterioration. This strategy is particularly useful as a bridge to
cardiac transplantation.
C A S E
13
Restrictive
Cardiomyopathy
CASE PRESENTATION
A 58-year old man presented to the cardiology facility with fatigue, difficulty in
breathing and increasing swelling over his ankles, for the last 3 months. His dyspnea
became worse at night and he required two or more pillows under his head to be
comfortable. In addition, he had noticed an increase in his abdominal girth and
had loss of appetite, but there was no weight loss. There was no history of fever,
productive cough, chest pain or hemoptysis. He also denied palpitations or episodes
of syncope. The patient was not hypertensive or diabetic and there was no history of
premature coronary artery disease in any of his family members.
On examination, he was tachypneic but not in any distress. The pulse was 90
beats /min with a BP of 114/72 mm Hg. There was no cyanosis or jaundice but pitting
edema was present around the ankles. The liver edge was palpable 6 cm below the
right costal margin and there was free fluid in the abdominal cavity. The JVP was
elevated 5 cm above the angle of Louis, with prominent a waves and without a fall
in its level during inspiration. The apex beat was normal in character and location,
without any displacement towards the axilla. The S1 and S2 were normal with an
audible S4 in late diastole. There was no murmur or pericardial friction rub. Crepts
were audible over the basilar lung fields posteriorly.
CLINICAL DISCUSSION
From the history and physical examination, this patient was clearly having
biventricular heart failure. ECG showed low QRS voltages with non-specific
T-wave changes but no Q waves. There was no tachyarrhythmia or conduction
block. X-ray chest findings were interstitial pulmonary edema and small bilateral
pleural effusion, but the heart size was normal. ECHO revealed relative small
ventricular chambers with bilateral atrial dilatation. The ventricular free walls
and the interventricular septum were thick and gave a “granular sparkling”
ground-glass appearance (Fig. 13.1). There was also thickening of the mitral and
tricuspid valve leaflets with minimal valvular regurgitation. The left ventricular
ejection fraction was normal but the transmitral Doppler showed a tall E-wave
with rapid deceleration and a small A-wave.
There were several unusual clinical findings in this case. There was biventri
cular heart failure but no clinical or radiological evidence of cardiomegaly.
Figure 13.1: ECHO showing bright echogenicity

with small ventricles and large atria
Prominent a waves in the JVP and presence of S4 on auscultation indicate atrial

contraction against a stiff, noncompliant right ventricle. Failure of the JVP to
fall during inspiration (or paradoxical rise) constitutes the Kussmaul’s sign and
also indicates ventricular diastolic restriction. Despite evidence of ventricular
hypertrophy on ECHO, the voltages of the QRS complexes on the ECG were low.
This indicates that myocardial infiltration was the cause of ventricular thickening
in this case and not myocardial hypertrophy, as is observed in systemic
hypertension and aortic stenosis. ECHO revealed relatively small ventricles and
large atria, findings that are typically seen in mitral stenosis with pulmonary
hypertension and secondary tricuspid regurgitation. However, in our case there
was no evidence of mitral stenosis.
This patient therefore has a restrictive ventricular filling abnormality or left
ventricular diastolic dysfunction (LVDD) or heart failure with preserved ejection
fraction (HFPEF). Clinically speaking, the most frequent causes of LVDD are
systemic hypertension and coronary artery disease, where the mitral inflow
Doppler signal shows a slow relaxation pattern with the E wave shorter than the
A wave. These conditions were clearly absent in our case. Therefore, the most
probable diagnosis in this case is restrictive cardiomyopathy.
There are several causes of restrictive cardiomyopathy (RCMP). The most
prominent cause of RCMP is endomyocardial fibrosis (EMF). Another reason is
hypereosinophilic syndrome, the so called Loeffler’s endocarditis. A variety of
systemic infiltrative disease such as amyloidosis, sarcoidosis and malignancy can
present with RCMP. Connective tissue disorders especially scleroderma can also
lead to myocardial restriction. Finally, RCMP may be observed in storage diseases
like hemochromatosis and glycogen storage disorders (Table 13.1).
Amyloidosis is an important cause of restrictive cardiomyopathy. Besides
cardiac involvement, there may be other systemic manifestations of amyloid
deposition. Macroglossia with periorbital ecchymosis is pathognomic of amyloi
dosis. Neurological manifestations are sensory neuropathy, carpal tunnel
syndrome and autonomic neuropathy with postural hypotension. Hepatomegaly
may be due to amyloid infiltration or due to congestive heart failure. Renal
Case 13 Restrictive Cardiomyopathy 61
Table 13.1: Causes of restrictive cardiomyopathy

Endomyocardial
Endomyocardial fibrosis
Hypereosinophilic syndrome
Infiltrative
Amyloidosis
Sarcoidosis
Malignancy
Gaucher’s disease
Non-infiltrative
Idiopathic
Scleroderma
Storage diseases
Hemochromatosis
Glycogen storage disorder
involvement may lead to nephrotic syndrome, which exacerbates the pedal

edema of heart failure.
It is often challenging to differentiate restrictive cardiomyopathy from
constrictive pericarditis and cardiac catheterization may be required for their
distinction. The diagnosis of pericardial constriction is suggested if there is
past history of tuberculosis, cardiac surgery or radiotherapy. Also, the X-ray
chest shows linear pericardial calcification and ECHO may reveal pericardial
thickening with multiple parallel lines, casting a bright reflection. Computed
tomography (CT) or magnetic resonance imaging (MRI) may be required to
demonstrate pericardiac thickening. Moreover, in constrictive pericarditis, the
cardiac chamber size, myocardial thickness, valve structure and ejection fraction
are all normal (Table 13.2). Clinically speaking, constrictive pericarditis presents
with sole or predominant right heart failure while restrictive cardiomyopathy
often presents with biventricular failure.
Table 13.2: Differences between constrictive pericarditis

and restrictive cardiomyopathy
Constrictive pericarditis Restrictive cardiomyopathy
Pericardium Thick Normal
Myocardium Normal Thick
Ventricles Normal Obliterated
Atria Normal Dilated
LV function Normal Mildly impaired
MV and TV Normal Regurgitation
MV inflow Abrupt halt Slow relaxation
MANAGEMENT ISSUES
Diuretics reduce dyspnea and edema and they are the mainstay of the medical
management of restrictive cardiomyopathy. However, vigorous diuresis should
be avoided since it would compromise ventricular filling, reduce cardiac output
and even cause syncope. It is crucial to maintain sinus rhythm and to cardiovert
atrial fibrillation by electrical (DC shock) or pharmacological (amiodarone)
means. This is because atrial fibrillation leads to loss of atrial contribution to
ventricular filling and shortens the diastolic filling time. Digoxin is best avoided
for rate control and heart failure treatment, due to the high risk of ventricular
arrhythmias in the presence of myocardial disease. Beta blockers and verapamil
improve diastolic dystunction and control tachyarrhythmias. Specific therapies
for restrictive cardiomyopathy are steroids for sarcoidosis and scleroderma as
well as iron chelation therapy with desferrioxamine for hemochromatosis.
RECENT ADVANCES
The diagnosis of cardiac amyloidosis can now be confirmed by tissue biopsy.
Amyloid shows birefringence when stained with Congo red and viewed under
polarized light. Biopsy material can be obtained either from abdominal fat pad
or from rectal and gingival sites. Endomyocardial biopsy is only performed if the
above tissue biopsies are inconclusive and yet the clinical suspicion is strong.
Of late, cardiac MRI imaging with gadolinium enhancement has shown high
sensitivity and specificity for amyloidosis. Recently, chemotherapy targeted
against clonal plasma cells, that produce monoclonal light chains, has been tried
as a treatment modality in cardiac amyloidosis. Also, sequential cardiac and stem
cell transplant has been optimistically attempted.
C A S E
14
Hypertrophic
Cardiomyopathy
CASE PRESENTATION
A 48-year old man was brought to the emergency room by his office colleagues,
because he had fainted while climbing stairs. There was no history of tonic-clonic
jerks, frothing at the mouth or tongue bite. On regaining consciousness, the patient
admitted having had two such episodes in the past, one while playing cricket with
his son and the other while changing a flat-tyre of his car. There was no sensation of
palpitation before these syncopal episodes. There was no past history of exertional
chest pain or breathlessness, although at times he did feel light-headed at the
end of the day. He did not suffer from hypertension or diabetes but he did smoke
occasionally and took alcohol over the weekends. His father had passed away due to
sudden cardiac death at the age of 42 and one of his elder cousin brothers was on
treatment for heart disease.
On examination, the patient was not tachypneic and there was no sweating. The
pulse rate was 88 beats/min. and regular, but two distinct peaks were felt in systole.
Vigorous pulsations were seen over the carotid arteries. The BP was 118/78 mm Hg in
the right arm. There was no anemia, cyanosis or sign of congestive heart failure. The
apex beat was forceful and normal in location but two separate impulses were felt
on palpation. The S1 and S2 were normal with a prominent S4 audible in late diastole.
A short systolic murmur was appreciated at the upper left sternal border that peaked
in mid-systole and ended abruptly. There was no ejection click or palpable thrill and
the murmur did not radiate to the neck. The lung fields were clear.
CLINICAL DISCUSSION
From the history and physical examination, this patient had syncope due to left
ventricular outflow tract (LVOT) obstruction. The cause of obstruction possibly
had a familial basis. ECG showed tall R waves with T wave inversion, in the lateral
precordial leads, but there was no S-T segment shift or presence of Q waves. The
rhythm was sinus and the Q-T interval was not prolonged. The X-ray chest did not
reveal any cardiomegaly or sign of pulmonary edema.
On ECHO, the left ventricle cavity was small in size with a normal ejection
fraction. There was significant hypertrophy of the left ventricular walls, with
hypertrophy of the interventricular septum (IVS) exceeding that of the LV
posterior wall (LVPW). The anterior mitral leaflet (AML) impinged on the
Figure 14.1: ECHO showing asymmetrical septal hypertrophy

impinging on the left ventricular outflow tract
hypertrophied septum during systole (Fig. 14.1). The aortic valve was structurally
normal. On Doppler, an LV outflow tract gradient was demonstrated, proximal to
the aortic valve. The high velocity jet had a typical concave appearance. On colour
flow mapping, a mitral regurgitation jet was seen entering the left atrium. These
ECHO findings are typical of hypertrophic obstructive cardiomyopathy (HOCM).
In retrospect, there were several pointers towards the diagnosis of HOCM,
in the patient’s history and physical examination. History of syncope with an
ejection systolic murmur raised the possibility of aortic stenosis, but there was
no ejection click, palpable thrill or radiation of murmur to the neck. Moreover
on ECHO, the aortic valve leaflets were structurally normal and the outflow
tract gradient was located proximal to the aortic valve. A positive family history
of sudden cardiac death may be due to congenital channelopathies that cause
ventricular tachyarrhythmias but the patient denied palpitation and the ECG
showed sinus rhythm with a normal Q-T interval and no evidence of WPW
syndrome. Moreover in most channelopathies, the heart is structurally normal.
Atherosclerotic coronary artery disease is a prominent cause of premature
sudden cardiac death which may run in families. However, coronary artery
disease is rarely if ever associated with left ventricular hypertrophy, unless there
is severe hypertension. Syncopal episodes are also uncommon, unless there is
severe calcific aortic stenosis.
A double-peaked radial pulse that consists of a percussion wave and a tidal
wave, is known as pulsus bisferiens. It indicates rapid early ejection of the left
ventricle, followed by slow delayed emptying, after recoil of the vascular bed.
Pulsus bisferiens is typical of HOCM but also occurs in combined aortic valve
disease (stenosis plus regurgitation). The strong and bifid apex beat indicates
forceful atrial contraction preceding ventricular systole. A prominent S4 sound
in presystole also carries the same significance. These are both clinical signs of
reduced left ventricular compliance due to myocardial hypertrophy.
Case 14 Hypertrophic Cardiomyopathy 65
Figure 14.2: ECHO showing isolated hypertrophy

of the left ventricular apex
Hypertrophic cardiomyopathy is inherited as an autosomal dominant trait,

caused by mutation of genes that encode for sarcomere proteins of the cardiac
myocyte. It is characterized by left ventricular hypertrophy (LVH), systolic anterior
motion (SAM) of the anterior mitral leaflet and dynamic left ventricular outflow
tract obstruction (LVOTO). The left ventricular hypertrophy (LVH) is typically
(but not exclusively) asymmetrical, involving the septum more than the free wall.
Other causes of LVH such as severe aortic stenosis or uncontrolled hypertension
are typically absent. This asymmetrical septal hypertrophy (ASH) superficially
resembles the LVH observed in athletes. There is associated diastolic dysfunction
of the left ventricle. In a specific variant known as apical cardiomyopathy, there
is prominent hypertrophy of the ventricular apex without outflow obstruction
(Fig. 14.2). The ECG typically shows deep inverted T waves in the precordial leads.
Systolic anterior motion (SAM) of anterior mitral leaflet (AML) is the hallmark
of hypertrophic obstructive cardiomyopathy (HOCM). During later part of
systole, the AML moves anteriorly to coapt with the IV septum (Fig. 14.3). This
occurs due to drag or Venturi effect caused by high velocity in the LV outflow tract
(LVOT). SAM occurs during late stage of systole when the LV cavity is smaller and
causes dynamic LVOT obstruction. Therefore, the term hypertrophic obstructive
cardiomyopathy (HOCM) is more appropriate. If no LVOT obstruction is
demonstrable, the terminology used is non-obstructive idiopathic hypertrophic
subaortic stenosis (IHSS).
Figure 14.3: ECHO showing systolic anterior motion of anterior mitral leaflet
LVOT obstruction may be present at rest or become more pronounced after

provocation. Provocation is provided by prolonged standing, Valsalva manoeuvre
or giving sublingual nitrate. All these methods reduce left ventricular size and
therefore increase the likelihood of LVOT obstruction. Conversely, squatting and
isometric exercise such as hand-grip, increase left ventricular size and reduce the
degree of LVOT obstruction.
MANAGEMENT ISSUES
The goals of treatment in hypertrophic cardiomyopathy are to control symptoms
and to prevent sudden cardiac death (SCD). Symptoms include exertional
dyspnea, typical or atypical angina, palpitations and syncope. Exertional
symptoms are generally because of left ventricular hypertrophy with diastolic
dysfunction and sometimes due to associated mitral regurgitation. Syncopal
episodes are generally related to outflow tract obstruction and sometimes due to
tachyarrhythmias. Patients should be advised to avoid strenuous exercise such as
weight-lifting and not to participate in competitive sports.
Beta-blockers are the agents of first choice. They control heart rate, improve
diastolic filling and reduce the outflow tract gradient. Calcium-channel blockers
like verapamil are preferable, if beta-blockers are contraindicated. These agents
are also used for rate-control if atrial fibrillation exists, although rhythm-control
with amiodarone is preferable. This is because the loss of atrial contribution to
ventricular filling, is often associated with clinical deterioration. All patients
in atrial fibrillation should also receive an anticoagulant to reduce the risk of
thromboembolism. Digitalis, diuretics and vasodilators should be avoided.
If the patient is symptomatic and refractory to medical treatment, surgical
myomectomy is offered to relieve the outflow obstruction. In a patient who is
at high-risk to undergo a surgical procedure, alcohol septal ablation can be
performed by the percutaneous route. An injection of ethanol is given into one
of the septal perforator branches to create an area of myocardial infarction and
thereby widen the narrow outflow tract.
Sudden cardiac death (SCD) can be prevented by using an implantable
cardioverter defibrillator (ICD), in selected high-risk candidates. These subjects
are selected on the basis of identified risk factors for SCD. Risk factors include
septal thickness >25 mm, outflow tract gradient >30 mm Hg, non-sustained
ventricular tachycardia on 48 hour ambulatory Holter monitoring, history of
recurrent syncope and atleast 1 SCD in a relative aged <45 years.
RECENT ADVANCES
Recently, cardiac magnetic resonance imaging (MRI) has been used to identify
myocardial fibre disarray, as a risk factor for ventricular arrhythmias. Those
subjects with this demonstrable abnormality should be candidates for implantable
cardioverter defibrillator (ICD), to prevent sudden cardiac death (SCD). Alcohol
septal ablation as an interventional strategy, has gained popularity.
C A S E
15
Takotsubo
Cardiomyopathy
CASE PRESENTATION
A 33-year old woman came on her own to the emergency room at 2 am, with
the complaint of severe central chest pain and suffocation sensation, of one hour
duration. She was also short of breath and sweating, but her chest pain did not
radiate to the arms or lower jaw. There was no past history of exertional angina or
dyspnea and she did not have diabetes or hypertension. There was also no history
of a “flu-like” illness in the preceding week. She was pre-menopausal and her blood
lipid profile had never been checked. She did not smoke, consume alcohol or abuse
illicit drugs, although she was on antidepressant medications off and on. There was
no history of premature coronary artery disease is any of her family members. Upon
close questioning, the lady disclosed that she lived alone ever since her acrimonious
divorce 3 months back, five years after a traumatic marriage.
On examination, the patient was of average built, anxious, sweating and
tachypneic. The extremities were warm and her palms were moist. The pulse was
rapid, fair in volume, at a rate of 110 beats/min. with a BP of 140/90 mm Hg. There was
no anemia, cyanosis or pedal edema and the JVP was not raised. The thyroid gland
was not enlarged and there was no tremor or eye-sign of thyrotoxicosis. The apex
beat was normal in location and character and the precordium was unremarkable.
The S1 was loud and S2 normal in intensity with a soft S3 in early diastole. No murmur
or friction rub was audible. There were few rales over the lower thirds of the lung
fields.
CLINICAL DISCUSSION
From the history and physical examination, this patient developed acute left
ventricular dysfunction preceded by severe chest pain. ECG showed sinus
tachycardia and S-T segment elevation in practically all the leads, with upright
T waves (Fig. 15.1). X-ray chest findings were borderline cardiomegaly and
pulmonary interstitial edema but there was no mediastinal widening or pleural
effusion. ECHO revealed a mildly dilated left ventricle with an ejection fraction of
35%. The distal septum and the left ventricular apex were hypokinetic (Fig. 15.2).
The mitral and aortic valves were structurally normal and no colour flow jet or
abnormal velocity was detected. No intracardiac mass or pericardial effusion was
seen. The proximal aorta did not show dilatation or any sign of aortic dissection.
Figure 15.1: ECG showing sinus tachycardia with ST segment elevation
Figure 15.2: ECHO showing hypokinesia

of distal septum and apex
Whenever a patient has severe chest pain with S-T segment elevation on ECG
and wall motion abnormality on ECHO, the first possibility to cross the mind is an
acute coronary syndrome. This patient had no major coronary risk factors barring
psycho-social stress, but her troponin-T test was positive. Since she came to a
tertiary-care hospital, primary coronary angioplasty was planned. To everyone’s
surprise, the coronary angiogram turned out to be completely normal.
Another cause of severe chest pain with ECG findings is acute dissection
of the aorta. These patients are usually hypertensive and develop acute aortic
regurgitation with left ventricular volume overload. Our patient had no evidence
of aortic root dilatation, cleavage of the aortic wall or aortic regurgitation. Rarely,
dissection of the aorta can cause myocardial infarction, if the coronary ostia get
occluded by the intimal flap.
Acute pericarditis also presents with chest pain and S-T segment elevation
on the ECG. The pain is seldom excruciating and generally increases on deep
inspiration. The troponin-T test is almost always negative. Presence of regional
wall motion abnormality and reduced ejection fraction are unusual except if
there is an additional component of myocarditis. Acute viral myocarditis is often
preceded by a flu-like illness. It presents with acute left ventricular dysfunction
but chest pain is unusual. Classical findings are global hypokinesia with
depressed ejection fraction but regional wall motion abnormalities can occur if
the myocardial inflammation is patchy in distribution.
Some other causes of acute left ventricular dysfunction also need to be
considered. Low levels of serum electrolytes such as potassium, calcium and
Case 15 Takotsubo Cardiomyopathy 69
magnesium can impair myocardial contractility. Similarly, nutritional deficiency

of vitamins and trace elements like thiamine, selenium and carnitine can also
cause ventricular dysfunction. At times, thyroid storm and pheochromocytoma
can present with left heart failure and should be ruled out by appropriate assays
including serum thyroid hormone levels and urinary catecholamines. In our
case, the left ventricular dysfunction was clearly related to psycho-social stress.
Therefore, the definite diagnosis is stress-induced cardiomyopathy which is also
termed as Takotsubo cardiomyopathy.
Table 15.1: Synonyms of Takotsubo cardiomyopathy

• Broken heart syndrome
• Stress induced cardiomyopathy
• Neurogenic myocardial stunning
• Transient ventricular apical ballooning
Takotsubo cardiomyopathy (Table 15.1) is a recently described entity also

called the “broken heart syndrome”, neurogenic myocardial stunning or transient
left ventricular apical ballooning. Takotsubo is a Japanese name given to a round-
bottomed narrow-necked fishing pot, used to trap an octopus. The shape of the
left ventricle after apical ballooning, resembles this fishing pot. It occurs more
often in postmenopausal, middle-aged women. Acute intense emotional stress
is the usual triggering factor and the surge of catecholamines either causes
microvascular vasospasm or direct myocyte injury. Additionally, release of free
fatty acids by adrenalin induced lipolysis and hypokalemia due to an excess of
cortisol, impair myocardial contractility.
These patients usually present with severe chest pain and clinical signs of
left ventricular dysfunction. The ECG shows S-T segment elevation in multiple
coronary artery territories and the biomarker of myocardial injury, the troponin-T
test, is generally positive. The ECHO shows wall motion abnormality of the
left ventricular apex which does not conform to any particular coronary artery
territory and typically spares the basal segments. Coronary angiography does not
show any major occlusive lesion in most patients.
MANAGEMENT ISSUES
Before the clinical profile of this entity was clearly elucidated, many women
with Takotsubo cardiomyopathy were probably managed as anxiety related
panic attack or menopausal syndrome. Some were thought to have acute viral
myocarditis while still others improved on their own while being investigated for
thyroid hyperfunction and pheochromocytoma. When the patients present with
chest pain, S-T segment elevation and positive troponin-T test, it is prudent to
manage them as acute coronary syndrome. Aspirin, clopidogrel, beta-blocker
and low molecular weight heparin (LMWH) can all be instituted. An angiotensin
converting enzyme (ACE) inhibitor with a diuretic can be added for heart failure
management. Most patients make a rapid and event-free recovery, without any
long-term disability or recurrence.
RECENT ADVANCES
Till a few years back, some stray case-reports of Takotsubo cardiomyopathy were
published from Japan. With growing awareness about this entity, it is now being
increasingly diagnosed across the globe. Recently carvedilol, a beta-blocker with
additional alpha-blockade property, has been studied in a small cohort of cases
and shown modest benefits.
SECTION
Aortic Diseases
C A S E
16
Aneurysm
of Aorta
CASE PRESENTATION
A young man 21 years of age, came to his family physician with the complaint of
progressively increasing shortness of breath of 2 months duration. There was no
history of fever, productive cough, chest pain, hemoptysis or wheezing. The severity
of his breathlessness had compelled him to discontinue basketball, his favorite
sport. In fact he had been a regular member of his school and college basketball
teams. Interestingly, he was inducted into his senior school team in class 8, when he
was only 13 years of age, because of his extraordinary height. There was no history of
nasal allergy, recurrent sore-throat, bronchial asthma or prolonged fever with joint
pains during his childhood. He denied abusing tobacco, alcohol or any illicit drug.
During a routine medical check-up before joining college 3 years back, he had been
told of a heart murmur.
On examination, pulse was 88 beats/ min. regular, good in volume, with a BP
of 160/70 mm Hg in the right arm. The JVP was not raised and there was no ankle
edema. The precordium was hyperdynamic and there were visible pulsations in the
aortic area. The apex beat was heaving in nature and located in the sixth intercostal
space, in the anterior axillary line. A soft early diastolic murmur was audible along
the left sternal border, accompanied by a S3 sound in diastole.
The ECG was normal and X-ray chest showed mild cardiomegaly, with a hump
over the upper right border of the heart. There was no sign of pulmonary congestion.
ECHO revealed a dilated left ventricular cavity, with an end-systolic diameter of
52 mm and an ejection fraction of 45%. The aorta was enormously dilated, with
a dimension of 68 mm between the leading edges of the anterior and posterior
aortic walls. There was loss of the acute angle between the sinus of Valsalva and the
ascending aorta. The aortic valve leaflets were not thickened, but appeared distant
from the aortic walls. The mitral valve was structurally normal.
The patient was unusually tall, thin and lanky, with a height of 6 feet 1 inch and
a body-weight of only 48kg. While examining the radial pulse, it was observed that
his fingers were excessively long and tapering, with an extremely flexible wrist joint.
Upon measurement, the distance between the finger-tips of the hands stretched
sideways, was 6 feet 5 inches. In other words, the arm-span exceeded the height of
the patient. Also, the pubis-to-heel length was more than the crown-to-pubis height.
This physical appearance, coupled with the typical X-ray chest and ECHO findings
of aortic root dilatation and aortic regurgitation, are highly suggestive of Marfan’s
syndrome.
74 Section 5 Aortic Diseases
CLINICAL DISCUSSION
The normal diameter of the aortic root in end-diastole, ranges from 20 to
37 mm. A dimension exceeding 40 mm constitutes aortic root dilatation, which
has several causes (Table 16.1). If the dilatation is due to advanced age or systolic
hypertension, the aortic annulus is normal in diameter. However, if the dilatation
is caused by medial necrosis of the aortic wall or an inflammatory aortitis, the
annulus is dilated with associated aortic regurgitation. In post-stenotic aortic
dilatation, the aortic valve leaflets are thickened and calcific.
Table 16.1: Causes of aortic root dilatation

• Atherosclerosis Unfolded aorta
Hypertension (elderly)
• Medial necrosis Marfan syndrome
Ehlers-Danlos syndrome
• Aortoarteritis Syphilitic (rare)
Takayasu disease
• Collagen disease Reiter’s syndrome
Ankylosing spondylitis
• Post-stenotic Aortic stenosis
• Post-traumatic Aortic aneurysm
An aortic root diameter exceeding 60 mm, is a feature of aortic aneurysm

which may be saccular or fusiform. The aneurysm appears as a balloon-like
stretching of the aortic wall, which expands in systole and compresses the left
atrium. The aortic valve cusps appear distant from the aortic walls, even when the
valve is open in systole (Fig. 16.1).
Figure 16.1: Diagram showing aneurysmal dilatation of the ascending aorta.

RV: Right ventricle; LV: Left ventricle; AO: Aorta; LA: Left atrium
Marfan’s syndrome is a connective tissue disorder, inherited as an autosomal

dominant trait. The defect lies in the elastic fiber nature of the connective tissue.
There is mutation in the gene encoding fibrillin-1, a connective tissue component
and regulatory protein of transforming growth factor (TGF). Several striking
Case 16 Aneurysm of Aorta 75
abnormalities of the skeletal system are observed. The typical body habitus is a
tall and slender figure with long tapering fingers and increased joint mobility.
The pubis-to-heel measurement exceeds the crown-to-pubis length. Associated
abnormalities include a high-arched palate, pectus carinatum, dislocated ocular
lenses (ectopia lentis) and presence of inguinal hernias (Table 16.2).
Table 16.2: Clinical features of Marfan’s syndrome

Skeletal Cardiac Others
Tall stature Aortic dilatation Dislocation of lens
Joint hypermobility Aortic aneurysm High-arched palate
Arm-span > height Aortic dissection Crowded dentition
Long digits (arachnodactyly) Aortic regurgitation Pectus carinatum
Scoliosis and sternal deformity Mitral valve prolapse Inguinal hernia
Cardiovascular complications are initiated by cystic medial necrosis of the

ascending aorta. There is dilatation of the aortic root with aortic valve insufficiency
and left ventricular volume overload (Fig. 16.2). Catastrophic dissection involving
the proximal aorta is a serious and potentially lethal complication. Mitral valve
prolapse is sometimes associated.
Figure 16.2: ECHO showing an aneurysmal aorta

with jet of aortic regurgitation
Although syphilitic aortitis is now distinctly rare, Takayasu’s aorto-arteritis

still afflicts young women in south Asia. It causes aneurysmal aortic dilatation
and stenosis of the major branches emerging from the aorta. Blood pressure
in the two arms may be different due to narrowing of subclavian artery on one
side. On the other hand, hypertension may be present in the lower limbs due to
reno-vascular stenosis. Therefore, Takayasu’s aorto-arteritis is also designated as
“reverse coarctation”.
MANAGEMENT ISSUES
The medical management of an aortic aneurysm depends upon its size, the blood
pressure and the presence or absence of aortic dissection. If the aneurysm is
small, stable in size and there is no dissection, it suffices to adequately control
the blood pressure. A drug with negative ionotropic effect such as a beta-blocker
is preferable, since it reduces the shearing force on the aorta. The patient is
counselled to avoid strenuous and burst activity like weight-lifting. Follow-up
echo is recommended every 6 to 12 months, to assess the size of aneurysm and
degree of aortic regurgitation.
Surgical intervention is considered if the aneurysm is growing in size on serial
imaging, there is evidence of impending rupture or dissection, or if the aortic
regurgitation is severe (Table 16.3). The affected segment of the ascending aorta
is resected and replaced with a prosthetic graft.
Table 16.3: Indications for aortic aneurysm surgery

• Severe dilatation more than 50 mm
• Rapid aneurysm growth > 10 mm/year
• Dissection of the proximal aorta
• Severe aortic regurgitation
RECENT ADVANCES
Newer imaging techniques such as computed tomography (CT) and magnetic
resonance imaging (MRI) have obviated the need for aortography, in the
evaluation of aortic aneurysms. Serial imaging can identify an expanding size,
impending rupture and an associated dissection.
C A S E
17
Dissection
of Aorta
CASE PRESENTATION
A 64-year old woman presented to the emergency department with sudden onset
of severe chest pain, one hour back. The chest pain was central in location and it
peaked instantly. The pain was also felt over the back between her scapulae, but
did not radiate to the arms or neck. There was no history of suffocation, choking,
palpitation or syncope and she had never experienced such chest pain before. She
had a history of hypertension for which she had been prescribed multiple drugs.
However, she was irregular with her medication and often skipped her doses. As a
result, her BP hovered around 150/100 mm Hg on most occasions. She was not a
diabetic but was advised to take atorvastatin for her elevated LDL-cholesterol. She
did not consume alcohol, but smoked a pack of cigarettes per day for the last 40
years. Although distressed by severe chest pain, she managed to tell the attending
doctor that the grip of her left hand felt weak since one hour.
On examination, she was clearly distressed but not dyspneic or diaphoretic. Pulse
rate was 104 beats/min., with a BP of 174/106 mm Hg in her right arm. All peripheral
pulses in the lower extremities were palpable, but a bruit was audible over the right
carotid artery. The JVP was not raised and there was no pitting edema around the
ankles. The apex beat was heaving in nature and slightly displaced outwards towards
the axilla. The S1 was normal and A2 was loud, but there was no gallop sound. A faint
early-diastolic murmur was audible along the left sternal border.
CLINICAL DISCUSSION
In an elderly patient, the most frequent but by no means the sole cause of
excruciating chest pain is myocardial infarction. Other causes of severe chest
pain are pulmonary embolism and spontaneous pneumothorax. The pain of
pleuro-pericarditis, oesophagitis or costo-chondritis is seldom excruciating.
Acute aortic dissection is an uncommon but potentially fatal cause of severe
chest pain that merits a high index of clinical suspicion.
The ECG showed sinus tachycardia but no S-T segment shift and the troponin-T
test was negative on admission. X-ray chest findings were mild cardiomegaly
with a left ventricular contour and slight mediastinal widening due to aortic root
dilatation. There was no basal atelactasis, pleural effusion or pneumothorax.
ECHO revealed dilatation of the aortic root, with a false lumen cleaving the
anterior aortic wall and having a blind end. An intimal flap separated the false
lumen from the true aortic lumen (Fig. 17.1). On colour Doppler, a mosaic jet
was observed in the left ventricular outflow tract, along the septum. There was no
pericardial effusion. These findings were consistent with the diagnosis of acute
dissection of the aorta.
Figure 17.1: ECHO showing cleavage of the anterior aortic wall

with intimal flap between true and false lumens
Acute myocardial infarction is an unlikely possibility in this case. The pain of

myocardial infarction does not reach its maximum intensity instantly, but builds
up gradually. Moreover, the radiation and accompaniments that are typical of the
pain of myocardial infarction, were absent in this case. The ECG did not show
S-T segment elevation and the troponin test was negative. On ECHO, cleavage of
aortic wall and presence of aortic regurgitation are not typical features of acute
myocardial infarction.
Table 17.1: Causes of aortic dissection

• Marfan’s syndrome
• Accelerated hypertension
• Hypertension in pregnancy
• Trauma; accidental or surgical
Dissection of aorta is often due to accelerated hypertension and may

complicate hypertension in pregnancy. Other causes are aortic coarctation and
Marfan’s syndrome (Table 17.1). Aortic dissection cleaves the media of the aortic
wall, with the adventitia and outer media forming the outer wall and the inner
media and intima forming the inner wall. The false lumen produced by cleavage
has a blind distal end while the proximal end communicates with the true lumen
at the site of aortic tear. The intimal flap oscillates between the true and false
lumens (Table 17.2). Other reasons for deformity of the anterior aortic wall are
aortic root abscess in infective endocarditis and an aneurysm of the sinus of
Valsalva.
Case 17 Dissection of Aorta 79
Table 17.2: Typical features of aortic dissection

• Dilatation of the proximal aortic root > 42 mm
• Anterior or posterior wall thickness >15 mm
• Double echo-line of the involved aortic wall
• Space between outer and inner wall >5 mm
• False lumen within aortic wall with blind end
• Intimal flap between true and false lumens.
There are various classifications of aortic dissection. The simplest classification

is of the Stanford group, which categorizes dissection as Type A (proximal), where
the ascending aorta is involved and Type B (distal), where the ascending aorta
is spared. This classification has prognostic as well as therapeutic implications.
DeBakey Type I dissection extends from the ascending to descending aorta while
Type II is confined to the ascending aorta. Type III is confined to the descending
aorta and akin to Stanford Type B (Table 17.3).
Table 17.3: Classification of aortic dissection according to location

DeBakey type Stanford group Location of dissection Incidence
I
II } A Ascending to descending aorta
Confined to ascending aorta
10%
70%
III B Confined to descending aorta 20%
The complications of aortic dissection are related to the involvement of the

aortic valve, pericardial sac and the arteries emerging from the aorta. Proximal
involvement of the aortic valve can cause acute aortic regurgitation and heart
failure. Hemorrhagic pericardial effusion can cause cardiac tamponade. Involve
ment of the coronary artery ostium can actually lead to myocardial infarction
(Table 17.4). Involvement of the subclavian arteries can cause upper limb ischemia
and discrepancy between the radial pulse volume on both sides. Involvement of
carotid arteries can cause cerebral ischemia as in our case, who had weakness of
the left hand and a right-sided carotid bruit. In distal aortic dissection, there may
be mesenteric, renal, spinal cord or lower limb ischemia (Fig. 17.2).
Table 17.4: Atypical features of aortic dissection

• Occlusion of neck vessels
• Aortic valve regurgitation
• Left ventricular dysfunction
• Myocardial infarction
• Pericardial effusion
Figure 17.2: Complications of aortic dissection
MANAGEMENT ISSUES
The management of aortic dissection depends upon its location and extent, the
presence or absence of complications and the hemodynamics of the patient.
In distal (Type B) dissection, it generally suffices to control the blood pressure
adequately. Agents with negative ionotropic effect such as a beta-blocker or
verapamil are preferable as they reduce the shearing force on the aorta and the
progression of dissection. A vasodilator such as sodium nitroprusside or an ACE-
inhibitor may be added if the blood pressure remains significantly elevated. In the
presence of abdominal, spinal or lower limb severe ischemia, an endovascular
stent graft may be deployed to cover the dissection.
In proximal (Type A) dissection, surgical intervention is preferable unless
the hemodynamics are compromised, in which case a conservative approach is
adopted. The affected segment of the ascending aorta is resected and replaced
with a prosthetic graft. The aortic valve and coronary ostia may need to be
reconstructed. In extensive aortic dissection (DeBakey Type I), a combined
approach that includes proximal resection with graft and distal endovascular
stenting may be used.
RECENT ADVANCES
Newer imaging techniques such as computed tomographic (CT) angiography
and magnetic resonance imaging (MRI) have obviated the need for aortography,
in the evaluation of aortic dissection. The sensitivity and specificity of these
tests, for a confirmatory diagnosis of aortic dissection, are in excess of 95%. The
technique of percutaneous endovascular stenting to manage DeBakey Type I and
III dissection, has also undergone considerable refinement.
C A S E
18
Coarctation
of Aorta
CASE PRESENTATION
A young man 27 years of age, presented to the out-patient clinic with general
fatigue and difficulty in climbing even two flights of stairs. He was diagnosed to have
systemic hypertension five years back and had been on antihypertensive drugs ever
since. Besides his fatigue, he also complained of episodic headache with dizziness.
These episodes were often related to undue physical exercise, emotional upset or
to missing of his medication. There was no history of cyanotic spells or fleeting joint
pains during childhood. He denied abusing tobacco, alcohol or illicit drugs.
On examination, there were no facial features of an endocrine disorder such as
thyrotoxicosis, acromegaly or Cushing’s syndrome, but his lower limbs were unusually
thin. The radial pulse was 84 beats/min. and bounding in nature. The BP in his right
arm was 170/100 mm Hg. Pulse amplitude over the femoral arteries was reduced
in volume and delayed compared to the brachial pulsation. Also, there were visible
pulsations in the suprasternal notch. The cardiac apex beat was heaving in nature
and slightly displaced towards the left axilla. On auscultation, S1 was normal, A2 was
loud and a presystolic S4 was audible. An ejection systolic murmur was heard at the
upper left parasternal area. Incidentally, the same murmur was also audible over the
interscapular region. Soft continuous murmurs were also heard over the scapulae.
On abdominal auscultation, there was no bruit heard over the renal arteries.
CLINICAL DISCUSSION
When confronted with a young patient with moderate to severe systemic
hypertension, the clinician should be alert to the possibility of secondary
hypertension. Incidentally, secondary hypertension constitutes less than 5% of
all hypertensive patients, while most have primary or essential hypertension.
Usual causes of secondary hypertension are:
• Endocrine disorders
• Renovascular disease
• Coarctation of aorta.
In this patient, ECG showed tall R waves in leads V5 and V6 with deep S
waves in leads V1 and V2, indicating the presence of left ventricular hypertrophy
(Fig. 18.1). X-ray showed mild cardiomegaly with a rounded contour of the left
heart border. There was “notching” of the undersurface of the 4th to 8th ribs and
Figure 18.1: ECG showing tall R waves in V5,V6

with deep S waves in V1,V2
a “figure-of-3” appearance of the aortic arch. ECHO revealed concentric left

ventricular hypertrophy with normal ejection fraction. Aortic valve was bicuspid
with thickened leaflets and an insignificant gradient across the valve. The mitral
valve was normal and there was no ventricular septal defect. Doppler signal from
the suprasternal notch showed a pulsatile aortic arch with a distal aortic gradient
that tapered into diastole. A mosaic jet was seen in the descending aorta, directed
away from the transducer (Fig. 18.2).
Figure 18.2: ECHO showing a mosaic jet

in the descending aorta
Coarctation of aorta is the most likely cause of hypertension in this case.

