In Hospital Outcomes in Adult Congenital Heart Disease Patients with Fontan Undergoing Heart Transplantation - A Decade Nationwide Analysis from 2004 until 2014
Hernandez GA, Lemor A, Clark D, Blumer V, Byrne R, Fowler R, Sandhaus E, Weingarten A, Frischhertz B, Schlendorf K, Zalawadiya S, Lindenfeld J, and Menachem J. In Hospital Outcomes in Adult Congenital Heart Disease Patients with Fontan Undergoing Heart Transplantation - A Decade Nationwide Analysis from 2004 until 2014. J Heart Lung Transplant 2019; 38(4 Suppl ):S110.
J Heart Lung Transplant
Purpose: Treatment of adult congenital heart disease (ACHD) patients who require heart transplantation (HT) remains challenging due to limited donors and high perioperative and wait-list mortality. Those patients with single ventricle physiology palliated with Fontan are at the highest risk of early mortality due to multi-organ involvement; in addition, few centers are capable of safely transplant them. We sought to evaluate trends and outcomes of HT in these patients. Methods: Using the U. S Nationwide Inpatient Sample (NIS) database, we identified all adults aged at least 18 years old who underwent HT from 2004-2014. We then identified those with specific ICD-9 codes to include tricuspid atresia, hypoplastic left heart syndrome and common ventricle, who in order to survive to age 18, must have been palliated with a Fontan procedure. Multivariate regression models were created to adjust for potential confounders. Results: A total of 93 Fontan patients underwent HT during the study time (0.5% of all heart transplants). Compared to non-Fontan heart transplantations, Fontan patients were younger, with higher incidence of liver disease and coagulopathy (baseline demographics are shown in table 1). Fontan patients receiving HT had higher mortality during transplant hospitalization compared to non-Fontan patients (26.3% vs. 5.3% OR 18.10, CI 5.06 - 65.0 p<0.001). ECMO usage and bleeding were also higher in the Fontan cohort with an OR of 5.30 (p=0.016) and 5.32 (p=0.015) for ECMO and bleeding, respectively. The remaining outcomes were similar for both cohorts. Conclusion: Fontan patients undergoing HT have exceptionally high inpatient mortality, which is nearly 5 times that of non-Fontan heart transplant recipients. The role of delayed referral, underlying liver disease, and other factors in this high mortality need to be explored.