Acute Decompensated Heart Failure among Patients Underwent Fontan Procedures

Most patients with single-ventricle (SV) heart disease can now reach adulthood after Fontan operation. Acute decompensated heart failure (ADHF) is one of the serious complications after Fontan operation, however, its incidence and outcome are not well understood. The study aims to investigate the incidence and clinical characteristics of patients diagnosed with ADHF requiring hospitalization after Fontan procedure in our institutions. A retrospective analysis of 655 patients (387 males) who have completed Fontan procedure in Fukuoka Children's Hospital (FCH) between 1984 and 2015 and followed at FCH and Kyushu University Hospital were conducted. The Fontan procedures included extracardiac conduit (82.7%), lateral tunnel (13.4%), atriopulmonary connection (2.3%), and others (1.5%). The mean observation period after Fontan procedure was 9.8 ± 6.8 years (0 - 31.6 years). Twenty-one patients (3.2%) were hospitalized due to right-sided ADHF with a mean time of 9.4 ± 6.6 years after Fontan procedure. Among them, 7 patients (1.1%) died and the most common cause of death was multiple organ failure. By univariate analysis, older age at Fontan operation, SV dysfunction, significant atrioventricular valve regurgitation, and asplenia were associated with the development of ADHF. In conclusion, ADHF is less common, but it remains a devastating complication after Fontal procedure.