There was clinical, radiological and cardiographic evidence of left ventricular
hypertrophy. There were brachio-femoral delay, suprasternal pulsations, a
parasternal and interscapular systolic murmur (coarctation itself ) and continuous
intercostal murmurs (collateral vessels). All these auscultatory findings are
pathognomic of aortic coarctation. The murmur of coarctation itself may be
continuous if the narrowing is severe, while presence of an ejection click indicates
an associated bicuspid aortic valve. On X-ray chest, notching of the lower border
of ribs indicates arterial collateralization, to by-pass the aortic obstruction. The
“figure-of-3” appearance is produced by indentation of the aorta at the site of
coarctation, with dilatation on either side of the narrowing. The suprasternal high
velocity Doppler signal is characteristic of aortic coarctation.
Coarctation of the aorta is a localized narrowing of the aortic arch (Fig. 18.3),
due to a fibrous ring encircling the wall or a shelf projecting into the lumen. The
narrowing is in the region of the ligamentum arteriosum (juxta-ductal) and may
be pre-ductal or post-ductal. The aorta distal to the narrowing is often dilated
and aneurysmal. Coarctation may present in the neonatal period (infantile type)
Case 18 Coarctation of Aorta 83
Figure 18.3: Coarctation of aorta
with heart failure, in which case the narrowing is diffuse, tubular and severe
(hypoplastic aorta). In adult type coarctation, the narrowing is discrete, ring-like,
less severe and detected during evaluation of systemic hypertension.
Some patients may complain of pain and/or weakness in the legs
(claudication), due to reduced femoral blood flow. Brachio-femoral delay pertains
to the difference in amplitude and timing between the brachial and femoral
pulsations. Therefore, the blood pressure is higher in the upper limbs than in the
lower limbs. If the left subclavian artery is also narrowed by coarctation, the blood
pressure is only elevated in the right arm. In Takayasu’s aorto-arteritis (pulseless
disease), which is also called “reverse coarctation”, the blood pressure is reduced
in the upper limbs compared to the lower limbs, because there is stenosis at the
origin of aortic arch branches.
Hypertension in coarctation of aorta is multifactorial. The aorta proximal to
the narrowing is stiff and the carotid sinus baroreceptors are set at a higher level.
Additionally there is a renal component, due to narrowing proximal to the renal
arteries. Coarctation of aorta is associated with a bicuspid aortic valve in half the
patients. Other cardiac anomalies are ventricular septal defect and mitral valve
prolapse (Table 18.1). In 10 to 20% patients, there are intracranial aneurysms
around the circle of Willis. Aortic coarctation may be associated with Turner’s
syndrome, characterized by a short-statured female with broad chest and a
Table 18.1: Abnormalities associated with coarctation

• Bicuspid aortic valve
• Mitral valve prolapse
• Ventricular septal defect
• Aneurysm sinus of Valsalva
• Intracranial Berry aneurysm
webbed neck. Long-term complications of coarctation are heart failure and stroke
due to hypertension, dissection of proximal aorta and ruptured aneurysm of the
sinus of Valsalva. The bicuspid aortic valve can develop stenosis or regurgitation
and be the site of endocarditis. Finally, sub-arachnoid hemorrhage can occur due
to ruptured intracranial aneurysm (Table 18.2).
Table 18.2: Complications associated with coarctation

• Congestive heart failure
• Endocarditis of aortic valve
• Dissection of proximal aorta
• Sub-arachnoid hemorrhage
• Stroke due to hypertension
• Aortic valve stenosis or regurgitation
• Ruptured sinus of Valsalva aneurysm
MANAGEMENT ISSUES
Management of secondary hypertension due to aortic coarctation, is governed
by the same principles that apply to the treatment of essential hypertension. This
involves the judicious use of a combination of antihypertensive agents. Several
drugs are used including diuretics, beta-blockers, calcium-antagonists and ACE-
inhibitors (or ARBs), since the hypertension is generally moderate to severe in
grade and severity. Definitive treatment of aortic coarctation (systolic gradient >
30 mm Hg) is surgical excision of the area of aortic narrowing with an end-to-end
anastomosis. A prosthetic tubular Dacron graft may also be interposed. Another
technique is patch aortoplasty at the site of coarctation.
RECENT ADVANCES
Recently, the technique of percutaneous balloon aortoplasty has been developed.
It is most suitable for discrete stenosis or if narrowing has recurred following
surgical correction of coarctation. The incidence of restenosis after aortoplasty is
reduced by stenting.
C A S E
19
Sinus of
Valsalva Aneurysm
CASE PRESENTATION
A 27-year old man was brought to the emergency room by his colleagues, with
history of severe chest pain followed by light-headedness. The patient worked as a
manual labourer in a construction company and his job involved strenuous activity,
including lifting heavy weights. However, there was no past history of exertional
chest pain or breathlessness. In fact, he had never suffered from any prolonged
illness necessitating medication. He denied addiction to tobacco, alcohol or any
other illicit drug. There was no past history of hypertension, diabetes, bronchial
asthma or pulmonary tuberculosis. Moreover, none of his family members had heart
disease or had succumbed to sudden cardiac death.
On examination, the patient looked anxious, but he was not in any distress. The
hands were cold and clammy and the pulse was feeble in volume. The heart rate
was 112 beats/min. with a BP of 96/64 mm Hg in the right arm, while lying in bed.
There was no pallor, cyanosis or ankle edema and the JVP was not raised. The apex
beat was normal in location and there was no parasternal heave. A continuous
systolo-diastolic murmur was heard along the left sternal border, more so in the
upper portion. The heart sounds could not be appreciated separately, because of
the tachycardia as well as the long murmur. The breath sounds were vesicular in
nature and air entry was normal and equal over both lung fields. There was no area
of bronchial breathing or muffled breath sounds. No rhonchi, crepitations or pleural
rub were audible.
CLINICAL DISCUSSION
From the history and physical examination, this previously healthy young man
had severe chest pain, hypotension and a continuous murmur. ECG showed sinus
tachycardia with narrow QRS complexes. There were no Q waves, S-T segment
shift or T wave inversion. X-ray chest showed a normal sized heart. There was no
basal atelectasis, pleural effusion or pneumothorax. The causes of severe chest
pain with hypotension are:
• Pulmonary embolism
• Pneumothorax
In this case, acute myocardial infarction is unlikely because the patient had
no coronary risk factors and the ECG did not show any Q waves or ST-T changes.
Dissection of the aorta is also unusual in the absence of prior uncontrolled
hypertension or coarctation of aorta. Spontaneous pneumothorax is a possibility,
but this patient had no history of a predisposing lung condition such as
bronchial asthma or pulmonary tuberculosis. Moreover, there was no clinical or
radiological evidence of pneumothorax. Pulmonary embolism generally occurs
after prolonged immobilization, but this patient was active and did not have any
predisposing factor for deep vein thrombosis of the leg veins. Moreover, there was
no area of collapse or consolidation on the chest X-ray.
On ECHO, all heart chambers were of normal size and all cardiac valves were
structurally normal. There were no vegetations over the leaflets or any mass or
thrombus in any chamber cavity. On careful examination of the short-axis view
at the level of the aortic valve, a flow velocity and colour flow map was seen
extending from the anterior aspect of aorta to the right ventricular outflow tract
(RVOT). Therefore, the definite diagnosis in this case is ruptured aneurysm of the
sinus of Valsalva.
An aneurysm of the sinus of Valsalva occurs as a result of congenital weakness
of one of the sinuses, known as annulo-aortic ectasia. Rupture of the aneurysm
may be spontaneous or due to increased aortic pressure, as occurs during
isometric exercise. Sometimes the rupture may be due to the presence of infective
endocarditis. Aneurysm of the noncoronary sinus ruptures into the right atrium,
while that of the right coronary sinus ruptures into the right ventricle.
Table 19.1: Causes of continuous murmur

• Venous hum
• Mammary souffle
• Aorto-pulmonary window
• Coarctation of the aorta
• Coronary arterio-venous fistula
• Ruptured aneurysm, sinus of Valsalva
In either case, there is continuous flow from the aorta to the right-sided
chamber, since the aortic pressure is higher than the chamber pressure. This
forms the basis of the continuous murmur heard when the aneurysm ruptures.
There are several causes of a continuous murmur over the precordium (Table
19.1). Ruptured aneurysm of the left coronary sinus is the least common of all and
may present with acute aortic regurgitation due to communication between the
aorta and the left ventricle. Other causes of acute aortic regurgitation are chest
wall trauma, infective endocarditis and dissection of aorta (Table 19.2).
Table 19.2: Causes of acute aortic regurgitation

• Annulo-aortic ectasia
• Blunt chest-wall trauma
Case 19 Sinus of Valsalva Aneurysm 87
Figure 19.1: Diagram showing sinus of Valsalva aneurysm, anterior to the aortic valve.
AV: Aortic valve; PA: Pulmonary artery; PV: Pulmonary valve; TV: Tricuspid
valve; SOV: Sinus of valsalva; RVOT: Right ventricular outflow tract
An aneurysm of sinus of Valsalva (SOV) appears as an outpouching of the

dilated coronary sinus anterior to the anterior aortic wall (Fig. 19.1), protruding
into the right ventricular outflow tract (RVOT). A rupture of this aneurysm into
the right ventricle produces right ventricular volume overload, of which there are
several causes (Table 19.3). Aneurysm of sinus Valsalva or the fistula created by its
rupture, is best visualized on transesophageal echocardiography (TEE). Cardiac
abnormalities associated with a sinus of Valsalva aneurysm are bicuspid aortic
valve and coarctation of aorta.
Table 19.3: Causes of right ventricular overload

• From right atrium
– atrial septal defect (ASD)
– tricuspid regurgitation (TR)
• From pulmonary artery
– pulmonary regurgitation (PR)
• From left ventricle
– ventricular septal defect (VSD)
• From aortic root
– ruptured aneurysm of sinus of Valsalva (SOV)
MANAGEMENT ISSUES
Newer imaging techniques such as computed tomography (CT) and magnetic
resonance imaging (MRI) have obviated the need for root aortography, to evaluate
the sinus of Valsalva. The size of the aneurysm, its precise location, the coronary
sinus involved and the fistula created by its rupture, can be accurately visualized
by these techniques. The management is surgical and involves resection of the
aneurysm and the created fistula, repair of the aortic annulus and replacement of
the affected segment with a prosthetic graft.
SECTION
6
Pulmonary
Diseases
C A S E
20
Pulmonary
Stenosis
CASE PRESENTATION
An 18- year old girl was taken to a neurologist by her parents. They had come back
from a hill-resort vacation two days back, where she had fainted while playing
badminton. There were no accompanying tonic-clonic movement, tongue-bite or
frothing at the mouth. There was no past history of seizures, syncopal episodes,
anoxic spells or squatting attacks during early childhood. A computed tomography
(CT) scan of the brain and an electroencephalogram (EEG) were taken which were
normal. The girl was referred to a cardiologist for evaluation of a heart murmur,
which was detected by the astute neurologist.
On examination, pulse was of low volume at a rate of 84 beats/min. with a BP of
104/66 mm Hg. Prominent a waves were observed in the venous pulsations at the
neck. The apex beat was normal in location and character. A sustained left parasternal
heave was palpable, with the inner edge of the palm. The S1 was normal and the S2
appeared single; a right-sided S4 was heard in diastole. A long and harsh ejection
systolic murmur was audible over the upper left parasternal area. The murmur was
not preceded by an ejection click or accompanied by a palpable thrill and it did not
radiate towards the carotid arteries.
CLINICAL DISCUSSION
From the history and physical examination, this young girl probably had some
cardiac outflow tract obstruction, which led onto syncope. The most likely
diagnosis in this case is pulmonary valve stenosis (Fig. 20.1). The a waves in the
jugular veins indicate forceful right atrial contraction against a noncompliant right
ventricle. The parasternal heave is indicative of right ventricular hypertrophy.
The S2 appears single because the P2 is muffled. If P2 is audible, the splitting of
S2 is wide due to prolonged right ventricular (RV) ejection time. Other reasons
for wide splitting of S2 are right bundle branch block (delayed RV activation) and
atrial septal defect (increased RV ejection volume).
The long and harsh ejection systolic murmur of pulmonary stenosis indicates
turbulent flow across the narrow valve. Length and loudness of the murmur
correlate with the severity of stenosis but once right ventricular failure sets
in, the murmur becomes short and soft. The systolic murmur of pulmonary
stenosis radiates towards the back and increases in intensity during inspiration.
92 Section 6 Pulmonary Diseases
Figure 20.1: Pulmonary valve stenosis
An ejection click and palpable thrill are less often observed compared to aortic
stenosis. Interestingly, the ejection click of pulmonary stenosis is the only right-
sided heart sound that decreases during inspiration.
The ejection click marks the end of isovolumic contraction and heralds the
onset of the ejection period. During inspiration, the increase in right ventricular
end-diastolic pressure (RVEDP) leads to partial opening of the pulmonary valve
and reduces the intensity of the ejection click. The ejection click is a feature
of valvular stenosis and is not heard in infundibular stenosis. It also helps to
differentiate pulmonary stenosis from atrial septal defect which produces a
similar murmur with wide splitting of the second heart sound or S2.
Figure 20.2: ECG showing tall R waves in V1 to V3,

with inversion of T waves
ECG showed tall R waves in right precordial leads with T wave inversion
(Fig. 20.2). There was right axis deviation of the QRS complex and right atrial
enlargement (P. pulmonale). X-ray chest findings were an enlarged right ventricle
to the right of midline, with a dilated pulmonary artery suggestive of post-stenotic
dilatation. The lung fields were oligemic (Fig. 20.3). On ECHO, the left ventricle
was normal in size with a normal ejection fraction and the free wall of the right
ventricle was thickened. The mitral and aortic valves were structurally normal.
The pulmonary valve leaflets were mildly thickened with mild systolic doming
and restricted excursion. On colour flow mapping, a jet was observed in the
proximal pulmonary artery, with increased systolic velocity across the valve on
Doppler (Fig. 20.4).
Case 20 Pulmonary Stenosis 93
Figure 20.3: X-ray showing dilated pulmonary artery,

with oligemic lung fields
The ECG findings of right ventricular hypertophy (RVH) are tall R waves in
lead V1, S-T segment depression and T wave inversion (RV strain) and right axis
deviation of the QRS vector. The voltage criteria of RVH are R in V1> 4 mm, R/S
ratio in V1> 1 and S in V6>7 mm. On chest X-ray, right ventricular enlargement and
pulmonary artery dilatation are also seen in pulmonary hypertension but in that
case there is pulmonary plethora and not oligemia. When pulmonary stenosis
is a component of Fallot’s tetralogy, the pulmonary artery is small (pulmonary
atresia) and not dilated. In pulmonary hypertension, ECHO findings are a
pulmonary regurgitant jet on color flow mapping and increased trans-tricuspid
flow velocity (TR Vmax).
Figure 20.4: ECHO showing a color flow map

in proximal pulmonary artery
Pulmonary stenosis needs to be clinically differentiated from other conditions

that produce a systolic murmur over the upper left parasternal area. These clinical
entities are pulmonary hypertension, aortic stenosis and a high cardiac output
state. Pulmonary hypertension is also associated with clinical, electrographic and
radiological signs of right ventricular hypertrophy (Table 20.1) but the ejection
murmur is accompanied by a loud P2 sound and sometimes the murmur of
pulmonary regurgitation. Moreover, the lung fields show pulmonary plethora
and not oligemia.
The systolic murmur of aortic stenosis is often accompanied by an ejection
click and a palpable thrill, radiates towards the neck and is better heard during
Table 20.1: Diagnostic criteria of right ventricular hypertrophy

• Clinical: Sustained left parasternal heave
• ECG: Tall R waves in right precordial leads
• X-ray: Cardiac silhouette to the right of midline
• ECHO: Thickening of right ventricular free wall
expiration. Moreover, there are clinical, electrographic and radiological signs of

left ventricular hypertrophy. An innocent hemic murmur known as Still’s murmur
is sometimes heard in anemia, pregnancy, thyrotoxicosis and beri-beri disease.
It is associated with a good volume pulse wide pulse-pressure and there is no
ejection click or palpable thrill. On electrographic and radiological investigations,
the cardiac chambers and valves are structurally normal.
Valvular pulmonary stenosis is often congenital in origin, as part of the
Rubella syndrome. Rarely, valvular pulmonary stenosis is due to rheumatic heart
disease (pulmonary valve involvement is least common) or carcinoid syndrome
(tricuspid valve involvement is more common). Subvalvular or infundibular
stenosis is often a part of Fallot’s tetralogy. Supravalvular stenosis due to a
discrete shelf-like band or a tunnel type deformity, is the least common form of
pulmonary stenosis (Table 20.2).
Table 20.2: Causes of pulmonary stenosis

• Congenital Rubella syndrome
• Acquired Carcinoid syndrome
• Subvalvular Fallot’s tetralogy
• Supravalvular Tunnel-type defect
MANAGEMENT ISSUES
There is no medical management of pulmonary stenosis. Surgery is indicated
in the presence of symptoms, right ventricular hypertrophy and a systolic
gradient above 50 mm Hg. The stenotic pulmonary valve has to be replaced
by a surgical procedure, if the valve anatomy is distorted. Artificial pulmonary
valve deployment is also feasible by the percutaneous route and expertise in this
technique is growing. In isolated subvalvular or infundibular pulmonary stenosis,
subvalvular muscle resection with valvotomy is the preferred procedure. When
infundibular stenosis is part of Fallot’s tetralogy, the procedure is combined
with patch closure of the ventricular septal defect. Pulmonary regurgitation is
the leading complication of valvotomy, which leads to dilatation of the tricuspid
valve annulus and enlargement of the right-sided cardiac chambers.
RECENT ADVANCES
As mentioned above, expertise in the technique of percutaneous artificial
pulmonary valve deployment has increased considerably. Also, percutaneous
native valve balloon valvuloplasty has gained popularity in recent years.
C A S E
21
Pulmonary
Hypertension
CASE PRESENTATION
A 23-year old unmarried girl was brought to a physician with the complaints of
progressively increasing breathlessness and fatigue on exertion, for the past 1 year.
There was no history of fever, cough with purulent sputum, chest pain or hemoptysis.
There was also no past history of seasonal allergy, bronchial asthma, anoxic spells or
squatting attacks during her childhood. The patient had normal appetite without
any weight-loss or history of night-sweats. On two occasions in the preceding
month, she had fainted for less than five minutes. The fainting episodes were not
preceded by palpitation, accompanied by tonic-clonic movements or followed by
any abnormal behaviour. The parents of the girl denied any history of prolonged
fever with painful joints, during her childhood or adolescence.
On examination, the pulse was feeble in volume at a rate of 92 beats/min. The BP
was 106/68 mm Hg in the right arm and the patient was afebrile. A tinge of cyanosis
was noticed over the tongue and lips but there was no clubbing of the finger-nails.
Prominent a waves were observed in the venous pulsations at the neck, but there
was no pedal edema. The apex beat of the heart was normal in position and had
no special character. A parasternal heave was palpable along the left sternal border,
with systolic pulsations felt over the 2nd left intercostal space. The S1 was normal but
the P2 was accentuated. Splitting of S2 could be appreciated upto the cardiac apex.
No S3 or S4 gallop sound was heard. A high-pitched diastolic murmur was audible
along the sternum. The breath sounds were vesicular in nature without any rhonchi
or crepitations.
CLINICAL DISCUSSION
From the history and physical examination, this young girl probably had cardiac
outflow tract obstruction, that was responsible for her dyspnea and fatigue and
also led to syncope. The most likely cause of low cardiac output in this case is
pulmonary arterial hypertension. The prominent a waves in the jugular veins
indicate forceful right atrial contraction against a noncompliant right ventricle.
A left parasternal heave indicates right ventricular enlargement while systolic
pulsations over the pulmonary area are due to dilatation of the main pulmonary
artery.
Accentuation of the P2 is characteristic of pulmonary arterial hypertension.
The fact that the P2 is audible even at the cardiac apex is itself indicative of the
fact that the P2 is loud and the pulmonary hypertension is severe. Pulmonary
hypertension causes pulmonary regurgitation even with a structurally normal
pulmonary valve. This produces a high-pitched diastolic murmur known as the
Grahm-Steel murmur. This murmur is short and does not increase during inspira
tion because the right ventricular end-diastolic pressure (RVEDP) is already high.
ECG showed tall R waves in right precordial leads and deep S waves in
left precordial leads, indicative of right ventricular hypertrophy (Table 21.1).
Table 21.1: Criteria for right ventricular hypertrophy

• Clinical: Sustained left parasternal heave
• ECG: Tall R waves in right precordial leads
• X-ray: Cardiac silhouette to the right of midline
• ECHO: Thickening of right ventricular free wall
Tall P waves (P. pulmonale) were due to right atrial enlargement. X-ray chest
findings were an enlarged right ventricle to the right of midline, with a dilated
main pulmonary artery and normal pulmonary vasculature. On ECHO, the
left ventricular size and ejection fraction were normal and the left atrium was
not dilated. The mitral and aortic valves were structurally normal without any
pressure gradient or abnormal jet on color flow mapping. There was no shunt
across the interventricular septum.
Figure 21.1: ECHO showing dilated right ventricle

with paradoxical septal motion
The right ventricle was enlarged and globular in shape. There was paradoxical
motion of the interventricular septum, that moved away from the left ventricular
cavity in systole (Fig. 21.1). On short-axis view, the dilated main pulmonary artery
with its right and left branches, gave a “pair of trousers” appearance (Fig. 21.2).
On color flow mapping, a pulmonary regurgitant jet was observed in the right
ventricular outflow tract (RVOT). The pulmonary artery pressure, as estimated
from the peak trans-tricuspid velocity (Vmax) by using the Bernoulli equation
(PG = 4 Vmax2), was markedly elevated (Fig. 21.3).
RVSP = PAP; RVSP – RAP = PG; RVSP = PG + RAP; RVSP = 4V2 + RAP
PG : Pressure Gradient
RAP : Right Atrial Pressure
PAP : Pulmonary Artery Pressure
RVSP : Right Ventricular Systolic Pressure
Case 21 Pulmonary Hypertension 97
Figure 21.2: ECHO showing dilatation of

the main pulmonary artery
Figure 21.3: Estimation of pulmonary artery pressure from tricuspid regurgitant velocity
JVP: Jugular venous pressure; TR: Tricuspid regurgitation; RA: Right atrium;
RV: Right ventricle; PA: Pulmonary artery
The clinical, electrographic and imaging evidence of right ventricular

enlargement with pulmonary artery dilatation is also present in pulmonary
valve stenosis but with certain differences. In pulmonary hypertension, the P2 is
loud while in pulmonary stenosis, the P2 is muffled. On chest X-ray, in pulmo
nary hypertension there is normal pulmonary vasculature or pulmonary plethora
while in pulmonary stenosis, the lung fields may be oligemic. On ECHO, thicken
ing of pulmonary valve leaflets with systolic doming and restricted excursion
is a feature of pulmonary valve stenosis. On color flow mapping, in pulmonary
hypertension, a regurgitant jet is seen in the right ventricular outflow tract. In
pulmonary stenosis, a systolic jet is observed in the proximal pulmonary artery.
There are several causes of pulmonary hypertension (Table 21.2). Elevated left
atrial pressure due to mitral valve disease (stenosis or regurgitation) is a prominent
cause. However, in our patient the mitral valve was structurally normal, without
any increased flow velocity or a regurgitant jet. Increased pulmonary blood flow
due to a left-to-right shunt is another important cause. But in our case there was
Table 21.2: Causes of pulmonary hypertension

• Increased pulmonary flow
Lt. to Rt. shunt; ASD, VSD, PDA
• Raised left atrial pressure
Mitral valve disease; MS, MR
• Chronic pulmonary disease
Chronic bronchitis, fibrosis
• Obstruction to pulmonary flow
Thrombo-embolic, veno-occlusive
• Primary pulmonary hypertension
no ventricular septal defect or patent ductus arteriosus. Chronic obstructive or

restrictive lung disease can also ultimately lead to pulmonary hypertension but
there was no history of long-standing cough with expectoration or wheezing in our
patient. Obstruction to pulmonary blood flow due to recurrent thromboembolism
can also cause pulmonary hypertension but that is not the likely diagnosis in our
case. Infrequent causes of pulmonary hypertension are intake of anorexigens,
systemic sclerosis, sleep disordered breathing and HIV infection which are also
unlikely possibilities in our patient. Therefore in all probability, our patient had
idiopathic or primary pulmonary hypertension. The mild cyanosis could be due
to minimal right-to-left shunting of blood across a patent foramen ovale.
MANAGEMENT ISSUES
In view of the pulmonary vasoconstriction and occlusion of the pulmonary vascu
lature associated with pulmonary hypertension, it is tempting to use vasodilators
as therapeutic agents. Historically, a plethora of drugs have been used which
include papaverine, nicotinic acid, isoxsuprine, cyclandelate and pentoxyphylline.
However, none of them has withstood the test of time or found to be beneficial
during evaluation in clinical trials. Among the established cardiovascular drugs,
isosorbide nitrate, hydralazine, prazosin and verapamil have also been tried in
the treatment of pulmonary hypertension but their benefits have been modest.
Antiplatelet drugs such as low dose aspirin, clopidogrel and the newer agent
cilostazol have also been used with mixed results. Phosphodiesterase (PDE)
inhibitors such as sildenafil and tadalafil are the only agents with proven clinical
benefit.
RECENT ADVANCES
Given the success of phosphodiesterase inhibitors in the treatment of pulmonary
hypertension, recently two new classes of vasodilators have been added to the
therapeutic armamentarium. The prostaglandin (PG) analogues epoprostenol
and iloprost have proven vasodilatory action. The endothelin (ET-1) antagonist
bosentan is the most promising out of the newer agents. It has a vasorelaxant as
well as antiproliferative action.
C A S E
22
Pulmonary
Embolism
CASE PRESENTATION
A 72-year old elderly gentleman was rushed to the medical room of Toronto airport
Canada, due to in-flight onset of severe chest pain, shortness of breath and profuse
sweating. This happened minutes before the landing of his long-haul flight from
New Delhi, India. He was a hypertensive on diuretic medication, but there was
no past history of heart or lung disease. His son who received him at the airport,
informed that the patient had undergone left knee replacement, six weeks prior to
undertaking this journey.
On examination, the patient was restless, tachypneic, pale and diaphoretic. The
pulse was rapid, irregular and feeble, at a rate of 110-120 beats/min. The BP in the
right arm was 90/60 mm Hg. with a respiratory rate of 32/min and his temperature
was 99.20 F. The JVP was elevated and there was edema, erythema and tenderness
over the left calf. The apex beat was normal in location and a parasternal lift was
felt. There was tachycardia, loud P2 and a right-sided S3. No murmur or pericardial
friction rub was audible. On chest auscultation, breath sounds were diminished over
the base of the left lung posteriorly.
CLINICAL DISCUSSION
From the history and physical examination, the most probable diagnosis in
this case is pulmonary embolism (PE) from deep vein thrombosis (DVT). ECG
showed atrial fibrillation with fast ventricular response and T wave inversion in
leads V1 to V3 (Fig. 22.1). There was no elevation of the S-T segment or presence of
Q waves. X-ray chest findings were mild cardiomegaly, left basal atelectasis and
a small left-sided pleural effusion (Fig. 22.2). There was no dilatation of the aortic
root or widening of the mediastinum.
Figure 22.1: ECG showing atrial fibrillation with fast ventricular response
Figure 22.2: X-RAY showing left basal atelectasis

with small pleural effusion
ECHO revealed normal left ventricular size and ejection fraction, without any
wall motion abnormality. There was a dilated and hypokinetic right ventricle with
mild tricuspid regurgitation. The mitral and aortic valves showed some annular
calcification, but there was no evidence of stenosis or regurgitation. There was
no sign of aortic root dissection. There were also no vegetations on leaflets,
ventricular thrombus or pericardial effusion.
There are several reasons for severe chest pain with dyspnea, sweating and
hypotension. Acute myocardial infarction is the leading possibility, but the ECG
did not show S-T segment elevation or presence of Q waves. Moreover, there was
no regional wall motion abnormality of any left ventricular myocardial segment.
Dissection of aorta may be considered, but there was no aortic root dilatation
or cleavage of the aortic wall with a false lumen. Spontaneous pneumothorax
generally occurs on a background of chronic lung disease with emphysema, but
there was no such history in this patient. Moreover, there was no evidence of
pneumothorax on the chest X-ray but only a small pleural effusion. Therefore,
pulmonary embolism remains the strongest diagnostic possibility.
Pulmonary embolism is common and potentially life-threatening but often
underdiagnosed clinical condition, associated with considerable morbidity and
mortality. There is obstruction of pulmonary vasculature by a thrombus, fat, air
or a tumour fragment. By and large, deep vein thrombosis (DVT) of the leg veins
is the leading source. Predisposing factors are venous stasis, hypercoaguable
state and injury to venous endothelium. Reasons for venous stasis are prolonged
bed rest and immobility due to trauma, surgery, congestive heart failure or
stroke. Long-distance air travel (economy-class syndrome) is also implicated.
Hypercoaguability can be due to several reasons which may be inherited
or acquired. Inherited defects are protein-C, protein-S or antithrombin-III
deficiency and excess of homocysteine or fibrinogen. Acquired factors are
estrogen therapy, malignancy, disseminated intravascular coagulation (DIVC)
and antiphospholipid antibodies (APLA).
The clinical presentation of pulmonary embolism (PE) is extremely variable
and is sometimes non-specific. Most patients present with pleuritic pain and
dyspnea, with a variable degree of circulatory embarassment. In massive PE,
Case 22 Pulmonary Embolism 101
there is hypotension and shock while in submassive PE, there is normotensive

right ventricular dysfunction. Rarely, PE may present with insiduous onset
of pulmonary hypertension. The clinical signs of acute PE are tachypnea and
tachycardia, with or without hypotension. There is evidence of right ventricular
strain in the form of elevated JVP, parasternal heave, loud P2 and right-sided
S3. Signs of lower limb deep vein thrombosis (DVT) are edema, erythema and
tenderness over the calf region.
A large variety of investigations are performed for the diagnosis and assessment
of pulmonary embolism. ECG invariably shows sinus tachycardia, sometimes
with P. pulmonale, unless the patient is in atrial fibrillation. Right bundle branch
block and T-wave inversion in leads V1 to V3 indicate right ventricular strain.
The S1Q3T3 pattern, although specific for pulmonary embolism (PE), is seen in a
minority of patients (Table 22.1). On chest X-ray, there may be basal atelactasis
Table 22.1: ECG features of pulmonary embolism

• Sinus tachycardia (invariable)
• Atrial fibrillation (sometimes)
• P. pulmonale (in sinus rhythm)
• Right bundle branch block
• Rightward QRS axis deviation
• Dominant R wave in lead V1
• T wave inversion in V1 to V3
• The S1 Q3 T3 Pattern
prominent S in LI
significant Q in LIII
inverted T in LIII
and a small pleural effusion. Classical finding of pulmonary infarction is a wedge-

shaped homogenous opacity above the diaphragm known as Hampton’s hump or
an oligemic lung segment which constitutes the Westermark’s sign (Table 22.2).
ECHO may reveal a dilated and hypokinetic right ventricle with mild tricuspid
regurgitation and sometimes a right ventricular thrombus. Echocardiography
is more often used to exclude other entities that mimic PE such as myocardial
infarction, aortic dissection and pericardial tamponade.
Table 22.2: X-ray findings in pulmonary embolism

