Pulmonary atresia with ventricular septal defect and situs inversus totalis

Case contributed by Hani M. Al Salam
Diagnosis certain


Patient presented with cyanosis since birth. Concurrent situs anomaly is observed.

Patient Data

Age: 3 months
Gender: Male

There is dextrocardia. 

The left lung shows increased vascularity.

The bulge of the liver shadow is in the left hemiabdomen.  The stomach bubble is seen in the right. 

Endotracheal and feeding tube are seen in place.


Cardiovascular findings:

  • heart is positioned within the right hemithorax with its apex directed towards the right.  
  • morphologic left atrium is positioned posteriorly with the morphologic right atrium located on the left
  • all pulmonary veins drain into the morphologic left atrium
  • left-sided superior and inferior vena cavae draining into the morphologic right atrium
  • pulmonary atresia with the confluent pulmonary arteries supplied by a vessel arising from the aorta (possibly a tortuous patent ductus arteriosus or a major aortopulmonary collateral artery)
  • stenosis at the proximal segment of the right pulmonary artery
  • the dilated ventricle on the right side is more trabeculated with a moderator band reflective of right ventricular morphology
  • aorta arising from the morphologic right ventricle
  • right-sided aortic arch and descending aorta
  • small morphologic left ventricle positioned to the left of the morphologic right ventricle
  • ventricular septal defect

Visceral situs inversus:

  • hyparterial bronchus is in the right while the eparterial bronchus is in the left
  • left-sided liver
  • right-sided stomach and spleen

Other findings:

  • atelectatic changes in the right lung and left upper lobe
  • endotracheal and feeding tubes are seen in place

Case Discussion

This case demonstrates pulmonary atresia with ventricular septal defect (PA-VSD) in a patient with situs inversus totalis.  In the past, PA-VSD with patent ductus arteriosus (PDA) or major aortopulmonary collateral arteries (MAPCA's) supplying the pulmonary arteries was classified as truncus arteriosus type IV or pseudotruncus.

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