Dextrocardia, DORV, VSD, persistent left SVC & PAPVR

Case contributed by Dr Mohammad A. ElBeialy

Presentation

18 months old cyanotic baby with complex congenital heart disease at echocardiography.

Patient Data

Age: 18 months
  • Situs solitus with isolated dextrocardia.
  • Atrio-ventricular disconcordance.
  • Great vessel relationship: Double outlet left-sided morphologically right ventricle (DORV) with malposed vessels: Aorta is anterior the main pulmonary artery (MPA).
  • Large subaortic VSD.
  • Left-sided aortic arch with normal three orderly branches. 
  • Cardiomegaly; predominantly right atrio-ventricular.
  • No evidence of aortic coarctation.
  • Confluent rather average calibered main pulmonary artery and its branches. No major aorto-pulmonary collaterals (MAPCAs) or peripheral pulmonary stenosis.
  • A very small patent ductus arteriosus (PDA) is seen just distal to the left subclavian artery origin and coursing anteroinferiorly to join the main pulmonary artery bifurcation. 
  • Partial anomalous pulmonary venous return with the left superior pulmonary vein is seen draining via a persistent left superior vena cava. The rest of the pulmonary veins are draining into the left atrium. 
  • Normal both coronary arteries arising from their corresponding cusps.
  • Normal hepatic veins and the inferior vena cava draining into the IVC.  

 

Case Discussion

This is a case of complex congenital heart disease with Dextrocardia, DORV, VSD, persistent left SVC & partial anomalous pulmonary venous return (PAPVR).

Double outlet right ventricle (DORV) is a rare congenital cardiac anomaly where the aorta and the pulmonary artery arise from the right ventricle. VSD is invariably present. 

The morhological right ventricle criteria are:

  • Triangular configuration.
  •  Moderator band at its apex. 
  • Coarse trabecular pattern at its apical septum.
  • Muscular band around its out ventricular tract with the its atrioventricular and ventriculo-arterial valves separated by muscle.

The septal surface of the morphological left ventricle is smooth.

The left atrial appendage is finger-like while the right atrial appendage is triangular. 

 

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Case information

rID: 34713
Case created: 6th Mar 2015
Last edited: 16th Dec 2015
System: Cardiac
Inclusion in quiz mode: Included

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