• Basal atelectasis
• Small pleural effusion
• Oligemic lung segment (Westermark’s sign)
• Wedge-shaped opacity (Hampton’s hump).
On arterial blood gas (ABG) analysis, hypoxemia is invariably present if

the PE is massive. Hypocapnia and respiratory alkalosis also occur unless
there is ventilatory limitation, in which case there is hypercapnia. D-dimer is
a degradation product of cross-linked fibrin and its quantitative assay is used
for the diagnosis of PE. However, it is more useful as a “rule-out” test, with a
high negative predictive value. Duplex ultrasound is a combination of Doppler
venous flow detection and real-time B-mode imaging. It plays a pivotal role in
the diagnosis of lower extremity deep vein thrombosis (DVT). The most reliable
finding of DVT is non-compressibility of a venous segment.
Ventilation-perfusion (V-Q) scanning used to be popular for the diagnosis
of PE, prior to the advent of high-resolution contrast-enhanced computed
tomography (CT). Its diagnostic accuracy is greater when it is clubbed with
a clinical probability score than when used alone. Presently, it is reserved for
those who cannot tolerate intravenous contrast because of allergy or renal
failure. Contrast enhanced computed tomography (CT) is now considered the
‘gold-standard’ imaging modality for the diagnosis of PE. New generation CT
scanners can visualize sixth-order branches of the pulmonary vasculature with
high resolution. They have virtually replaced the older technique of invasive
pulmonary angiography. Classical findings on CT are filling defect and abrupt
cut-off of a vessel. Magnetic resonance (MR) pulmonary angiography can only
detect large proximal PE and is not reliable for segmental and sub-segmental
emboli.
MANAGEMENT ISSUES
The choice of therapy in pulmonary embolism is largely individualized and
depends upon the clinical severity. Needless to say, hemodynamic and
respiratory support are of paramount importance in those patients who present
with shock or hypotension. Anticoagulants are the mainstay of treatment
and should be initiated instantly pending investigations, to prevent further
extension of an already formed thrombus. Sub-cutaneous low molecular weight
heparin (LMWH) such as Enoxaparin is preferable, because of predictable dose
response and no need for laboratory monitoring. The new-drug Fondaparinux,
a factor Xa inhibitor can also be used. When unfractionated heparin is used, the
international normalized ratio (INR) should be 1.5 to 2.5 times the control, for full
therapeutic effect. Importantly, certain conditions in the differential diagnosis of
PE, such as aortic dissection and pericardial tamponade, are contraindications to
anticoagulation.
Thrombolytic therapy accelerates the lysis of acute pulmonary embolism,
but is associated with an increased risk of major hemorrhage. It is reserved
for massive PE, with hypotension and severe right ventricular dysfunction. If
thrombolysis fails or is contraindicated, percutaneous embolectomy can be
performed. However, it can only be used in the main arteries and if attempted
in smaller branches, can cause perforation. Finally, if there is an absolute
contraindication to anticoagulation or failure of the same and there is a proximal
venous thrombus, an inferior vena caval (IVC) filter can be placed. It provides a
screen to prevent lower limb or pelvic emboli from travelling to the lungs.
C A S E
23
Obstructive
Pulmonary Disease
CASE PRESENTATION
A 63-year old man visited a chest physician with the complaints of fever,
breathlessness, cough and wheeze, for the past 1 week. The fever was of moderate
grade, without chills, rigors or night-sweats, but was associated with body-ache
and fatigue. He did feel breathless for the past several years, but his dyspnea had
worsened since the onset of fever. He was orthopneic in bed but denied paroxysms
of nocturnal dyspnea. The cough was associated with copious purulent sputum
especially during morning hours, but there was no hemoptysis. The patient also
experienced tightness in the chest on walking and he was aware of wheezing.
His appetite was moderate and there was no history of significant weight loss. On
specific questioning, he admitted smoking about 20 cigarettes daily, for the last over
35 years.
On examination, he was obviously dyspneic and orthopneic in bed and his
accessory muscles of respiration were working. The face was puffy and hyperemic
and there was mild cyanosis over the tongue and lips. The extremities were warm
and sweaty. Clubbing of the finger-nails was noticed and there was edema over the
ankles. The JVP was raised, trachea was central and there were no palpable lymph
nodes in the neck. The pulse rate was 104 beats/min with a good volume that
decreased appreciably during inspiration. The BP was 136/84 mm Hg, temperature
101.60 F and the respiratory rate was 28 per minute.
The antero-posterior diameter of the chest was increased, giving it the shape
of a barrel. The percussion note over both lung fields was hyper-resonant and the
upper border of the liver was percussed in the 6th right intercostal space. Bilateral
expiratory rhonchi with scattered crepts were audible over the entire lung fields,
with normal air-entry. The cardiac apex beat could not be located but there was a
palpable parasternal heave. The S1 was normal with a loud P2. A soft early-diastolic
murmur followed the P2. A pansystolic murmur was also audible over the lower left
parasternal area, that did not radiate towards the axilla. On abdominal examination,
the liver edge was palpable 5 cm below the costal margin and was pulsatile. There
was no other organomegaly or sign of free fluid in the abdominal cavity.
CLINICAL DISCUSSION
From the history and physical examination, the most likely diagnosis in this case
is chronic obstructive pulmonary disease (COPD). The fever with worsening
dyspnea and purulence of sputum indicates acute exacerbation of COPD, which
is not uncommon among smokers. There were signs of respiratory distress
(working accessory muscles), polycythemia (facial hyperemia), deoxygenation
(cyanosis) and chronic lung suppuration (finger-nail clubbing). The raised JVP,
enlarged liver and ankle edema indicate right heart failure which, when it is due
to COPD, is known as corpulmonale. An appreciable decline in pulse volume
during inspiration, is termed as pulsus paradoxus. Besides COPD, other causes of
pulsus paradoxus are status asthmaticus and constrictive pericarditis.
A barrel-shaped chest with hyper-resonance over the lung fields and a
“pushed-down” liver are indicative of hyperinflation, which is termed as
pulmonary emphysema. Because of hyperinflated lungs, the cardiac apex beat
is difficult to locate. In acute exacerbation of COPD, the adventitious breath
sounds (rhonchi and crepts) are audible over the entire lung fields and the air-
entry is equal. A parasternal heave with a loud P2 and an early-diastolic murmur
of pulmonary regurgitation are indicative of pulmonary arterial hypertension.
Pulmonary hypertension leads to secondary tricuspid regurgitation which
produces a lower parasternal pansystolic murmur and an enlarged liver which is
sometimes pulsatile. The neck veins are distended with a prominent y descent.
Figure 23.1: ECG showing non-progression of R wave

in the left precordial leads
The ECG shows low voltage of the QRS complexes, with non-progression of
the R wave in precordial leads from V1 to V6 (Fig. 23.1). Due to clock-wise rotation
of the heart, the right ventricle underlies most of the precordium, producing
a rS pattern in most precordial leads. Other ECG features are tall R waves in
right precordial leads (right ventricular hypertrophy), tall P waves (right atrial
enlargement) and rightward deviation of the QRS vector (Table 23.1). The cardiac
Table 23.1: ECG abnormalities in COPD

• Low voltage QRS complexes (rS pattern)
• Right ventricular hypertrophy (tall R in V1)
• Right atrial enlargement (P. pulmonale)
• Right axis deviation of QRS vector (RAD)
• Atrial tachyarrhythmias e.g.fibrillation (AF)
Case 23 Obstructive Pulmonary Disease 105
Figure 23.2: X-ray showing a tubular heart

with hyperlucent lung-fields
rhythm can be sinus tachycardia, atrial fibrillation or even multifocal atrial

tachycardia.
The chest X-ray shows a tubular-shaped heart which appears small because of
hyperinflation of the lungs (Fig. 23.2). The main pulmonary artery is often dilated.
The lung fields are hyperlucent with prominent broncho-vascular markings. Due
to hyperinflation, the intercostal spaces are wide and the domes of the diaphragm
are flat and pushed downwards (Table 23.2).
Table 23.2: X-ray chest findings in COPD

• Tubular heart
• Hyperinflated lungs
• Dilated pulmonary artery
• Wide intercostal spaces
• Flat domes of diaphragm
Transthoracic ECHO is sometimes difficult to perform in patients of chronic

obstructive lung disease, because of a poor acoustic window due to emphy
sema. Nevertheless, ECHO may reveal a dilated right ventricle that loses its
triangular shape and becomes more globular. There is paradoxical motion of
the interventricular septum, that moves away from the left ventricular cavity
in systole. On short-axis view, the dilated main pulmonary artery with its right
and left branches, gives a “pair of trousers” appearance (Fig. 23.3). On colour
flow mapping, a pulmonary regurgitation (PR) jet may be observed in the right
ventricular outflow tract (RVOT). The pulmonary artery pressure, estimated from
the tricuspid regurgitant velociy, is elevated.
Figure 23.3: ECHO showing dilatation of

main pulmonary artery
Besides an ECG, X-ray and ECHO, other pertinent investigations in COPD
are hematological, biochemical and bacteriological. The hemoglobin is high
due to polycythemia while the leucocyte count is elevated with predominant
neutrophils, because of the bacterial infection. The ESR is also increased
because of chronic pulmonary suppuration but if very high, should alert us to
the possibility of pulmonary tuberculosis. Blood glucose and liver and kidney
function tests are done to rule out diabetes mellitus and other organ dysfunction.
The sputum is examined by Gram staining to identify the causative organism.
Sputum is also microscopically examined for acid fast bacilli (AFB) and fungal
hyphae. Appropriate cultures are performed to identify suitable antimicrobial
therapy. For patients in respiratory distress, arterial blood gas (ABG) analysis is
done which may show hypoxemia, hypercapnia and respiratory acidosis.
MANAGEMENT ISSUES
The treatment of acute exacerbation of COPD includes antibiotics to treat the
infection and bronchodilators to relieve airway obstruction. The antibiotic choice
may be empirical or based on sputum culture report. Bronchodilators are given
systemically or by the inhalation route and include theophylline, salbutamol,
ipratropium and corticosteroids. Supportive treatment includes antipyretics,
expectorants and oxygen therapy. Among the cardiovascular drugs, calcium
blockers like verapamil and diltiazem may be used to control the heart rate.
Betablockers and digoxin are avoided because of the risk of bronchospasm and
ventricular arrhythmias respectively. Pulmonary hypertension can be treated
with the phosphodiesterase (PDE) inhibitor sildenafil, prostaglandin (PG)
analogue epoprostenol or an endothelin (ET-1) receptor antagonist bosentan.
SECTION
7
Pericardial
Infections
C A S E
24
Acute
Pericarditis
CASE PRESENTATION
A 26-year old man presented to the emergency department, with a dull precordial
chest pain of 12 hours duration. The chest pain was not associated with suffocation
or choking sensation and did not radiate to the arms. But the pain worsened when
the patient took a deep breath and it hurt to cough or sneeze. About five days back,
he had a “flu-like” illness with low grade fever, aches and pains along with dry cough.
There was no history of purulence in sputum, hemoptysis or wheeze. He smoked
5 cigarettes a day, consumed about a litre of beer over the week-end, but denied
abusing any illicit drug. There was no history of premature coronary artery disease in
his family. He also denied any history of sore-throat with joint pains during childhood
and he had not undergone any recent dental or endoscopic procedure.
On examination, the patient was in obvious discomfort and preferred to sit up
in bed and lean forward. The pulse rate was 110 beats/min. regular, with a BP of
110/70 mm Hg and the temperature was 100.60 F. At the neck, JVP was not raised,
thyroid gland was normal and there were no palpable lymph nodes. There were
also no erythematous areas, skin nodules, petechial spots or swollen joints. Throat
examination revealed mild pharyngeal congestion, but there were no pustules over
the tonsils. On inspection, the precordium was quiet and the apex beat normally
located. Upon auscultation, a high-pitched scratchy sound was audible all over
the precordium. The lung fields were clear without any rhonchi or crepts. The ECG
showed sinus tachycardia and narrow QRS complexes, with elevation of the S-T
segment in most leads (except aVR) and depression of the P-R segment (Fig. 24.1)
Figure 24.1: ECG showing sinus tachycardia with concave S-T elevation
110 Section 7 Pericardial Infections
CLINICAL DISCUSSION
When a patient presents with a short febrile illness, clinical signs of heart disease
and some ECG abnormalities, possibilities that need to be considered are
rheumatic fever, infective endocarditis, acute myocarditis and acute pericarditis.
Rheumatic fever generally presents with a migratory polyarthritis, with or without
cutaneous nodules or an evanescent skin rash. Infective endocarditis generally
follows a dental or surgical procedure with a pre-existing valvular disease or a
septal defect. Acute myocarditis is characterized by myocardial dysfunction
and clinical signs of heart failure. Our patient presented with chest pain, had
an audible pericardial rub and the ECG showed elevation of the S-T segment.
Therefore, the most likely diagnosis in this case is acute pericarditis.
The chest pain of acute pericarditis is sharp in character and central in
location. Therefore, it resembles the chest pain of myocardial infarction, but lacks
the characteristic radiation and accompaniments of ischemic chest pain. The
pain increases on deep breathing and coughing and therefore, patients prefer
shallow respiration. The pain also worsens on lying supine and hence, the patient
prefers to sit and lean forward.
The pericardial friction rub is a high-pitched, scratchy or squeaky sound,
audible over the precordium along the lower left sternal border. It is better audible
during inspiration, with the patient sitting up and leaning forward. Occasionally,
the rub is accompanied by a palpable thrill, especially in patients with uremic
pericarditis. The classical pericardial rub is triphasic and has three components,
each related to a discrete phase of the cardiac cycle. Accordingly, there is a
presystolic rub during atrial systole and a systolic and diastolic component
related to ventricular systole and diastole respectively. Sometimes, the rub is a
one-component or two-component sound, if there is associated atrial fibrillation
or ventricular dysfunction. Sometimes the rub is evanescent and changes its
quality on a day-to-day basis.
Table 24.1: Causes of S-T segment elevation

• Prinzmetal angina
• Ventricular aneurysm
• Dressler’s syndrome
• Acute viral pericarditis
• Early repolarization variant
The most common and significant cause of S-T segment elevation on the ECG
is coronary artery disease. S-T elevation occurs in acute myocardial infarction
and in coronary vasospasm (Prinzmetal’s angina). In patients with prior
myocardial infarction, reasons for S-T segment elevation are Dressler’s syndrome
and ventricular aneurysm. S-T elevation also occurs in the early repolarization
variant in which case the clinical profile is entirely normal (Table 24.1). The S-T
Case 24 Acute Pericarditis 111
Table 24.2: ECG features of acute pericarditis

• S-T segment elevation is concave upwards
• S-T elevation is observed in nearly all leads
• T waves invert after S-T returns to base-line
• Q waves do not appear with ST-T changes
• R wave height is maintained in chest leads
• P-R segment is depressed in the limb leads
• Reciprocal S-T segment depression not seen
• Sinus tachycardia is almost invariably present
• Arrhythmias and conduction defects are unusual
• ECG changes do not evolve but resolve rapidly
segment elevation of acute pericarditis can be differentiated from that of acute

myocardial infarction by several ECG features such as concave upward shape and
concomitant depression of the P-R segment (Table 24.2).
In patients with chest pain, the most frequently requested test after ECG is
cardiac troponin-T. Troponin may be elevated in upto 50% of patients with acute
pericarditis, thus limiting its diagnostic value. The total leucocyte count (TLC) and
C-reactive protein (CRP) level may be elevated in acute pericarditis. A four-fold
rise in antiviral antibody titre may occur, but is not diagnostic and often futile in the
absence of specific antiviral therapy. Anti-streptolysin O (ASLO) titre and throat
swab culture for beta-hemolytic Streptococcus are appropriate, if rheumatic fever
is suspected. Suitable blood cultures are obtained, if the possibility of infective
endocarditis is being entertained. In acute pericarditis, ECHO is useful to identify
pericardial effusion and features suggestive of associated myocarditis. Usually,
only a small rim of effusion is present and the left ventricular systolic function
is normal. In myo-pericarditis, the ventricular function is mildly impaired but is
generally reversible.
MANAGEMENT ISSUES
Treatment of acute pericarditis should first be targeted against a specific cause, if
there is one. Examples include withdrawal of the offending drug in drug-induced
pericarditis, hemadialysis for uremic pericarditis, thyroid hormone replacement
for myxedema and suitable chemotherapy in malignant disease (Table 24.3).
In most cases, non-steroidal anti-inflammatory drugs (NSAIDs) are first-line
treatment, in the absence of a specific cause. In post-infarction Dressler’s
syndrome, aspirin is preferable over other NSAIDs. It is given in high-doses to
begin with, to be tapered down over a period of time. Concomitant use of a proton
pump inhibitor is recommended to reduce the risk of gastrointestinal bleeding.
Table 24.3: Causes of acute pericarditis

• Infective: viral, bacterial, tubercular
• Traumatic: accidental, cardiac surgery
• Malignant: metastasis, radiotherapy
• Autoimmune: rheumatoid arthritis, SLE
• Metabolic: uremia and myxedema
• Drug-induced: procainamide, hydrallizine
• Infarction: post-MI Dressler’s syndrome
Colchicine may be used along with a NSAID in refractory and recurrent cases
or alone if NSAIDs are contraindicated. Steroids should only be considered in
the context of a systemic inflammatory disease. Steroids should not be used in
bacterial or tubercular infection since they cause immunosuppression and in
post-MI patients, as they interfere with scar formation. Pericardiocentesis and
pericardectomy are rarely required in the treatment of acute pericarditis.
C A S E
25
Pericardial
Effusion
CASE PRESENTATION
A 66-year old gentleman visited the out-patient department of a tertiary-care hospital,
with two months history of progressively worsening exertional breathlessness and
increasing ankle swelling. There was no history of orthopnea or nocturnal dyspnea
and he denied complaints of fever, productive cough, chest pain or hemoptysis. The
patient was a known case of long-standing diabetes and hypertension. Recently, he
was diagnosed to have chronic kidney disease and was advised peritoneal dialysis.
His daily medication included lisinopril, frusemide, digoxin and glimepiride. There
was no past history of angina pectoris or myocardial infarction, although he was told
to have an “enlarged heart”.
On examination, the patient was pale and mildly tachypneic, but not in any
distress. The pulse was rapid, regular and low in volume, with an appreciable fall
in pulse volume during inspiration. The pulse rate was 110 beats/min. with a BP of
104/66 mm Hg and respiratory rate of 24/min. The JVP was elevated without any
noticeable descent during inspiration and there was pitting ankle edema. The
precordium was silent and the apex beat was not visible but could be located only
on palpation. On percussion, the area of cardiac dullness extended beyond the
cardiac apex. The S1 and S2 were faintly audible on auscultation, but no murmur,
gallop rhythm or friction rub was appreciated. Breath sounds were diminished over
the left lung base posteriorly, with an area of bronchial breathing just above it. The
rest of the lung fields were clear.
CLINICAL DISCUSSION
From the case history and particularly from the physical examination, the most
likely diagnosis in this case is pericardial effusion. ECG showed sinus tachycardia
with generalized low QRS voltages. The R wave amplitude varied on a beat-to-
beat basis. X-ray chest findings were a markedly enlarged globular cardiac
silhouette with a narrow basal vascular pedicle, giving the heart a “money-bag”
appearance. The pulmonary broncho-vascular markings were normal. ECHO
revealed a 2.5 cm wide echo-free space around the heart. There was noticeable
collapse of the right atrium and right ventricle during diastole. The left ventricular
function was normal.
In retrospect, there were several clinical pointers towards the diagnosis of
pericardial effusion. A decline in pulse volume (fall in systolic BP>10 mm) during
Figure 25.1: X-ray showing large cardiac silhouette

with a narrow basal vascular pedicle
inspiration, is known as pulsus paradoxus. Increase in venous return shifts the

interventricular septum towards the left ventricle, thereby reducing stroke
volume. Besides pericardial effusion with cardiac tamponade, other reasons for
pulsus paradoxus are constrictive pericarditis and status asthmaticus. A raised
JVP without noticeable descent, indicates inadequate venous emptying during
inspiration. Besides pericardial effusion, a raised and fixed JVP is a feature of
superior vena caval obstruction.
A silent precordium with a non-palpable apex beat and muffled heart sounds
indicate some intervening substance, may be fluid (pericardial effusion), air
(pulmonary emphysema) or fat (morbid obesity). The combination of low BP,
raised JVP and muffled heart sounds is known as Beck’s triad and is characteristic
of cardiac tamponade. The area of diminished breath sounds, dull percussion
note and bronchial breathing just above it, indicate compression of the left lower
lobe by the pericardial effusion. This constitutes the Ewart’s sign.
On ECG, the beat-to-beat variability of QRS amplitude is known as electrical
alternans. Its clinical counterpart is pulsus alternans. Total electrical alternans
involves the QRS complex as well as the P and T waves. Besides cardiac tamponade,
electrical alternans is also observed in severe left ventricular dysfunction. The
“money-bag” heart, with enlarged cardiac silhouette and narrow vascular
pedicle, is characteristic of pericardial effusion (Fig. 25.1). When cardiomegaly
is due to heart failure, there is cephalization of pulmonary veins, along with hilar
congestion, Kerley B lines and pulmonary edema.
Echocardiography is extremely valuable not only to confirm the presence of
pericardial effusion (Fig. 25.2), but also to assess its magnitude and to identify
signs of cardiac tamponade. It is also useful to exclude other causes of heart failure
and to guide drainage of pericardial fluid. The quantity of pericardial fluid can
be gauged from the width of the echo-free space around the heart (Table 25.1).
The nature of pericardial fluid can be judged from careful analysis of the echo-
free space. Transudative effusion is sonolucent while sanguinous fluid has high
echodensity, sometimes with thrombus formation. Exudative effusion shows
Case 25 Pericardial Effusion 115
Figure 25.2: ECHO showing an echo-free space

all around the heart
Table 25.1: Quantification of pericardial effusion

Amount Volume Posterior space Anterior space
Small < 100 ml < 1 cm —
Moderate 100-500 ml 1-2 cm < 1 cm
Large > 500 ml > 2 cm > 1 cm
fibrinous strands while malignant effusion may show echo-dense metastasis

deforming the smooth pericardium.
A large pericardial effusion imposes constraint on filling of the cardiac
chambers and hence impairs cardiac output. This serious clinical situation is
known as cardiac tamponade. Tamponade results from a large volume of effusion,
or rapid collection of a small effusion. A large effusion may accumulate gradually
without causing tamponade, if the pericardial sac is compliant and gets adequate
time to stretch itself. Therefore, rapidity of fluid accumulation is more important
than absolute volume. The right-sided chambers are low-pressure structures and
can easily get compressed by a large pericardial effusion. Right atrial collapse is a
sensitive but less specific sign of cardiac tamponade. Right ventricular collapse,
particularly if it lasts for more than one-third of diastole, is a less sensitive but
more specific sign of cardiac tamponade.
Due to the constraint imposed by the effusion on the filling of the cardiac
chambers, increased filling of the right ventricle (during inspiration) reduces
filling of the left ventricle (hence cardiac output). Therefore during inspiration,
tricuspid flow velocity increases while mitral flow velocity decreases. The
converse occurs during expiration. A change in flow velocity exceeding 25% is
considered to be diagnostic. This phenomenon of ventricular interdependence
also forms the basis of pulsus paradoxus, which is observed clinically. This
reciprocal relationship is also seen in constrictive pericarditis, where it is an
important differentiating feature from restrictive cardiomyopathy.
Table 25.2: Causes of pericardial effusion

• Infective : Tubercular pericarditis
• Traumatic : Accidental, surgical
• Malignant : Metastasis, irradiation
• Metabolic : Uremia, myxedema
• Autoimmune : Rheumatoid arthritis
There are several causes of pericardial effusion but inflammatory causes

lead the list (Table 25.2). Tuberculosis is the commonest infection that causes
pericardial effusion in the developing countries. Auto-immune disorders like
rheumatoid arthritis and systemic lupus are also sometimes responsible for
pericardial effusion. Idiopathic or viral pericarditis is usually associated with only
a small effusion. Hemorrhagic effusion may follow accidental or surgical trauma
or may be due to metastatic deposits in malignant disease. Metabolic disorders
that cause pericardial effusion are uremia and myxedema (hypothyroidism).
Pertinent investigations in pericardial effusion are completed blood count,
tuberculin test, antinuclear antibodies, renal function tests and thyroid profile.
The pericardial fluid is tested by cytology, bacterial culture and for adenosine
deaminase activity and tumour markers when tuberculosis or malignancy are
suspected.
MANAGEMENT ISSUES
Drainage of a large pericardial effusion by means of echo-guided (for safe needle
entry) pericardiocentesis is the treatment of choice, particularly if there is cardiac
tamponade. Most often the hemodynamic response to drainage is dramatic
and gratifying. Fluid resuscitation may cause only transient improvement, but
ionotropes has no role as cardiac contractility is not compromised. If there is no
evidence of tamponade and the hemodynamics are stable, immediate drainage
is unnecessary and the patient is managed conservatively. However, careful
periodic assessment is mandatory. In tamponade due to aortic dissection,
pericardiocentesis worsens hemodynamic stability and surgical drainage is
preferable.
Antitubercular drugs are sometimes prescribed empirically if tubercular
etiology is likely, or definitely if it is proven upon pericardial fluid analysis.
Antinflammatory agents including steroids are employed for the treatment of
auto-immune and inflammatory disorders. Malignant disease is treated with
suitable chemotherapy or even radiotherapy which however, carries the risk of
pericardial constriction.
C A S E
26
Constrictive
Pericarditis
CASE PRESENTATION
A 52-year old man visited the out-patient clinic of the department of internal
medicine, with complaints of generalized fatigue, reduced appetite, increased
abdominal girth and swelling over both ankles. He denied history of heavy alcohol
intake, prolonged jaundice, hematemesis or altered bowel habits. About one year
back, he was diagnosed to have pulmonary tuberculosis, for which he was prescribed
anti-tubercular drugs for 6 months. But he was able to take his full medication only for
3 months, because he developed drug-induced hepatitis and therefore his rifampicin
and isoniazid were stopped. However, he did take ethambutol and levofloxacin for
the remaining 3 months. At present there were no complaints of fever, night-sweats,
productive cough or hemoptysis. There was no history of diabetes, hypertension or
heart disease in the patient or any of his family members.
On examination, the patient looked ill with a pinched face, thin emaciated arms,
swelling around the ankles and a protuberant abdomen. He was not tachypneic or
orthopneic and not in any form of distress. His conjunctiva and tongue were pale
but there was no cyanosis or icterus. The pulse was fair in volume at a rate of 92
beats/min., with an appreciable fall in pulse volume during inspiration. The JVP
was elevated 5 cm above the angle of Louis and it failed to fall appreciably during
inspiration. The abdomen was distended, but there were no dilated veins or spider
naevi on the skin surface. Shifting dullness was demonstrated, indicating free fluid
in the abdominal cavity. The liver edge was palpable 8 cm below the right costal
margin; it was slightly tender but not pulsatile. The precordium was silent and the
apex beat was difficult to locate. There was no murmur or pericardial rub, but a high-
pitched sound was audible in mid-diastole. There was some retraction of the apex of
right lung on inspection, but both the lung fields were clear on auscultation.
CLINICAL DISCUSSION
From the history and physical examination, this patient definitely was in right
heart failure. ECG showed sinus rhythm with low QRS voltages and non-specific
T wave inversion. X-ray chest did not show any cardiomegaly, but there was a
striking area of linear calcification along the left heart border (Fig. 26.1). There
was fibrosis over the apical segment of the right lung. ECHO revealed normal
size of all cardiac chambers, with normal left ventricular ejection fraction. There
Figure 26.1: X-ray showing linear pericardial calcification
was no myocardial thickening or wall-motion abnormality and all cardiac valves

were structurally normal. However, there was thickening of the pericardium,
with multiple parallel echo-lines casting a bright reflection. Therefore, the most
probable diagnosis in this case is constrictive pericarditis.
There were several typical clinical findings in this case. These were a variable
pulse volume, raised JVP, silent precordium, no cardiac murmur and a sharp
third heart sound in diastole. An appreciable decrease in pulse volume during
quiet respiration is known as pulsus paradoxus. Besides constrictive pericarditis,
pulsus paradoxus is observed in cardiac tamponade, chronic obstructive lung
disease and status asthmaticus. A raised JVP that paradoxically rises even further
during inspiration is known as Kussmaul’s sign. Besides constrictive pericarditis,
the Kussmaul’s sign is observed in restrictive cardiomyopathy and after right
ventricular infarction. The deep x descent on the JVP, represents the rapid phase
of early diastolic ventricular filling. This is known as the “dip and plateau” pattern
or the “square-root” sign of the ventricular pressure trace.
A silent precordium with a ‘lost’ apex-beat is a feature of pericardial effusion
or constriction, morbid obesity and pulmonary emphysema. The third heart
sound in constrictive pericarditis is the pericardial knock. It is a sharp and high-
pitched sound, when compared to the classical S3. The pericardial knock marks the
termination of rapid early-diastolic phase of ventricular filling. Due to pericardial
constriction, the inferior vena cava is dilated (Fig. 26.2) and there is congestive
hepatomegaly. The spleen is also enlarged and ascites is present. These findings
are picked up on clinical examination as well as by ultrasonography.
Constrictive pericarditis is a masquerader of several clinical conditions. Signs
and symptoms are similar to those of congestive heart failure, but right-sided
failure is more prominent and ascites is out of proportion to the degree of pedal
edema. Hepatomegaly, ascites and edema may be misdiagnosed as cirrhosis of
liver, if the neck veins are not observed carefully. Finally, if a patient is in atrial
fibrillation, the diastolic knock may be misinterpreted as an opening snap and
the diagnosis of mitral stenosis may be entertained.
Case 26 Constrictive Pericarditis 119
Figure 26.2: Abdominal ultrasound showing dilatation

of the inferior vena cava
Table 26.1: Causes of constrictive pericarditis

• Tubercular pericarditis
• Bacterial pericarditis
• Cardiac surgery
• Chest irradiation
Constrictive pericarditis is most often preceded by a tubercular infection.

Sometimes it may follow bacterial pericarditis but never viral pericarditis.
Rheumatic pancarditis rarely causes constriction because the effusion is sero
fibrinous in nature and usually gets absorbed. Constrictive pericarditis may also
follow chest irradiation or cardiac surgery (Table 26.1).
It is often difficult if not impossible to differentiate constrictive pericarditis
from restrictive cardiomyopathy and cardiac catheterization may be required
for their distinction. Restrictive cardiomyopathy is suggested by the presence of
certain subtle echo features which are not observed in pericardial constriction.
These features are small ventricles and large atria, thick mitral and tricuspid valve
leaflets and mild impairment of left ventricular systolic function (Table 26.2).
Table 26.2: Differences between constrictive pericarditis and restrictive cardiomyopathy

Constrictive pericarditis Restrictive cardiomyopathy
Pericardium Thick Normal
Myocardium Normal Thick
Ventricles Normal Obliterated
Atria Normal Dilated
LV function Normal Mildly impaired
MV and TV Normal Regurgitation
Moreover, the ventricular free walls may give a “granular-sparkling” appear

ance. Clinically speaking, restrictive cardiomyopathy generally presents with
biventricular heart failure while constrictive pericarditis presents with sole or
predominant right heart failure.
MANAGEMENT ISSUES
A two to three month period of conservative treatment can be tried in constrictive
pericarditis, if the symptoms are mild. In the presence of signs indicating systemic
venous congestion, it is naturally tempting to prescribe a diuretic to a patient
of constrictive pericarditis. However, this approach is often counterproductive
since ventricular filling declines further and cardiac output falls. Ionotropes like
digoxin are unhelpful as there is no impairment of myocardial contractility. It is
crucial to maintain sinus rhythm and to cardiovert atrial fibrillation by electrical
or pharmacological means. This is because atrial fibrillation leads to loss of
atrial contribution to ventricular filling and shortens the diastolic filling time.
Pericardectomy by surgical means is the only effective treatment of constrictive
pericarditis. It releases the ventricles from the restriction to diastolic filling,
imposed by the rigid pericardial sac.
RECENT ADVANCES
The classical hemodynamic findings of constrictive pericarditis on cardiac
catheterization not only helps to arrive at a definitive diagnosis but also help to
differentiate between pericardial constriction and restrictive cardiomyopathy.
These findings have been recently ellucidated. Due to the restriction imposed
by the rigid pericardium on right ventricular filling, there is a sharp rise in early
diastolic pressure followed by a flat pressure profile in mid and late diastole. This
is reflected as a “dip and plateau” wave form on right ventricular pressure tracing
and constitutes the “square-root sign”. On inspection of the JVP, this pattern is
observed as a prominent x descent.
Another hemodynamic finding is the reciprocal variation in the mitral and
tricuspid inflow velocities with the phases of respiration, due to pericardial
constraint. This reflects ventricular interdependence wherein right ventricular
filling increases during inspiration at the expense of left ventricular filling. The
converse happens during expiration when right ventricular filling decreases and
left ventricular filling increases. This is reflected as an exaggerated (>25%) rise or
fall of mitral and tricuspid inflow velocities, during inspiration and expiration.
SECTION
8
Myocardial
Infections
C A S E
27
Rheumatic
Fever
CASE PRESENTATION
A 19-year old girl visited her family physician with the complaints of high-grade
fever, pain in lower-limb joints and rash over the arms and legs, for the past 1 week.
The fever started with pain in her throat and dry cough and was associated with
chills but not rigors. There was no history of purulent sputum, chest pain, dyspnea,
wheezing or hemoptysis. She also denied pain abdomen, vomiting, loose stools or
burning sensation during micturition. The pain in her joints started in her right wrist
and went on to affect both her ankles, which limited her walking ability. The skin
rash was macular and erythematous, but there were no petechial spots or history of
pruritus. The girl had not undergone dental extraction or any surgical procedure in
the recent past. She did have sore-throat frequently during her childhood, but there
was no history of anoxic spells or squatting attacks while playing.
On examination, the young girl looked ill, toxic and anxious. The pulse rate was
104 beats/min. with a BP of 104/66 mm Hg and her temperature was 100.6o F. Her
extremities were warm and dry and there was no tremor or clubbing of the fingers.
She was mildly anemic but not cyanosed or icteric. The erythematous rash over the
extremities blanched on pressure. Her both ankles were mildly swollen and tender
to touch, but there was no redness over the skin. On throat examination, there
was mild pharyngeal congestion with enlarged tonsils that had few pustules. At
the neck, the thyroid gland was not enlarged, JVP was not raised and there was no
significant lymphadenopathy. The apex beat was normal in location and character
and the precordium was quiet. The S1 was soft and S2 normally audible; no S3 gallop
or pericardial friction rub was appreciated. A low-pitched, mid-diastolic murmur was
heard at the cardiac apex. The murmur was not preceded by an opening snap and
did not undergo pre-systolic accentuation. The lung fields were clear without any
rhonchi or rales.
CLINICAL DISCUSSION
From the history and physical examination, this young girl had a febrile illness
with arthralgias, erythematous rash and a diastolic murmur. The obvious
diagnosis with this constellation of clinical findings is acute rheumatic fever.
The ECG showed sinus rhythm with a heart rate of 86 beats per minute and a
124 Section 8 Myocardial Infections
Figure 27.1: ECG showing prolongation of the P-R interval
prolonged P-R interval (Fig. 27.1). The QRS complexes were narrow and there was
no S-T segment shift or T wave inversion. The X-ray chest did not show increased
cardio-thoracic ratio or signs of pulmonary edema. ECHO revealed normal left
ventricular size and systolic function, mild left atrial dilatation and a normal sized
right ventricle. The mitral valve leaflets were mildly thickened (Fig. 27.2) with
normal excursion and there was no diastolic doming of either leaflet.
Figure 27.2: ECHO showing thickened

mitral valve leaflets
The hemoglobin was 10.4 g/dL with a leucocyte count of 11,800/cu.mm. of

which 73% were neutrophils. The ESR was 55 mm in the 1st hour. Urine analysis
showed traces of albumin but no WBCs or RBCs. The peripheral blood smear did
not show any plasmodium parasite. The anti-streptolysin O (ASLO) titre was 366
IU with a C-reactive protein(CRP) value of 66 mg/L. Biochemical parameters
including blood glucose, serum cholesterol and the renal and liver function tests
were within normal limits. Urine and blood culture did not yield any bacterial
growth. However, culture of the swab taken from the throat was positive for beta-
hemolytic Streptococcus.
The clinical diagnosis of acute rheumatic fever is based on the presence of
Jone’s criteria (Table 27.1). Two major criteria or one major and two minor
criteria, along with evidence of recent Streptococcal infection, are required to
clinch the diagnosis. Major Jone’s criteria are carditis, polyarthritis, chorea,
erythema marginatum and subcutaneous nodules. Minor criteria are fever,
arthralgia, history of rheumatic fever, elevated ESR, CRP and prolonged P-R
interval. Evidence of a recent Streptococcal infection include positive throat swab
culture and increased anti-Streptolysin O (ASLO) titer.
Case 27 Rheumatic Fever 125
Table 27.1: Jone’s criteria for the diagnosis of rheumatic fever

