Abstract 14415: Clinical Characteristics and Predictors of the “Good Fontan”: A Multi-Institutional Study

Abstract

Introduction: Fifteen-year survival after Fontan operation approaches 95%, but morbidities are increasingly prevalent. It is unknown how well cardiologists can predict which pts are at risk for major adverse events (MAE), but awareness is critical to enable appropriate counseling. We asked cardiologists the “surprise” question: would you be surprised if your patient has a MAE in the next year? Methods: We performed a prospective, multi-center study across New England. The attending cardiologist was asked the yes/no “surprise” question. Inclusion criteria included lateral tunnel or extracardiac Fontan and age >10 yrs; individuals were excluded for current transplant evaluation, history of Fontan conversion, or pregnancy. Baseline clinical data were collected. After 12 months, cardiologists were surveyed to assess for MAE (see Table) and multivariate analysis (MVA) was performed for those deemed to be at risk and those with MAE. Results: From 2018-19, 146 pts and 40 cardiologists were enrolled at 9 sites. 99/146 pts (68%) were predicted to be a “Good Fontan.” After 1 yr, 17 (12%) patients experienced a MAE (Table). The simple kappa coefficient of ability to predict MAE was 0.17 (95% CI 0.02-0.32), suggesting ability to predict MAE was only 17% better than random chance. In MVA of those at risk (47/146), diuretic/beta blocker use (p=<0.0001) and systolic dysfunction (p=0.0046) were associated with identified risk. MVA of pts who experienced MAE (n=17) found prior unplanned cardiac admission (p=0.0062), diuretic/beta blocker use (p=0.028) and significant AV valve regurgitation (p=0.049) to be associated with MAE. Conclusions: Cardiologists are only marginally able to identify which Fontan pts are at risk for MAE in the next year. There was overlap between factors associated with prediction of “at risk” group and MAE, namely use of diuretic/blocker and the linked systolic dysfunction/AV valve regurgitation, which merits close attention in follow-up and further study.