The Evolving Surgical Burden of Fontan Failure: An Analysis of The Society of Thoracic Surgeons Congenital Heart Surgery Database

Background: Fontan failure often requires surgical therapy in the form of Fontan revision or heart transplantation. We sought to characterize national trends in the surgical burden of Fontan failure and identify risk factors for adverse outcomes. Methods: Fontan patients undergoing Fontan revision or transplantation from January 2010 to June 2018 were included. We evaluated baseline characteristics and outcomes and used multivariable logistic regression to identify risk factors for operative mortality and composite mortality and major morbidity. Results: A total of 1135 patients underwent Fontan revision (n = 598) or transplantation (n = 537) at 100 centers. Transplantations increased from 34 in 2010 to 76 in 2017, largely owing to an increase in patients with hypoplastic left heart syndrome (HLHS) (18 in 2010 to 49 in 2017), while Fontan revision decreased (75 in 2010 to 49 in 2017). Transplantation patients were younger (median 14 years of age vs 18 years of age; P <. 001), more often had preoperative risk factors (66% vs 40%; P <. 001), and more often had HLHS (51% vs 15%; P <. 001). Operative mortality and composite major morbidity and mortality were 7.6% and 35% for transplantation and 7.1% and 22% for Fontan revision, respectively. Multivariable risk factors for mortality included older age (odds ratio [OR], 1.08/y; P = .007), presence of preoperative risk factors (OR, 3.33; P = .002), and concomitant pulmonary artery reconstruction (OR, 2.7; P = .029) for Fontan revision but only older age (OR, 1.06/y; P = .020) for transplantation. Conclusions: Both transplantation and Fontan revision are associated with high morbidity and mortality. There has been evolution of practices in surgical therapy for Fontan failure, perhaps related to rising prevalence of HLHS staged palliation.