Uncorrected Tetralogy of Fallot With Pulmonary Atresia in an Adult

Management of tetralogy of Fallot with pulmonary atresia (ToF-PA) in adults is not well established.

A 37-year-old woman with ToF-PA presented with dyspnea. Left and right heart catheterization revealed a large major aortopulmonary collateral artery (MAPCA) to the left pulmonary artery supplying confluent pulmonary arteries and with normal pulmonary vascular resistance. She underwent fenestrated ventricular septal defect closure, ligation of the MAPCA, and right ventricle-to-pulmonary artery conduit placement. She developed acute right heart failure, with distal conduit stenosis with the residual MAPCAs. A 26 × 12 mm stent was deployed, with relief of conduit stenosis. The MAPCAs were closed with vascular plug and coils.

Surgical repair can be recommended with confluent pulmonary arteries of adequate size, MAPCAs in which unifocalization or ligation/coiling can occur, and absence of severe pulmonary vascular disease. Surgery in adults may contribute significantly to mortality, with unclear benefit.

Unrepaired ToF-PA in adults is rare, and careful consideration must be given to each patient given the lack of standardized treatment protocols. Multidisciplinary teams are essential for preoperative planning and management of postoperative complications.