Abstract 17794: Modes of Failure and Cause of Death in Children with Functional Single Ventricle Physiology Following Contemporary Total Cavopulmonary Connection Completion

Background: We hypothesized that modes of Fontan failure may predict the mode and timing of subsequent death following the contemporary form of Fontan completion. Methods: Patient records were reviewed for 38 (7.6%) patients that died after staged palliation and subsequent total cavopulmonary connection (TCPC) (n=500; extracardiac, n=326; lateral tunnel, n=174) performed from 1985 to 2012. Patient characteristics, modes of failure, and causes of death were classified. Results: There were 23 (4.6%) hospital deaths and 15 (3%) late deaths. There has been no hospital death since 2000. Survival has significantly improved over time (p<0.001, Figure 1A). Primary modes of failure included ventricular systolic dysfunction (n=19, 50%), ventricular diastolic dysfunction (n=7, 18%), pulmonary vascular dysfunction (n=2, 5%), thromboembolism (n=2, 5%), and other modes (n=7, 18%). Cardiac-related Fontan failure is mainly associated with early death in the early era (p=0.048, Figure 1A,B). Direct cause of death was hear...