Singapore Med J 2018; 59(5): 279-283

Medical Education
https://doi.org/10.11622/smedj.2018054

CMEArticle
Clinics in diagnostic imaging (186)
Li Ching Lau1, MBBS, FRCR, Hui Liang Koh2, MBBS, FRCR, Wei Luen James Yip3, MBBS, MRCP, Ching Ching Ong2, MBBS, FRCR

Fig. 1 Frontal chest radiograph.

Fig. 2 Coronal contrast-enhanced CT image of the abdomen. Fig. 3 Axial contrast-enhanced CT image of the appended lower chest.

CASE PRESENTATION S2 heart sounds, with no cardiac murmur. There were crepitations
A 61-year-old Chinese woman presented to the emergency in the lung bases and tenderness over the right hypochondrium.
department with fever, vomiting and anorexia of three days’ Initial blood investigations showed leucocytosis, conjugated
duration. She had a past medical history of hypertension, hyperbilirubinaemia, and elevated alkaline phosphatase and
hyperlipidaemia and a previous cerebrovascular accident. transaminase levels. Electrocardiography showed right bundle
On examination, the patient was toxic looking, confused and branch block with right axis deviation.
jaundiced. Her vital signs were as follows: temperature 40.5°C, Chest radiography (Fig. 1) was performed, followed
blood pressure 134/67 mmHg, respiratory rate 20 breaths per by computed tomography (CT) of the abdomen and pelvis
minute, pulse rate 149 beats per minute and oxygen saturation (Figs. 2 & 3) to evaluate the suspected hepatobiliary sepsis. What
98% on room air. Physical examination revealed soft S1 and loud do the images show? What is the diagnosis?

Department of Diagnostic Imaging, KK Women's and Children's Hospital, 2Department of Diagnostic Imaging, 3Department of Cardiology, National University Hospital, Singapore
1

Correspondence: Dr Ong Ching Ching, Consultant, Department of Diagnostic Imaging, National University Hospital, 5 Lower Kent Ridge Road, Singapore 119074.
ching_ching_ong@nuhs.edu.sg

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IMAGE INTERPRETATION of the left and right ventricles.(2) For example, a left-to-right shunt
Anterior-posterior chest radiograph (Fig. 1) shows cardiomegaly is increased with left ventricular hypertrophy or mitral stenosis.(4)
with probable dilatation of the right atrium (arrows). The The majority of adults with ASDs remain asymptomatic. Early
central pulmonary arteries are dilated (arrowheads), suggesting signs may be picked up on health screening examinations,
pulmonary arterial hypertension. No pruning of pulmonary electrocardiography or chest radiography. Auscultation may
arteries was seen. reveal a faint to moderately loud systolic ejection murmur best
The CT images of the abdomen and pelvis show biliary heard at the upper left sternal border; wide split fixed S2; absent
obstruction secondary to a calculus in the distal common bile duct thrill; and a barely audible to faint diastolic flow rumble at the
(arrowhead, Fig. 2). There are also incidental findings of an atrial lower left sternal border.(4) When patients with ASDs eventually
septal defect (ASD) measuring at least 27 mm (asterisk, Fig. 3), become symptomatic, they may commonly present with
as well as dilatation of the right atrium (black arrowhead), right symptoms such as exertional dyspnoea, decreased effort tolerance
ventricle (black arrow) and pulmonary arteries (white arrows). and palpitations from arrhythmias.(2)
Radiographic evidence of ASD is often subtle. The normal
DIAGNOSIS cardiomediastinal outline is reviewed in Fig. 4. The right atrium
Atrial septal defect with pulmonary arterial hypertension. forms the right cardiac border. The right ventricle, an anterior
structure located just underneath the sternum, forms the inferior
CLINICAL COURSE border of the heart on the frontal chest radiograph. The left atrium
The patient required intubation and intensive care on admission. is a posterior structure located just below the carina; only its
She also developed complications of Gram-negative septicaemic appendage forms part of the left heart border. The left ventricle
shock, pneumonia and acute kidney injury. A cardiologist forms the left heart border. The normal upper limit of the diameter
reviewed her in the intensive care unit. Echocardiography that of the right interlobar pulmonary artery is 15 mm in women and
was performed confirmed a large secundum ASD with left-to-right 16 mm in men.(5)
shunt flow, severe right ventricular systolic dysfunction, severe On the chest radiograph, the first sign of underlying ASD
tricuspid regurgitation and severe pulmonary hypertension. is right heart dilatation. Increased convexity and enlargement
Pulmonary artery systolic pressure (PASP) was 70 mmHg and left of the right heart border suggest right atrial dilatation. When
ventricle ejection fraction was 55%. She was unlikely to have the right ventricle dilates, the left ventricle is displaced
Eisenmenger’s syndrome, as her oxygen saturation level was superolaterally, which results in increased convexity of the
normal on room air. left heart border and cardiac apex. (6) Long-standing ASD
The patient initially responded to the treatment of hydration, results in pulmonary hypertension (PH), which is an abnormal
intravenous antibiotics and biliary stenting. However, her elevation of pressure in the pulmonary circulation (mean
condition subsequently deteriorated when she developed critical pulmonary arterial pressure > 25 mmHg). Features of PH on
illness neuropathy and recurrent desaturations. She eventually chest radiographs include central pulmonary artery dilatation
passed away. (due to increased pulmonary flow), pruning of the peripheral

