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Ethnic disparities in long-term survivors of the Fontan procedure: A population-based study.
Juliet Emamaullee1, Sean Martin1, Cameron Goldbeck1, Brittany Rocque1, Arianna Barbetta1, Rohit Kohli2, Vaughn Starnes1
1Surgery, University of Southern California, Los Angeles, California, United States, 2Pediatrics, Childrens Hospital Los Angeles, Los Angeles, California, United States

Objectives: Fontan-associated liver disease (FALD) has emerged as an unintended secondary comorbidity of the Fontan circulation in patients with univentricular physiology congenital heart disease. With multidisciplinary management, it is estimated that 80% or more of patients post-Fontan may survive into adulthood, leading to concurrent, often silent, FALD progression. Development of cirrhosis can complicate patient management, especially among those requiring heart transplant for a failing Fontan. There is a paucity of data describing long-term outcomes and the impact of FALD in this population. The aim of this study was to perform a population-based analysis of 1) factors associated with overall survival and 2) the prevalence of FALD in patients post-Fontan.

Methods: Patients who underwent the Fontan procedure between 1992-2018 were identified by ICD and CPT codes in the California Office of Statewide Health Planning and Development (OSHPD) Patient Discharge Dataset. Patients were stratified by race/ethnicity and presence of liver disease. Primary outcomes were mortality and transplant. Multivariable Cox regression and Kaplan-Meier survival analyses were performed.

Results: A total of 1436 patients met inclusion criteria. The mean age at Fontan was 4.94.2 years and the median time to last follow-up was 12.6 [8.4, 17.3] years. Overall, 75.9% of patients studied were ?18 years of age at the time of last follow up. There were 864 (60.2%) males and 665 (46.3%) were Hispanic. The overall 20-year survival was 91% (95% CI: 88%-93%), but mortality was higher in Hispanic patients when compared to non-Hispanic White patients (HR 1.49 [1.09-2.03], p=0.012). Only 225 patients (15.7%) were diagnosed with liver disease, although this was more prevalent as patients aged (Figure 1). While there was no difference in the overall number of deaths between patients with and without liver disease (11/225, 4.9% vs 85/1211, 7.0%, p=0.24), liver disease was associated with late death (median 9.6 [6.4, 13.2] years post-Fontan) when compared to patients who died without liver disease (4.1 [1.4, 10.4] years, p=0.02). During the study period, 64 patients underwent heart transplant, including 5 combined heart-liver transplants. Patients with liver disease were more likely to have undergone heart transplant (p<0.01) and have protein losing enteropathy (p<0.01).

Conclusions: In this large, highly diverse population-based study in long term survivors of the Fontan procedure, Hispanic ethnicity was associated with increased all-cause mortality. This finding suggests that strategies designed to overcome ethnic disparities in this population are urgently needed. Further, the prevalence of FALD is currently underrecognized, and our data raises the concern that its incidence increases as this population ages and may be associated with late deaths. These data emphasize the need for development of FALD-specific liver surveillance strategies in patients post-Fontan.


Figure 1: Cumulative incidence of FALD in patients post-Fontan.


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