Selection of revision technique for suboptimal total cavopulmonary connection based on computational fluid dynamics: a case report

Unequal pulmonary blood flow in Fontan circulation is a known contributor to development of pulmonary arteriovenous fistula (PAVF). However, optimal revision strategies remain debated, especially in patients with a pedicled autologous pericardial conduit. A 23-year-old woman with a history of pulmonary atresia and previous total cavopulmonary connection (TCPC) using a pedicled pericardial extracardiac lateral tunnel developed hemoptysis and cyanosis due to left-sided PAVF. Cardiac catheterization, 3-dimensional computed tomography and 4-dimensional flow magnetic resonance imaging demonstrated unequal inferior vena cava flow preferentially directed to the right pulmonary artery. Preoperative computational fluid dynamics (CFD) simulation was used to evaluate three reconstructive options to rectify the issue. A configuration created with a prosthetic vascular graft was selected for optimal flow balance with minimal energy loss and phrenic nerve preservation. The patient tolerated the procedure well, and has been in a satisfactory condition and free of hemoptysis with reduction of PAVF over a 5-year follow-up. CFD simulation can serve as a useful adjunctive preoperative tool for comparing hemodynamic characteristics of different TCPC revision strategies, particularly in anatomically complex cases involving pedicled pericardial conduits.