Major criteria Minor criteria Streptococcal infection
• Carditis • Fever • History of scarlet fever
• Arthritis • Arthralgia • Throat swab culture + ve
• Chorea • High ESR • Streptococcal antigen + ve
• Eryth. marginatum • Raised CRP • High anti-Streptolysin O titre
• Subcut. nodules • P-R interval
A migratory or “fleeting” type of polyarthritis with fever and extreme weakness

is the commonest manifestation of rheumatic fever. The arthritis typically
involves the medium-sized joints such as the elbows, ankles and wrists. Chorea
is usually observed in young children as an isolated entity, with rheumatic heart
disease occuring a few days later. Erythema marginatum, which is hardly seen, is
a pink rash on the trunk which blanches on pressure and is neither painful nor
indurated. Rheumatic carditis is a pancarditis. Endocarditis manifests as a new
murmur due to inflamed valve leaflets. Myocarditis presents as newly developed
myocardial dysfunction. Evidence of pericarditis is a pericardial friction rub or
presence of an effusion.
The major sequel of acute rheumatic fever is chronic valvular heart disease.
Antibodies against streptococcal polysaccharide (carbohydrate cell wall) cross-
react with cardiac tissues (myosin and laminin) to cause valve damage by
molecular mimicry. During the acute stage, the mitral valve leaflets are inflamed
resulting in a diastolic Carey-Coomb’s murmur. Later on, due to smouldering
disease, the valves are chronically damaged. Mitral stenosis, aortic regurgitation,
mitral regurgitation and aortic stenosis, follow in that order of decreasing
incidence.
MANAGEMENT ISSUES
The two pillars in the management of acute rheumatic fever are aspirin and
penicillin. Aspirin can be given in a dose of 325 mg 4 times a day, during the
stage of acute inflammation. The dose is gradually brought down, as the fever
and pain subside. Care should be taken to co-prescribe a proton pump inhibitor
like pantoprazole and to advise to take aspirin soon after a meal. If the patient is
sensitive to aspirin, an alternative agent like ibuprofen or diclofenac may be used.
Crystalline penicillin is administered in full doses parenterally, during the stage
of acute inflammation. If the patient is hypersensitive to penicillin, a macrolide
antibiotic such as erythromycin or azithromycin is chosen and the dose is 500
mg 6 hourly or 300 mg daily, respectively. Once the acute flare has settled down,
long-term prophylaxis is suggested, particular to women, until they attain the age
of 40 years. Antibiotic prophylaxis against infective endocarditis before a dental
or surgical procedure, depends upon the nature and degree of chronic valvular
involvement.
RECENT ADVANCES
As per the revised Jone’s criteria for rheumatic activity in developing countries,
erythema marginatum has been dropped as it is hardly ever seen. Polyarthralgia
with raised ESR and high ASLO titre is no less common than objective arthritis
and has been included as a major criteria.
C A S E
28
Acute
Myocarditis
CASE PRESENTATION
A 32-year old man presented to the emergency department with worsening
breathlessness, over the preceding 3 days. Since the previous night, he was finding
it difficult to lie flat in bed and had to sit up frequently to breathe. The patient was
quite well until 5 days prior to his hospital visit, when he developed a “flu-like” illness.
The low grade fever, headache, myalgia and mild sore throat had now settled down.
There was no history of productive cough, chest pain or hemoptysis. There was also
no history of skin rash, petechial spots or painful joints. He had not undergone any
recent dental or endoscopic procedure. He did not suffer from recurrent pharyngo-
tonsillitis during his childhood.
On examination, the patient was ill-looking and tachypneic. Pulse was fast and
feeble and his extremities were sweaty. His heart rate was 116 beats/min with
a BP of 104/68 mm Hg and the temperature was 98.80 F. There were no petechial
hemorrhages under the finger-nails or in the conjunctiva and his joints were not
swollen. The apex beat was displaced outwards and the S1 and S2 were normal. A soft
S3 was audible in early diastole, but there was no murmur. No pericardial friction rub
was heard. There were few basilar rales over the lung fields.
CLINICAL DISCUSSION
From the history and physical examination, it is obvious that the patient had a
short febrile illness, culminating in left ventricular dysfunction within a week.
ECG showed sinus tachycardia, low QRS amplitude and non-specific T wave
inversion. X-ray chest findings were enlarged cardiac silhouette, hilar congestion
(Fig. 28.1), Kerley B lines and pulmonary edema. ECHO revealed a dilated left
ventricle with global hypokinesia (Fig. 28.2). There was no abnormality of the
cardiac valves, septal defect or presence of pericardial effusion.
When a patient presents with a febrile illness and ECG abnormalities, there
are four main diagnostic possibilities. These are:
• Rheumatic fever
• Acute pericarditis
• Acute myocarditis
• Infective endocarditis.
Figure 28.1: X-ray showing cardiomegaly with

left pleural effusion
Figure 28.2: ECHO showing an enlarged

globular left ventricle
Acute pericarditis is unlikely because the patient had no chest pain or S-T
segment elevation on the ECG or pericardial friction rub on clinical examination.
Moreover, left ventricular dysfunction is unusual in pericarditis, unless there is
an associated component of myocarditis. Infective endocarditis is also unlikely
because the ECHO did not show any abnormality of the cardiac valves or presence
of a septal defect. Rheumatic fever is a pancarditis where myocardial dysfunction
is accompanied by pericarditis (friction rub) and endocarditis (inflammed
valve leaflets). Moreover, rheumatic fever generally presents with a migratory
polyarthritis with or without cutaneous nodules and an evanescent skin rash.
Therefore, the most probable diagnosis in this case is acute myocarditis.
Acute myocarditis is an inflammatory disease afflicting the heart muscle and
causing myocardial dysfunction over a short period of time. Although global
hypokinesia is the hallmark of myocarditis, regional wall motion abnormalities
can occur, if myocardial inflammation is patchy in distribution. The myocardial
damage and necrosis is immune-mediated and the cardiac troponin levels are
often elevated. The etiology is most often viral and Coxsackie B virus or Echovirus
Case 28 Acute Myocarditis 129
Table 28.1: Pathogens implicated in acute myocarditis

• Viral Coxsackie B, Echovirus, HIV
• Bacterial C. diphtheriae, Mycoplasma
• Spirochetal Lyme’s disease, Leptospirosis
• Protozoal Chaga’s disease
are commonly implicated. Myocarditis may be bacterial as in Mycoplasma

infection and Diphtheria. Rarely, it may be caused by a spirochete as in Lepto
spirosis or protozoa as in Chaga’s disease (Table 28.1).
The patient of acute myocarditis must be investigated to establish the inflammatory
nature of the disease and the type of inflammation. The total leucocyte count
(TLC), erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP)
helps to establish inflammation. Anti-streptolysin O (ASLO) titre and throat
swab culture for beta-hemolytic Streptococcus are indicated, if rheumatic fever
is suspected. Suitable blood cultures are obtained, if the possibility of infective
endocarditis is being entertained.
Viral serology for the diagnosis of viral myocarditis is generally unhelpful,
because the implicated viruses are ubiquitous in nature and antiviral drugs
have no role in the treatment. Endomyocardial biopsy is rarely performed
because of its low diagnostic yield, except in situations where a specific etiology
is suspected on clinical grounds and needs to be confirmed on tissue analysis.
These conditions are Loeffler’s endocarditis, hemochromatosis and amyloidosis.
Besides inflammation, electrolyte imbalance and nutritional deficiency are
sometimes responsible for acute left ventricular dysfunction. These need to be
excluded by appropriate tests such as serum potassium, calcium and magnesium
levels. Thyroid hormone assay and urinary catecholamine levels are measured if
thyrotoxicosis or pheochromocytoma are suspected.
MANAGEMENT ISSUES
The clinical features of myocarditis are similar to those of dilated cardiomyopathy,
except that the onset of symptoms and signs is rapid. Therefore, the management
of acute myocarditis is similar to that of dilated cardiomyopathy and follows the
principles of standard heart failure treatment. The clinical manifestations not only
evolve rapidly but also resolve within a short period of time. Loop diuretics are
the mainstay of therapy to relieve pulmonary edema. An angiotensin converting
enzyme (ACE) inhibitor or angiotensin receptor blocker (ARB) is added to reduce
cardiac afterload and to improve ventricular performance. An aldosterone
antagonist (spironolactone or eplerenone) may be added if symptoms persist
on the above treatment. Digoxin has no role in the acute setting since atrial
fibrillation is unusual. Antiviral drugs and immunomodulatory agents do not
improve clinical outcomes.
RECENT ADVANCES
The role of auto-immunity in the pathophysiology of myocarditis and subsequent
onset of dilated cardiomyopathy is being extensively investigated. However, so
far neither anti-inflammatory drugs nor immunomodulatory therapy have been
found to be successful. Auto-antibodies against beta-adrenergic receptors may
also be relevant and patients who test positive for autoantibodies may respond
more favourably to beta-blocker treatment.
SECTION
9
Endocardial
Infections
C A S E
29
Aortic Valve
Endocarditis
CASE PRESENTATION
A 64-year old gentleman presented to the hospital emergency service with fever
for the past 3 days. The fever was high grade and associated with chills but there
were no rigors. The patient was discharged 2 weeks back from this very hospital,
having undergone an aortic valve replacement with a St. Jude’s prosthesis for severe
valve stenosis. Besides fever, he complained of headache, malaise, arthralgias and
anorexia. He had not felt pain or noticed any redness at the sternal wound or at
the site of intravenous cannula insertion. There was no history of productive cough,
burning micturition, vomiting, pain abdomen or loose stools. He did not experience
any significant chest pain or breathlessness. The patient denied having undergone
any dental extraction or endoscopic procedure, after his surgery. He admitted
having been noncompliant with the medication prescribed to him, at the time of
discharge from the hospital.
On examination, he was ill-looking, toxic, febrile (101.80 F) and mildly tachypneic.
Pulse was 110 beats/min with a BP of 130/60 mm Hg and his extremities were warm
and sweaty. There was no sign of inflammation at the sternotomy wound or the site
of intravenous line. The S1 was loud, S2 was sharp but no S3 or S4 was heard. There
was an ejection systolic murmur along the left sternal border, but no pericardial
friction rub was audible. Breath sounds were normal without any rhonchi or crepts.
The abdomen was soft without hepato-splenomegaly or ascites. Additionally, there
were petechiae under the finger nails (splinter hemorrhages) and sub-conjunctival
spots (Roth spots) as well as tender nodules on the finger tips (Osler nodes).
The hemoglobin was 10.8 g/dL with a leucocyte count of 14,800/cu.mm; 86%
were neutrophils and the ESR was 42 mm in 1st hour. Urine analysis showed albumin
+1 with few RBCs but no WBC. ASLO titre was 180 IU with a CRP value of 48 mg/L.
The biochemical parameters were Glucose 88 mg/dl, Creatinine 1.1 mg/dl, Bilirubin
2.2 mg/dl SGOT 24, SGPT 30 and Cholesterol 178 mg/dl. Urine culture did not yield
any bacterial growth and throat swab culture was negative for beta-haemolytic
Streptococcus. No pus was obtainable from the sternotomy wound for culture.
Three sets of blood cultures were obtained, using aseptic skin precautions and all of
them grew Staphylococcus epidermidis, after 72 hours of incubation.
ECG showed sinus tachycardia with few ventricular premature beats but there was
no conduction block. The X-ray chest did not show any cardiomegaly, parenchymal
lung lesion (pneumonitis) or mediastinal widening (mediastinitis). ECHO revealed
normal left ventricular size and ejection fraction. There were irregular echo-reflective
masses attached to the aortic valve leaflets with mild aortic regurgitation (Fig. 29.1).
134 Section 9 Endocardial Infections
Figure 29.1: ECHO showing nodular masses

attached to aortic valve leaflets
Keeping in mind the history of a febrile illness with a prosthetic heart valve,
positive blood cultures and demonstrable vegetations with aortic regurgitation,
the most likely diagnosis in this case is aortic valve endocarditis.
CLINICAL DISCUSSION
Infective endocarditis may be caused by typical organisms, atypical bacteria
and sometimes by fungi (Table 29.1). Non-infective endocarditis is observed in
malignant disease (marantic), collagen disorder (Libman-Sacks) and rheumatic
fever (pancarditis). High-risk lesions for endocarditis are regurgitant left-side
valves (MR, AR), intracardiac shunts (VSD, PDA) and a prosthetic valve. Moderate-
risk lesions are stenotic valves (MS, AS), MVP with MR and right-sided valves (TR
and PS). Low-risk lesions are ASD, HOCM and MVP without MR. Modified Duke’s
criteria are popularly used for the diagnosis of endocarditis (Table 29.2). Fever is
associated with various vascular and immunological phenomena. Blood cultures
may be negative due to prior antibiotic therapy, fastidious or atypical pathogen
and in case of fungal endocarditis. ECHO is used to detect vegetations, diagnose
the underlying lesion, to look for complications and to evaluate the response
to therapy. Transesophageal echocardiography (TEE) is a better approach for
detecting complications, such as prosthetic valve dehiscence or aortic root
abscess and for the timing of surgical intervention.
Table 29.1: Pathogens implicated in infective endocarditis

BACTERIA • Typical Staphylococcus, Streptococcus
HACEK * group bacteria
• Fastidious Legionella, Brucella
Neisseria, Nocardia
• Intracellular Chlamydia, Coxiella
Mycoplasma, Bartonella
FUNGI • Typical Candida, Aspergillus
*HACEK group: Haemophilus, Actinobacillus, Cardiobacterium, Eikenella, Kingella
Case 29 Aortic Valve Endocarditis 135
Table 29.2: Modified Duke’s diagnostic criteria of infective endocarditis

Pathological criteria
• Micro-organism demonstrated by bacterial culture
• Active endocarditis on histological examination
Major criteria
• Positive blood cultures
• Typical organism consistent with endocarditis, on two separate blood cultures
• Organism consistent with endocarditis, from persistently positive blood culture
• Positive serology for Coxiella burnetti, Chlamydia psitacci or Mycoplasma
Evidence of endocardial involvement
• Prosthetic valve dehiscence, aortic root abscess, new valvular regurgitation
Minor criteria
• Predisposing heart condition e.g. prosthetic heart valve or intravenous drug use
• Fever more than 380C
• Vascular phenomenon (embolic complication, mycotic aneurysm, Janeway lesions)
• Immunological phenomenon (rheumatoid factor, nephritis, Osler’s nodes, Roth spots)
• Microbiological evidence not meeting major criteria
• Echocardiographic changes not meeting major criteria
DEFINITE ENDOCARDITIS
• Pathological criteria plus 2 major, or 1 major and 3 minor, or 5 minor criteria
POSSIBLE ENDOCARDITIS
• 1 major and 1 minor, or 3 minor criteria
REJECTED DIAGNOSIS
• Firm alternative diagnosis
• Does not meet above criteria
• Resolution within 4 days of antibiotic therapy
MANAGEMENT ISSUES
The mainstay of the effective treatment of infective endocarditis (barring fungal
endocarditis) is antibacterial drugs. Bactericidal drugs with low minimum
inhibitory concentrations (MIC90) against the identified organism are chosen.
The intravenous route of administration is preferable to achieve high serum
levels of the drug. This ensures adequate penetration of the antibiotic, since the
vegetations are rich in necrotic tissue debris and therefore resist entry of the drug.
A combination of 3rd generation cephalosporin with an aminoglycoside may be
initiated, even when culture reports are pending, and are generally effective
against Streptococcal endocarditis. Infection caused by Staphylococcus aureus
is treated with flucloxacillin or vancomycin along with gentamycin or amikacin.
Usually 2 to 4 weeks of treatment suffices in native valve Streptococcal infection
while 6 weeks are required to treat Staphylococcal prosthetic valve endocarditis.
The indications for surgical intervention in endocarditis are prosthetic valve
dehiscence, aortic root abscess, fistula formation, refractory heart failure and
recurrent embolic events (Table 29.3).
Table 29.3: Indications for surgery in endocarditis

• Fungal or resistant organism causing endocarditis
• Prosthetic valve dehiscence or aortic root abscess
• Heart block (septal abscess) or a fistula formation
• Valve regurgitation leading to refractory heart failure
• Recurrent systemic emboli despite adequate therapy
RECENT ADVANCES
The clinical spectrum of infective endocarditis has undergone a major change in
the last two decades, for several reasons. Prevalence of Staphylococcal infection
has increased with the widespread use of catheters, venous lines and pacing leads
and the rising incidence of intravenous drug abuse. An entirely new HACEK group
of bacteria has been identified as also a range of intracellular bacteria that can
cause endocarditis. With the growing number of valve replacements, prosthetic
valve endocarditis is on the rise. Finally, immunocompromised hosts such as
HIV-infected patients and organ transplant recipients on immunosuppressive
drugs are more likely to be infected by fungal organisms.
C A S E
30
Tricuspid Valve
Endocarditis
CASE PRESENTATION
A 32-year old male came to the out-patient department of a charitable hospital, with
history of fever for the last 3 weeks. The fever was high-grade and associated with
chills and night sweats, but there were no rigors. He also felt fatigued and had lost his
appetite, leading to significant weight loss. However, he was not aware of his exact
past body-weight. He also had cough with scanty mucoid sputum for one month,
but there was no history of burning micturition, vomiting, pain abdomen or passing
loose stools. There was also no history of dyspnea, chest pain or hemoptysis. The
patient was unmarried and he was not accompanied by any attendant. He was a
college drop-out, presently not engaged in any gainful employment. He admitted
smoking and taking alcohol as well as drug-snorts, whenever he could afford them.
He also sometimes abused intravenous illicit drugs.
On examination, the patient was emaciated, ill-looking, confused and febrile.
The pulse rate was 110 beats/min. with a BP of 96/70 mm Hg and a temperature
of 101.60F. He was mildly anemic but not cyanosed or icteric and there was pitting
edema over the ankles. The neck veins were engorged and showed rapid y descent.
There were multiple needle-prick marks over the veins on his forearms. On cardiac
auscultation, a pansystolic murmur was heard over the lower left parasternal area;
no gallop sound was audible. There were scattered rhonchi and crepts over the
lung fields. On examination of the abdomen, there was hepatomegaly. The liver
edge was palpable 5 cm below the costal margin and it was pulsatile. There was no
splenomegaly or ascites.
The hemoglobin was 9.2 g/dL with a total leucocyte count of 13,600/cumm. of
which 78% were neutrophils; the ESR was 64 mm in the 1st hour. Urine analysis
showed albumin +1 with 2-3 WBCs and 8-10 RBCs per high power field. ASLO
titre was 110 IU with a CRP value of 62 mg/L. The biochemical parameters were
Glucose 78 mg/dl, Creatinine 1.4 mg/dl, Bilirubin 1.8mg/dl, SGOT 79 and SGPT
53 and Cholesterol 144 mg/dl. The hepatitis B surface antigen was negative but
his HIV-status was unknown. Urine culture did not yield any bacterial growth.
Throat swab culture was negative for beta-hemolytic Streptococcus. Three sets
Figure 30.1: ECHO showing a nodular mass

attached to valve leaflet
of blood cultures were obtained, including from the veins of the forearms that
bore needle-prick marks. Two out of the three cultures grew coagulase negative
Staphylococcus aureus. One of the cultures grew Candida albicans.
ECG showed sinus tachycardia with narrow QRS complexes and no ST-T
changes. X-ray chest did not show any cardiomegaly, but the broncho-vascular
markings were prominent over both lung fields. ECHO revealed normal left
ventricular size and ejection fraction. The mitral and aortic valves were normal.
An irregular echo-reflective mass was observed, which appeared to arise from the
tricuspid valve (Fig. 30.1). There was moderate degree of tricuspid regurgitation.
Keeping in mind the history of a prolonged febrile illness with intravenous drug
abuse, positive blood cultures and demonstrable vegetations with tricuspid
regurgitation, the most likely diagnosis in this case was of tricuspid valve
endocarditis.
CLINICAL DISCUSSION
Endocarditis of the left-sided cardiac valves is more common than that of the right-
sided valves. This is because of greater turbulence of blood flow in the left side of
the heart and the fact that mitral and aortic valve disease is far more common
than tricuspid valve disease. Tricuspid valve endocarditis usually occurs due
to intravenous drug abuse. Staphylococcal aureus introduced by contaminated
needles from the skin into the venous system, is the most common causative
organism (Table 30.1).
Table 30.1: Pathogens implicated in right-sided endocarditis

• Typical Staphylococcus aureus
• Atypical HACEK* group bacteria
• Fungal Candida, Aspergillus
*HACEK group: Haemophilus, Actinobacillus, Cardiobacterium, Eikenella, Kingella
Case 30 Tricuspid Valve Endocarditis 139
Table 30.2: Masses in right atrium

• Right atrial thrombus
• Right atrial myxoma
• Tricuspid vegetation
• Metastatic deposits
• Chiari network remnant
• Eustachian valve of IVC*
* IVC: Inferior Vena Cava
In an immuno-compromised host, HACEK group bacteria and fungal

pathogens may be implicated, as in our case. Fungal endocarditis causes large
vegetations, which produce a mass in the right atrium. Other causes of a mass in
the right atrium are thrombus, myxoma and metastasis (Table 30.2). Tumors that
grow along the blood vessels, such as hypernephroma of the kidney and hepato-
cellular carcinoma, extend to the right atrium via the inferior vena cava.
The Duke’s diagnostic criteria are popularly used for the definite diagnosis of
endocarditis. Major criteria are microbiological and echocardiographic criteria.
Microbiological evidence is positive blood culture or bacterial serology, of a
typical organism consistent with endocarditis. Echocardiographic evidence is
endocardial involvement in the form of an oscillating valvular structure or new
valvular regurgitation (Table 30.3).
Table 30.3: Diagnostic criteria of infective endocarditis

Microbiological • positive blood culture
• positive bacterial serology
Echocardiographic • oscillating valvular structure
• new valvular regurgitation
MANAGEMENT ISSUES
Antimicrobial agents are the mainstay of treatment of infective endocarditis.
A bactericidal drug with a low MIC90 value against the identified organism is
chosen and given intravenously in full doses for an adequate period of time. For
Staphylococcal infection, flucloxacillin along with gentamycin is the preferred
regimen. For fungal infection, itraconazole or liposomal amphotericin B is
chosen. Both these regimens are followed for 4 to 6 weeks. Septic pulmonary
emboli which can turn into lung abscess, are treated with antimicrobial drugs
and not by anticoagulants.
RECENT ADVANCES
The incidence of Staphylococcal endocarditis has risen due to the widespread use
of catheters, venous lines and pacing leads. Methicillin resistant Staph. aureus
(MRSA) has emerged as a notorious pathogen. Fungal endocarditis is been
recognized in immunocompromised hosts such as HIV-infected patients and
organ-transplant recipients on immunosuppressive drugs.
SECTION
10
Intracardiac
Masses
C A S E
31
Atrial
Myxoma
CASE PRESENTATION
A 42-year old woman presented to the out-patient department with frequent
fainting spells in the preceding two weeks, against a background history of exertional
breathlessness. Besides being dyspneic, she had felt fatigued and lethargic over
the last 3 months. She had also noticed a low-grade fever and an unintentional
weight loss of 4 kg in this period. She gave no history of chills, rigors, night-sweats
or nocturnal dyspneic episodes. There was no history of cyanotic spells, squatting
attacks or fleeting joint pains during her childhood. She denied smoking tobacco,
consuming alcohol or abusing illicit drugs.
On examination, she was mildly pale and dyspneic, but not in any distress. Her
heart rate was 96 beats/min. with a BP of 128/84 mm Hg and her temperature was
99.8°F. There were no signs of congestive heart failure. On auscultation, a mid-
diastolic rumble was heard at the mitral area, preceded by a high-pitched third
heart sound. The intensity and character of the murmur changed with the patient’s
position. There were no crepitations over the lung fields.
CLINICAL DISCUSSION
A history of dyspnea, constitutional symptoms and febrile illness with a cardiac
murmur, raises several diagnostic possibilities. These are:
• Rheumatic fever
• Occult malignant disease
• Atrial myxoma.
The most likely reason for a mid-diastolic murmur in the mitral area is
rheumatic mitral stenosis. The murmur is preceded by an opening snap, if the
leaflets are pliable. The murmur is best audible with the patient in the left lateral
position but does not change its character. ECG and X-ray chest of this patient
were unremarkable. ECHO revealed a round mobile mass in the left atrium,
arising from the fossa ovalis of the inter-atrial septum and prolapsing into the
mitral valve orifice (Fig. 31.1). The mass was lobulated in outline and variegated
144 Section 10 Intracardiac Masses
Figure 31.1: ECHO showing left atrial myxoma

prolapsing into the mitral orifice
in echogenicity. The left atrium was mildly dilated but the mitral valve leaflets
were normal in structure and excursion. This appearance was highly suggestive
of an atrial myxoma.
An atrial thrombus closely resembles a myxoma with certain subtle differences.
The thrombus arises from the posterior atrial wall, not the septum and it is never
pedunculated. A myxoma is usually pedunculated and rarely sessile. Myxoma
generally prolapses into the mitral valve orifice in diastole (Fig. 31.2), unless it
is too large in size or sessile. Thrombus is fairly rounded in outline and uniform
in echogenicty, while a myxoma is often lobulated and variegated in appearance
due to areas of hemorrhage, cystic necrosis and calcification. Moreover, a left
atrial thrombus is often associated with structural abnormality of the mitral valve.
The differences between an atrial myxoma and an atrial thrombus are given in
Table 31.1. Besides an atrial myxoma and an atrial thrombus, other structures
that may be rarely seen in the left atrium are supravalvular ring (cor triatriatum),
dilated coronary sinus, anomalous pulmonary veins, and large mitral leaflet
vegetations.
Figure 31.2: Figure showing left atrial myxoma (A), prolapsing into the mitral orifice (B)
RV: Right ventricle; LV: Left ventricle; AV: Aortic valve; MV: Mitral valve;
AO: Aorta; LA: Left atrium
Case 31 Atrial Myxoma 145
Table 31.1: Differences between atrial myxoma and atrial thrombus

Atrial myxoma Atrial thrombus
Site Atrial septum Posterior wall
Attachment Pedunculated Free
Shape Lobulated Rounded
Echogenicity Echolucent Echogenic
Prolapse in MV Often Rare
Mitral valve Normal Diseased
In retrospect, the mid-diastolic murmur in our case was not due to mitral
stenosis but caused by the atrial myxoma obstructing the mitral valve (ball-valve
effect). Also, the third heart sound preceding the murmur was not the opening
snap of the valve but the ‘tumour-plop’ of the myxoma. On M-mode scan of the
mitral valve, there is an early echo-free zone, before the myxoma obstructs the
valve. This echo-free zone is not seen in case of mitral stenosis.
In general, cardiac tumours are rare. Secondary metastases are far more
common than primary neoplasms of the heart. Metastasis from lung and breast
are the commonest due to their overall prevalence and direct extension to the
heart (Table 31.2). Majority (75%) of primary cardiac tumours are benign of
which nearly half (50%) are myxomas. Other benign tumours are rhabdomyoma
and fibroelastoma. Majority of the malignant tumours are the sarcomas such as a
rhabdomyosarcoma and fibrosarcoma.
Table 31.2: Various types of cardiac tumours

Primary (rare) Benign (common) Myxoma
Rhabdomyoma
Fibroelastoma
Malignant (rare) Angiosarcoma
Fibrosarcoma
Rhabdomyosarcoma
Secondary (common) Metastasis Lung, breast
Stomach, ovary
Lymphoma
A myxoma is a friable and gelatinous tumour of connective tissue origin,

related to germ-line mutation. It is more common (75%) in women than men
and is generally diagnosed in the fourth or fifth decade of life. It occurs 3 times
more often in the left atrium than in the right atrium. Although usually single and
2 to 8 cm in size, myxomas may also be multiple, familial and part of a clinical
syndrome with neurofibromas, naevi and lentigines.
NAME syndrome: Naevi, Atrial myxoma, Myxoid neurofibroma, Ephilides

LAMB syndrome: Lentigines, Atrial Myxoma, Blue naevi
Myxomas may present with any or all of the triad of symptoms which are
constitutional, obstructive and embolic in nature. Systemic symptoms may
be vague and include low grade fever, lethargy, arthralgias and weight loss.
Obstructive symptoms are due to mitral inflow occlusion and resemble the
symptoms of mitral stenosis such as pulmonary congestion, hemoptysis and
syncope. Finally, fragments of myxoma tissue may embolize to remote vascular
locations.
Transesophageal echocardiography (TEE) may provide vital additional informa
tion, over and above that obtained from the transthoracic approach. This pertains
to the size, site, attachment of the myxoma and its valvular involvement. This
information serves as a guide for surgical resection. Advanced imaging techniques
such as computed tomography (CT) and magnetic resonance imaging (MRI),
provide further useful anatomical information.
MANAGEMENT ISSUES
Surgical resection of the myxoma is the only definitive form of treatment and
most myxomas are amenable to surgery. Complete full thickness resection is
preferable, in order to prevent recurrence. Myxomas are removed even if they do
not cause any symptoms, in order to avoid embolic events. The atrial septum to
which the myxoma is often attached, is reconstructed using a pericardial patch.
Long-term surveillance by annual follow-up ECHO is recommended to detect
recurrence.
RECENT ADVANCES
Newer sophisticated imaging techniques such as computed tomography (CT)
and magnetic resonance imaging (MRI) can improve upon the anatomical
information about myxomas provided by echocardiography (ECHO). More
recently, contrast ECHO has been used to differentiate myxomas from thrombi on
the basis of their vascularity. Intravenous injection of microbubbles will perfuse
the tumour but not the thrombus.
C A S E
32
Atrial
Thrombus
CASE PRESENTATION
A 32-year old married woman, a domestic servant by profession, came to a general
hospital with sudden weakness of the right hand and inability to walk, since the
last 10 hours. There was no history of recent febrile illness, trauma to the head or
purulent discharge from the ear. However, she always felt tired and short of breath
and she had curtailed her working hours for the past 1 year. She also experienced
episodic palpitations and dizzy spells, ever since the birth of her second child, 3
years back. She recollected being extremely breathless in the last trimester of her
pregnancy and her obstetrician strictly advising her not to bear any more children.
The patient also distinctly remembered having been incapacitated by severe joint
pains for over a month, when she was 15 years of age.
On examination, she was anxious and tachypneic. Her tongue and palms were
pale but there was no cyanosis or icterus. The pulse was irregular, low in volume at
a rate of about 90 beats/min. while the heart rate was around 110 beats/min. The
BP was 104/72 mm Hg in the right arm and all peripheral pulses were palpable. The
JVP was raised and there was mild pitting edema over the ankles. The apex beat
was normal in character and a left parasternal heave was felt. The S1 was variable
in intensity and the P2 was loudly audible. A low-pitched mid-diastolic rumble was
heard over the cardiac apex, which did not accentuate in pre-systole. The lung fields
were clear on auscultation. On neurological examination, power in the right hand
and at the elbow was grade 3 while power in the right knee was grade 4. Sensations
were preserved and the deep tendon reflexes were diminished on the right side. The
was also a right supranuclear facial nerve palsy and slight slurring of speech.
CLINICAL DISCUSSION
From the history and physical examination, this woman had rheumatic heart
disease with mitral stenosis and atrial fibrillation. In all likelihood, she had
an embolic stroke due to a dislodged fragment of a left atrial thrombus, which
occluded the left middle cerebral artery and caused hemiparesis. An irregular
pulse, with a pulse rate deficit compared to the heart rate, are signs of atrial
fibrillation. The S1 is variable in intensity, because of a beat-to-beat variation
of the diastolic filling period. In the presence of atrial fibrillation, the mid-
diastolic murmur does not undergo presystolic accentuation, because the atrial
contribution to ventricular filling is absent.
Figure 32.1: ECG showing atrial fibrillation with fast ventricular response
As expected, the ECG showed atrial fibrillation with a fast ventricular response
(Fig. 32.1). Additionally, there was evidence of right ventricular hypertrophy and
right-ward deviation of the QRS vector. X-ray chest findings were cardiomegaly,
straightening of the left heart border with a prominent atrial appendage as well
as signs of interstitial pulmonary edema. On ECHO, the left ventricle was normal
but the left atrium and right ventricle were dilated. The mitral valve leaflets were
thickened and showed limited excursion with diastolic doming (Fig. 32.2). The
aortic valve was structurally normal. No mass or thrombus was demonstrated
in any cardiac chamber. The pulmonary artery pressure, as estimated from the
tricuspid regurgitant jet, was elevated.
Figure 32.2: ECHO showing thickened mitral leaflets,

diastolic doming and dilated left atrium
Enlargement of the left atrial appendage is typical of mitral valve disease,

be it stenosis or regurgitation, especially when due to rheumatic heart disease.
Probably acute rheumatic carditis damages the left atrial wall and its appendage,
causing them to bulge when the atrial filling pressure rises. Enlargement of the
left atrial appendage causes straightening of the left heart border on chest X-ray
(Fig. 32.3). The “4-bump heart” is formed above downward by the aortic knuckle,
pulmonary artery, left atrial appendage and the left ventricular lateral wall.
The risk of thromboembolism in mitral stenosis is very high, particularly if
atrial fibrillation is present and more so if it is intermittent. Mitral stenosis can be
safely assumed to be the cause of cerebral infarction, even if a left atrial thrombus
is not demonstrable. A thrombus that is too small for detection, thrombus in
the atrial appendage or one that has already embolised, may be missed on
Case 32 Atrial Thrombus 149
Figure 32.3: X-RAY showing cardiomegaly with

straightening of left heart border
transthoracic echo. Transesophageal echocardiography (TEE) vastly improves

the detection of a thrombus in the left atrial cavity or its appendage. Occasionally,
an ECHO may show a very large atrial thrombus, which is potentially fatal if it
suddenly obstructs the mitral valve. Such a ‘ball-valve’ thrombus is an indication
for urgent surgical intervention.
A left atrial thrombus sometimes needs to be differentiated from a left atrial
myxoma, since both can cause distal embolism. Thrombus usually arises from the
posterior atrial wall or floats freely, while myxoma is usually pedunculated and is
attached to the inter-atrial septum. Thrombus is rounded in shape and uniform
in echogenicity, while myxoma is often lobulated with variable echogenicity due
to areas of hemorrhage, necrosis or calcification. Finally, thrombus uncommonly
prolapses into the diseased mitral valve while myxoma commonly prolapses
into the normal mitral valve. The differences between atrial thrombus and atrial
myxoma are given in Table 32.1.
Besides an atrial thrombus and an atrial myxoma, other structures that may be
sometimes seen in the left atrium are supravalvular ring (cor triatriatum), dilated
coronary sinus, anomalous pulmonary veins, large mitral leaflet vegetation and
reverberation artefacts from a calcified mitral annulus.
Table 32.1: Differences between atrial thrombus and atrial myxoma

Atrial thrombus Atrial myxoma
Site Posterior wall Atrial septum
Attachment Free Pedunculated
Shape Rounded Lobulated
Echogenicity Echogenic Echolucent
Prolapse in MV Rare Often
Mitral valve Diseased Normal
MANAGEMENT ISSUES
In a patient of mitral stenosis and atrial fibrillation with a thrombo-embolic stroke,
the first priority is anticoagulation. Low molecular-weight heparin(LMWH) such
as enoxaparin is preferred because of predictable pharmacokinetics, without the
need of prothrombin time (PT) and aPTT monitoring. An oral anticoagulant like
warfarin is initiated almost simultaneously, since it requires 3 to 5 days to produce
optimal anticoagulation. Oral anticoagulation is required on a long-term basis in
a patient of mitral stenosis with history of thromboembolism.
Other cardiovascular drugs are used to reduce the symptomatology of mitral
stenosis. Diuretics reduce pulmonary congestion, especially in those with
concomitant mitral regurgitation. Digoxin, beta-blockers and verapamil control
the ventricular response in atrial fibrillation and improve left ventricular diastolic
filling. Percutanenous balloon mitral valvuloplasty (BMV) in not advocated in
patients with a demonstrable left atrial thrombus. Mitral valve replacement is
required for critical stenosis (mitral valve area < 1 cm2).
C A S E
33
Ventricular
Thrombus
CASE PRESENTATION
A 67-year old gentleman was brought to the cardiology department by his son for a
follow-up visit, 1 week after discharge from the hospital. The patient had sustained an
anterior wall myocardial infarction 2 weeks ago, for which he received thrombolytic
therapy. However, the streptokinase infusion was stopped at the half-way stage,
because the patient developed hypotension. Thereafter, his course in the hospital
was by-and-large uneventful, barring a extrasystolic ventricular bigeminy lasting
36 hours, which was treated with lignocaine infusion. Upon returning home, he did
not experience any angina, dyspnea, palpitation or syncope. Medications prescribed
to him were aspirin 150 mg, clopidogrel 75 mg, ramipril 5 mg, atorvastatin 20 mg
and metoprolol 50 mg.
On examination, the patient was alert, comfortable and not tachypneic. The pulse
was fair in volume, at a rate of 92 beats/min. and few ectopic beats were noticed. The
BP was 110/66 mm Hg in the right arm. All peripheral pulses were well palpable. The
JVP was not raised and there was no hepatomegaly or ankle edema. On precordial
examination, the apex beat was diffuse in nature and displaced towards the left
axilla. The S1 and S2 heart sounds were normal but a soft S3 was audible in diastole.
There was no murmur or pericardial rub audible. Few inspiratory crackles were heard
over the lower lung fields posteriorly.
ECG showed sinus rhythm at a rate of 92 beats/min. and an occasional unifocal
ventricular premature beat was seen. No couplet or bigeminal rhythm was noticed.
There was complete attenuation of the R waves with deep Q waves in the precordial
leads V1 to V4. Small q waves were also seen in leads V5 and V6 (Fig. 33.1). There was
coving of the S-T segment with inversion of the T wave in all the precordial leads. The
Q-T interval was normal.
ECHO showed a dilated left ventricle with depressed systolic function (ejection
fraction 35%). The mid and distal segments of the interventricular septum, the left
ventricular apex and the distal segment of the lateral wall were hypokinetic but no
dyskinetic area was identified. A well-defined rounded mass was seen arising from
the ventricular apex and protruding into the left ventricular cavity (Fig. 33.2). The
echogenicity of the mass was variable and it did not move in synchrony with the
ventricle. The posterior mitral leaflet failed to reach the plane of the mitral annulus
and the leaflet coaptation point was distally located in the ventricle. The aortic valve
leaflets were mildly thickened but had normal excursion without systolic doming.
No pericardial effusion was noted.
Figure 33.1: ECG showing R wave attenuation, deep Q waves

with S-T segment coving and T wave inversion
Figure 33.2: ECHO showing a dilated, hypokinetic left ventricle

with a mural thrombus arising from the apex
CLINICAL DISCUSSION
From the history and physical examination as well as from the ECG and ECHO
findings, this elderly gentleman had sustained a recent extensive anterior wall
myocardial infarction. He had now developed left ventricular systolic dysfunction
and a ventricular thrombus. Two weeks after acute myocardial infarction (MI)
usual complications include post-MI angina, malignant ventricular arrhythmias
and left ventricular failure. Sometimes, a ventricular thrombus may form on an
infarcted and scarred myocardial segment or within a left ventricular aneurysm.
This patient did not demonstrate any dyskinetic myocardial segment and there
was no outward bulge of the left ventricular cavity. A fragment of the ventricular
thrombus may break away and travel through the systemic circulation to cause
embolization in any distal vascular bed.
A dilated left ventricle with reduced systolic wall motion and stagnated
blood flow, is an ideal setting for ventricular thrombus formation. A ventricular
thrombus may form on a dyskinetic, infarcted and scarred myocardial segment or
within a left ventricular aneurysm. Idiopathic dilated cardiomyopathy is another
important reason for ventricular thrombus formation.
A pedunculated ventricular thrombus appears as a well-defined, rounded,
mobile stalked mass, that protrudes into the ventricular cavity. The mobility of
the thrombus is not synchronous with the left ventricular free wall motion. It is
Case 33 Ventricular Thrombus 153
more likely to embolize if the echodensity is variable due to areas of necrosis.

