Tetralogy of Fallot with pulmonary atresia and total anomalous pulmonary venous connection: Surgical management of a rare combination of congenital heart defects

Introduction: The combination of Tetralogy of Fallot with pulmonary atresia (ToF/PA) and total anomalous pulmonary venous connection (TAPVC) is an extremely rare congenital anomaly. We report on a patient with ToF/PA with multifocal lung perfusion through a PDA and major aortopulmonary collaterals (MAPCAs) and obstructed supracardiac TAPVC who underwent surgical treatment.

Aims: The main goal of the initial surgery was re-routing of the pulmonary venous return to the left atrium and establishment of antegrade pulmonary blood flow through a valved conduit (9mm femoral vein homograft) in order to promote growth of the severely hypoplastic pulmonary arteries.

Because of the unusual location of the pulmonary venous confluence, the confluence was anastomosed to the left atrial appendage instead of the generally preferred location (back wall) of the LA. The vertical vein connecting to the SVC was left open to allow decompression of the pulmonary veins in case of postoperative obstruction at the LA anastomotic site.

The progression of the stenosis of the vertical vein at the SVC junction and obstruction of the PV-LA anastomosis necessitated a second surgery. A new wide anastomosis of the PV confluence to the undersurface of the SVC was created.

Discussion: There are very few, if any, reports of the anatomy and successful surgical treatment of ToF/PA/MAPCA with supracardiac TAPVC in the literature. A staged surgical approach with initial RV-PA connection and relief of TAPVC obstruction seems to be a safe option and allows for growth of the diminutive pulmonary arteries before unifocalization and VSD closure.