As in Pulmonary Atresia with VSD, this defect is also associated with complete obstruction of the Pulmonary Artery. However, as there is no associated VSD, blood is diverted from the right atrium to the left atrium via the Foramen Ovale. The right ventricle (RV) is usually small (Hypoplastic), though its wall may be thickened (Hypertrophied). Survival depends on the ductus remaining open in the early days of life (in order for blood to reach the lungs), 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 may be possible, but often necessitates several operations.

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