DISEASES OF MYOCARDIUM AND

PERICARDIUM
 DISEASES OF MYOCARDIUM. CARDIOMYOPTHIES

Cardiomyopathies are heart muscle disorders that cause mechanical

and electrical dysfunction of the myocardium.
Based on the anatomic appearance and abnormal physiology of the LV
3 main types are classified : Dilated ( DCM), Hypertrophic (HCM),
Restrictive Cardiomyopathy.
 DILATED CARDIOMAIOPATHY ( DCM)

DCM is characterized by ventricular chamber enlargement , with

impaired systolic contractile function. Myocyte damage can result
from a wide spectrum of genetic and other causes.
Many cases are currently classified as idiopathic, several familial
forms of DCM have been identified and are believed to be
responsible for 20% to 30% of what were once classified as
idiopathic DCM . Autosomal recessive, autosomal dominant,
X- linked, and mitochondrial patterns of inheritance have been
identified.
 PATHOPHYSIOLOGY OF DCM

The hallmark of DCM is ventricular dilation with decreased contractile

function. Most often both ventricles are involved , sometimes
dysfunction is limited to LV or RV. As ventricular SV and CO decline
because of impaired contractility, 2 compensatory effects are activated
1. Frank-Starling Mechanism, increased stretch due to elevated LV
volume. 2. Neurohormonal activation, contributing to increased HR
and contractility. With persistent reduction of CO the decline in renal
blood flow prompts the kidney to increase renin secretion.
 HYPERTROPHIC CARDIOMYOPATHY

HCM is characterized by asymmetric LV hypertrophy, with vigorous

LV contraction and systolic function, but stiff muscle and impaired
relaxation and high diastolic pressures. Prevalence is high 1 in 500.
It is the most common cardiac abnormality found in athletes who die
suddenly during vigorous physical exertion.
HCM is a familial disease in which inheritance follows an autosomal
dominant pattern. The proteins encoded by mutant genes are all part
of sarcomere.
 PATHOLOGY OF HCM

Asymmetric hypertrophy of the ventricular septum is most commonly

found. Less often, involves ventricular walls symmetrically , or is
localized to apex or mid region of LV.
The histology HCM is unusual. the myocardial fibers are in extensive
disarray. Short , wide, hypertrophied fibers are oriented in chaotic
directions and are surrounded by numerous cardiac fibroblasts and
extracellular matrix.
 PATHOPHYSIOLOGY OF HCM

The predominant feature is marked LV hypertrophy that reduces

the compliance and diastolic relaxation properties of chamber,
such that the feeling becomes impaired.
Patients 1/3 of total who suffer from HCM and have asymmetric
hypertrophy of proximal IVS exhibit transient outflow tract
obstruction. During ventricular contraction ejection of blood
toward the aortic valve is more rapid than usual. ( because of
the narrowed outflow tract, this rapid flow causes Venturi forces
that abruptly draw the anterior mitral leaflet toward the septum,
causing transient obstruction to blood flow into the aorta .
 DISEASES OF THE PERICARDIUM

The pericardium is a 2- layered sac that encircles the heart.

The pericardium appears to serve 3 functions. 1. It fixes the
heart within the mediastinum and limits its motion.2. It prevents
extreme dilation of the heart during sudden rise of intracardiac
volume. 3. It may function as a barrier to limit the spread of
infection from the adjacent lungs.
In the healthy heart, intrapericardial pressure varies during the
respiratory cycle from -5 (inspiration) to +5 mm hg ( expiration).
 ACUTE PERICARDITIS

The most common affliction of the pericardium is acute pericarditis.

The most common etiology : Idiopathic, Viral , Tuberculosis.
Viral causes : Echovirus, Coxsackievirus B.
Tuberculosis: Is an important cause in immunosuppressed patients.
Arises from the reactivation of the organisms in mediastinal lymph
nodes, hematogenous spread or directly from the lungs.
Pericarditis is the most common manifestation of cardiovascular
disease in patients with AIDS. Bacterial pericarditis is a fulminant
illness, most likely to occur in immunocompromised patients, those
with burns and malignances. Pneumococci and Staphylococci are
responsible most frequently.
 NONINFECTIOUS PERICARDITIS

