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 ( = 0.70), transplant-free survival ( = 0.70), or freedom from renal disease ( = 0.60). However, estimated 10-year freedom from liver disease was greater in BiV patients (82% BiV vs 71% Fontan,  = 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,  < 0.001 and 13.09 vs 9.6,  = 0.002), while catheter-based interventions were higher among Fontan patients (8.41 vs 4.63,  < 0.001).

CONCLUSIONS

Among a contemporary cohort of patients with single ventricle anatomy, BiV provide comparable long-term survival and lower risk of liver disease when compared to patients who have undergoing Fontan operations.