This is a complex heart disease that presents since birth. In this heart defect, there isno direct connection between right atrium and right ventricle along with small sized right sided ventricle.

In this situation, there is big hole between two upper chambers of heart (atrial septal defect, ASD) which leads to shunting of impure blood to left sided chambers which leads to decreased oxygen content in the body.

This disease can be associated with ventricular septal defect (VSD), pulmonary artery stenosis (PS) or pulmonary atresia; transposition of great vessels etc.

An atrial septal defect (ASD) and a ventricular septal defect (VSD) must both be present to maintain blood flow-from the right atrium, the blood must flow through the ASD to the left atrium to the left ventricle and through the VSD to the right ventricle to allow blood supply to the lungs.

Clinical Manifestations

  • Bluishness of fingers and lips in severe PS or pulmonary atresia
  • Signs of heart failure if there is large VSD with no PS
  • Murmur etc
  • Sometimes it can present in the form of critical disease when there is very small ASD.

For diagnosis, Echocardiography is must.


  • Medical management: by giving Prostaglanding infusion in cases of pulmonary atresia like situation, so that Patent ductusarteriosus can be open and can supply pulmonary arteries.
  • Surgical Management: Total 3 surgeries need to be done in 3 stages:

First Stage: done in first few weeks of life

  • If severe PS is there with increasing cyanosis, then modified Blalock-Taussig  shunt (BT shunt) is advised to maintain pulmonary blood flow.
  • Pulmonary artery banding (PA banding): When there is high flow through the lungs with signs of heart failure, then by creating artificial PS by putting a tissue in main pulmonary artery, we can decrease blood supply to lungs

For second and third stage surgeries, kindly see double outlet ventricle section of common cardiac condition in

Tricuspid Atresia

Tricuspid Atresia