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 heart failure (n=18, 47%), multi-organ failure (n=4, 11%), pulmonary failure (n=3, 8%), cerebral complications (n=2, 5%), arrhythmia (n=1, 3%), renal failure (n=1, 3%), sudden death (n=6, 16%), and unknown (n=3, 8%). The causes of the 11 late deaths (>1 year post-Fontan) were sudden death (n=3, 27%), heart failure (n=3, 27%) and others (n=5, 45%). Among 5 patients with protein-losing enteropathy (PLE), 2 (40%) died from PLE and its complications. Modes of failure were directly related to cause of death in 19 (50%) patients. Autopsy in 10 patients showed chronic myocardial change in 9 and lung congestion or edema in 7.
Conclusion: Early deaths due to acute ventricular failure seen in the earlier eras of TCPC experience have been largely overcome. Late death up to 15 years following TCPC completion is uncommon and the mode of failure and subsequent cause of death are variable. The mode of Fontan failure predicted the cause of death in approximately half of the patient group.
- © 2012 by American Heart Association, Inc.