Following MI:1.Early after MI, results from inflammation extending

from epicardial surface of injured myocardium. It’s more common
in patients with transmural infarctions. It doesn’t affect the prognosis
of MI, it’s major importance is to differentiate it from the pain of
reccurent myocardial ischemia. This form of pericarditis occurs in <
5% of patients with acute MI who undergo acute reperfusion, and
common in those who aren’t. Also it’s common in patients with large
MI.
The second form of post MI pericarditis is known as Dressler Syndrome.
It can develop 2 weeks to several months after MI. Cause is unknown
but thought to be of autoimmune origin. Dressler has become very rare
since the advent of reperfusion therapies. Similar form of pericarditis
may occur weeks to months following heart surgery, termed postpericar
diotomy pericarditis.
Uremic pericarditis is potentially serious complication of untreated
chronic renal failure. It has become rare with the widespread availability
of dialysis therapy.
 OTHER FORMS OF PERICARDITIS

Neoplastic pericarditis most commonly results from metastatic spread

or local invasion by cancer of the lung, breast or lymphoma. Primary
tumors of the pericardium are rare. Neoplastic effusions are usually
large and hemorrhagic and frequently lead to cardiac tamponade.
Radiation induced pericarditis may complicate radiation therapy to the
thorax if cumulative dose has exceeded 4000 cGy. Radiation can cause
local inflammation and can result in fibrosis. Cytology helps to distinguish
it from tumor invasion.
 PATHOGENENSIS AND PATHOLOGY

Similar to other inflammatory processes pericarditis is characterized

by 3 stages: 1. Local vasodilation with transudation of protein-poor
cell- free fluid into the pericardial space. 2. Increased vascular
permeability with leak of protein into the pericardial space. 3. Leukocyte
exudation. The leukocytes are of critical importance because they help
contain or eliminate the offending infectious o autoimmune agent.
 PATHOLOGY OF PERICARDITIS

The pathologic appearance of the pericardium depends on the

underlying cause and severity of inflammation.
Serous pericarditis is characterized by scant polymorphonuclears,
lymphocytes and histiocytes. Early inflammation.
Serofibrinous pattern is the most commonly observed pattern. The
pericardial exudate contains plasma proteins, including fibrinogen
yielding a grossly rough and shaggy appearance. ( bread and butter
pattern. Occasionally this process may lead to scarring.
Suppurative ( purulent ) associated with bacterial pericarditis, the serosal
surfaces are erythematous and coated with purulent exudate.
Hemorrhagic pericarditis refers to a grossly bloody form, most often tuberculosis and malignancy.
 PERICARDIAL EFFUSION

The normal pericardial space contains 15-50 ml of pericardial fluid.

A larger volume of fluid may accumulate in association of pericarditis.
Noninflammatory serous effusions may result from conditions of :
1. Increased capillary permeability ( hypothyroidism).
2. Increased capillary hydrostatic pressure ( congestive heart failure).
3. Decreased plasma oncotic pressure( cirrhosis,nephrotic syndrome).
Three factors determine whether a pericardial effusion remains
silent or symptoms of compression ensure:
1. The volume of fluid.
2. The rate at which the fluid accumulates.
3. The compliance characteristics of pericardium.
CLINICAL AND DIAGNOSTIC FINDINGS: PERICARDIAL EFFUSION

Dull , constant ache in the left side of the chest.

Dysphagia , Because of esophageal compression.
Dyspnea, resulting from lung compression.
Hoarseness, recurrent laryngeal nerve compression.
Hiccups , phrenic nerve stimulation.
Chest X-ray, symmetrically enlarged heart silhouette, it fluid > 250ml.
ECG: Reduced QRS voltage, or if large effusions are present the
height of QRS may vary from beat to beat ( electrical alterans), resulting
from constantly changing electrical axis as heart swings within large
effusions from side to side.
CLINICAL MANIFESTATION OF CONSTRICTION

In constrictive pericarditis the negative intrathoracic pressure generated

during inspiration can’t be transmitted through rigid pericardial sac.
Inspiratory augmentation of RV filling is more limited. When a patient
with severe pericardial constriction inhales , the negative intrathoracic
pressure draws blood toward the thorax, where it can’t be accommodated
by the constricted right-sided chambers. As a result the increased venous
return accumulates in the intrathoracic systemic veins , causing the JV
to become more distended during inspiration. The Kussmauls sign.