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Year : 2020  |  Volume : 4  |  Issue : 3  |  Page : 395-396

The Triad of Lefts: Left Isomerism, Left-Sided Continuation (Hemiazygos) of Interrupted Inferior Vena Cava Draining into Left Superior Vena Cava


1 Department of Cardiology, Apex Heart Institute, Ahmedabad, Gujarat, India
2 Department of Cardiology, VS General Hospital, Ahmedabad, Gujarat, India
3 Department of Anesthesia, P D Hinduja Hospital, Mumbai, Maharashtra, India

Date of Submission13-Jan-2020
Date of Acceptance19-Feb-2020
Date of Web Publication18-Dec-2020

Correspondence Address:
Dr. Pradyot Tiwari
Apex Heart Institute, Ahmedabad, Gujarat
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiae.jiae_4_20

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How to cite this article:
Tiwari P, Runwal S, Dev M, Patel B, Patel T, Shah S, Banerji A. The Triad of Lefts: Left Isomerism, Left-Sided Continuation (Hemiazygos) of Interrupted Inferior Vena Cava Draining into Left Superior Vena Cava. J Indian Acad Echocardiogr Cardiovasc Imaging 2020;4:395-6

How to cite this URL:
Tiwari P, Runwal S, Dev M, Patel B, Patel T, Shah S, Banerji A. The Triad of Lefts: Left Isomerism, Left-Sided Continuation (Hemiazygos) of Interrupted Inferior Vena Cava Draining into Left Superior Vena Cava. J Indian Acad Echocardiogr Cardiovasc Imaging [serial online] 2020 [cited 2021 Jan 15];4:395-6. Available from: https://www.jiaecho.org/text.asp?2020/4/3/395/303935

A 6-year-old female was referred to us in view of dyspnea, fatigue, and central cyanosis with a provisional diagnosis of congenital cyanotic heart disease. The patient had an oxygen saturation of 78% at room air. On auscultation, she had a single first heart sound, wide and fixed split-second heart sound, and Grade III pansystolic flow murmur heard at the apex. Echocardiography revealed situs ambiguous, atrioventricular canal defect seen as large ostium primum atrial septal defect and small inlet ventricular septal defect with moderate bilateral atrioventricular valve regurgitation, a large patent ductus arteriosus, left-sided aortic arch, hepatic veins draining directly into the right atrium [Figure 1], interrupted inferior vena cava on the left of abdominal aorta, and persistent left superior vena cava (SVC) [Figure 1].
Figure 1: (a) Hepatic veins draining into right atrium. (b) Persistent left superior vena cava

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Computed tomography scan of the thorax and abdomen with contrast venography showed findings of situs ambiguous with left isomerism including trachea bifurcating into bilateral morphologically similar bronchi and bilateral bilobed lungs, transverse liver, polysplenia, nonrotated gut, persistent left SVC [Figure 2], hemiazygos vein continuous with left-sided inferior vena cava [Figure 2] and [Figure 3], and draining into persistent left SVC [Figure 2], which is a very rare finding in the reported cases of left isomerism. The patient was advised surgical correction but refused for any surgical intervention.
Figure 2: (a) Persistent left superior vena cava. (b) Hemiazygous continuation of inferior vena-cava draining into persistent left superior vena cava

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Figure 3: (a) Hemiazygous continuation in thorax. (b) Interrupted inferior venacava to the left of spine

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This is an extremely rare anomaly,[1],[2] and although hemodynamically insignificant (barring increased risk of deep venous thrombosis[1]), it requires changes in venous cannulation for cardiopulmonary bypass, wherein cannulation of both the SVCs and right atrial becomes mandatory.[1] Furthermore, in patients planned for a single ventricle repair, a Kawashima procedure is equivalent to a Fontan procedure.[3]

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.
Ojha V, Pandey NN, Jagia P. Hemiazygos continuation of isolated left-sided inferior vena cava into persistent left superior vena cava: Rare association of left isomerism. BMJ Case Rep 2019;12: e230350.  Back to cited text no. 1
    
2.
Kim YJ, Kwon SH, Ahn SE, Kim SJ, Shin SJ, Oh JH. Interrupted inferior vena cava with hemiazygos continuation in an adult with a persistent left superior vena cava and left single coronary artery: A case report. J Korean Soc Radiol 2016;74:394-8.  Back to cited text no. 2
    
3.
Van der Horst RL, Hastreiter AR. Congenital interruption of the inferior vena cava. Chest 1981;80:638-40.  Back to cited text no. 3
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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