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 Table of Contents  
INTERESTING CASE REPORT
Year : 2021  |  Volume : 5  |  Issue : 2  |  Page : 162-165

Imaging Insights into Eisenmenger Syndrome: From Basics to Sophistication


Department of Cardiology, St. John's Medical College Hospital, Bengaluru, Karnataka, India

Date of Submission17-Jul-2020
Date of Decision18-Sep-2020
Date of Acceptance27-Sep-2020
Date of Web Publication04-Feb-2021

Correspondence Address:
Dr. Srilakshmi M Adhyapak
Department of Cardiology, St. John's Medical College Hospital, Bengaluru - 560 034, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiae.jiae_33_20

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  Abstract 

We describe an unusual case of a large aortopulmonary window which was not very symptomatic and presented as Eisenmenger syndrome at the age of 20 years in a male patient. We take this opportunity to highlight some of the basic investigations like chest X-ray and contrast echocardiography which can delineate the level of the shunt. Attention should be given to the subtle bedside clinical signs which can give clues to the diagnosis. We did a cardiac computed tomography scan for confirmation of the diagnosis.

Keywords: Aortopulmonary window, cyanosis, Eisenmenger syndrome


How to cite this article:
Adhyapak SM, Anand P, Rao T, Shaikh JU, Varghese K. Imaging Insights into Eisenmenger Syndrome: From Basics to Sophistication. J Indian Acad Echocardiogr Cardiovasc Imaging 2021;5:162-5

How to cite this URL:
Adhyapak SM, Anand P, Rao T, Shaikh JU, Varghese K. Imaging Insights into Eisenmenger Syndrome: From Basics to Sophistication. J Indian Acad Echocardiogr Cardiovasc Imaging [serial online] 2021 [cited 2021 Dec 5];5:162-5. Available from: https://www.jiaecho.org/text.asp?2021/5/2/162/308732


  Introduction Top


Eisemenger syndrome is a syndrome complex constituting reversal of large left to right shunts with increasing pulmonary vascular resistance. These shunts can be at the atrial septum, ventricular septum, or at the great arterial levels.

We wish to highlight certain basic teaching pearls in a patient with Eisenmenger syndrome. We also want to stress that basic investigations such as chest X-ray and contrast echocardiography can be used to delineate the site of the shunt, while sophisticated imaging investigations such as cardiac computed tomography (CT) and magnetic resonance imaging can confirm the diagnosis. Certain subtle clinical features have been defined as hallmarks of individual lesions, mainly involving the second heart sound. A single second sound is a feature of reversal of flow through a ventricular septal defect (VSD), while a normally split second sound with loud P2 features reversal of flow through a patent ductus arteriosus (PDA). In an atrial septal defect (ASD), reversal of flow may shorten the gap between the two components of the second heart sound with a fixed split, the P2 being loud. The split in the second heart sound remains fixed although it may shorten.[1]

Differential cyanosis is a feature of reversal of flow in a PDA. ASD and VSD usually exhibit uniform pandigital cyanosis and clubbing. The cardiac size usually regresses with reversal of flow in a VSD, while in an ASD, cardiomegaly persists.

The chest X-ray may feature subtle signs, which may help the diagnosis further. Peripheral pruning of pulmonary vasculature with enlarged prominent main pulmonary artery is a sign of Eisenmenger syndrome. The presence of cardiomegaly may help distinguish a pretricuspid shunt (ASD), while normal or decreased cardiac diameter signifies a posttricuspid shunt which can be either a VSD or PDA.

A contrast echocardiogram can define the anatomical position of the shunt. Injection of agitated saline into the antecubital vein can define the right to left shunt when the bubbles appear in the left heart after 5–7 cardiac cycles. In the aorta, if the bubbles appear in the descending aorta, the reversal of flow is through a PDA, while if the bubbles appear in the ascending aorta, the reversed flow is through an aortopulmonary window.


  Case Report Top


We describe this unusual case of an aortopulmonary window presenting with Eisenmenger syndrome at the age of 20 years.

A 20-year-old male presented to us with 2–3 episodes of hemoptysis per day of 10 ml each for 1 week. He had a childhood history of breathlessness and failure to thrive which was noticed when he was 5 months of age. He was hospitalized for the same and administered medications. The details of which were not available. Further, he had a history of breathlessness on exertion at the age of 5 years due to which he could not play and keep up with his peers in outdoor activities. Besides, he developed repeated lower respiratory tract infections requiring hospitalization and systemic antibiotics. After the age of 14 years, the episodes of productive cough and breathlessness decreased. Clinical examination revealed a right ventricular apex, left parasternal heave, single second heart sound which was a loud P2, and a long early diastolic murmur along his left parasternal region. He had uniform pandigital cyanosis and clubbing.

