Complex congenital heart disease: DORV, Malposed great arteries, VSD, PDA, PAPVR, Persistent left SVC & Interrupted IVC.

Case contributed by Dr Mohammad A. ElBeialy


Cyanotic baby with congenital heart disease at echocardiography.

Patient Data

Age: 11 months
Gender: Male
  • Situs solitus with the apex to the left.
  • Atrio-ventricular concordance.
  • Great vessel relationship: Double outlet right ventricle (DORV) with malposed vessels: Aorta is anterior the main pulmonary artery (MPA).
  • Large subaortic VSD.
  • Right-sided aortic arch with normal three orderly branches. 
  • Cardiomegaly; predominantly right atrio-ventricular.
  • A small tubular tortuous  PDA is seen between the proximal descending aorta and proximal LPA.
  • No evidence of aortic coarctation.
  • Confluent good size main pulmonary artery and its branches. No major aorto-pulmonary collaterals (MAPCAs) or peripheral pulmonary stenosis.
  • Partial anomalous pulmonary venous return of the two right pulmonary veins that are seen draining into the right atrium. The two attenuated left sided pulmonary veins are draining into the left atrium.
  • Double SVC. Right SVC opens into the right atrium and the persistent left SVC opens into the left atrium.
  • Interrupted IVC with prominent hemi-azygous vein draining into the left SVC. Hepatic veins drain directly into the left atrium.

Case Discussion

Complex congenital heart disease: DORV, Malposed great arteries, VSD, PDA, PAPVR & persistent left SVC as well as interrupted IVC with prominent hemiazygous vein draining into the left SVC.

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Case information

rID: 34608
Published: 1st Mar 2015
Last edited: 14th Aug 2019
System: Cardiac
Inclusion in quiz mode: Included

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