Biventricular Repair of Univentricular Heart Lowers Risk of Liver Disease Compared With the Fontan Operation

Background: The Fontan operation is associated with chronic venous hypertension, liver and renal disease, and several other sequelae. The alterative surgical approach, when feasible, a biventricular conversion (BiV), may diminish some of these long-term risks. Objectives: The aim of this study was to compare long-term outcomes of patients undergoing BiV with those undergoing a destination Fontan operation. Methods: We identified all patients with univentricular physiology cared for at Boston Children’s Hospital between 2007 and 2022 and divided them into those who received BiV or Fontan operations. Outcomes included 10-year incidences of modified major adverse cardiovascular events (MACE), liver dysfunction, renal dysfunction, and transplant-free survival. Outcomes in the 2 groups were compared using propensity matching. Results: A total of 927 patients were evaluated, 341 BiV and 586 Fontan. Following propensity matching, 258 patients from each group were compared. There were no differences between groups in estimated 10-year freedom from MACE (P = 0.70), transplant-free survival (P = 0.70), or freedom from renal disease (P = 0.60). However, estimated 10-year freedom from liver disease was greater in BiV patients (82% BiV vs 71% Fontan, P = 0.02). Incidence rate per 100 person-years follow-up of surgical interventions and readmissions was higher among BiV patients (10.11 vs 1.85, P