His chest X-ray (PA view) demonstrated that the cardiac apex was formed by the right ventricle, with an aneurismal main pulmonary artery segment. There was peripheral pruning of pulmonary vasculature. An interesting observation was that the right pulmonary artery was conspicuous by its absence, while a branching pulmonary artery was seen through the cardiac silhouette. The aortic arch was right sided as it coursed to the right of the vertebral column [Figure 1].
Figure 1: A chest X-ray (PA view) of the patient demonstrating cardiomegaly. The cardiac silhouette shows apex formed by the right ventricle, with the aneurismal main pulmonary artery segment. There is peripheral pruning of pulmonary vasculature. An interesting observation is that the right pulmonary artery is conspicuous by its absence, while a branching pulmonary artery is seen through the cardiac silhouette. The aortic arch is right sided as it courses to the right of the vertebral column

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The two-dimensional echocardiography demonstrated a dilated right atrium, right ventricle, and main pulmonary artery, with normal valves and normal biventricular function. There was no ASD, VSD, or PDA.

A contrast echocardiography with agitated saline injected into the antecubital vein showed contrast bubbles in the ascending aorta after 5 cardiac cycles. The contrast bubbles were seen in the descending aorta much later [Figure 2].
Figure 2: This is an apical four-chamber view on two-dimensional echocardiography demonstrating a dilated right atrium and right ventricle. The color flow is laminar across both atrioventricular valves with no evidence of atrioventricular valve stenosis or regurgitation. The interatrial septum and interventricular septum are intact

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Further, a cardiac CT scan was done which confirmed a Type 1 aortopulmonary window with enlarged pulmonary arteries [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8].
Figure 3: This is the parasternal long-axis view on two-dimensional echocardiography demonstrating the enlarged right ventricle with intact interventricular septum

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Figure 4: This is a suprasternal view of the aortic arch. This is a contrast echocardiography with agitated saline injected through the antecubital vein. The contrast bubbles are seen in the ascending aorta after 5 cardiac cycles

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Figure 5: This is a computed tomography scan at the base of the heart demonstrating a large Type 1 aortopulmonary window. The short main pulmonary artery with branch pulmonary arteries arising from the aorta is visualized

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Figure 6: The computed tomography scan demonstrates the right-sided thoracic aortic arch

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Figure 7: These reconstructed computed tomography scan images demonstrate the large Type 1 aortopulmonary window arising from the ascending aorta, which then turns to continue as a right aortic arch

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Figure 8: These reconstructed computed tomography scan images demonstrate the large Type 1 aortopulmonary window arising from the ascending aorta, which then turns to continue as a right aortic arch

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  Discussion Top


The clinical history of heart failure in childhood which regressed during adolescence in this 20-year-old patient was a marker of this syndrome. His clinical examination revealed a right ventricular pressure overload with severe pulmonary regurgitation. The second heart sound was single and loud. He had pandigital clubbing. His chest X-ray (PA view) demonstrated that the cardiac apex was formed by the right ventricle, with an aneurysmal main pulmonary artery segment. There was peripheral pruning of pulmonary vasculature. An interesting observation was that the right pulmonary artery was conspicuous by its absence, while a branching pulmonary artery was seen through the cardiac silhouette. This in itself leads us to a differential diagnosis of absent right pulmonary artery suggestive of hemitruncus or truncus arteriosus.

The contrast echocardiography points to a diagnosis of aortopulmonary window as the contrast bubbles were first seen in the ascending aorta and later in the descending aorta.

Here, the clinical history and examination pointed toward Eisenmenger syndrome. The chest X-ray showed that the shunt was at the great arterial level. The contrast echocardiography pointed toward a diagnosis of aortopulmonary window. This was confirmed by a cardiac CT scan [Figure 9], [Figure 10], [Figure 11].
Figure 9: These reconstructed computed tomography scan images demonstrate the large Type 1 aortopulmonary window arising from the ascending aorta, which then turns to continue as a right aortic arch

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Figure 10: These reconstructed computed tomography scan images demonstrate the large Type 1 aortopulmonary window arising from the ascending aorta, which then turns to continue as a right aortic arch

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Figure 11: These reconstructed computed tomography scan images demonstrate the large Type 1 aortopulmonary window arising from the ascending aorta, which then turns to continue as a right aortic arch

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We wish to emphasize the importance of bedside clinical examination and basic imaging techniques which are still relevant in their contribution to the diagnosis of Eisenmenger syndrome defining the level of the intracardiac shunt.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Wood P. The eisenmenger syndrome. Br Med J 1958;2:701-9.  Back to cited text no. 1
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10], [Figure 11]



 

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