This defect is a form of Tetralogy of Fallot in which there is complete obstruction of the Pulmonary Artery resulting in total diversion of blood from the right ventricle into the aorta. Survival depends on the ductus remaining open in the early days of life (in order for blood to reach the lungs), or on the presence of other connecting blood vessels between the Aorta and the Pulmonary Arteries in the lungs (Collaterals). Most babies will need a 'Shunt' operation during infancy, involving insertion of a tiny piece of artificial tube (made from Goretex) between the Aorta, or a branch (usually one of the arm arteries), and one of the branch Pulmonary Arteries. Complete Repair is carried out at two to three years. Complete repair for Pulmonary Atresia is usually carried out after the first year of life, though sometimes it may be performed earlier.

Repair

Complete repair for Pulmonary Atresia usually necessitates the insertion of a 'Conduit', which is a tube containing a valve, placed to connect the right ventricle to the pulmonary artery.  The VSD is also closed and the pulmonary arteries may require enlargement with one or more patches.

Go to Normal Heart