Dextrocardia, DORV, Malposed great arteries, VSD, severe PS, TAPVR & Interrupted IVC with azygos continuation of the IVC

Case contributed by Dr Mohammad A. ElBeialy


A 4 months old cyanotic baby with complex congenital heart disease at echocardiography.

Patient Data

Age: 4 months
Gender: Male

  • Situs solitus with isolated dextrocardia.
  • Atrio-ventricular disconcordance with the right atrium is attached to a right-sided morphologically left ventricle and the left atrium is attached to a left-sided morphologically right ventricle.
  • 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. 
  • Cardiology.
  • A small tubular tortuous PDA is seen between the proximal descending aorta and proximal LPA.
  • No evidence of aortic coarctation.
  • Confluent small-sized main pulmonary artery and its branches with severe valvular and subvalvular pulmonary stenosis. No major aorto-pulmonary collaterals (MAPCAs) or peripheral pulmonary stenosis.
  • Total anomalous pulmonary venous return with the pulmonary veins are seen draining into the right atrium.
  • Interrupted IVC with prominent azygous vein draining into the SVC. Hepatic veins drain directly into the right atrium.


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Case information

rID: 34679
Case created: 4th Mar 2015
Last edited: 16th Dec 2015
System: Cardiac
Inclusion in quiz mode: Included

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