A mural ventricular thrombus is a flat, laminated mass, contiguous with the
ventricular wall with which it moves synchronously. It is more echogenic than
the adjacent myocardium and less likely to embolize than a pedunculated
thrombus. Thrombus always has a clear identifiable edge while an artefact caused
by stagnated blood has a hazy appearance. On color flow mapping, the flow stops
abruptly at the edge of a thrombus but not at the edge of an artefact.
Left ventricular thrombus is a masquerader of several other mass lesions.
Causes of a mass in the left ventricle are rhabdomyoma, false tendon and
prominent papillary muscle. Mural thrombus can be distinguished from localized
myocardial thickening by the fact that myocardium thickens during systole while
a thrombus does not. Thrombus can be differentiated from a cardiac tumor by the
fact that adjacent wall motion is almost always abnormal in case of thrombus and
often normal in case of tumor.
MANAGEMENT ISSUES
Presence of a ventricular thrombus is an established indication for oral
anticoagulant therapy. Other clear indications for anticoagulation are mechanical
prosthetic heart valve, left atrial thrombus with mitral stenosis and ventricular
aneurysm. When an oral anticoagulant like warfarin is initiated, it takes 3 to 5
days for the onset of full therapeutic effect. In this interim period, heparin therapy
may be given. Unfractionated heparin requires monitoring but it is cost effective.
Low molecular weight heparin (LMWH) is more expensive, but it does not require
prothrombin time (PT) or aPTT monitoring.
It is now widely recommended that every myocardial infarction patient
must receive aspirin, a statin, an ACE-inhibitor and a beta-blocker. Our patient
had already been prescribed all these drugs. A diuretic can be added to reduce
cardiac work-load, if pulmonary congestive symptoms develop. An aldosterone
antagonist such as spironolactone or eplerenone can also be used. These drugs,
besides reducing cardiac work-load, improve ventricular remodelling and lower
cardiovascular mortality.
SECTION
11
Typical ECG
Abnormalities
C A S E
34
Left Ventricular
Hypertrophy
CASE PRESENTATION
A 56-year old obese gentleman visited his physician with the complaints of frequent
headaches and spells of dizziness. He also found it difficult to carry out routine
tasks and felt fatigued and breathless even on mild physical exertion. The patient
was diagnosed to have systemic hypertension about 30 years back. At that time, he
was extensively investigated for secondary hypertension, but no renal or endocrine
disorder was detected. He was prescribed antihypertensive medications, but did
not take them regularly and was not on periodic medical follow-up. The patient had
always been overweight even at a young age and was diagnosed to have diabetes
mellitus about 10 years back. He had not got his lipid profile checked recently. The
man was sedentary and did not follow any particular diet plan. He smoked 8 to 10
cigarettes per day and consumed 2 to 3 large pegs of whiskey, on most days of the
week.
On examination, the patient was grossly overweight, with a body mass index
(BMI) of 34 kg/m2. He was tachypneic and afebrile. The pulse was regular, good in
volume and the heart rate was 84 beats/min. All peripheral pulses were palpable
and there was no carotid bruit or brachio-femoral delay. The BP was 164/106 mm
Hg over the right arm in the sitting position. The JVP was not raised but mild edema
was present over the ankles. The cardiac apex beat was sustained and heaving in
nature and systolic pulsations were observed in the suprasternal notch. The S1 was
normal but the A2 sound was accentuated; a S4 sound was heard in presystole. A soft
systolic murmur was audible over the upper left parasternal area. Few basilar rales
were audible over the lung fields. An ECG was obtained (Fig. 34.1).
Figure 34.1: ECG showing tall R waves in left chest leads

with deep S waves in right chest leads
158 Section 11 Typical ECG Abnormalities
ECG INTERPRETATION
The ECG showed tall R waves exceeding 25 mm in the left precordial leads and
deep S waves in the right precordial leads. There also was S-T segment depression
and T-wave inversion in the lateral leads. These findings are consistent with the
diagnosis of left ventricular hypertrophy. The tall R waves in the lateral precordial
leads, reflect the increased electrical forces generated by the thickened left
ventricular myocardium. S-T segment depression and T wave inversion in these
leads indicate left ventricular strain. Additional findings can include notched
bifid P waves (P. mitrale) due to left atrial enlargement and left axis deviation of
the QRS vector.
Voltage criteria are often employed for the electrical diagnosis of left
ventricular hypertrophy (Table 34.1). The Sokolow and Lyon criteria of S in V1
plus R in V5 or V6 greater than 35 mm is popularly used. Another criteria used
is of Framingham wherein, R in V5 or V6 >25 mm or R in aVL >11 mm is taken as
criteria for left ventricular hypertrophy. The left ventricular (LV) strain pattern
of S-T segment depression and T-wave inversion is observed in LV pressure
overload. In case of LV volume overload, the S-T segment is isoelectric and the T
wave is upright and tall.
Table 34.1: Voltage criteria for left ventricular hypertrophy

• Sokolow and Lyon Criteria
S. in V1 + R in V5 or V6 >35 mm
• Framingham Criteria
R in V5 or V6 >25 mm; R in aVL >11 mm
CLINICAL DISCUSSION
Causes of left ventricular hypertrophy are classified into those of systolic
overload and the causes of diastolic overload. Systolic overload is due to
systemic hypertension, aortic stenosis, coarctation of aorta and hypertrophic
cardiomyopathy. Causes of diastolic overload are mitral and aortic valve regurgi
tation and an intracardiac left-to-right shunt such as ventricular septal defect or
patent ductus arteriosus (Table 34.2).
Echocardiography is 5 to 10 times more sensitive than an ECG, in detecting
left ventricular hypertrophy (LVH). Presence of LVH is the most common ECHO
abnormality in a hypertensive patient. Conversely, systemic hypertension is the
most common cause of LVH. Besides detecting LVH, ECHO is used to evaluate
left ventricular systolic and diastolic function and for the diagnosis of associated
coronary artery disease. Other abnormalities seen on ECHO are mitral and aortic
valve degeneration, dilatation of the aortic root and rarely aortic coarctation
(Table 34.3). ECHO typically shows thickening of the interventricular septum (IVS)
and LV posterior wall (LVPW) greater than 11 mm with virtual obliteration of the
left ventricular cavity during systole (Fig. 34.2). Left ventricular hypertrophy is an
Case 34 Left Ventricular Hypertrophy 159
Table 34.2: Causes of left ventricular hypertrophy

Systolic overload
• Aortic valve stenosis
• Coarctation of the aorta
• Hypertrophic cardiomyopathy
Diastolic overload
• Mitral regurgitation
• Aortic incompetence
• Ventricular septal defect
• Patent ductus arteriosus.
Figure 34.2: ECHO showing thick septum and posterior wall

with obliteration of left ventricular cavity
independent predictor of cardiovascular morbidity and mortality, as a risk factor

for myocardial infarction, stroke, heart failure and sudden arrhythmic death.
Serial echoes are performed annually to monitor the progress of hypertensive
heart disease, particularly to assess the regression of LVH with antihypertensive
drug therapy.
Table 34.3: ECHO findings in systemic hypertension

• Left ventricular hypertrophy (LVH)
• Diastolic and systolic LV dysfunction
• Regional wall motion abnormality
• Mitral and aortic valve calcification
• Dilatation of aortic root
• Coarctation of aorta.
MANAGEMENT ISSUES
Current practice guidelines recommend that all hypertensive patients with left
ventricular hypertrophy, target organ damage and associated cardiovascular risk
factors particularly diabetes, should be offered antihypertensive drug treatment.
The choice of first-line therapy has been the subject of debate, as well as the
study design of several clinical trials. It is being believed that newer agents like
calcium channel blockers (e.g. amlodipine) or an ACE inhibitor (e.g. ramipril) are
more effective for regression of hypertrophy than older agents like diuretics (e.g.
thiazide) or beta blockers (e.g. atenolol). Moreover, newer agents are less likely to
increase the risk of diabetes than the older agents. The increased risk of diabetes
with older drugs is particularly relevant in patients of South Asian origin, who are
already at higher risk of developing diabetes and the metabolic syndrome.
C A S E
35
Left Bundle
Branch Block
CASE PRESENTATION
A 63-year old man was brought to the emergency room past midnight, with the
complaint of shortness of breath and difficulty in lying flat on the bed. The patient
had sustained an anterior wall myocardial infarction 5 months back and ever since,
he had complained of easy fatiguability and dyspnea on exertion. For the past
1 week, he required 2 or 3 pillows in bed to catch sleep and yet woke up often because
of air-hunger. There was no recent history of chest pain, palpitation or syncope. His
current daily medication was ramipril 5 mg, torsemide 20 mg, metoprolol 25 mm,
aspirin 150 mg, clopidogrel 75 mg and atorvastatin 10 mg.
On examination, the patient was obviously tachypneic and looked anxious. He
was pale and diaphoretic. The extremities were cold but there was no cyanosis. The
JVP was not raised and there was no pitting edema around the ankles. The pulse
was fast and low in volume with variable volume in successive beats. The BP was
104/74 mm Hg over the right arm in the supine position. The cardiac apex beat was
diffuse and displaced towards the left axilla. The S1 was normal and the A2 was loud.
There was paradoxical splitting of S2, that shortened during the phase of inspiration.
A S3 was also appreciated in early diastole. A pansystolic murmur was audible over
the cardiac apex, that radiated towards the axilla. Auscultation over the lung fields
revealed bilateral crepts over the bases posteriorly. An ECG was obtained (Fig. 35.1).
ECG INTERPRETATION
The ECG showed broad ventricular complexes, that measured 0.14 sec. in width.
In leads L1 and V6, each ventricular complex had two peaks, producing a M-shaped
RsR’ pattern. The broad complex was followed by S-T segment depression and
T-wave inversion. These findings are consistent with the diagnosis of left bundle
branch block. Bundle branch block denotes delayed or interrupted conduction
down the right or left branch of the bundle of His (Fig. 35.2). It leads to widening
of the ventricular complex, due to delayed depolarization of the ventricle whose
bundle branch is blocked. The QRS complexes of both the ventricles are “out of
sync” with each other and produce two R waves in sequential order. Incomplete
bundle branch block (BBB) results in QRS width of 0.11 to 0.12 sec while in
complete BBB, the QRS width exceeds 0.12 sec. The S-T segment depression and
Figure 35.1: ECG showing wide QRS complexes

due to left bundle branch block
Figure 35.2: Diagram to represent left bundle

branch block (LBBB)
T-wave inversion are repolarization abnormalities, secondary to the abnormal

pattern of ventricular depolarization.
In left bundle branch block (LBBB), the M-shaped RsR’ pattern is observed
in lead V6. The notched R wave represents abnormal sequence of septal and
left ventricular free wall depolarization. There is total distortion of the normal
QRS complex. In right bundle branch block (RBBB), the M-shaped rsR pattern
is observed in lead V1. The septal (denoted by r) and free wall (denoted by S)
depolarization through the left bundle branch are normal. Right ventricular
depolarization (denoted by R’) is delayed. Therefore RBBB does not distort the
normal QRS complex, but only adds a terminal R’ deflection.
Block in the conduction of impulses down one of the two fascicles of the
left bundle branch constitutes a hemiblock (Fig. 35.3). Left anterior hemiblock
(LAHB) is far more common than left posterior hemiblock (LPHB). Anterior
hemiblock is more common because the anterior fascicle is compact, has a single
blood-supply and lies close to the disease prone aortic valve. On the other hand,
the posterior fascicle is fan-like, has a dual blood-supply and is far less likely to
be involved in septal pathology. Hemiblock (LAHB) causes an abnormal pattern
of left ventricular activation, resulting in left axis deviation of the QRS vector
(Fig. 35.4).
Case 35 Left Bundle Branch Block 163
Figure 35.3: Diagram to represent left anterior

hemiblock (LAHB)
Figure 35.4: ECG showing left axis deviation

due to left anterior hemiblock
CLINICAL DISCUSSION
Left bundle branch block (LBBB) often indicates the presence of organic heart
disease. A variety of cardiac conditions can cause LBBB including myocardial
infarction, systemic hypertension, aortic valve disease, cardiomyopathy and
fibrocalcerous degeneration (Table 35.1). On the other hand, right bundle branch
block (RBBB) is sometimes observed in normal individuals. Typical causes of
RBBB are atrial septal defect, acute pulmonary embolism and chronic obstructive
pulmonary disease.
RBBB does not distort the QRS complex, but only adds a terminal deflection
due to delayed right ventricular depolarization. Therefore, changes of myocardial
infarction such as appearance of Q waves and loss of R wave height, can be readily
diagnosed in the presence of RBBB. However, it is difficult to diagnose myocardial
infarction in the presence of LBBB, since the QRS complex is completely distorted.
Table 35.1: Causes of left bundle branch block

• Fibrocalcerous degeneration.
Table 35.2: Criteria for diagnosing MI in presence of LBBB

• Presence of q wave in L1, V5 and V6
• Terminal S wave in leads V5 and V6
• S-T segment drift greater than 5mm
• Upright T wave concordant with QRS
The criteria for the diagnosis of myocardial infarction in the presence of LBBB are
given in Table 35.2.
In left bundle branch block (LBBB), there is paradoxical splitting of the second
heart sound (S2). The A2 component of S2 is delayed due to late depolarization
of the left ventricle and follows the P2. During inspiration, when P2 gets delayed
due to increased venous return, the splitting of S2 becomes narrow instead of
physiological widening. Besides LBBB, another cause of paradoxical splitting of S2
is aortic valve stenosis in which left ventricular ejection time is prolonged. Other
reasons for paradoxical splitting of A2 are pre-excitation of the right ventricle
through an accessory bypass tract or premature activation of the right ventricle
by an external pacemaker. LBBB causes a jerky motion of the interventricular
septum on echocardiography. This should not be diagnosed as septal infarction,
unless the septum fails to thicken during systole.
MANAGEMENT ISSUES
There is no specific treatment of left bundle branch block. The underlying heart
disease is to be managed on its own merit. There is a word of caution regarding
the use of drugs which block the A-V node such as verapamil, diltiazem and
beta blockers. They may cause complete heart block, particularly if the block is
bifascicular or trifascicular to begin with. Bifasicular block includes right bundle
branch block and left anterior hemiblock. Trifasicular block is bifasicular block
with a prolonged P-R interval.
C A S E
36
Features of
Hypokalemia
CASE PRESENTATION
A 48-year old man sought consultation from a physician for pain and weakness in
both arms, since the last 3 days. There was no history of chest pain, breathlessness,
palpitation or sweating. The heaviness in the arms was aggravated by lifting a light
weight, but not by movement at the neck. The patient also complained of fatigue and
pain over his calf muscles while walking. He had systemic hypertension for several
years for which he was presently prescribed losartan 50 mg and hydrochlorthiazide
25 mg. The patient also suffered from bronchial asthma, for which he used an
inhaler containing a combination of salmeterol and fluticasone. Recently, he had
a gastro-intestinal infection with profuse vomiting and diarrhea that lasted 2 days.
Besides age and hypertension, other cardiovascular risk factors in the patient were
prediabetes and a modestly elevated serum cholesterol.
On examination, the patient was coherent, comfortable and in no distress. There
was no anemia, cyanosis, icterus or ankle edema. The JVP was not raised, thyroid
gland was normal and there were no palpable lymph-nodes. The pulse rate was 72
beats/min with a BP of 130/80 mm Hg. The precordium was unremarkable and the
apex beat was normally located. The S1 and S2 were normal and no gallop sound was
heard. No murmur or pericardial friction rub was audible. The breath sounds were
normal without rhonchi or crepts. An ECG was obtained (Fig. 36.1) following which
he was asked to immediately see a cardiologist. His blood biochemistry was Glucose
128 mg/dl, Urea 38 mg/dl, Creatinine 1.2 mg/dl, LDL cholesterol 147 mg/dl, Sodium
131 m Eq/L and Potassium 2.9 m Eq/L.
Figure 36.1: ECG showing flat T waves with prominent U waves

ECG INTERPRETATION
The ECG showed normal sinus rhythm. The P wave and QRS complex were
normal in morphology. There were no significant Q waves and the S-T segment
was isoelectric. The T wave was reduced in amplitude, while the U wave was
prominent. The Q-T interval seemed to be prolonged. These findings are consistent
with the diagnosis of hypokalemia. Hypokalemia is an important cause of T wave
change. The T wave is either reduced in amplitude, flattened or inverted. This is
associated with prominence of the U wave that follows the T wave. The low T wave
followed by a prominent U wave produces a ‘camel-hump’ effect.
In hypokalemia, the T wave is flattened and the prominent U wave may
be mistaken for the T wave. This may falsely suggest prolongation of the Q-T
interval, whereas it is actually the Q-U interval. Hypokalemia therefore causes
pseudo-prolongation of the Q-T interval, at the expense of T wave. The U wave
that is exaggerated and approximates the size of the T wave is considered to be a
prominent U wave. Other causes of prominent U wave are cardiovascular drugs
e.g. digitalis, quinidine and psychotropic agents e.g. phenothiazines, tricyclic
antidepressants.
Figure 36.2: ECG features of progressively increasing hypokalemia
The ECG features of hypokalemia depend upon its severity (Fig. 36.2). In mild
hypokalemia, only the T wave amplitude is reduced. In moderate hypokalemia,
the U wave becomes more prominent than the T wave. In severe hypokalemia,
there is sagging of the S-T segment and only the U wave is visible (Table 36.1).
Table 36.1: ECG features of progressive hypokalemia

• Reduced amplitude of the T wave
• Flat T wave with prominent U wave
• S-T segment sagging; only U wave.
Case 36 Features of Hypokalemia 167
CLINICAL DISCUSSION
The ECG is sometimes helpful in the diagnosis of an electrolyte abnormality, even
before the blood biochemistry results from the laboratory are available. Variation
in the blood levels of potassium and calcium are mainly responsible for these
ECG changes. Hypokalemia causes pseudo-prolongation of the Q-T interval, at
the expense of the T wave. Hypocalcemia causes true prolongation of the Q-T
interval, with true lengthening of the S-T segment.
Table 36.2: Causes of hypokalemia

• Body fluid loss
Diuresis
Vomiting
Diarrhea
• Redistribution
Metabolic alkalosis
• Drug-induced
Beta-agonist
Insulin therapy
• Hyperaldosteronism
Cushing’s disease
Conn’s syndrome
• Genetic causes
Hypokalemic periodic paralysis
Renal tubular acidosis Type 2
There are several causes of hypokalemia which have been enlisted in

Table 36.2. True loss of potassium is due to vomiting, diarrhea, naso-gastric
suction and diuretic therapy (see Table 36.2). Redistribution of potassium occurs
in metabolic alkalosis (intracellular shift) and with beta-agonist or insulin
therapy. Hypokalemia is a feature of cortisol excess due to Cushing’s disease or
steroid therapy as well as a feature of hyperaldosteronism in Conn’s syndrome.
Genetic causes of hypokalemia are Type 2 renal tubular acidosis (RTA-2) and
hypokalemic periodic paralysis. Typical clinical features of hypokalemia are
fatigue and leg cramps. In severe cases, neuromuscular paralysis and cardiac
arrhythmias may occur. In cardiac patients on diuretic treatment, hypokalemia
aggravates digitalis toxicity and increases the likelihood of serious ventricular
arrhythmias.
In our case, the hypokalemia was multi-factorial. Firstly, the patient was
prescribed a diuretic for his hypertension. Secondly, he was using an inhaled
beta-agonist for asthma, which is known to cause hypokalemia. Finally, he had
a recent episode of gastro-enteritis, which might have caused substantial loss of
potassium from his body.
Table 36.3: Management of hypokalemia

• Potassium replacement
Dietary supplements
Oral K preparations
Intravenous infusion
• Treatment of the cause
Anti-emetics
Anti-diarrheals
Diuretic withdrawal
MANAGEMENT ISSUES
Management of hypokalemia includes potassium replacement and correction
of the underlying cause (Table 36.3). Potassium can be replaced through dietary
supplementation of potassium-rich foods. Oral proprietary supplements of
potassium citrate can also be prescribed. If potassium deficiency is severe or if
the patient is vomiting, potassium chloride is administered as an intravenous
infusion. Generally, potassium deficiency is more severe, if there is true loss of
body fluids than if there is only a transcellular shift of potassium.
C A S E
37
Features of
Hyperkalemia
. CASE PRESENTATION
A 64-year old woman was wheeled into the emergency room, with generalized
weakness and shortness of breath of one week duration. She also complained of
swelling around the eyes and over the feet, loss of appetite and occasional vomiting.
The lady was a known case of diabetes mellitus since 25 years and systemic
hypertension for the last 12 years. She sustained an anterior wall myocardial
infarction four years back, for which she was thrombolysed. At that time, coronary
angiography showed triple-vessel disease, but she declined a revascularization
procedure. Her serum creatinine value was found to be high and therefore, she
was switched over from oral antidiabetic drugs to insulin therapy. The patient also
underwent laser photocoagulation for proliferative retinopathy, one year back. She
was presently undergoing maintenance hemodialysis, thrice a week.
On examination, the patient was drowsy, disoriented and obviously dyspneic.
The complexion was pale and sallow with dry skin that bore marks of pruritus.
There was periorbital puffiness and pitting edema over the ankles and lower legs.
The neck veins were engorged but there was no cyanosis or icterus. The pulse rate
was 92 beats/min. with a BP of 160/94 mm Hg. The precordium was unremarkable
and the apex beat was displaced to the left. The S1 was normal with a loud A2 and
a S3 sound in early diastole. An ejection murmur was audible along the left sternal
border. No pericardial friction rub was audible. There were bilateral basilar rales over
the lung fields. An ECG was obtained (Fig. 37.1) following which she was immediately
given an injection. Her laboratory reports were Hemoglobin 9.2 g/dL% Urine sugar
+1 albumin +2, Glucose 144 mg/dl, Urea 124 mg/dl, Creatinine 5.2 mg/dl, Sodium
129 mEq/L, Potassium 6.8 mEq/L and Calcium 7.4 mg%.
Figure 37.1: ECG showing tall T waves with flat P waves

ECG INTERPRETATION
The ECG showed normal sinus rhythm. The P wave was flattened and the P-R
interval was prolonged. There were no significant Q waves and the S-T segment
was isoelectric. The T wave was upright, tall and peaked. The Q-T interval was
short. These findings are consistent with the diagnosis of hyperkalemia. A
T-wave that exceeds a voltage of 5 mm in the standard leads and 10 mm in the
precordial leads is considered tall. Besides hyperkalemia, causes of tall T wave
are myocardial ischemia and the hyperacute phase of myocardial infarction. The
T wave of hyperkalemia is tall, peaked symmetrical and has a narrow base, the so
called ‘tented’ T wave. The Q-T interval is short. On the other hand, the T wave of
coronary insufficiency is tall but broad-based and the Q-T interval is prolonged.
The normal Q-T interval is 0.39 + 0.04 sec. and ranges from 0.35 to
0.43 sec. A Q-T interval measuring less than 0.35 sec is considered short. Besides
hyperkalemia, causes of short Q-T interval are hypercalcemia and digitalis toxicity.
Hyperkalemia shortens the Q-T interval and is associated with tall T waves, wide
QRS complexes and diminished P waves. Hypercalcemia also shortens the Q-T
interval but there are no changes in the morphology of the QRS deflection. The
proximal limb of the T-wave has an abrupt upslope to its peak.
Figure 37.2: ECG features of progressively increasing hyperkalemia
The ECG features of hyperkalemia depend upon its severity (Fig. 37.2). When
the serum level exceeds 6.8 mEq/L, tall T waves and short Q-T interval are seen.
When it exceeds 8.4 mEq/L, additionally the P wave gets flattened and the P-R
interval gets prolonged. At a serum level which is in excess of 9.1 mEq/L, the QRS
complex also becomes wide and ventricular arrhythmias occur (Table 37.1).
Table 37.1: ECG features of progressive hyperkalemia

A. Serum K>6.8 mEq/L tall T waves;
short Q-T interval
B. Serum K>8.4 mEq/L (A) plus flat P waves;
prolonged P-R interval
C. Serum K>9.1 mEq/L (B) plus wide QRS complex;
A-V block and arrhythmias
Case 37 Features of Hyperkalemia 171
CLINICAL DISCUSSION
The electrocardiogram often shows changes due to an electrolyte abnormality.
These changes are observed even before the biochemical reports from the
laboratory are available. Variation in the blood levels of potassium and calcium
are chiefly responsible for these ECG changes. Abnormal levels of calcium and
magnesium produce similar changes. Hypercalcemia shortens the Q-T interval
while hypocalcemia prolongs it.
Table 37.2: Causes of hyperkalemia

• Potassium gain
Hemolysis, tumour lysis
Rhabdomyolysis, burns
• Redistribution
Metabolic acidosis
Hyperglycemia
• Hypoaldosteronism
Addison’s disease
Acute renal failure
• Drug induced
Potassium sparing diuretics
Non-steroidal anti-inflammatory drugs
Anti-angiotensin drugs (ACEi & ARBs)
• Genetic Causes
Hyperkalemic periodic paralysis
Renal tubular acidosis Type 4.
There are several causes of hyperkalemia which have been enlisted in

Table 37.2. True gain of potassium is often due to hemolysis, rhabdomyolysis,
burns or tumour lysis. Redistribution of potassium occurs in metabolic acidosis
(extracellular shift), with beta-blocker therapy and in severe insulin deficiency.
Hyperkalemia is a feature of hypoaldosteronism due to Addison’s disease or
acute on chronic renal failure. Drugs known to cause hyperkalemia include
potassium-sparing diuretics, non steroidal anti-inflammatory drugs (NSAIDs),
and the angiotensin converting enzyme (ACE) inhibitors. Genetic causes of
hyperkalemia are Type 4 renal tubular acidosis (RTA-4) and hyperkalemic
periodic paralysis.
The clinical picture of hyperkalemia depends upon the cause. The most
common scenario is of acute on chronic renal failure with fluid overload,
hypertension and metabolic acidosis, which are characteristic features of uremia.
Sometimes, hyperkalemia is a part of diabetic ketoacidosis. At other times, burns
and crush-injuries with rhabdomyolysis are associated with hyperkalemia.
Severe hyperkalemia can cause serious ventricular arrhythmias.
Table 37.3: Management of hyperkalemia

• Calcium gluconate injection
• Glucose-insulin infusion
• Nebulized salbutamol
• Oral polystyrene resin
• Hemodialysis
MANAGEMENT ISSUES
Since hyperkalemia can cause serious ventricular arrhythmias, the first priority
is to protect the heart. Calcium gluconate has membrane stabilizing properties
and is administered intravenously as 10 ml of 10% injection over 10 minutes. The
next step is to drive potassium into the cells using 100 ml of 25% glucose with
10 units of insulin given by an infusion. Nebulized salbutamol increases the
urinary excretion of potassium by increasing the Na-K-ATPase pump activity.
Excess potassium in the body can be depleted by potassium-binding resin like
polystyrene sulphonate administered orally. Finally, if severe hyperkalemia is
associated with metabolic acidosis and fluid overload, hemodialysis is the answer
(Table 37.3).
SECTION
12
Electrocardiac
Syndromes
C A S E
38
Prolonged Q-T
Syndrome
. CASE PRESENTATION
A 62-year old woman visited her cardiologist along with her daughter, because of two
episodes of syncope in the preceding week. The fainting episodes were preceded by
palpitation and associated with blurring of vision. The patient was a known case of
coronary heart disease and she had sustained an anterior wall myocardial infarction
ten months back. At that time, she did not receive thrombolytic therapy because of
late presentation to the hospital and she had also declined coronary angiography.
The patient also felt breathless on exertion and her echocardiogram showed a
large anterior wall motion abnormality with an ejection fraction of 30+5%. About
a month back, she underwent 24-hour Holter monitoring which showed multifocal
ventricular premature beats, with short runs of ventricular tachycardia. Therefore,
she was prescribed amiodarone, in addition to her usual medication which included
ramipril, frusemide, aspirin, atorvastatin and isosorbide mononitrate. An initial
loading dose of 1200 mg per day of amiodarone, was tapered to a maintenance dose
of 400 mg daily after one week.
On examination, the patient was apprehensive and tachypneic. The pulse was
fast and low in volume with a few “missed beats”. The heart rate was 104 beats/min.
with a BP of 110/66 mm Hg. The cardiac apical impulse was diffuse and displaced
towards the axilla. The S1 and S2 were normal with an S3 gallop sound in diastole. No
murmur or friction rub was audible. There were bilateral basilar rales over the lung
fields. An ECG was obtained (Fig. 38.1).
Figure 38.1: ECG showing broad T waves with prolonged Q-T interval
ECG INTERPRETATION
The ECG showed normal regular sinus rhythm. The P waves were normal in
morphology and large Q waves were seen in the anterior precordial leads. The
176 Section 12 Electrocardiac Syndromes
T wave was upright, tall and broad, which occupied most of the R-R interval. The
measured Q-T interval was 0.60 sec. This finding is consistent with the diagnosis
of long Q-T syndrome.
The duration of the QRS complex represents ventricular depolarization time
and the width of the T wave represents ventricular repolarization time. Therefore,
the Q-T interval is a measure of the total duration of ventricular electrical systole.
The Q-T interval is measured on the horizontal axis, from the onset of the Q
wave to the termination of the T wave (not the U wave). The duration of the QRS
complex, the length of the S-T segment and the width of the T wave are included
in the measurement of the Q-T interval (Fig. 38.2).
Figure 38.2 : Measurement of the Q-T interval
The upper limit of normal Q-T interval depends upon several variables,
including the age, gender and the autonomic tone. It tends to be shorter in young
individuals (0.44 sec) and longer in the elderly (0.45 sec). It is slightly shorter in
males than in females, the upper limit being 0.43 sec in men. The Q-T interval
shortens at fast heart rates and lengthens at slow heart rates. Since the Q-T interval
varies with tachycardia and bradycardia, the measured Q-T interval needs to be
corrected for heart rate.
The corrected Q-T interval is known as the Q-Tc interval. For heart rate
correction, the Bazett’s formula is generally used, where Q-Tc interval is equal to
the measured Q-T interval divided by the square-root of the R-R interval. When
the heart rate is 60 beats/min. and the R-R interval is 1 sec (25 x 0.04sec), the Q-Tc
interval and the Q-T interval are the same. As a general rule, a Q-T interval that
exceeds half of the R-R interval, is taken as a prolonged Q-T interval.
CLINICAL DISCUSSION
There are several causes of Q-T interval prolongation. These have been enlisted
in Table 38.1. Congenital long Q-T syndromes may present dramatically
with syncope. Indeed, congenital long Q-T syndromes are characterized by
prolongation of the Q-T interval, syncope, ‘seizures’ and sudden death due to
ventricular arrhythmias (Torsade de pointes), in apparently healthy children
and young adults. Some cases of sudden infant deaths have been attributed to
congenital long Q-T syndrome. Therefore, an ECG should be performed in all
Case 38 Prolonged Q-T Syndrome 177
Table 38.1: Causes of prolonged Q-T interval

