Biventricular repair versus Fontan completion for patients with d- or l-transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction

Objectives: D-transposition of the great arteries and l-transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction are complex biventricular congenital heart diseases for which decision-making regarding surgical strategy remains challenging. We investigated the intermediate-term outcomes of Fontan versus biventricular procedures in these patients. Methods: We analyzed 129 patients with d-transposition of the great arteries/ventricular septal defect/left ventricular outflow tract obstruction (n = 85) or l-transposition of the great arteries/ventricular septal defect/left ventricular outflow tract obstruction (n = 44) and 2 functional ventricles from Australia who had primary surgical management (29 Fontan, 100 biventricular repair) undertaken between 1990 and 2015. Results: Median operative age of patients was 2.9 years (range, 0.2-26.8 years). During a median follow-up of 6.2 years (range, 2 days to 25.8 years), 9 patients died after biventricular repair (3 early and 6 late deaths). One patient received a transplant 1.2 years after Fontan completion. Overall transplant-free survivals at 1, 5, 10, and 15 years were 95%, 93%, 92%, and 90%, respectively. Overall reintervention-free survivals at 1, 5, 10, and 15 years were 79%, 64%, 45%, and 29% respectively. Biventricular repair tended to be associated with a higher rate of death, transplantation, or reintervention than the Fontan pathway (hazard ratio, 1.83; 95% confidence interval, 0.90-3.71; P = .10). Some 73% of transplant-free survivors had New York Heart Association class I. Functional status was similar between the Fontan and biventricular groups. Conclusions: Intermediate-term outcomes were comparable between patients with d-transposition of the great arteries/ventricular septal defect/left ventricular outflow tract obstruction and patients with l-transposition of the great arteries/ventricular septal defect/left ventricular outflow tract obstruction. Both Fontan and biventricular pathways are associated with excellent mortality and functional outcomes. Biventricular patients have a greater risk of reintervention. The Fontan procedure is a viable option when anatomic risk factors preclude biventricular repair.