DISCUSSION
Our patient was incidentally diagnosed with ASD in late
adulthood when she presented with hepatobiliary sepsis. The
presence of a large ASD with severe pulmonary hypertension,
in this case, was deemed a significant contributor to her poor
clinical outcome. ASD can present at any age and is also the most
common congenital heart disease presenting in adulthood.(1,2) This
article aimed to highlight the imaging features of ASD, with special
emphasis on the routine chest radiograph. The pathophysiology
and clinical manifestations of ASD were also briefly discussed.
An ASD is an abnormal communication between the left
and right atria of the heart. It produces a constant left-to-right
interatrial shunt flow and also intermittent right-to-left shunt flow
from transient increases in right atrial pressure during a Valsalva-
like activity such as forceful coughing. This right-to-left shunt
Fig. 4 Normal frontal chest radiograph shows the right and left mediastinal
flow can result in paradoxical embolisation. A patent foramen outlines. The right mediastinal outline is formed by the superior vena cava
ovale, on the other hand, produces only intermittent right-to-left (cyan dashed line), right pulmonary artery (black solid line), right atrium
(green dashed line) and inferior vena cava (blue dashed line). The left
interatrial shunt flow.(3) Constant left-to-right shunting due to the mediastinal outline is formed by the aortic knuckle (red dashed line), main
ASD may result in right heart dilatation and pulmonary arterial pulmonary artery (white dashed line), left pulmonary artery (white solid
line), left atrial appendage (yellow dashed line) and left ventricle (magenta
hypertension over time. The magnitude and direction of shunting dashed line). The right atrium should only show mild convexity. The normal
are related to the defect size and relative diastolic filling properties size of the pulmonary arteries has been likened to the little fingers of adults.

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Table I. Classification of pulmonary hypertension, adapted from the Dana Point classification system.(7)

Group Classification
1 Pulmonary arterial hypertension
• Idiopathic
• Heritable
• Drug and toxin induced
• Associated with: connective tissue disease, HIV infection, portal hypertension, congenital heart diseases and schistosomiasis
Pulmonary veno‑occlusive disease and/or pulmonary capillary haemangiomatosis
Persistent pulmonary hypertension of the newborn
2 Pulmonary hypertension due to left heart disease
• Left ventricular systolic/diastolic dysfunction
• Valvular disease
• Left heart inflow/outflow tract obstruction
3 Pulmonary hypertension due to lung diseases and/or hypoxia
• Chronic obstructive pulmonary disease
• Interstitial lung disease
• Other pulmonary diseases with mixed restrictive and obstructive pattern
• Sleep-disordered breathing
• Alveolar hypoventilation disorders
• Chronic exposure to high altitude
• Developmental lung diseases
4 Chronic thromboembolic pulmonary hypertension
5 Pulmonary hypertension with unclear multifactorial mechanisms
• Haematological disorders
• Systemic disorders
• Metabolic disorders
• Others
HIV: human immunodeficiency virus