Congenital causes
• Jervell-Lange-Neilsen syndrome
(autosomal recessive with deafness)
• Romano-Ward syndrome
(autosomal dominant without deafness)
Acquired causes
• Electrolyte deficiency e.g. calcium, potassium
• Antiarrhythmic drugs e.g. quinidine, amiodarone
• Coronary artery disease e.g. myocardial infarction
• Myocarditis e.g. viral myocarditis, rheumatic fever
• Intracranial event e.g. head injury, brain hemorrhage
• Bradyarrhythmias e.g. A-V block, sinus bradycardia
• Drug induced e.g. terfenadine, cisapride, olanzapine
infants on anticonvulsants for seizure prophylaxis. The prognosis and triggers of

sudden cardiac death in patients with congenital long Q-T syndrome, are related
to the Q-T interval and the genotype.
Figure 38.3 : ECG showing pseudo-prolongation

of the Q-T interval
In hypokalemia, the T wave is flattened and the prominent U wave may

be mistaken for the T wave. This may falsely suggest prolongation of the Q-T
interval, whereas it is actually the Q-U interval. Hypokalemia therefore, cause
pseudo-prolongation of the Q-T interval (Fig. 38.3). Antiarrhythmic drugs such
as quinidine, procainamide and amiodarone can prolong the Q-T interval. They
also cause widening of the QRS complex which, if it exceeds 25% of baseline, is
an indication for withdrawing the culprit drug. Since Q-T interval prolongation
predisposes to arrhythmias, this is the mechanism to explain the arrhythmia
enhancing property or proarrhythmic effect of antiarrhythmic drugs. Besides
antiarrhythmic agents, certain non-cardiovascular drugs can also prolong the
Q-T interval. These drugs are listed in Table 38.2.
The clinical importance of Q-T interval prolongation lies in the fact that
it predisposes to a typical type of polymorphic ventricular tachycardia.This
tachycardia is known as “Torsade de pointes”, a ballet term which literally means
“torsion around a point”. This term explains the morphology of the ventricular
tachycardia, which consists of polymorphic QRS complexes that keep changing
Table 38.2: Drugs causing Q-T interval prolongation

Antiarrhythmics Anti-infectives
Quinidine Erythromycin
Procainamide Gatifloxacin
Amiodarone Ketoconazole
Psychiatry drugs Miscellaneous
Imipramine Cisapride
Haloperidol Terfenadine
Amitryptyline Ketanserin
in amplitude and direction (Fig. 38.4). The polymorphic QRS complexes give the
appearance of periodic torsion or twisting around the isoelectric line.
Long Q-T syndrome (LQTS) belongs to a class of congenital channelopathies,
that are responsible for about 5 to 10% cases of sudden cardiac death (SCD). Other
conditions belonging to this class are the Brugada syndrome and catechola
minergic ventricular tachycardia (CVT). Besides these above channelopathies,
structural heart diseases responsible for SCD are hypertrophic cardiomyopathy
and arrhythmogenic right ventricular dysplasia.
Figure 38.4: Polymorphic ventricular tachycardia

( ‘Torsades de pointes’)
MANAGEMENT ISSUES
The treatment of long Q-T syndrome depends upon the cause. When the cause
is reversible, it suffices to correct the electrolyte abnormality or to withdraw the
offending drug. The Q-T interval should be carefully assessed at peak plasma
concentration, if multiple drugs with Q-T prolonging effect are used. In Q-T
interval prolongation due to a cardiovascular or intracranial event, the underlying
condition has to be managed. Since patients with congenital long Q-T syndrome
are prone to syncope and sudden death due to ventricular arrhythmias, an
implantable cardioverter defibrillator (ICD) is advocated.
The management of polymorphic ventricular tachycardia (“Torsades de
pointes”) due to long Q-T interval, is different from the management of mono
morphic ventricular tachycardia. In the acute setting, an infusion of magnesium
sulphate or isoproterenol (a beta-blocker) is used. If the patient does not
respond, overdrive ventricular pacing or electrical cardioversion is performed.
An implantable cardioverter defibrillator (ICD) is offered to those who develop
recurrent syncope, have family history of sudden cardiac death (SCD) and to
survivors of cardiac arrest. To patients of congenital long QT syndrome (LQTS),
cervical sympathetic ganglionectomy can be offered.
C A S E
39
Sick Sinus
Syndrome
. CASE PRESENTATION
A 78-year old elderly gentleman was paid a domiciliary visit by his physician, to
evaluate frequent spells of dizziness over the past 2 weeks. The patient felt light-
headed when he stood up from the sitting or lying down position. Fortunately, he
had never fallen down or fainted because he used a walking aid and was regularly
looked after by a personal attendant. There was no history of breathlessness, chest
pain or palpitation. The patient was hypertensive for over 40 years and took his
medicines regularly. There was no past history of myocardial infarction or paralytic
stroke. The only time he was hospitalized was for prostate surgery five years back.
The patient’s family members had also noticed recent mental confusion and lapses
in his memory.
On examination, the patient was conscious and cooperative but somewhat
confused. The pulse was irregular, good in volume, at a rate of 48 to 52 beats/min.
The dorsalis pedis pulsations were diminished on both sides and a bruit was audible
over the left carotid artery. The BP was 160/82 mm Hg in the supine position and
150/74 mm Hg while standing. The JVP was not raised and there was no pitting
edema over the ankles. The S1 was normal and A2 was loud; no S3 or S4 sound was
audible. A short harsh systolic murmur was heard over the upper parasternal area. A
different blowing pansystolic murmur was heard over the cardiac apex, that radiated
towards the axilla. Breath sounds were vesicular, without any crepitations. An ECG
was obtained (Fig. 39.1). At the time of prostate surgery, an ECHO had shown mild
concentric left ventricular hypertrophy with an ejection fraction of 52% and no
regional wall motion abnormality. CT scan of the head at the same time showed
periventricular lacunar infarcts and changes of diffuse cerebral atrophy.
Figure 39.1: ECG showing sinus bradycardia, asystole and junctional escape
ECG INTERPRETATION
The ECG showed sinus bradycardia at a rate of 50 beats/min., with short periods
of asystole. At times the R-R interval was twice the usual R-R interval, suggesting
2:1 sino-atrial exit block (S-A block). At other times, the period of asystole
was followed by a delayed beat without a preceding P wave, consistent with a
junctional escape beat. This constellation of ECG findings which includes sinus
bradycardia, S-A block and junctional escape, is consistent with the diagnosis of
sinus node dysfunction, the so called sick sinus syndrome (Table 39.1).
Table 39.1: ECG findings in sick sinus syndrome

• Sinus bradycardia
• Sino-atrial exit block
• Slow atrial fibrillation
• Junctional escape rhythm
The sick sinus syndrome usually presents with bradyarrhythmias such as

those mentioned above. At times the indications of sinus node dysfunction are
an inadequate tachycardia with sympathomimetic drugs, excessive sensitivity
to beta-blocker drugs and atropine resistant bradycardia. At other times, there
may be atrial fibrillation with slow ventricular response or a junctional rhythm.
The coexistence of fast and slow cardiac rhythms constitutes the so called
“tachy-brady” syndrome.
Figure 39.2: ECG showing 2:1 atrio-ventricular (A-V) block
In a pause due to 2:1 sino-atrial (S-A) exit block, the entire P-QRS-T complex
is missing, since neither atrial nor ventricular activation occurs. By contrast
in 2:1 atrio-ventricular (A-V) block, the P wave is inscribed normally but not
followed by a QRS complex (Fig. 39.2). S-A block may coexist with A-V block and
even left bundle branch block, if fibrocalcerous degeneration involves the entire
conduction system. Quite often, sinus node dysfunction is suspected clinically
but difficult to prove because the ECG is normal and Holter monitoring does
not show up the arrhythmia, during the period of observation. A prolonged
sinus node recovery time (SNRT) and sino-atrial conduction time (SACT) on
electrophysiological studies (EPS) is then taken as a diagnostic criteria for sick
sinus syndrome (SSS).
Case 39 Sick Sinus Syndrome 181
CLINICAL DISCUSSION
The “sick sinus syndrome” (SSS) is a clinical condition caused by a diseased
sinus node, which fails to produce or successfully conduct a sufficient number of
cardiac impulses. It is observed in elderly patients and is believed to be caused by
a degenerative condition like amyloidosis or infiltration of the atrium by a fibro
calcerous process. Certain cardiovascular drugs notably beta-blockers (atenolol,
metoprolol), calcium-blockers (verapamil, diltiazem) and digoxin may also cause
sinus node dysfunction, which is reversible after discontinuation of therapy.
Table 39.2: Symptoms of sick sinus syndrome

• Dizziness and syncope
• Dyspnea and fatigue
• Palpitation and angina
• Confusion and dementia
The most frequent symptoms of sick sinus syndrome are dizziness, mental
confusion and fainting attacks (Table 39.2). Spells of dizziness and syncope in sick
sinus syndrome are due to transient ventricular asystole, causing a precipitous
decline in stroke volume and cerebral perfusion. Such episodes are known as
Stokes-Adams attacks. Besides SSS, other causes of Stokes-Adam’s attacks are
advanced atrio-ventricular block, malignant ventricular arrhythmias, carotid
sinus hypersensitivity and subclavian steal syndrome (Table 39.3).
Table 39.3: Causes of Stokes Adams Attacks

• Advanced atrio-ventricular block
• Serious ventricular arrhythmias
• Carotid sinus hypersensitivity
• Subclavian steal syndrome
• Sick sinus syndrome
Since patients are in the advanced age group, many of them have had
a cerebrovascular accident or a prior myocardial infarction. They may also
complain of dyspnea and fatigue due to heart failure. Palpitation and angina
pectoris may occur due to tachyarrhythmias or associated coronary artery disease.
Dizziness and syncope in an elderly patient may be multifactorial. Even those
with documented sick sinus syndrome may additionally have volume depletion,
electrolyte imbalance or hypoglycemia. They may have orthostatic hypotension
due to autonomic failure or vertebro-basilar insufficiency. Still others may have
cardiac outflow obstruction due to aortic sclerosis or ventricular arrhythmias due
to an old myocardial scar or heart failure. Therefore, a detailed evaluation of these
patients is warranted.
MANAGEMENT ISSUES
Drugs that can increase the rate of discharge from the sinus node such as
sympathomimetic (adrenaline) and vagolytic (atropine) drugs, can only tempo
rarily increase the heart rate. Permanent pacemaker implantation (PPI) is
the definitive form of treatment, particularly if the symptoms are severe and
disabling. Dual-chamber pacemakers are more physiological and carry a lower
risk of atrial fibrillation and stroke. They also have a distinct advantage over
single-chamber pacing if there is coexistent A-V nodal disease or bundle branch
block. Pacemaker implantation makes it possible to use antiarrhythmic drugs to
treat tachyarrhythmias, which otherwise would have caused severe bradycardia.
C A S E
40
Early Repolarization
Syndrome
. CASE PRESENTATION
A 29-year old well-built man of African origin, sought an appointment with the
cardiologist, for opinion on an abnormal ECG. The ECG was performed as part
of routine pre-employment medical evaluation. The man vehemently denied
complaints of fatigue, breathlessness, chest pain, palpitation or syncope. He had
been actively involved in competitive sports during his college days and still played
tennis on week-ends. The man did not smoke or take alcohol, but was fond of calorie-
dense food. He did not suffer from diabetes or hypertension and had never got a
serum lipid analysis done. There was no family history of coronary heart disease or
of cerebrovascular accident.
On examination, the man was of stocky built, with a muscular physique. His body
mass index (BMI) was 28 kg/m2. He was fully conscious, comfortable and cheerful.
There was no anemia, cyanosis, icterus or edema. The trachea was central, thyroid
gland was not palpable and the JVP was not raised. The pulse rate was 58-62 beats/
min. with a normal pulse volume and no special character. The BP was 120/80 mm
Hg over the right arm. The precordium was unremarkable, with a normally located
apex beat. The S1 and S2 were normal without any gallop sound. No murmur or
pericardial friction rub was audible. The breath sounds were vesicular without any
audible rhonchi or crepitations. A fresh ECG was performed in the cardiologist’s office
(Fig. 40.1).
Figure 40.1: ECG showing features of early repolarization syndrome

ECG INTERPRETATION
The ECG showed tall R waves in the lateral precordial leads, preceded by narrow
Q waves. The S-T segment was elevated concave upwards, with an initial slur on
the S-T segment (J wave). The T waves were upright, tall and symmetrical in the
lateral leads, with prominent U waves in the mid-precordial leads. These findings
are consistent with the diagnosis of early repolarization syndrome (Table 40.1).
Since early repolarization is frequently observed in healthy athletic persons, this
entity is also known as the “athlete’s heart”.
There are several causes of S-T segment elevation (Table 40.2), of which acute
myocardial infarction is the leading cause. The S-T segment elevation of early
repolarization, can simulate the injury pattern of acute myocardial infarction.
However, there are several classical differentiating features:
• S-T segment elevation is concave upwards in lead V6
• Ratio of S-T elevation : T wave height is below 0.25
• There is no reciprocal S-T depression in other leads
• ECG changes do not evolve as in case of infarction
• ECHO does not show abnormal regional wall motion
• Serial level of cardiac enzyme titers are not increased.
Table 40.1: ECG features of early repolarization syndrome

• Tall R wave in lead V6
• Narrow and deep Q wave
• Concave S-T segment elevation
• Initial J wave on the S-T segment
• Upright and tall symmetrical T wave
CLINICAL DISCUSSION
The “early repolarization” variant is an alarming electrocardiographic entity,
which presents with S-T segment elevation. It represents early repolarization of
a portion of the ventricle, before the entire myocardium has been depolarized.
There is an early uptake of the S-T segment, before the descending limb of the
R wave has reached the baseline. This causes an initial slur on the S-T segment,
known as the J wave. The S-T segment is elevated and concave upwards. There is
an associated increased amplitude of the R wave. The T wave is also tall, but the
ratio of S-T segment elevation to T wave height is less than 0.25. Interestingly,
the degree of S-T elevation and T wave height may vary on a day-to-day basis
and the S-T segment may normalize after exercise. Besides the features already
mentioned, other characteristics of this syndrome are sinus bradycardia with
sinus arrhythmia, voltage criteria of left ventricular hypertrophy and persistent
juvenile pattern of T wave inversion in leads V1 to V3.
Case 40 Early Repolarization Syndrome 185
Table 40.2: Causes of S-T segment elevation

Coronary artery disease
• Prinzmetal’s angina
• Dressler’s syndrome
• Ventricular aneurysm
Non-coronary disease
• Acute pericarditis
• Pulmonary embolism
• Early repolarization
• Brugada syndrome
Early repolarization is more frequently observed among young athletic males

of Africo-Carribean descent. They are healthy subjects who are free of symptoms
and their clinical examination is entirely normal. Acute viral pericarditis also
presents with concave-upwards S-T segment elevation but sinus tachycardia
is almost invariably present. Moreover, patients of acute pericarditis have a
preceding flu-like illness, they present with chest pain and there is an audible
pericardial rub.
MANAGEMENT ISSUES
Individuals who have features of early reploarization on the ECG, are healthy
asymptomatic subjects without any objective evidence of organic heart disease.
Therefore, no specific treatment apart from reassurance is advocated. However,
lack of awareness about this entity may lead to unnecessary investigations
including stress-testing, myocardial perfusion imaging and coronary angiography.
C A S E
41
Brugada
Syndrome
. CASE PRESENTATION
A 28-year old man was asked to see a cardiologist by a general physician, for expert
opinion on an abnormal ECG. The ECG had been performed by the physician
empanelled with an insurance company, as part of the pre-insurance medical
check-up. The man clearly denied any history of chest pain, dyspnea, palpitation or
syncope. He was physically very active and undertook a brisk walk for 40 minutes, on
most days of the week. He had also been a regular member of his school and college
cricket teams. The man smoked about 5 cigarettes a day and consumed about 1 litre
of beer on week-ends. His blood pressure and blood sugar levels were normal but
his lipid profile showed an elevated LDL value. One of his cousin brothers had died
of cardiac arrest, at the age of 32 years.
On examination, the man was well built and had a healthy appearance with a
body mass index (BMI) of 26 kg/m2. He was conscious, comfortable and cooperative
during examination. There was no anemia, cyanosis, jaundice or ankle edema. The
pulse rate was 72 beats/min.; pulse was fair in volume and had no special character.
The BP in the right arm was 120/80 mm Hg. The precordium was unremarkable and
the cardiac apex beat was normally located. The S1 and S2 were normal and no S3 or
S4 sound was heard. No murmur or pericardial friction rub was audible. The breath
sounds were normal and no rhonchi or crepts were heard over the lung fields. A
fresh ECG was obtained in the office of the cardiologist (Fig. 41.1).
ECG INTERPRETATION
The ECG showed a triphasic rSR’ pattern in lead V1, which was 0.10 second in
duration. Additionally, there was a “tent-like” coved S-T segment elevation of
0.2 mV with large inverted T waves (Table 41.1). These findings are consistent
with the diagnosis of Brugada syndrome. Three types of S-T segment elevation are
described in Brugada syndrome, depending upon the ventricular repolarization
pattern.
• Type 1: “Tent-like” coved S-T segment, elevation >0.2 mV, negative T wave
• Type 2: “Saddle-back like” S-T segment, elevation >0.1 mV, positive T wave
• Type 3: “Saddle-back like” S-T segment, elevation <0.1 mV, positive T wave.
Case 41 Brugada Syndrome 187
Figure 41.1: ECG showing features of the Brugada syndrome.
Table 41.1: ECG Features of Brugada Syndrome

• rSR’ pattern in lead V1
• rSR’ duration < 0.12 sec
• Large and inverted T wave
• Coved S-T segment elevation
Besides Brugada syndrome, other causes of S-T segment elevation in lead V1

are right ventricular infarction, acute pulmonary embolism and arrhythmogenic
right ventricular dysplasia (Table 41.2). The rSR pattern in lead V1 observed in
case of Brugada syndrome, superficially resembles right branch block (RBBB).
But unlike in RBBB, the ventricular complex is not more than 0.12 sec wide and
there are no broad S waves in lead L1 and V6. The characteristic ECG abnormalities
observed in Brugada syndrome may only be transient and not observed
constantly. These may become exaggerated or unmasked after drug challenge
with antiarrhythmic agents such as flecainide and procainamide.
Table 41.2 : Causes of S-T segment elevation in lead V1

• Right ventricular infarction
• Acute pulmonary embolism
• Arrhythmogenic RV dysplasia.
CLINICAL DISCUSSION
The Brugada syndrome is a rare but striking electrocardiographic abnormality. It is
believed to be a genetic disorder of sodium transport, across ion channels located
in the right ventricle. This produces an abnormal pattern of right ventricular
depolarization. Patients who have this abnormality are prone to develop sudden
syncope because of malignant ventricular tachycardia or even cardiac arrest due
to ventricular fibrillation. The genetic defect underlying Brugada syndrome may
exist in more than one family member and form the basis of familial ventricular
arrhythmias. The disorder is transmitted down subsequent generations by
autosomal dominant inheritance.
Brugada syndrome belongs to a class of congenital channelopathies which

are responsible for nearly 5 to 10% cases of sudden cardiac death (SCD). Other
members of this class are long Q-T syndrome (LQTS) and catecholaminergic
ventricular tachycardia (CVT), as given in Table 41.3. Besides these channelo
pathies, congenital structural heart diseases responsible for SCD are hypertrophic
cardiomyopathy and arrhythmogenic right ventricular dysplasia.
Table 41.3: Congenital channelopathies

• Long Q-T syndrome
• C
atecholaminergic VT*
(*ventricular tachycardia)
MANAGEMENT ISSUES
There is no specific treatment of the underlying disorder in Brugada syndrome.
However, insertion of an automatic implantable cardioverter defibrillator (AICD)
may be considered in those patients with history of recurrent syncope, after
cardiac resuscitation from ventricular fibrillation, or if there is history of sudden
cardiac death in a family member.
C A S E
42 WPW Syndrome
. CASE PRESENTATION
A 24-year old unmarried female arrived at the emergency-room, with the complaints
of palpitation and light-headedness for the last 15 minutes. She drove herself to the
hospital and admitted that she felt dizzy while driving. The patient also gave history
of several such episodes in the past. At times, she was able to abort the attack by
splashing cold water on her face, or by applying firm pressure over the eyes. At
other times, she had to rush to the hospital, as on that day. During the “machine-
like” sensation over the chest, she never experienced any chest pain or difficulty in
breathing. However, after the palpitation stopped, she would pass a large volume
of urine. The onset of these episodes was unrelated to physical exercise, emotional
stress or to the intake of any particular food or beverage. There was no history of
tremor, heat-intolerance, undue fatigue or significant weight-loss.
On examination, the patient was apprehensive but not tachypneic. The
extremities were not cold but her palms were sweaty. The pulse rate was extremely
rapid and exceeded 150 beats/min, although it could not be counted accurately.
The BP was 91/64 mm Hg over the right arm and she was apyrexial. There were no
signs of congestive heart failure. There was no goitre, bruit over the thyroid gland or
any clinical sign of Grave’s disease. The precordium was normal and the apex beat
was normally located. There was extreme tachycardia and the character of S1 and
S2 could not be appreciated. The attending doctor performed right carotid sinus
massage which resulted in sudden termination of the patient’s symptoms and a
remarkable change in her heart rate. An ECG was obtained afterwards (Fig. 42.1).
Figure 42.1: ECG showing features of the WPW Syndrome

ECG INTERPRETATION
The ECG showed normal P waves with a P-R interval of 0.08 sec. The width of the
QRS complex was 0.12 sec with a notch on the ascending limb of the R wave. There
was depression of the S-T segment with inversion of the T wave. These findings
are consistent with the diagnosis of WPW syndrome. The Wolff-Parkinson-
White (WPW) Syndrome is a distinct electrocardiographic entity wherein an
accessory pathway, the bundle of Kent, directly connects the atrial myocardium
to the ventricular myocardium, bypassing the A-V node (Fig. 42.2). This produces
abnormalities of the QRS complex, P-R interval, S-T segment and the T wave.
Figure 42.2: Diagram to illustrate the Bundle of Kent
The P-R interval is short because ventricular depolarization through the

accessory pathway, bypasses the normal conduction delay at the A-V node.
The notch on the ascending limb of R wave is the delta wave. It indicates pre-
excitation of the ventricle through the accessory pathway, before depolarization
of the entire ventricle by the normal conduction system. The QRS complex is
wide because it is a fusion beat, which is the sum of ventricular prexcitation and
normal ventricular depolarization. The S-T segment and T wave inversion are
repolarization abnormalities, secondary to the abnormal pattern of ventricular
depolarization (Fig. 42.3).
Figure 42.3: ECG in case of WPW syndrome

Case 42 WPW Syndrome 191
Three types of QRS configuration are described in the WPW syndrome,

depending upon the direction of the accessory pathway.
• Type A (left septal connection) produces upright QRS complexes in all the
precordial leads. It resembles right bundle branch block or true posterior wall
myocardial infarction.
• Type B (right-sided connection) has negative QRS complexes in V1 and
positive complexes in lead V6. It resembles left bundle branch block or left
ventricular hypertrophy.
• Type C (left lateral connection) has positive QRS complexes in lead V1 and
negative complexes in lead V6. It resembles right ventricular hypertrophy.
CLINICAL DISCUSSION
The standardized nomenclature of pre-excitation syndromes uses the term “tract”
for pathways that insert into specialized conduction tissue and “connection”
for pathways that enter the general myocardium. The Wolff-Parkinson-White
(WPW) syndrome involves an atrio-ventricular connection (Kent bundle). Lown-
Ganong-Levine (LGL) syndrome involves an atrio-fascicular bypass tract (James
bundle) (Fig. 42.4). Mahaim fibers constitute a fasciculo-ventricular connection.
Figure 42.4: Diagram to illustrate the various pre-excitation syndromes

A: Atrium; V: Ventricle; A-V: Atrio-ventricular
The WPW syndrome is a masquerader of several other cardiac conditions:

• The delta wave as separate from the R wave can mimic bundle branch block.
• The dominant R wave in lead V1 may resemble right ventricular hypertrophy.
• Negative delta wave with S-T segment depression and T wave inversion, may
appear as myocardial infarction.
• Antidromic A-V re-entrant tachycardia conducted anterogradely through the
accessory pathway may be mistaken for ventricular tachycardia.
The clinical importance of the WPW syndrome lies in the fact that it predisposes
to paroxysmal atrial tachycardia, since the bypass tract forms a re-entrant circuit
with the regular conduction pathway. Paroxysmal atrial tachycardia (PAT) in the
presence of WPW syndrome needs to be differentiated from a PAT without an
accessory pathway, since the management is somewhat different.
MANAGEMENT ISSUES
Presence of the WPW syndrome predisposes an individual to paroxysmal supra
ventricular tachycardia (PSVT), wherein the bypass tract constitutes a re-entrant
circuit along with the normal conduction pathway. In almost 90% patients,
conduction proceeds anterogradely down the A-V node and returns retrogradely
through the accessory pathway to the atrium. This is known as orthodromic
tachycardia and here the QRS complex is narrow. This arrhythmia responds to
vagal manoeuvres and to drugs used in the treatment of A-V nodal re-entrant
tachycardia (AVNRT). These drugs include verapamil, diltiazem, digitalis and
adenosine.
In less than 10% patients, anterograde conduction proceeds down the
accessory pathway and returns retrogradely through the normal A-V nodal
pathway to the atrium. This is known as antidromic tachycardia and here the QRS
complex is wide and demonstrates the WPW syndrome. This arrhythmia does
not respond to vagal manoeuvres, but only to cardioversion with DC shock or to
antiarrhythmic drugs that block the accessory pathway such as amiodarone.
SECTION
13
Cardiac Arrhythmias
C A S E
43
Supraventricular
Tachycardia
. CASE PRESENTATION
A 36-year old woman had been experiencing episodes of “fluttering” sensation in the
chest, since two years. The episodes were described as a “machine-like” feeling over
the precordium, that was not accompanied by chest pain or shortness of breath.
During these episodes she did feel dizzy, but she never lost her consciousness. Once
the episode was over, she would pass urine frequently. Her episodes were unrelated
to physical exertion, food intake or to mental stress. At times she was able to abort
the attack on her own, either by splashing cold water on her face or by applying
firm pressure over her eyes. At other times, she had to visit a cardiologist who either
performed carotid sinus massage or administered intravenous diltiazem. Initially,
episodes would occur only once in a month or two but recently, their frequency
had increased significantly. Therefore, although she was prescribed verapamil
120 mg twice a day which she took regularly, the patient was advised to undergo
electrophysiological studies (EPS).
On examination, the patient was apprehensive but not tachypneic or in any
distress. The heart rate exceeded 150 beats/min., with a BP of 96/70 mm Hg and
she was afebrile. The JVP was not raised and there was no edema over the ankles.
There was no tremor over fingers, thyromegaly or eye prominence to suggest
thyrotoxicosis. The precordium was hyperkinetic in nature, with a normally located
apex beat. On auscultation, S3 or S4 sound could not be appreciated because of
extreme tachycardia, but no murmur or friction rub was audible. Breath sounds were
vesicular without any rhonchi or crepts. A long-strip ECG recording of lead LII was
obtained (Fig. 43.1).
Figure 43.1: ECG showing abrupt onset of narrow QRS tachycardia

196 Section 13 Cardiac Arrhythmias
ECG INTERPRETATION
The ECG showed an abrupt onset of regular tachycardia at a rate exceeding 150
beats/min., with an R-R interval of less than 10mm. The QRS complexes were
narrow and the P or T waves were not visible. These findings are consistent with
the diagnosis of paroxysmal supraventricular tachycardia (PSVT).
Figure 43.2: Diagram to illustrate circus movement

in a closed re-entrant circuit
Supraventricular tachycardia is most often (in 90% cases) based on repeti

tive circus movement of impulses in a closed re-entrant circuit (re-entrant
tachycardia). In 50% cases, the circuit is composed of two pathways within the
atrioventricular node (AV nodal reentrant tachycardia-AVNRT). In 40% cases, the
circuit consists of an AV nodal pathway and an accessory bypass tract along its side
(AV re-entrant tachycardia-AVRT). An atrial impulse first passes anterogradely
down one of the two pathways, the other pathway being in the refractory period.
The impulses then returns retrogradely through the other pathway, which has
by now recovered its conductivity. In this way, repetitive circulation of impulses
occurs, to produce a sustained atrial tachycardia (Fig. 43.2). Least often (in about
10% cases), supraventricular tachycardia is due to rapid discharge of impulses
from an ectopic atrial focus (ectopic atrial tachycardia).
The heart rate in paroxysmal atrial tachycardia is 150 to 200 beats per minute,
if a re-entrant circuit is involved. It tends to be slower in ectopic atrial tachycardia
(120 to 150 beats/min), as the A-V node cannot conduct more than 150 atrial
impulses per minute. The heart rate can exceed a rate of 200 beats/min, if an
accessory bypass tract is involved, as in case of WPW syndrome. This is because
in WPW syndrome, the impulses can bypass the decremental influence of the
A-V node, by travelling down the accessory pathway. An atrial tachycardia arising
Table 43.1: Differences between ectopic and re-entrant atrial tachycardia

Ectopic tachycardia Re-entrant tachycardia
Heart rate 120-150/min More than 150/min
Onset and offset Gradual Sudden
P wave Ectopic, visible Inverted, Rarely visible
A-V block Can coexist Never, 1:1 conduction
Effect of vagal manoeuvre Slowing Termination
Past history Not significant Previous episodes
Organic heart disease May be present Generally absent
Case 43 Supraventricular Tachycardia 197
from an ectopic focus can be differentiated from a tachycardia due to a re-entrant

mechanism, by certain subtle features (Table 43.1).
Most often, a supraventricular tachycardia is characterized by narrow QRS
complexes, due to synchronized ventricular activation through the specialized His
bundle conduction system. Occasionally, the supraventricular impulses find one
Table 43.2: Differences between ventricular tachycardia

and aberrant ventricular conduction
Ventricular tachycardia SVT with aberrancy
Regularity of rhythm Slightly irregular Clock-like regularity
P waves Not seen May be seen
P-QRS relationship Unrelated Related
QRS width > 0.14 sec 0.12-0.14 sec
QRS morphology Bizarre Triphasic
QRS in V1 to V6 rS in V1 to V6 RsR’ in V1; Rs in V6
R-R’ height R > R’ R’ > R
QRS axis Leftward Normal
Capture/fusion beats May be seen Not seen
Hemodynamics Compromised Stable
Organic heart disease Often present Often absent
Response to carotid massage No response Termination
of the two bundle branches refractory to conduction. In that case, the impulses
are conducted only through the other bundle branch, producing a situation of
aberrant ventricular conduction. This needs to be differentiated from a wide QRS
ventricular tachycardia. The differences between ventricular tachycardia and
supraventricular tachycardia with aberrant ventricular conduction are given in
Table 43.2.
CLINICAL DISCUSSION
Paroxysmal re-entrant atrial tachycardia is most often based on a reciprocal
mechanism involving a bypass tract or a dual intranodal pathway. Episodes of
atrial tachycardia are one of the manifestations of pre-excitation (WPW synd
rome). In the absence of WPW syndrome, paroxysmal atrial tachycardia (PAT)
is generally not associated with organic heart disease. If properly managed, PAT
does not alter life-expectancy and carries an excellent prognosis. PAT coexisting
with the WPW syndrome carries a poorer prognosis, because of the risk of
degeneration into ventricular tachycardia. A paroxysm of atrial tachycardia in the
presence of an underlying WPW syndrome is suggested, if it meets one of the
following criteria:
• ECG during sinus rhythm shows short P-R interval and wide QRS complex
• The ventricular rate exceeds 200 beats/minute, indicating the absence
of physiological A-V block
• Inverted P waves are observed indicating retrograde atrial activation.
Symptoms due to atrial tachycardia depend upon the atrial rate, the duration of
the tachycardia and the presence of heart disease. A fast atrial tachycardia causes
palpitation and neck pulsations. Angina pectoris may occur due to increased
myocardial oxygen demand and reduced coronary filling time. Prolonged atrial
tachycardia can cause dizziness or syncope due to decline in cardiac output
(shortened ventricular filling time) and loss of atrial contribution to ventricular
filling. Termination of the tachycardia is often followed by polyuria due to the
release of atrial natriuretic peptide (ANP) by the stretching of atrial myocardium.
MANAGEMENT ISSUES
The first step in the management of supraventricular tachycardia is to attempt
vagal stimulation, to block the atrio-ventricular (AV) node. Vagal manoeuvres
include carotid sinus massage, supraorbital pressure, Valsalva manoeuvre and
splashing ice-cold water on the face (Table 43.3). Before commencing carotid
sinus massage, the carotid artery should be auscultated for a bruit. If a bruit
is present, carotid massage should not be performed on that side, or else an
embolus may dislodge from the plaque. Massage should be done on one side at a
time and not simultaneously on both sides. If vagal manoeuvres fail to abort the
tachycardia, a drug to block the A-V node is administered intravenously. Drugs
that are effective include adenosine, diltiazem and amiodarone. After restoration
of sinus rhythm, oral diltiazem, verapamil, amiodarone or a beta-blocker such as
metoprolol is prescribed to prevent recurrence.
Table 43.3: Management of supraventricular tachycardia

PSVT with nodal re-entry
• Vagal manoeuvres
• Adenosine, diltiazem
PSVT with bypass tract
• Amiodarone therapy
• Radiofrequency ablation
The management of paroxysmal atrial tachycardia in the presence of WPW

syndrome, is somewhat different. Vagal manoeuvres are useful, only if anterograde
conduction proceeds through the A-V node. Digitalis is contraindicated as
it enhances conduction down the accessory pathway and may precipitate
ventricular fibrillation. Diltiazem and metoprolol reduce the tolerance to the
high ventricular rate and can precipitate congestive heart failure. Amiodarone is
the antiarrhythmic agent of choice for the long-term treatment and prevention of
arrhythmias associated with the WPW syndrome (Table 43.3).
The availability of sophisticated electrophysiological studies (EPS) to identify
and locate bypass tracts and the development of latest ablative techniques,
have revolutionalized the management of WPW syndrome. For ablation of the
bypass tract, high-frequency AC current is delivered through a thermister tipped
catheter, which leads to localized heat coagulation. Radiofrequency ablation
(RFA) of the bypass tract can be offered to patients who report recurrent and
frequent symptomatic episodes of PAT which are refractory to drug therapy or
those that cause hemodynamic compromise.
C A S E
44
Atrial
Fibrillation
. CASE PRESENTATION
A 42-year old woman presented with the complaints of palpitation and extreme
fatigue. She denied history of chest pain or dizziness, but she felt tired and breathless
after routine activities. During the preceding 6 months, she had unintentionally
lost 4 to 5 kg of weight, despite having a good appetite. There was no history of
excessive thirst or frequent urination, but she passed 3 to 4 semiformed stools
everyday. The patient felt particularly uncomfortable during the summer months
when her restlessness, fatigue and palpitations increased considerably. There was
no history of prolonged febrile illness with joint pains during her childhood and she
had never received monthly shots of penicillin. The patient was married since 16
years and had two sons who were 13 and 9 years of age. Both of them were born
after normal delivery and were in good health.
On examination, the patient was restless, anxious and tachypneic. Her extremities
were warm and her palms were dry. There was no anemia, cyanosis, jaundice or
ankle edema. The pulse was fast, irregular and of good volume, with a pulse rate
of 90 to 100 beats/min. The heart rate by auscultation was 110 to 120 beats/min,
with a BP of 144/92 mm Hg over the right arm. Her temperature was 99.20F and the
respiratory rate was 24/min. There was tremor over her outstretched hand and the
eye-balls were prominent with lid-retraction. The JVP was not raised and there were
no palpable lymph-nodes, but the thyroid gland was diffusely enlarged. The goiter
was not tender, but a bruit was audible. The precordium was hyperkinetic with a
forceful apical impulse. The S1 was variable in intensity with a loud S2. No murmur
or gallop sound was audible. A long-strip ECG recording of lead LII was recorded
(Fig. 44.1).
Figure 44.1: ECG showing irregular rhythm with fine fibrillatory waves
ECG INTERPRETATION
The ECG showed a fast irregular rhythm, with a variable R-R interval. The QRS
complexes were narrow, but no P waves were visible. Instead, there were fine
fibrillatory waves between the QRS complexes. These findings are consistent with
the diagnosis of atrial fibrillation (AF). Atrial fibrillation is a grossly irregular fast
rhythm produced by functional fractionation of the atria into numerous tissue
islets, in various stages of excitation and recovery. Consequently, atrial activation
is chaotic and ineffectual in causing atrial contraction (Fig. 44.2). Although 400
to 500 fibrillatory impulses reach the A-V node per minute, only 100 to 150 of
them succeed in eliciting a ventricular response, while others are blocked in the
A-V node. The random activation of the ventricles produces a grossly irregular
ventricular rhythm.
Figure 44.2: Diagram to illustrate atrial islets

with chaotic atrial activation
The hallmark of atrial fibrillation is the absence of discrete P waves. Instead,

there are numerous, small, irregular fibrillatory waves (f waves) that are difficult
to identify individually but produce a ragged baseline. In long-standing atrial
fibrillation, these undulations are minimal and produce a nearly flat baseline.
As mentioned, the ventricular rate is grossly irregular and varies from 100 to 150
beats per minute. Atrial fibrillation can be differentiated from atrial flutter by the
absence of P waves and an irregular ventricular rate (Table 44.1). At times, precise
differentiation between the two may be difficult and the rhythm is then known as
“flutter-fibrillation”, “coarse fibrillation” or “impure flutter”.
Table 44.1: Differences between atrial flutter and atrial fibrillation

Atrial flutter Atrial fibrillation
Atrial rate 220-350 beats/min Over 350 beats/min
Ventricular rate Regular. Half to one fourth Variable. No relation
of atrial rate to atrial rate
Atrial activity Flutter (F) waves Fibrillatory (f ) waves
Saw-toothed baseline Ragged baseline
Ventricular activity Constant R-R interval Variable R-R interval
In atrial fibrillation, the ventricular rate generally varies from 100 to 150 beats
per minute. Faster rates are observed in children, patients of thyrotoxicosis and in
the presence of WPW syndrome. Slower rates are observed during drug treatment
Case 44 Atrial Fibrillation 201
with beta-blockers (propranolol, atenolol) or calcium-blockers (verapamil,

diltiazem), as these drugs block the A-V node. Elderly patients with A-V nodal
disease may also manifest slow atrial fibrillation. Regularization of the ventricular
rate in a patient on digitalis for atrial fibrillation, indicates the onset of junctional
tachycardia and is a manifestation of digitalis toxicity.
CLINICAL DISCUSSION
Atrial fibrillation (AF) can be classified into three groups. In paroxysmal AF,
discrete episodes are self-terminating and last less than 48 hours. In persistent AF,
fibrillation continues unabated for atleast 7 days, but can be converted to sinus
rhythm by electrical or chemical cardioversion. In permanent AF, fibrillation
continues indefinitely and conversion to sinus rhythm is not possible. Atrial
fibrillation can occur in virtually any form of heart disease. Common causes of
atrial fibrillation are given in Table 44.2.
Table 44.2: Causes of atrial fibrillation

Persistent atrial fibrillation
• Constrictive pericarditis
• Cardiac trauma/surgery
• Hypertensive heart disease
• Valvular heart disease (MS)
• Coronary artery disease (MI)
• Congenital heart disease (ASD).
Paroxysmal atrial fibrillation
• Thyrotoxicosis
• WPW syndrome
• Sick sinus syndrome
• Lone atrial fibrillation
• Acute alcoholic intoxication
• Pulmonary thrombo-embolism.
The symptoms of AF depend upon the ventricular rate, the nature and severity
of underlying heart disease and the effectiveness of treatment. Palpitation is due
to fast heart rate while syncope is caused by reduced cerebral perfusion. Angina
may occur because of increased myocardial oxygen demand as well as shortened
coronary filling time due to tachycardia. Dyspnea is due to pulmonary congestion,
secondary to loss of atrial contribution to ventricular filling. Sometimes, regional
ischemia in the form of limb gangrene, hemiparesis or blindness may occur
because of systemic embolization from a left atrial thrombus.
Atrial fibrillation can be recognized clinically by several subtle signs. The
pulse is irregularly irregular, with the pulse rate on palpation being less than the
heart rate on auscultation (pulse deficit). The a waves are not observed in the
neck veins. There is a beat-to-beat variability in the pulse pressure as well as the
intensity of first heart sound, because of a variable ventricular diastolic filling
period.
MANAGEMENT ISSUES
The treatment of atrial fibrillation (AF) is governed by the patient’s symptoms
and hemodynamic status. In the majority of patients, long-term rate control
along with oral anticoagulation, is a reasonable therapeutic strategy. Drugs that
prolong the refractory period of the atrio-ventricular (AV) node such as digoxin,
diltiazem and metoprolol are effective for rate control in persistent AF. In patients
of paroxysmal AF with infrequent episodes, a “pill in the pocket” (taken when
symptomatic) strategy is preferable. Drugs used for this purpose are amiodarone,
sotalol, flecainide and propafenone.
Long-standing atrial fibrillation produces stasis of blood in the left atrium and
promotes the development of thrombi in the atrial cavity and the atrial appendage.
Dislodged fragments of these thrombi can enter the systemic circulation as emboli
and settle down in any arterial territory. Anti-coagulants such as caumarin and
warfarin are required for long-term use in chronic atrial fibrillation, to reduce
the likelihood of systemic embolization. This particularly applies to patients with
rheumatic heart disease and to prosthetic valve recipients. Those patients with
a previous history of thrombo-embolism (stroke or TIA) and those who have a
documented atrial thrombus, are also candidates for long-term anticoagulant
therapy.
Table 44.3: Management of atrial fibrillation

• Heart-rate control
• Anticoagulation
• Electrical cardioversion
• Radiofrequency ablation
If the patient’s clinical status is poor and hemodynamics are unstable, electrical
cardioversion with 100 to 200 Joules of energy is the treatment of choice, in an
attempt to restore sinus rhythm. There are two requisites before cardioversion is
attempted. Firstly, the patient should not have received digitalis in the previous
48 hours. Digitalis not only decreases the threshold for defibrillation, but also
predisposes to the risk of life-threatening arrhythmias. Secondly, an oral anti-
coagulant should be initiated before cardioversion and continued for atleast
four weeks. This is because atrial thrombi are more likely to dislodge as emboli,
once sinus rhythm is restored. Cardioversion should not be attempted if there is a
documented left atrial clot.
In patients who are symptomatic but either refractory or intolerant to several
antiarrhythmic agents, the final option is of radiofrequency ablation (RFA).
Advantages of RFA are not only freedom from symptoms but also avoidance of
the toxic effects of antiarrhythmic drugs and the need to monitor anticoagulant
therapy.
C A S E
45
Ventricular
Premature Beats
. CASE PRESENTATION
A 72-year old man arrived at the emergency room with shortness of breath,
palpitation, anorexia and vomiting. He had been repeatedly hospitalized over the
past 6 months, because of congestive heart failure due to ischemic cardiomyopathy.
The patient had sustained myocardial infarction twice and his left ventricular ejection
fraction was 25%. During his previous admission a month back, frequent multifocal
ventricular premature complexes (VPCs) were detected, for which amiodarone
200 mg daily was added to his ongoing therapy. His dose of frusemide was escalated
from 40 mg to 60 mg per day because of worsening heart failure. He was also
receiving digoxin 0.25 mg once a day, 6 days in a week.
On examination, the patient was restless, orthopneic and in respiratory distress.
There was mild pallor and the extremities were cold and clammy. The pulse was
rapid, irregular and low in volume, with a BP of 104/66 mm Hg. The JVP was raised
5 cm above the angle of Louis and there was edema over the ankles and lower legs.
The lower border of the liver was felt 4 cm below the costal margin and ascites was
present. Fine inspiratory crackles were heard over the bases of lung fields bilaterally.
The cardiac apical impulse was diffuse and displaced towards the axilla. On
auscultation, both S3 and S4 were audible, producing a summation gallop rhythm.
A soft pansystolic murmur was also heard at the cardiac apex, that radiated towards
the left axilla and scapula. A 12-lead ECG was obtained, with a long-strip recording
of lead LII (Fig. 45.1)
Figure 45.1: ECG showing multifocal ventricular premature beats

ECG INTERPRETATION
The ECG showed a fast irregular rhythm with frequent premature beats. The
premature beats had a wide and bizarre QRS morphology, were not preceded
by P waves, but were followed by a compensatory pause. The morphology of
the premature beats, as well as the interval between the premature beat and the
preceding sinus beat (coupling interval), were variable. At times, a very premature
beat was superimposed upon the T wave of the preceding sinus beat. These
findings are consistent with the diagnosis of multifocal ventricular premature
complexes (VPCs) exhibiting the R-on-T phenomenon.
VPCs can be qualified on the basis of their pattern of occurrence and their
location in the cardiac cycle. VPCs with an identical morphology and constant
coupling interval are called unifocal VPCs. Those VPCs that have a variable
morphology and changing coupling interval are called multifocal VPCs. That
VPC which occurs late in the diastolic period of the preceding sinus beat (long
coupling interval), just about when the next sinus beat is expected, is called an
end-diastolic VPC. A VPC that is so premature (very short coupling interval)
that it is superimposed upon the T wave of the preceding sinus impulse, is said
to exhibit the R-on-T phenomenon. A VPC during a slow rhythm, that does not
allow any sinus beat to be missed and is not followed by a compensatory pause,
is called an interpolated VPC. VPCs alternating with sinus beats constitute a
bigeminal rhythm (extrasystolic ventricular bigeminy). VPC after every two sinus
beats represents trigeminy and a VPC after every third sinus beat constitutes a
quadrigeminy. The degree of ventricular ectopy has been categorized as per the
Lown’s classification given in Table 45.1.
Table 45.1: Lown’s classification of ventricular ectopy

Category Degree of ectopy
Class 0 No ectopy
Class 1 Less than 30/hour
Class 2 More than 30/hour
Class 3 Multiform VPCs
Class 4A Couplets
Class 4B Runs of 3 or more
Class 5 R-on-T phenomenon
CLINICAL DISCUSSION
Ventricular premature complexes (VPCs) can occur even in normal individuals,
although they are more often due to organic heart disease. Causes of VPCs in
normal persons are:
• Drugs e.g. beta-agonists, theophylline
• Emotional stress and physical exercise
• Smoking and high intake of tea/coffee
• Anxiety neurosis and thyrotoxicosis.
Case 45 Ventricular Premature Beats 205
Cardiac conditions in which VPCs are observed are:

• Coronary Artery Disease
Ischemia
Infarction
Reperfusion
• Congestive heart failure
Hypertension
Cardiomyopathy
Ventricular aneurysm
• Mitral Valve Prolapse Syndrome
• Digitalis Treatment and Intoxication
• Cardiac Surgery and Catheterization.
Figure 45.2: ECG showing a premature beat that exhibits

the R-on-T phenomenon
A VPC that occurs very prematurely (very short coupling interval) super
imposes on the T wave of the preceding sinus beat and is said to exhibit the
‘R-on-T’ phenomenon (Fig. 45.2). This represents the occurrence of ventricular
stimulation, during the vulnerable phase or the period of supernormal excitability
and is likely to precipitate ventricular fibrillation. The ‘R-on-T’ phenomenon is
observed in these situations:
• VPCs after acute myocardial infarction
• Very premature stimuli during external pacing
• Electrical cardioversion during digitalis therapy
• VPCs with underlying Q-T interval prolongation.
VPCs may be asymptomatic or associated with palpitation and a sensation of
“missed beats’. Awareness of VPCs is due to the post-VPC compensatory pause
and increased force of contraction of the beat following the VPC. Neck pulsations
may be felt due to atrial systole occurring with a closed tricuspid valve, since the
atria and ventricles are activated almost synchronously.
MANAGEMENT ISSUES
The management of ventricular premature beats is governed by the severity of
ectopy, symptoms of the patient and the presence of structural heart disease. Few
isolated beats without symptoms and in the absence of heart disease are mostly
left alone. If symptoms are present, causative factors need to be corrected. This
includes alleviating anxiety, withdrawal of adrenergic drugs and reduction of
smoking and beverage intake. If these measures do not suffice, beta-blockers are
the drugs of choice to treat VPCs associated with anxiety, mitral valve prolapse
and thyrotoxicosis.
VPCs occurring within 24 hours of myocardial infarction indicate reperfusion
and carry a better prognosis than those that appear after 24 hours. Lidocaine and
amiodarone are the drugs of choice to treat VPCs after myocardial infarction,
heart surgery or after cardiac catheterization. Patients who have structural heart
disease in the form of left ventricular hypertrophy (LVH), dilated cardiomyopathy
(DCMP) or arrhythmogenic right ventricular dysplasia (ARVD), almost always
deserve an antiarrhythmic drug such as amiodarone. When a patient of
congestive heart failure on digitalis develops significant VPCs, it has to be decided
on clinical grounds whether digitalis should be continued to manage the heart
failure or withdrawn in view of drug intoxication. If digitalis is to be withdrawn,
the antiarrhythmic drug of choice for digitalis induced VPCs is phenytoin
sodium which should be used in conjunction with standard therapy of digitalis
intoxication.
Antiarrhythmic drugs may be prescribed to control VPCs but before initiating
treatment, the following issues need to be addressed:
• Aggravation of bradycardia and left ventricular dysfunction
• Proarrhythmic effects and likelihood of other tachyarrhythmias
• Potential systemic side-effects of long-term antiarrhythmic therapy
• Inappropriate drug usage, since the cause-and-effect relationship
between VPCs and fatal arrhythmias has not been established.
C A S E
46
Ventricular
Tachycardia
. CASE PRESENTATION
A 66-year old woman was brought to the emergency department with history
of recurrent syncope preceded by palpitation and dizziness. She had sustained
an anterior wall myocardial infarction 8 months back and also complained of
exertional breathlessness with paroxysms of nocturnal dyspnea. She did not receive
thrombolytic therapy and had not undergone primary angioplasty because she
presented to the hospital, over 24 hours after the onset of chest pain. The patient
was practically home-bound with limited physical activity and did not complain of
exertional angina. An echocardiogram was performed a month after her myocardial
infarction, which revealed a large wall motion abnormality involving the mid and
distal septum, ventricular apex and the distal lateral wall. The left ventricular ejection
fraction was 25%.
On examination, the patient was fully conscious and cooperative but tachypneic.
The pulse was fast and irregular, low in volume, at a rate of 96 beats/min. with
“missed beats”. The BP was 110/66 mm Hg with a respiratory rate of 28/min. The JVP
was not raised and there was no pitting edema over the ankles. The cardiac apical
impulse was diffuse and displaced towards the axilla. Besides S1 and S2, an S3 gallop
sound was audible in early diastole. A soft holosystolic murmur was also heard at the
cardiac apex. Bilateral basilar rales were audible over the lung fields. Along with a
12-lead ECG, a rhythm strip of lead LII was obtained, which showed an alarming but
transient abnormality (Fig. 46.1).
Figure 46.1: ECG strip showing monomorphic ventricular tachycardia

ECG INTERPRETATION
The ECG rhythm strip showed an abrupt onset of irregular tachycardia, at a rate
of about 200 beats/min., with R-R interval of 7 to 8 mm. The QRS complexes were
bizarre and wide, exceeding 0.14 sec in width, but did not conform to a bundle
branch block pattern. The P and T waves were not discernable. These findings are
consistent with the diagnosis of ventricular tachycardia. Ventricular tachycardia
is due to enhanced automaticity of a latent ventricular pacemaker, that fires
impulses rapidly. Alternatively, it is based on a closed re-entrant circuit around a
fixed anatomical substrate in the ventricular myocardium (Fig. 46.2). Ventricular
tachycardia may be sustained (lasting >30 sec) or non-sustained (lasting
<30 sec). It is classified as monomorphic (similar QRS complexes) or polymorphic
(variable QRS complexes) in nature.
Figure 46.2: Diagram to illustrate ventricular re-entrant circuit
Polymorphic ventricular tachycardia is characterized by phasic variation

of the QRS direction. A series of ventricular complexes are first up-pointing
then down-pointing and this phenomenon occurs in a repetitive continuum
(Fig. 46.3). Since this gives the appearance of rotation around the isoelectric line,
it is called “Torsades de pointes”, a ballet term which literally means “twisting
around a point”. Torsades de pointes is generally, associated with prolongation
of the Q-T interval. The prolonged Q-T interval favours the occurrence of a
ventricular premature beat that coincides with the T-wave of the preceding beat
(R-on-T phenomenon) and initiates the ventricular tachycardia.
Figure 46.3: ECG showing polymorphic ventricular tachycardia

Case 46 Ventricular Tachycardia 209
Ventricular tachycardia superficially resembles a supraventricular tachycardia

conducted aberrantly to the ventricles. Features in favour of a supraventricular
tachycardia are clock-like regularity, triphasic QRS morphology, normal QRS
axis, stable hemodynamic parameters and response to carotid sinus massage.
CLINICAL DISCUSSION
A series of three or more successive ventricular ectopic beats constitutes a ventri
cular tachycardia. A ventricular tachycardia that lasts for more than 30 seconds and
requires cardioversion for termination is called sustained ventricular tachycardia.
A non-sustained ventricular tachycardia lasts for less than 30 seconds and ends
spontaneously. Ventricular tachycardia is considered repetitive or recurrent
if three or more discrete episodes are documented, while chronic ventricular
tachycardia is that in which recurrent episodes occur for over a month. Sustained
ventricular tachycardia (scar VT) is often based on structural heart disease
wherein a fixed anatomical substrate facilitates a re-entrant mechanism. Causes
of sustained ventricular tachycardia are:
• Myocardial scar
Infarction
Aneurysm
• Myocardial disease
Myocarditis
Cardiomyopathy
• Congestive failure
Ischemic
Hypertensive
• Valvular abnormality
Mitral valve prolapse
Rheumatic heart disease.
Symptoms due to ventricular tachycardia are palpitation because of fast heart
rate and angina due to increased oxygen demand and shortened coronary filling
time. Lack of atrio-ventricular synchrony can cause dyspnea (pulmonary edema)
and syncope (low cardiac output). The hallmark of ventricular tachycardia is the
dissociation between atrial systole and ventricular systole (A-V dissociation). On
auscultation, the intensity of S1 is variable because of changing diastolic filling
period. Cannon a waves are observed in the neck veins due to atrial contraction
against a closed tricuspid valve. An atrial impulse may occasionally find the
ventricle receptive to depolarization and is able to “capture” the ventricle. A
capture beat (complete capture) or a fusion beat (incomplete capture) is irrefut
able evidence of A-V dissociation. Markers of electrical instability in survivors of
acute myocardial infarction are:
• Documented ventricular arrhythmias on 24-hour Holter monitoring
• Reproducible tachycardia on programmed electrical stimulation (PES)
• Late depolarizations on signal averaged electrocardiogram (SAECG).
MANAGEMENT ISSUES
The management of ventricular tachycardia (VT) depends upon the etiology,
the nature of heart disease and the hemodynamics of the patient. Ventricular
tachycardia due to sympathetic stimulation (catecholaminergic VT), caused
by stress, exercise or adrenergic drugs and in the absence of structural heart
disease, responds well to beta-blockers. In the presence of heart disease and if
hemodynamics are stable, chemical cardioversion with antiarrhythmic drugs
is initiated. An intravenous bolus of lignocaine or amiodarone is followed by a
maintenance infusion. Oral amiodarone is later continued indefinitely to prevent
recurrence. Alternative agents are sotalol, propafenone, flecainide and ibutilide.
If the hemodynamics are unstable due to hypotension, heart failure, or ongoing
ischemia, electrical cardioversion with DC shock is the procedure of choice. An
initial energy of 50 to 100 Joules is followed up with higher voltage, until sinus
rhythm is restored. If there is circulatory shock to begin with, upto 360 Joules are
used. Once sinus rhythm is restored, prophylactic pharmacological therapy is
initiated and continued indefinitely.
The management of polymorphic ventricular tachycardia with Q-T
prolongation is quite different. An infusion of magnesium sulphate or isoprotere
nol is used for chemical cardioversion. If the patient responds to beta-blockade,
either long-term antiadrenergic therapy is initiated or cervical sympathetic
ganglionectomy is offered. If the hemodynamics are unstable with hypotension
or shock, electrical cardioversion is the procedure of choice, as per the protocol
mentioned above. Alternatively, overdrive ventricular pacing is done, which is
often successful. To patients with history of recurrent syncope or family history
of sudden cardiac death (SCD) and to survivors of cardiac arrest, an implantable
cardioverter defibrillator (ICD) device is offered.
SECTION
14
Coronary Artery
Disease
C A S E
47
Chronic Stable
Angina
. CASE PRESENTATION
A 38-year old gentleman paid a visit to the cardiologist, with the complaint of
retrosternal heaviness during brisk walking. The discomfort was described as a
feeling of tightness in the chest and was associated with a sensation of suffocation
and choking. The chest pain radiated towards the left shoulder, down the inner aspect
of the left arm as well as to the neck and lower jaw. There was no history of dyspnea,
palpitation, excessive sweating or syncope. The discomfort typically occurred when
the patient undertook any form of physical activity, soon after a meal. He denied
any chest pain at rest or during sleep. The frequency and severity of his episodic
chest discomfort had not increased over the last three months. The patient was not
taking any cardiovascular medication and had never undergone a blood glucose or
lipid analysis. He smoked about 8 to 10 cigarettes a day for the last 20 years and
took 5 to 6 alcoholic drinks over weekends. He did not follow any particular diet
plan or exercise regime. His executive job entailed long hours of working and caused
considerable mental stress. There was history of premature coronary artery disease
as well as of diabetes mellitus, in several of his family members.
On examination, the patient was alert and in no distress. He was modestly
overweight with a body mass index (BMI) of 27kg/m2. The pulse rate was 84 beats/
min. with a BP of 134/82 mm Hg and there were no signs of heart failure. Findings
on general examination were xanthelasma on the upper eyelids, arcus senilis
around the cornea and acanthosis nigricans over the nape of neck. The precordium
was unremarkable with a normally located apex beat. There was no S3 or S4 gallop,
murmur or friction rub upon auscultation. The lung fields were clear without any
rhonchi or crepts. An ECG was obtained (Fig. 47.1).
Figure 47.1: ECG showing S-T segment depression with

T wave inversion in the lateral chest leads
214 Section 14 Coronary Artery Disease
ECG INTERPRETATION
The ECG showed normal sinus rhythm with normal P wave morphology and
no prolongation of the P-R interval or Q-T interval. The QRS complexes were
narrow, without significant Q waves or attenuation of the R wave height. A 2 mm
horizontal depression of the S-T segment was observed in leads V4, V5 and V6. The
T wave was also inverted in leads V5 and V6. These findings are consistent with the
diagnosis of lateral wall myocardial ischemia, a common feature in a patient who
has angina pectoris.
Coronary artery disease is the most important cause of S-T segment
depression. The degree of S-T segment depression (greater than 1 mm) correlates
with the severity of coronary insufficiency. Besides being depressed, the morpho
logy of the S-T segment, with increasing severity of myocardial ischemia, can be
classified as shown in Figure 47.2.
Figure 47.2: Nature of S-T segment depression with increasing severity of ischemia
A. Isolated J point depression (upsloping S-T segment)

B. Horizontality of S-T segment (sharp ST-T junction)
C. Plane S-T depression (horizontal S-T depression)
D. Sagging depression (hammock-like S-T segment).
Depression of the S-T segment constitutes the most useful criterion for the
positivity of the stress ECG test, performed by exercising on a treadmill or bicycle
ergometer. The degree of positivity of the stress test (mild, moderate or severe) can
be gauged from several parameters of S-T segment depression. The magnitude
of S-T segment depression and its shape are associated with the severity of
coronary artery disease. Additionally, early appearance of S-T depression in the
exercise period and longer persistence into the recovery period correlate with the
positivity of the stress test.
Angina pectoris is described as a feeling of retrosternal heaviness or tightness,
with a sensation of suffocation or choking. The pain is of a crushing or squeezing
nature along with restlessness, sweating, palpitation, shortness of breath or
extreme weakness. The pain may radiate to both shoulders and arms, down the
inner aspect of left arm or to the neck and lower jaw. In contrast, the pain arising
from the musculo-skeletal system of the chest is described as a dull ache or a
sharp pricking sensation. The pain increases on deep inspiration, turning to one
side, or on bending forwards. The pattern of radiation and accompaniments that
characterize angina pectoris, are typically absent in muscular chest pain.
Stable angina is caused by an imbalance between the supply of and demand
for oxygenated blood to the myocardium. The commonest cause is obstructive
Case 47 Chronic Stable Angina 215
coronary artery disease due to atherosclerosis. Stable angina is due to coronary

occlusion by one or more atherosclerotic plaques that have a small lipid core,
a thick fibrous cap and are less prone to rupture. Conventional risk factors for
coronary disease are advanced age, male sex, being overweight, tobacco abuse,
diabetes mellitus, hypertension, hyperlipdemia, sedentary lifestyle, psychosocial
stress and family history of coronary artery disease (Table 47.1).
Table 47.1: Conventional risk factors for coronary artery disease

• Male Sex • Hypertension
• Advanced age • Hyperlipidemia
• Being overweight • Diabetes mellitus
• Tobacco abuse • Sedentary lifestyle
• Family history • Psychosocial stress
Besides atherosclerotic coronary artery disease, there are some other atypical
and uncommon causes of angina pectoris, which too cause an imbalance between
myocardial oxygen supply and demand. These uncommon causes of angina are
given in Table 47.2.
Table 47.2: Atypical causes of angina pectoris

Non-cardiac
• Severe anemia
• Thyrotoxicosis
Vascular
• Coronary artery spasm
• Microvascular disease
Cardiac
• Hypertensive heart disease
• Hypertrophic cardiomyopathy
MANAGEMENT ISSUES
The first step in the management of stable angina pectoris is suitable life-style
modification and control of cardiovascular risk factors. Patients should be
advised to cut-down on calorie-dense foods, follow a regular exercise regimen,
to quit smoking and manage psycho-social stress. Major risk factors such as
hypertension, diabetes and hyperlipidemia should be treated with suitable drugs,
in addition to correction of life-style.
Glyceryl trinitrate (GTN) is used sub-lingually for rapid relief from angina,
while long-acting isosorbide mononitrate (ISMN) is given for long-term
prophylaxis. Beta-blockers are the mainstay of therapy, as they reduce the rate
and force of cardiac contraction and therefore the workload and oxygen demand.
In the scenario of beta-blocker contraindication or intolerance, a rate-limiting

calcium antagonist like verapamil or diltiazem is used. Metabolic agents like
nikorandil and trimetazidine are sometimes used. Low-dose aspirin prevents
thrombotic events. If the patient is allergic or intolerant to aspirin, clopidogrel
is employed. All patients should receive a statin, irrespective of their cholesterol
level. Statins have amply proven their worth in preventing cardiovascular events.
The ACE-inhibitor ramipril has also been shown to reduce CV events and all-
cause mortality in patients with cardiovascular disease.
RECENT ADVANCES
Besides the conventional risk factors of coronary artery disease, certain new
risk factors have been recently identified (Table 47.3). These include high levels
of C-reactive protein (CRP), homocysteine (Hcy), lipoprotein (a) [Lp (a)], and
fibrinogen (Fgn). However, the INTERHEART study clearly showed that majority
of heart attacks across the globe can be conveniently explained on the basis of
conventional risk factors. Modest intake of alcohol with high intake of fruits and
vegetables were found to be important “anti-risk” factors.
Ranolazine is a new metabolically active agent used for the treatment of stable
angina. Ivabradine is a recently introduced drug for heart-rate control, in patients
who cannot tolerate uptitration of beta-blocker dose.
Table 47.3: Non-conventional risk factors

for coronary artery disease
• C-reactive protein (CRP)
• Lipoprotein (a) [Lp(a)]
• Homocysteine (Hcy)
• Fibrinogen (Fgen)
C A S E
48
Acute Coronary
Syndrome
. CASE PRESENTATION
A 56-year old gentleman was brought to the emergency department by his wife, in
the wee hours of the morning. The man had complained of severe central chest pain
with profuse sweating, during sexual intercourse. He was a known case of diabetes
mellitus and hypertension. However, he was irregular with his medication and did
not follow-up with his doctor periodically. The patient had not undergone a blood
lipid analysis despite advice from his doctor and persistent request by his wife. He
had a sedentary life-style and was on no particular dietary restrictions. He smoked
8 to 10 cigarettes everyday and took 3 to 4 pegs of whiskey, on most days of the
week. A treadmill test performed one year earlier had been inconclusive, because
the patient had failed to achieve the target heart rate. Recently, he had been
complaining of frequent episodes to restlessness and belching, which he attributed
to acidity and indigestion.
On examination, the patient was tachypneic and diaphoretic. He was markedly
overweight with a body mass index (BMI) of about 32 kg/m2. The pulse was irregular
and low in volume with “skipped” beats. The extremities were cold and clammy. His
heart rate was 104 beats/min. with a BP of 104/74 mm Hg. Skin tags and acanthosis
nigricans were seen at the nape of the neck and xanthelasma were observed over
the upper eyelids. The precordium and apex beat were unremarkable. The S1 and S2
were loud with a S3 gallop; no murmur was audible. Few basilar rales were heard over
the lung fields bilaterally. An ECG was obtained (Fig. 48.1).
Figure 48.1: ECG showing S-T segment elevation in inferior leads

with S-T segment depression in lateral leads
ECG INTERPRETATION
The ECG showed sinus rhythm with normal P waves and no prolongation of the
Q-T interval. There was a 5 mm elevation of the S-T segment in leads LII, LIII and
avF. There was also S-T segment depression (reciprocal changes) in leads LI and
avL. There was no attenuation of the R wave or appearance of significant Q waves.
These findings are consistent with the diagnosis of hyperacute phase of inferior
wall myocardial infarction.
The phases of acute myocardial infarction are divided into hyperacute phase
(0 hr to 6 hrs), recent phase (7 hrs to 7 days), evolved phase (8 days to 28 days) and
stabilized phase (more than 29 days). In the hyperacute phase, the S-T segment
is markedly elevated, which blends with the proximal limb of the tall T wave. In
the evolved phase, the S-T segment elevation begins to settle down, T waves get
inverted and Q waves appear. In the stabilized phase, there is normalization of
the S-T segment and T waves but the Q waves persist (Fig. 48.2).
Figure 48.2: Diagram to illustrate the various phases of

S-T elevation myocardial infarction
Myocardial infarction due to coronary occlusion, consists of a central

necrotic core surrounded by a zone of injury and skirted by a water-shed area of
ischemia. These areas form the pathological basis of the ECG changes observed in
myocardial infarction. Q wave represents necrosis, S-T elevation is due to injury
and T wave inversion is indicative of myocardial ischemia (Fig. 48.3).
Figure 48.3: The pathological basis of ECG changes

after acute myocardial infarction
Case 48 Acute Coronary Syndrome 219
The location of an infarct is identified from the particular ECG leads that show
the classical findings of myocardial infarction (Table 48.1).
Table 48.1: Location of infarction from leads with Q waves

Leads with Q waves Location of infarction
V1V2 Septal
V3V4 Anterior
V5V6LIaVL Lateral
V1-4 Anteroseptal
V3-6LIaVL Anterolateral
V1-6LIaVL Extensive anterior
LIaVL High lateral
LIILIIIaVF Inferior
CLINICAL DISCUSSION
Acute coronary syndrome is classified into ST elevation myocardial infarction
(STEMI), non-ST elevation myocardial infarction (NSTEMI) and unstable angina
(UA). Since myocardial infarction (not angina) involves tissue necrosis, the levels
of cardiac enzymes (creatine kinase and troponin), are elevated. The distinction
between unstable angina and NSTEMI is therefore based on the cardiac enzyme
levels. STEMI is also referred to as Q wave or transmural MI. NSTEMI is also
designated as non-Q or subendocardial MI. This classification has therapeutic
as well as prognostic implications. Acute coronary syndrome is due to coronary
occlusion by an atherosclerotic plaque, which has a large lipid core, a thin fibrous
cap and is more prone to rupture. A thrombus forms over the ruptured vulnerable
plaque.
In variant angina, which is also known as Prinzmetal’s angina, the basis
of myocardial ischemia is not coronary thrombosis but arterial spasm. In an
ischemic episode of vasospastic angina, the ECG changes are similar to those of
hyperacute phase of infarction with S-T segment elevation and tall T waves. The
difference is that the ECG changes do not evolve serially but settle down rapidly.
Q wave never appear and levels of cardiac enzymes are not raised as there is no
myocardial necrosis.
Myocardial infarction is classified on the basis of the age of infarct (recent or
stabilized), site of infarct (anterior or inferior wall) and type of infarct (transmural
or subendocardial). There may paucity of ECG findings if the infarction is
small, atrial, posterior in location, or right ventricular MI. ECG findings of
infarction are difficult to interpret in the presence of left bundle branch block,
left ventricular hypertrophy, WPW syndrome and digoxin therapy. Reasons for
a disparity between ECG changes and the clinical findings are left circumflex
disease, hibernating myocardium, attenuation phenomenon or the presence of
a mechanical complication.
Table 48.2: Types of regional wall motion

• Normal motion: full inward motion
• Hypokinesia:<50% inward motion
• Akinesia: no inward wall motion
• Dyskinesia: outward movement
• Aneurysmal: outpouching of wall.
On echocardiography, the systolic inward motion of the infarcted myocardial

segment is reduced in extent, altogether absent or may even be paradoxically
outward. The regional wall motion abnormality can be classified as given in
Table 48.2. From the location of the regional wall motion abnormality (RWMA), it
is possible to identify the coronary artery which is occluded (Fig. 48.4).
Figure 48.4: Identification of the occluded coronary artery from

the location of wall motion abnormality
PLAX: Parasternal long axis; PSAX: Parasternal short axis; RCA:
Right coronary artery; LAD: Left anterior descending artery; LCX:
Left circumflex artery
MANAGEMENT ISSUES
All patients with an acute coronary syndrome (ACS), should ideally be managed
in a coronary care unit (CCU), where the speed of instituting treatment improves
myocardial salvage (time is muscle). The long-term survival after myocardial
infarction heavily depends upon the extent of myocardial salvage (muscle is
time). The essentials of treatment are bed-rest, oxygen, opiate analgesia, aspirin
Case 48 Acute Coronary Syndrome 221
162.5 mg stat and clopidogrel 300 mg stat. Patient of STEMI may be taken up for
percutaneous coronary intervention (PCI), if there is rapid access to a Cath-Lab
facility. If not, the patient should receive thrombolytic therapy, provided there are
no contraindications.
Patient of NSTEMI should receive heparin, nitrate and a beta-blocker. High
risk patients are taken up for coronary angiography with revascularization.
Coronary artery revascularization is undertaken by percutaneous transluminal
coronary angioplasty (PTCA) or by coronary artery bypass graft (CABG) surgery.
PTCA is suitable for significant (>70% stenosis) single-vessel disease or 2-vessel
disease. CABG is preferable for 3-vessel disease, 2-vessel disease including
proximal LAD and left main-stem occlusion.
RECENT ADVANCES
A number of scoring systems have been developed, to risk-stratify patients
presenting with an acute coronary syndrome. The most commonly used is the
TIMI (Thrombolysis In Myocardial Infarction) Risk score, that was derived from
trials of low molecular weight heparin. The TIMI system assigns a binary score
(0 or 1) to seven independent risk factors, which have a similar predictive value.
Higher the score, greater is the risk of death or recurrent ischemia at 14 days. The
seven risk factors of the TIMI score, are given in Table 48.3.
Table 48.3: TIMI risk score after acute coronary syndrome