Fig. 5 A 19-year-old woman presented with shortness of breath on exertion Fig. 6 A 46-year-old lady presented with palpitations. Frontal chest
and palpitations. Frontal chest radiograph shows dilated central pulmonary radiograph shows dilatation of the right atrium (black arrowhead)
arteries (white arrows) with a small aortic knuckle (white arrowhead), and central pulmonary arteries (white arrowheads). A large secundum
which suggests a left-to-right shunt. There is also right atrial dilatation ASD (25 mm × 30 mm) and elevated PASP of 52 mmHg were seen on
(black arrowhead) and elevation of the cardiac apex (black arrow) due echocardiography (not shown).
to right ventricular dilatation. A large secundum atrial septal defect (ASD;
29 mm × 35 mm) and elevated pulmonary artery systolic pressure (PASP)
of 44 mmHg were seen on echocardiography (not shown). Nice, France, in 2013 updated the clinical classification of PH
in the following groups: disorders that cause pulmonary arterial
arteries and increased diameter of the right interlobar artery hypertension (Group 1); PH due to left heart disease (Group 2),
(15 mm in women and 16 mm in men), as measured from its PH due to chronic lung disease or hypoxia (Group 3); chronic
lateral aspect to the interlobar bronchus. In addition, a small thromboembolic pulmonary hypertension (Group 4); and PH
aortic knuckle can be seen in patients with ASD, a manifestation due to unclear multifactorial mechanisms (Group 5).(7) The
of decreased systemic flow. classification of PH is listed in Table I. Case examples of ASD are
There are many causes of PH and it is important to look for illustrated in Figs. 5 & 6. Comparison with previous radiographs
clues to the underlying causes on chest radiographs, such as may be helpful in identifying subtle and gradual changes in the
interstitial lung disease, emphysema and chest wall deformities. size and configuration of the right heart chambers and central
The Fifth World Symposium on Pulmonary Hypertension held in pulmonary arteries.

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is especially important in excluding a concomitant partial

anomalous pulmonary venous drainage.
Haemodynamically significant ASDs are associated with
increased morbidity and mortality.(2) Early detection and closure
of significant ASDs can help reduce morbidity by preventing
some of the associated complications. These complications
include arrhythmias, paradoxical embolism, stroke, brain abscess,
pulmonary hypertension, pulmonary artery aneurysm, right
ventricle failure and Eisenmenger’s syndrome.(8,9) Transcatheter
device closure of suitable secundum ASDs (Fig. 7 & 8) is now
an available option that is associated with less morbidity than
open surgical closure.(10)

ACKNOWLEDGEMENTS
Fig. 7 A 20-year-old woman was asymptomatic with an incidental finding of The authors would like to acknowledge Dr Lynette Teo, Dr Lee
a heart murmur. Echocardiography (not shown) revealed a large secundum
ASD (20 mm × 20 mm), elevated PASP of 41 mmHg, and mitral valve Chin Hwee and all colleagues at National University Hospital,
prolapse, which was the cause of the murmur. She underwent ASD closure Singapore, who were involved in the care of our patient.
with an Amplatzer septal occluder (area between black arrowheads).
Frontal chest radiograph shows the typical double-disc configuration of
the Amplatzer septal occluder device. There are also features of dilatation
of the right heart and pulmonary arteries. ABSTRACT We report a case of a 61-year-old woman
with a large atrial septal defect (ASD) that was
detected incidentally on chest radiography and
computed tomography when she presented with sepsis.
Echocardiography confirmed a large secundum ASD
with left-to-right shunt flow, right heart dilatation and
severe pulmonary hypertension. The patient had a poor
clinical outcome despite intensive care and eventually
passed away. Haemodynamically significant ASDs have
a known association with increased morbidity and
mortality, and their early detection and closure cannot be
understated. This article aimed to highlight the imaging
features of ASD, with special emphasis on the routine
chest radiograph. The pathophysiology and clinical
manifestations of ASD are also briefly discussed.

Keywords: atrial septal defect, cardiomegaly, chest x-ray, pulmonary hypertension,