• Sixty-five years of age or older
• Three or more CV risk factors
• Significant coronary stenoses
• S-T segment deviation, > 2 mm
• Use of aspirin prior to admission
• Two or more anginal episodes
within previous 24 hours
• Elevated cardiac enzyme levels
(creatine kinase or troponin)
C A S E
49
Papillary Muscle
Rupture
. CASE PRESENTATION
A 63-year old woman was wheeled into the emergency room, with the complaints
of vague chest discomfort, profuse sweating and sinking sensation lasting about 5
hours. She also had retching and vomiting for which she took some antacid, thinking
that it was “gas-trouble”. When there was no relief, the patient’s daughter deemed
it necessary to get an ECG done. The ECG findings were alarming and therefore
she was admitted to the coronary care unit (CCU). There she received thrombolytic
therapy and remained stable for the next 3 days. On the 4th day of her admission, she
felt breathless and was unable to lie flat in bed. There was no fresh chest pain and
the cardiac monitor did not show any serious arrhythmia, except for an occasional
ventricular premature complex.
On examination, the patient was tachypneic, diaphoretic and appeared to be
in distress. She looked pale and her extremities were cold and clammy. The pulse
was fast, irregular and of low volume, with a rate of 104 beats/min. Her BP was
106/74 mm Hg in the right arm and the respiratory rate was 28/min. The JVP was not
raised and there was no edema over the ankles. The precordium was hyperkinetic
and the cardiac apex beat was displaced towards the left axilla. The S1 and S2 were
normal but an S3 gallop sound was appreciated in diastole. A soft systolic murmur
was audible over the cardiac apex. The murmur radiated towards the axilla and could
be heard upto the left scapula. Bilateral coarse crackles were heard over the lung
fields.
ECG showed sinus tachycardia with 5 mm elevation of the S-T segment in leads
LII, LIII and aVF. There was reciprocal S-T segment depression in leads LI and aVL. These
findings were consistent with the diagnosis of hyperacute inferior wall myocardial
infarction (Fig. 49.1). Additionally, there was elevation of the S-T segment in the
right-sided chest leads V3R and V4R. This was indicative of right ventricular infarction.
ECHO revealed a normal sized left ventricle with an ejection fraction of 45%. There
was dilatation of the right ventricle and right atrium. The postero-basal segment of
the left ventricle and free wall of the right ventricle were hypokinetic. There was no
mass or thrombus in any chamber. The posterior leaflet of the mitral valve exhibited
an exaggerated whip-like motion (Fig. 49.2). The tip moved past the mitral annular
plane and deep into the left atrium. It failed to coapt with the anterior leaflet at the
end of diastole. On colour flow mapping, an eccentric jet was seen in the left atrium,
that was directed towards the posterior left atrial wall. These findings are consistent
with the diagnosis of flail mitral leaflet due to papillary muscle rupture.
Case 49 Papillary Muscle Rupture 223
Figure 49.1: ECG showing the hyperacute phase of

inferior wall myocardial infarction
Figure 49.2: ECHO showing exaggerated motion

of a flail posterior mitral leaflet
CLINICAL DISCUSSION
Acute mitral regurgitation (MR) in a setting of acute myocardial infarction, occurs
either due to papillary muscle rupture or because of papillary muscle dysfunction.
Rupture of a papillary muscle due to ischemic necrosis causes a flail mitral
valve leaflet (Fig. 49.3). Since rupture of the postero-medial papillary muscle
is more common than that of the antero-lateral muscle, often it is the posterior
mitral leaflet (PML) that is flail. It generally follows inferior wall infarction due
to occlusion of the posterior descending branch of the right coronary artery.
Papillary muscle dysfunction is due to ischemic restriction of papillary function
or akinesia of the infero-basal wall that does not adequately shorten in systole. As
Figure 49.3: Diagram to illustrate whip-like motion

of a flail posterior mitral leaflet
LV: Left ventricle; LA: Left atrium
a result, the posterior mitral leaflet fails to reach the plane of the valve annulus
and the coaptation point of mitral leaflets is distally located into the left ventricle.
In acute MR due to myocardial infarction (MI), the left ventricular (LV) systolic
function often remains unimpaired. In pump failure due to extensive MI, there is
severe LV dysfunction. Although there is no time for left ventricular dilatation to
develop, an acute rise in left ventricular end-diastolic pressure (LVEDP) rapidly
produces frank pulmonary edema. The systolic murmur of acute MR is short
and soft compared to the long and loud murmur of MR due to chronic valvular
disease. This is because of two reasons. Firstly, the large mitral orifice created
by acute MR, does not generate much turbulence across the valve. Secondly,
the rapid rise in left atrial pressure and decline in pressure gradient impedes
regurgitation, during the later half of systole. Besides papillary muscle rupture
due to myocardial infarction, other causes of acute mitral regurgitation are blunt
trauma to the chest wall and valve dehiscence in case of infective endocarditis
(Table 49.1). Another cause of a systolic murmur after myocardial infarction is
ventricular septal rupture.
Table 49.1: Causes of acute mitral regurgitation

• Chest wall trauma
On Doppler or on color flow mapping, the MR flow velocity or color jet is

eccentric and directed towards the posterior left atrial wall (Fig. 49.4). The jet
area may be much less than the actual amount of MR; hence there is a risk of
underestimating the severity of acute MR. The flail leaflet of papillary muscle
rupture resembles the floppy leaflet of mitral valve prolapse with subtle
differences. The prolapsing leaflet just buckles but does not flap freely and it
enters the left atrium only for a brief period (Table 49.2).
Figure 49.4: ECHO showing an eccentric jet

of mitral regurgitation
Case 49 Papillary Muscle Rupture 225
Table 49.2: Differences between flail mitral leaflet and mitral valve prolapse
Flail leaflet Prolapsed leaflet
Range of motion flaps freely just buckles
Entry into left atrium
extent deep into just enters
duration for long time for short time
Direction of tip towards LA towards LV
MANAGEMENT ISSUES
Mitral regurgitation due to coronary heart disease is multifactorial and papillary
muscle dysfunction or rupture is one of the causes. Other reasons are dysfunctional
ventricular remodelling with increased sphericity and mitral annular dilatation
due to ventricular enlargement. Medical management includes vasodilators
along with a diuretic, provided the blood pressure allows their use. In surgical
treatment, since the valvular anatomy is generally not distorted, mitral valve
replacement is not the answer. Instead restrictive annuloplasty, which involves
insertion of an undersized ring to improve leaflet apposition, is preferable.
Moreover, annuloplasty preserves left ventricular geometry and spares the patient
from the problems of anticoagulation (thrombosis, bleeding and monitoring).
C A S E
50
Left Ventricular
Aneurysm
. CASE PRESENTATION
A 68-year old man came with his son to his treating cardiologist, for a periodic heart
check-up. Three months back, the patient has sustained an anterior wall myocardial
infarction (MI), for which he was treated in this very hospital. At that time, he received
oral aspirin, sub-cutaneous enoxaparin and an infusion of nitroglycerine. He was not
given thrombolytic therapy because he presented over 24 hours after the onset of his
chest pain and Q waves had already appeared on the ECG. The patient also declined
coronary angiography, because he was not ready to undergo a revascularization
procedure. Thereafter, he did not develop post-MI angina, but he did complain of
some fatigue and exertional breathlessness.
On examination, the patient was tachypneic while lying on the couch in the
doctor’s chamber. The pulse rate was 96 beats/min. with a BP of 110/74 mm Hg
over the right arm. The JVP was not raised and there was no edema over the ankles.
The precordium was remarkable because of a prominent bulge and a double apical
impulse. The apex beat was diffuse and sustained and extended medially and
upwards, upto the 3rd intercostal space. On auscultation, the S1 and S2 were normal
but a soft S3 sound was audible in early diastole. No murmur or pericardial friction
rub was appreciated. The breath sounds were vesicular with few crepitations audible
over the lower lung fields.
ECG showed attenuation of R waves in the antero-septal precordial leads. There
was coving and elevation of the S-T segment with inversion of the T waves (Fig.
50.1). The cardiac Troponin -T test was negative. X-ray chest findings were increased
cardio-thoracic ratio with a large bulge on the left border of the heart. An arc-like
hemispherical calcification was seen within the bulge (Fig. 50.2).
ECHO revealed a dilated left ventricle with an ejection fraction of 35%. There was
a large dyskinetic area involving the mid and distal interventricular septum as well as
the left ventricular apex. The dyskinetic area underwent outward systolic expansion
with a persistent deformity during diastole. The wall of the dyskinetic area was
more echogenic than the adjacent myocardium. A laminated mass was observed
contiguous with the dyskinetic area, with which it moved synchronously (Fig. 50.3).
These findings are consistent with the diagnosis of left ventricular aneurysm with
mural thrombus.
Case 50 Left Ventricular Aneurysm 227
Figure 50.1: ECG showing R wave attenuation,

S-T coving and T wave inversion
Figure 50.2: X-ray showing an arc-like calcification within a ventricular bulge
Figure 50.3: ECHO showing a mural thrombus

arising from the ventricular apex
CLINICAL DISCUSSION
A ventricular aneurysm usually develops after an anterior wall myocardial
infarction, due to occlusion of the left anterior descending coronary artery. It
rarely follows inferior wall infarction. On ECG, if the typical pattern of evolved
myocardial infarction (MI) persists for three or more months after acute MI,
ventricular aneurysm should be suspected. On precordial inspection, the
ventricular aneurysm produces a double left ventricular apical impulse. If the
aneurysm is particularly large, it causes a sustained and diffuse apical impulse
that extends medially and upwards, by two or more intercostal spaces.
Table 50.1: Differences between true LV aneurysm and Pseudo-aneurysm

LV aneurysm Pseudo-aneurysm
Shape Wide neck Narrow neck
Location Apex of LV Posterior wall
Motion Dyskinetic Expansile
Wall Myocardium Pericardium
Rupture Unlikely Liable
Thrombus Laminar Fills cavity
A ventricular aneurysm is a large bulge-like deformity with a wide neck,

located at or near the apex of the left ventricle. It is more common after damage
to the anterior wall than after inferior wall infarction. The aneurysm exhibits
dyskinesia or outward systolic expansion and a persistent deformity during
diastole. The wall of the aneurysm is made of myocardium and is more echogenic
than adjacent areas because it is made of fibrous scar tissue. It does not rupture
but is often associated with a pedunculated or laminated ventricular thrombus.
A false aneurysm (pseudo-aneurysm) follows rupture of the left ventricular
free wall, where the resultant hemopericardium clots and seals the breach by
pericardial adhesions. The neck of the pseudo-aneurysm that communicates
with the left ventricle is narrower than the aneurysm itself. The pseudo-aneurysm
appears as a globular extracardiac pouch. A false aneurysm is located on the
posterolateral LV wall and is more common after inferior wall than after anterior
wall infarction. It is not expansile and remains constant in size. The wall of the
aneurysm is made of pericardium and it is less echogenic than adjacent areas.
It is friable, more liable to rupture and it is often filled with a thrombus caused
by hemopericardium. The differences between a true aneurysm and a pseudo-
aneurysm are given in Table 50.1.
The development of a left ventricular aneurysm after acute myocardial
infarction is a serious complication. Most patients go on to develop intractable
congestive heart failure requiring aggressive decongestive therapy. There is a
higher likelihood of ventricular tachycardia originating from the myocardial scar.
The incidence of post-infarction unstable angina is also increased. Moreover,
fragments of the mural thrombus in the aneurysmal pouch may dislodge to
produce distal embolism (Table 50.2).
Table 50.2: Complications of ventricular aneurysm

• Recurrent ventricular tachycardia
• Post-MI unstable angina pectoris
• Intractable congestive heart failure
• Mural thrombus with distal embolism
Case 50 Left Ventricular Aneurysm 229
MANAGEMENT ISSUES
Presence of a thrombus within a ventricular aneurysm is an established indication
for oral anticoagulant therapy. Other definite indications for anticoagulation are
mechanical prosthetic valve and left atrial thrombus with mitral stenosis. While
it takes 3 to 5 days for the therapeutic effect of an oral anticoagulant like warfarin
to take over, heparin is given in this interim period. Unfractionated heparin
requires monitoring of prothrombin time (PT) and aPTT, but it is cost-effective.
Low molecular weight heparin (LMWH) like enoxaparin is more expensive but
does not require monitoring.
It is established practice to prescribe aspirin, a statin, an ACE-inhibitor
and a beta-blocker to every survivor of myocardial infarction, unless there is
a specific contraindication. A diuretic is added to reduce cardiac workload,
if there are symptoms of pulmonary congestion. An aldosterone antagonist
like spironolactone or eplerenone can reduce cardiac workload and improve
ventricular remodelling. A nitrate is prescribed for post-infarction angina while
amiodarone is used if serious ventricular arrhythmias are documented.
It is not uncommon for patients who develop a ventricular aneurysm, to
have left main or triple-vessel coronary artery disease (TVD). Resection of the
aneurysm can be contemplated at the time of coronary artery bypass grafting
(CABG) surgery which is performed for either unstable angina or for refractory
heart failure.
Index
Page numbers followed by f refer to figure and t refer to table, respectively.
A Aortic
aneurysm 74,,75, 76t
Acid-fast bacilli 106 dilatation 75
Acute diseases 71
alcoholic intoxication 201 dissection 75, 79t
AR 46 regurgitation 19, 26, 43, 44f, 45, 46t,
coronary syndrome 39,,217, 219, 75, 125
221t, 220 root abscess 136
mitral regurgitation 32, 223
sclerosis 48
myocardial infarction 32, 152,,184, 20
stenosis 39, 42, 50,,74, 125
5, 218, 218f
valve 87f, 144f
myocarditis 127, 128, 129t
calcification 50
pericarditis 109, 127, 185
endocarditis 133
pulmonary embolism 187
leaflets 134f
renal failure 171
regurgitation 79
rheumatic fever 125
replacement 42,,46, 51
valvular regurgitation 39
sclerosis 50
viral pericarditis 110
stenosis 39, 40f, 84, 215
Addison’s disease 171
Aortoarteritis 50, 74
Adenosine 198
Aortopulmonary window 20, 86
Amiodarone 18, 177
Arrhythmogenic right ventricular
therapy 198
dysplasia 178
Amyloidosis 61
Arterial blood gas 102, 106
Aneurysm of
Arteriovenous fistula 20, 44
aorta 73
Arthralgia 125
sinus of Valsalva 83, 86, 87
Assessment of severity of mitral
Aneurysmal
regurgitation 31t
aorta with jet of aortic regurgitation 75f
dilatation of ascending aorta 74f stenosis 27t
Angiosarcoma 145 Asymmetrical septal hypertrophy 65
Angiotensin converting enzyme 129 Atherosclerosis 74
inhibitor 69, 171 Athlete’s heart 184
Ankylosing spondylitis 46,,50, 74 Atrial
Annuloaortic ectasia 86 fibrillation 101, 199-202
Anomalous left coronary artery arising flutter 200t
from pulmonary artery 22 myxoma 143, 145, 145t, 146, 149
Anterior mitral leaflet 63 natriuretic peptide 198
Antiarrhythmic drugs 177 septal defect 7, 8, 8f, 9f, 13, 16, 22, 87
Antistreptolysin O 111, 124 tachyarrhythmia 104
Anxiety neurosis and thyrotoxicosis 204 thrombus 145, 145t, 147, 149
Aorta 13f Atriofascicular bypass tract 191
232 50 Cases in Clinical Cardiology: A Problem Solving Approach
Atrioventricular aortic
block 181 dissection 78t
connection 191 regurgitation 46t
node 18, 198 root dilatation 50t, 74t
Atypical causes of angina pectoris 215t atrial fibrillation 201t
Austin-Flint murmur 26 constrictive pericarditis 119t
Automatic implantable cardioverter continuous murmur 86t
defibrillator 188 dilated cardiomyopathy 57t
Autosomal dominant without deafness 177 hyperkalemia 171t
AV nodal re-entrant tachycardia 192, 196 hypokalemia 167t
mitral regurgitation 32t
pericardial effusion 116t
B prolonged Q-T interval 177t
Bacterial pericarditis 119 pulmonary
Barlow’s syndrome 35 hypertension 98t
Basal atelectasis 101 regurgitation 12t
Beri-beri disease 19, 44 stenosis 94t
Bicuspid aortic valve 46, 83 restrictive cardiomyopathy 61t
Billowing valve 35 right ventricular overload 87t
Blalock-Taussig shunt 14 ST segment elevation 110t, 185f
Blunt chest wall trauma 32, 86 Stokes Adams attacks 181t
Body mass index 186, 213, 217 tricuspid regurgitation 18f
Bradyarrhythmias 177 Chaga’s disease 129
Brain hemorrhage 177 Chest
Broken heart syndrome 69 irradiation 119
Brugada syndrome 185, 186, 187f, 188 wall trauma 46, 224
Buckling of mitral leaflets 34f Chiari network remnant 139
Bundle of Kent 190f Childhood asthma 11
Chronic
C MR 32
obstructive pulmonary disease 104
Calcification of aortic valve leaflets 49f pulmonary disease 98
Calcium gluconate injection 172 stable angina 213
Candida albicans 138 Circle of Willis 83
Carcinoid syndrome 12, 18, 94 Cisapride 177
Cardiac Classification of
arrhythmias 193 aortic dissection 79t
computed tomography 46 mitral valve prolapse 35t
magnetic resonance imaging 66 Cleavage of anterior aortic wall 78f
resynchronization therapy 58 Coarctation of aorta 20, 22, 78, 81,
surgery 119, 205 83f, 86, 159
trauma 201 Collagen disease 74
Cardiomegaly with Complications of
prominent pulmonary artery 20f aortic dissection 80f
pulmonary congestion 45f ventricular aneurysm 228t
Carey-Coomb’s murmur 26, 125 Computed
Carotid sinus hypersensitivity 181 tomographic angiography 80
Catecholaminergic ventricular tachycardia tomography 14, 28, 61, 76, 87, 146
178, 188 Confusion 181
Causes of Congenital
acute bicuspid aortic valve 41
aortic regurgitation 86t cardiomyopathy 22
mitral regurgitation 224t endocardial cushion defects 31
pericarditis 112t heart disease 201
Index 233
long QT syndrome 178 E
parachute valve 27
Congestive heart failure 14, 84, 205, 209 Ebstein’s anomaly 15, 16, 18
Conn’s syndrome 167 Eccentric jet of mitral regurgitation 224f
Connective tissue Ectopia lentis 75
disease 18 Ectopic and re-entrant atrial tachycardia
disorder 27, 32, 46, 143 196t
Constrictive pericarditis 61, 61t, 117, 119, Ehlers-Danlos syndrome 74
119t, 201 Electrical cardioversion 202, 205
Contrast enhanced computed tomography Electrolyte deficiency 177
102 Endocardial
Coronary cushion defect 18
arteriovenous fistula 20, 86 infections 131
artery 220f Endocarditis of aortic valve 84
bypass graft 32, 46, 51, 221, 229 Endocrine disorders 81
disease 177, 185, 205, 201, 214, Endomyocardial fibrosis 18, 31, 32, 60, 61
216t, 229 Enlarged globular left ventricle 128f
spasm 215 Erythrocyte sedimentation rate 129
vasospasm 110 Ewart’s sign 114
Corrigan sign 44 Eye-signs of Grave’s disease 15, 16
C-reactive protein 111, 124, 129, 216
Cushing’s F
disease 167
Fallot’s tetralogy 11, 13f, 14, 94
syndrome 81
Fatigue 181
Cyanotic spells 3
Fever 125
Fibrinogen 216
D Fibroelastoma 145
De Musset sign 44 Fibrosarcoma 145
Deep vein thrombosis 99, 100, 101, 102 Fistula formation 136
Dementia 181 Flail mitral leaflet 225t
Diabetes mellitus 215 Flat domes of diaphragm 105
Dilatation of Floppy valve 35
aortic root 159 Functional tricuspid regurgitation 11
inferior vena cava 119f Fungal endocarditis 140
main pulmonary artery 106f
proximal aortic root 79 G
Dilated Gaucher’s disease 61
ascending aorta with prominent aortic Gibson’s murmur 21
knuckle 49f Glucose-insulin infusion 172
cardiomyopathy 18, 32, 55, 56, 201 Glyceryl trinitrate 215
pulmonary artery 105, 93f Glycogen storage disorder 61
Dislocation of lens 75 Granular-Sparkling appearance 120
Dissection of Grave’s disease 15
aorta 46, 77, 85, 86
proximal aorta 76, 84
Disseminated intravascular coagulation
H
100 Hammock appearance 34f
Diuresis vomiting diarrhea 167 Hampton’s hump 101
Documented ventricular arrhythmias 209 Head injury 177
Dressler’s syndrome 110, 185 Heart
Duroziez sign 44 block 136
Dyspnea 181 failure 22
Hemochromatosis 61 Jugular venous pressure 97f
Hemodialysis 172 Junctional escape rhythm 180
Hemoptysis 27
High-arched palate 75
Holter monitoring 209
K
Homocysteine 216 Kent bundle 191
Hurler’s Kerley B lines and pulmonary edema 114
mucopolysaccharidosis 27 Kussmaul’s sign 118
syndrome 27
Hypercalcemia 171
L
Hypereosinophilic syndrome 61
Hyperinflated lungs 105 Lamb syndrome 146
Hyperkalemia 169, 171 Left
Hyperlipidemia 215 anterior descending artery 220f
Hypertension 74, 205, 215 atrial
in pregnancy 78 myxoma 26, 144f
Hypertensive heart disease 215 radiofrequency ablation 28
Hypertrophic thrombus 28
cardiomyopathy 63, 178, 215 bundle branch block 58
obstructive cardiomyopathy 64, 65 ventricular
Hypocalcemia 167 aneurysm 226
Hypokalemia 165, 167, 177 diastolic dysfunction 60
Hypokinesia of distal septum and apex 68f dysfunction 79
Hypoplastic aorta 83 end-diastolic pressure 39, 224
Hypothyroidism 116 hypertrophy 65, 157-159, 206
outflow tract obstruction 39, 50,
63, 65
I posterior wall 40
Idiopathic hypertrophic subaortic systolic dysfunction 56f
stenosis 65 Leptospirosis 129
Implantable cardioverter defibrillator 58, Linear pericardial calcification 118f
66, 178, 210 Lone atrial fibrillation 201
Infarction 112, 205, 209 Long Q-T syndrome 178, 188
Infective endocarditis 32, 46, 86, 127, 134t, Low
139t, 143, 224 molecular weight heparin 69, 150,
Inferior vena cava 9f 153, 229
Inguinal hernia 75 voltage QRS complexes 104
Interventricular septum 40, 63, 158 Lower limb deep vein thrombosis 101
Intracardiac masses 141 Lown’s classification 204
Intracranial berry aneurysm 83 of ventricular ectopy 204t
Intractable congestive heart failure 228 Lown-Ganong-Levine syndrome 191
Intraventricular conduction defect 58 Lyme’s disease 129
Ischemia 205
Ischemic cardiomyopathy 56
M
Isolated hypertrophy of left ventricular
apex 65f Magnetic resonance imaging 14, 46, 58, 61,
76, 80, 87, 146
Main pulmonary artery 20f
J Management of
James bundle 191 atrial fibrillation 202t
Jervell-Lange-Neilsen syndrome 177 Fallot’s tetralogy 14
Joint pains 3 hyperkalemia 172t
Jone’s criteria 125t, 126 hypokalemia 168t
Index 235
supraventricular tachycardia 198t Occult malignant disease 143
ventricular tachycardia 210 Olanzapine 177
Marfan’s syndrome 35, 46, 50, 73, 74, 75t, 78 Oligemic lung segment 101
Maternal rubella syndrome 21 Oral polystyrene resin 172
Medial necrosis 74 Organic mitral regurgitation 56
Metabolic acidosis hyperglycemia 171 Ostium
Methicillin resistant Staph. aureus 140 primum ASD 9
Microvascular disease 215 secundum ASD 9, 35
Mid-diastolic murmur of mitral stenosis 26 Overriding aorta 12, 13, 13f
Minimum inhibitory concentrations 135
Mitral
and aortic valve calcification 159
P
annular calcification 27, 32, 50 Paget’s disease 19
regurgitation 29, 29f, 35, 36, 125 Papillary muscle
stenosis 25, 125 dysfunction 32
valve 28, 144f rupture 222
diseases 23 Paradoxical
prolapse 16, 32, 33, 35, 75, 83, 205, embolism 10
209, 225t septal motion 96f
replacement 28 atrial fibrillation 201
stenosis 25, 25f supraventricular tachycardia 192, 196
Modified Duke’s diagnostic criteria of Patent ductus arteriosus 19, 20, 21f, 22,
infective endocarditis 135t 44, 86
Monomorphic ventricular tachycardia 207f Peak tricuspid velocity 26
Mosaic jet in descending aorta 82f Pectus carinatum 75
Multifocal ventricular premature beats 203f Percutaneous
Muscular VSD 5 balloon mitral valvuloplasty 28, 150
Myocardial coronary intervention 221
disease 209 transluminal coronary angioplasty 221
infarction 79, 85, 110, 177, 185, 224, 227 Pericardial infections 107
infections 121 Perimembranous VSD 5
scar 209 Permanent pacemaker implantation 182
Myocarditis 177, 209 Persistent atrial fibrillation 201
Myxoid neurofibroma 146 Pheochromocytoma 36
Myxoma 145 Phosphodiesterase inhibitors 98
Myxomatous Pneumothorax 85
degeneration 35 Polymorphic ventricular tachycardia
valve 35 178f, 208f
Posterior
mitral leaflet 223
N motion of mitral leaflets 34f
NAME syndrome 146 Post-MI
Neurogenic myocardial stunning 69 Dressler’s syndrome 112
Non-coronary disease 185 unstable angina pectoris 228
Non-ST elevation myocardial infarction Pre-excitation syndrome 16
219 Pressure gradient across valve 26
Non-steroidal anti-inflammatory drugs Primary pulmonary hypertension 98
111, 171 Prinzmetal’s angina 110, 185, 219
Procainamide 112
Progressive
O hyperkalemia 170t
Obstructive pulmonary disease 103 hypokalemia 166t
Occlusion of neck vessels 79 Prolonged Q-T syndrome 175
Prosthetic Rheumatic
tubular dacron graft 84 aortic stenosis 41
valve dehiscence 136 carditis 125
Proximal pulmonary artery 93f fever 123, 125t, 127, 143, 177
Pulmonary heart disease 18, 27, 25, 28, 32, 35, 41,
artery 13f, 14, 87f, 97f 46, 94, 209
pressure 26, 96, 97f, 105 mitral regurgitation 30
balloon angioplasty 14 Rheumatoid arthritis 112, 116
congestion 27 Right
diseases 89 atrial
embolism 85, 99-101, 101t, 185 enlargement 104
emphysema 114 myxoma 139
hypertension 11, 12, 18, 93, 95 pressure 96
regurgitation 13f, 18, 87 thrombus 139
stenosis 12, 13, 13f, 91, 93 axis deviation of QRS vector 104
thromboembolism 201 bundle branch block 15, 16, 17, 101
valve 87f, 92 coronary artery 220f
involvement 94 heart failure 27
stenosis 92f sided endocarditis 18
valvotomy 12 ventricular
Pulseless disease 83 dysplasia 206
Pulsus end-diastolic pressure 92, 96
paradoxus 114 hypertrophy 12, 13, 26, 93, 94t, 96t,
parvus 39 104
tardus 39 infarction 187
outflow tract 13, 86, 87, 87f, 96, 105
Q systolic pressure 96
Role of percutaneous aortic balloon
Quantification of pericardial effusion 115t valvuloplasty 42
Quincke sign 44 Romano-ward syndrome 177
Quinidine 177 Root dilatation 46
Rubella syndrome 94
R Ruptured aneurysm of sinus of Valsalva 20,
84, 86, 87
Radiofrequency ablation 198, 202
Raised left atrial pressure 98
Recurrent S
systemic emboli despite adequate
therapy 136 Sarcoidosis 61
ventricular tachycardia 228 Senile calcific degeneration 41
Regional wall motion abnormality 159, 220 Septal abscess 136
Regurgitant jet in outflow tract 45f Serious ventricular arrhythmias 181
Reiter’s syndrome 74 Several causes of
Renal tubular acidosis 171 dilated cardiomyopathy 57
Renovascular disease 81 mitral regurgitation 31
Reperfusion 205 Severe
Respiratory infections 27 anemia 19, 27, 215
Restrictive cardiomyopathy 59-61, 61t, annular calcification 27
119, 119t aortic regurgitation 76
Reverse coarctation 83 Severity of ischemia 214f
Rhabdomyoma 145 Sick sinus syndrome 179, 180, 180t, 181,
Rhabdomyosarcoma 145 181t, 201
Index 237
Sinoatrial Technique of percutaneous aortic valve
conduction time 180 replacement 42, 51
exit block 180 Terfenadine 177
Sinus Tetralogy of Fallot 12
bradycardia 177, 179, 180 Thickened mitral valve leaflets 124f
node recovery time 180 Thyrotoxicosis 19, 27, 201, 215
of Valsalva 78, 86, 87 Torsades de pointes 178
aneurysm 85, 87f Total leucocyte count 111, 129
tachycardia 68f, 101, 109f Transesophageal echocardiography 6, 9,
venosus defect 9 10, 28, 87, 134, 146, 149
Slow atrial fibrillation 180 Transient ventricular apical ballooning 69
Small pleural effusion 101 Transthoracic echocardiography 41
Square-root sign 118, 120 Traube sign 44
ST elevation myocardial infarction 218f, Tremor of fingers 15
219 Tricuspid
Stable angina 214 regurgitation 16f, 87, 97f
Staphylococcal infection 139 valve 87f, 94
Staphylococcus aureus 135, 138 endocarditis 137
Still’s murmur 7, 94 prolapse 18
Storage diseases 61 vegetation 139
Streptococcal endocarditis 135 Triple-vessel coronary artery disease 229
Stress induced cardiomyopathy 69 Tubercular pericarditis 116, 119
Subarachnoid hemorrhage 84 Tubular heart 105
Subclavian Turner’s syndrome 35, 83
artery 14 Types of
steal syndrome 181 atrial septal defect 9t
Subcutaneous low molecular weight cardiac tumors 145t
heparin 102 regional wall motion 220t
Subpulmonic VSD 5 ventricular septal defect 5t
Subvalvular
fusion 28
pulmonary stenosis 14
V
stenosis 12 Valvular AR 46
Sudden cardiac death 58, 66, 188, 210 heart disease 201
Superior vena cava 9 regurgitation 18
Supracristal VSD 5 Vena cava defect 9
Supraventricular tachycardia 195 Ventricular
Systemic aneurysm 110, 185, 205
hypertension 46, 159t premature
thromboembolism 27 beats 203
Systolic complexes 203, 204
anterior motion of anterior mitral leaflet septal defect 3, 4, 4f, 5f, 12, 13, 13f, 22,
65, 65f 83, 87
murmur of aortic stenosis 93 tachycardia 197t, 207
thrombus 151
Viral myocarditis 177
T
Tachy-brady syndrome 180
Takayasu’s
W
aorto-arteritis 75, 83 Westermark’s sign 101
disease 50, 74 Wolff-Parkinson-White syndrome 16, 18,
Takotsubo cardiomyopathy 67, 69t, 70 35, 189, 189f, 190, 190f, 191, 197, 201

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50 Cases in

Atul Luthra MBBS MD DNB

JAYPEE BROTHERS MEDICAL PUBLISHERS (P) LTD

Jaypee Brothers Medical Publishers (P) Ltd.

© 2014, Jaypee Brothers Medical Publishers

Inquiries for bulk sales may be solicited at: jaypee@jaypeebrothers.com

50 Cases in Clinical Cardiology: A Problem Solving Approach

First Edition: 2014

With the widespread availability of sophisticated cutting-edge technology, the

Present-day cardiology is replete with a bewildering array of sophisticated

I am extremely grateful to:

Section 1: Congenital Heart Diseases

Section 2: Mitral Valve Diseases

Section 3: Aortic Valve Diseases

Section 4: The Cardiomyopathies

Section 5: Aortic Diseases

Section 6: Pulmonary Diseases

Section 7: Pericardial Infections

Section 8: Myocardial Infections

Section 9: Endocardial Infections

Section 10: Intracardiac Masses

Section 11: Typical ECG Abnormalities

Section 12: Electrocardiac Syndromes

Section 13: Cardiac Arrhythmias

Section 14: Coronary Artery Diseases

Figure 1.2: Ventricular septal defect

In VSD, a breach in the continuity of the interventricular septum creates a

Figure 1.3: Various locations of ventricular septal defect (VSD)

Table 1.1: Types of ventricular septal defect

A small VSD (Maladie de Roger) generates a loud pansystolic murmur in a

VSD. Complications of VSD in childhood are growth retardation and repeated

Table 1.2: Indications for surgical closure of VSD

Figure 2.1: Color flow map extending from

Figure 2.2: Atrial septal defect

In ASD, breach in the continuity of the interatrial septum creates a left-to-

Figure 2.3: Various locations of atrial septal defect (ASD)

Table 2.1: Types of atrial septal defect

is sinus venosus defect, which is accompanied by anomalous pulmonary venous

in males. Complications of ASD in adults are effort intolerance and pulmonary

Table 2.2: Indications for surgical closure of ASD

Ostium primum atrial septal defects with atrioventricular valvular abnor­

Table 3.1: Causes of pulmonary regurgitation

Figure 3.1: ECG showing tall R waves in leads V1 to V3.

Figure 3.2: Pulmonary regurgitation

The primary developmental abnormality is of the pulmonary subvalvular or

In a typical unrepaired case of Fallot’s tetralogy, auscultatory findings are a

exaggerated excursion. On colour flow mapping, a regurgitant jet was seen in

Figure 4.1: Tricuspid regurgitation

Figure 4.2: X-ray showing cardiomegaly due

increases in intensity during inspiration, provided the right ventricular function

Figure 4.3: ECHO showing enlarged right atrium

leaflet is attached to the IV septum, 10 mm or more distal to the anterior mitral

Table 4.1: Causes of tricuspid regurgitation

Figure 5.1: X-ray showing cardiomegaly with

Figure 5.2: ECHO showing retrograde jet from left

Figure 5.3: Patent ductus arteriosus

The continuous murmur of PDA is classically described as a “machinery”

A mammary soufflé is heard widely over the precordium in pregnant women. It

Figure 6.1: Mitral valve stenosis

Figure 6.2: M-mode ECHO showing reduced excursion

Figure 1.3: Various locations of ventricular septal defect (VSD)

Figure 2.1: Color flow map extending from

Figure 2.3: Various locations of atrial septal defect (ASD)

Ostium primum atrial septal defects with atrioventricular valvular abnor

Figure 4.2: X-ray showing cardiomegaly due

Figure 4.3: ECHO showing enlarged right atrium

Figure 5.1: X-ray showing cardiomegaly with

Figure 5.2: ECHO showing retrograde jet from left

Figure 6.2: M-mode ECHO showing reduced excursion

Figure 6.3: 2-D ECHO showing restricted opening with

Figure 7.2: X-ray showing cardiac enlargement

Figure 7.3: ECHO showing a color-flow map

Figure 8.1: ECHO showing buckling of mitral leaflets,

Figure 8.2: M-mode scan showing posterior motion of

Atypical chest pain, palpitation, fatigue, orthostatic symptoms and neuro

Figure 9.3: ECHO showing a mosaic colored jet

Figure 10.2: X-ray showing cardiomegaly

Figure 10.3: ECHO showing regurgitant jet

Figure 11.1: ECG showing tall R waves with

Figure 11.2: X-ray showing dilated ascending aorta

Figure 12.1: X-ray showing the classical findings of

Figure 12.2: ECHO showing reduced excursion

Figure 13.1: ECHO showing bright echogenicity

Figure 14.1: ECHO showing asymmetrical septal hypertrophy

Figure 14.2: ECHO showing isolated hypertrophy

Figure 15.2: ECHO showing hypokinesia

Figure 16.1: Diagram showing aneurysmal dilatation of the ascending aorta.

Figure 16.2: ECHO showing an aneurysmal aorta