septal occluder device
Fig. 8 Axial contrast-enhanced CT image of the same patient from Fig. 7.
The Amplatzer septal occluder (area between black arrowheads) is in situ,
apposing the atrial septal wall on each side of the defect. REFERENCES
1. Rojas CA, El-Sherief A, Medina HM, et al. Embryology and developmental
defects of the interatrial septum. AJR Am J Roentgenol 2010; 195:1100-4.
The diagnostic modality of choice for ASD is echocardiography, 2. Webb G, Gatzoulis MA. Atrial septal defects in the adult: recent progress and
as it provides both structural and functional information.(8) overview. Circulation 2006; 114:1645-53.
3. Kedia G, Tobis J, Lee MS. Patent foramen ovale: clinical manifestations and
Transthoracic echocardiography may be limited in assessing the treatment. Rev Cardiovasc Med 2008; 9:168-73.
right ventricle due to poor acoustic windows in some patients. 4. Frank JE, Jacobe KM. Evaluation and management of heart murmurs in children.
Am Fam Physician 2011; 84:793-800.
Transoesophageal echocardiography may be performed to 5. Peña E, Dennie C, Veinot J, Muñiz SH. Pulmonary hypertension: how the
obtain a better acoustic window, but it is invasive in nature. radiologist can help. Radiographics 2012; 32:9-32.
6. Cook AL, Hurwitz LM, Valente AM, Herlong JR. Right heart dilatation in adults:
Cardiovascular magnetic resonance (CMR) imaging, CT and
congenital causes. AJR Am J Roentgenol 2007; 189:592-601.
diagnostic cardiac catheterisation are alternative modes of 7. Simonneau G, Gatzoulis MA, Adatia I, et al. Updated clinical classification of
imaging. CMR imaging is the gold standard for the assessment pulmonary hypertension. J Am Coll Cardiol 2013; 62(25 Suppl):D34-41.
8. Martin SS, Shapiro EP, Mukherjee M. Atrial septal defects - clinical
of right ventricular volumes and function. It also allows for shunt manifestations, echo assessment, and intervention. Clin Med Insights Cardiol
quantification (Qp/Qs ratio; ratio of total pulmonary blood flow 2014; 8(Suppl 1):93-8.
9. Tartan Z, Cam N, Ozer N, Kaşikçioğlu H, Uyarel H. Giant pulmonary artery
to total systemic blood flow) by using velocity flow mapping. aneurysm due to undiagnosed atrial septal defect associated with pulmonary
A Qp/Qs ratio of 1:1 is normal and usually indicates that there hypertension. Anadolu Kardiyol Derg 2007; 7:202-4.
10. Quek SC, Hota S, Tai BC, Mujumdar S, Tok MY. Comparison of clinical outcomes
is no shunting. CT and CMR imaging both have the advantage and cost between surgical and transcatheter device closure of atrial septal defects
of allowing the visualisation of pulmonary venous return, which in Singapore children. Ann Acad Med Singapore 2010; 39:629-33.

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SINGAPORE MEDICAL COUNCIL CATEGORY 3B CME PROGRAMME

(Code SMJ 201805B)

Question 1. Regarding the clinical auscultation findings of atrial septal defects (ASDs): True  False
(a) ASDs do not cause cardiac murmurs. □ □
(b) ASDs can cause a systolic ejection murmur that is best heard at the upper left sternal border. □ □
(c) ASDs do not cause continuous machinery murmurs. □ □
(d) ASDs do not cause widely split fixed S2. □ □
Question 2. Regarding ASD:
(a) It is the most common congenital heart disease presenting in adulthood. □ □
(b) Early detection and closure of haemodynamically significant ASDs can help to reduce morbidity and □ □
mortality.
(c) The diagnostic modality of choice is cardiovascular magnetic resonance imaging. □ □
(d) Transcatheter device closure is now an available treatment option. □ □
Question 3. Regarding the haemodynamics of ASDs:
(a) It can allow right-to-left interatrial shunt flow, which explains paradoxical embolism. □ □
(b) It usually produces left-to-right interatrial shunt flow, because the left atrium has higher pressure than □ □
the right atrium.
(c) Most ASDs are haemodynamically well tolerated and remain asymptomatic into adulthood. □ □
(d) A left-to-right interatrial shunt flow is decreased with left ventricular hypertrophy. □ □
Question 4. Regarding the imaging features of ASDs on chest radiographs:
(a) Imaging features include right atrial dilatation, right ventricular dilatation and pulmonary arterial □ □
dilatation.
(b) The first sign of ASD is pulmonary arterial dilatation. □ □
(c) A large aortic knuckle is seen due to increased systemic flow. □ □
(d) Atrial septal occluder devices are visible on the chest radiograph. □ □
Question 5. The following are known complications of ASDs:
(a) Stroke. □ □
(b) Arrhythmia and palpitations. □ □
(c) Eisenmenger’s syndrome. □ □
(d) Heart failure. □ □

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