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Clinical complications of liver disease in adults after the Fontan operation
Bhavesh Patel1, Marina Serper2, Emily Ruckdeschel3,4
1Internal Medicine and Pediatrics Residency Program, Hospital of the University of Pennsylvania and Children's Hospital of Philadelphia, 3400 Spruce St, Philadelphia, PA, 19104, USA.
Insights
Fontan-associated liver disease (FALD) complications are not well-understood. This study identified prior diuretic use, thrombocytopenia, and cyanosis as risk factors for adverse outcomes in adult Fontan patients.
Area of Science:
- Cardiology
- Hepatology
- Adult Congenital Heart Disease
Background:
- Fontan-associated liver disease (FALD) natural history and complications require further characterization.
- Understanding FALD is crucial for managing adult congenital heart disease survivors.
Purpose of the Study:
- To describe the incidence, prevalence, and risk factors for liver-related complications in Fontan-associated liver disease.
- To identify clinical factors associated with adverse outcomes in FALD.
Main Methods:
- Single-center, retrospective cohort study of adult Fontan patients (2006-2019).
- Primary outcome: clinical complications of liver disease (CCLD) including ascites, GI bleeding, hepatic encephalopathy, or HCC.
- Secondary outcome: composite of CCLD, transplantation, or death.
Main Results:
- 182 adult Fontan patients studied with median follow-up of 4.1 years.
- Cumulative incidence of CCLD was 2.6 per 1000 person-years; composite outcome was 6.2 per 1000 person-years.
- Prior diuretic use associated with CCLD (HR 10.03). Diuretic use (HR 16.47), thrombocytopenia (HR 2.30), and cyanosis (HR 5.68) associated with composite outcome.
Conclusions:
- Clinical factors associated with adverse outcomes in FALD were identified.
- Findings may aid in early recognition and management of FALD complications.
- This research highlights the importance of monitoring liver health in Fontan patients.
Background:
The natural history and complications of Fontan-associated liver disease (FALD) are not well-characterized. We aim to describe the incidence and prevalence of and risk factors for adverse liver-related complications in FALD.
Methods:
This is a single-center, retrospective cohort study of adult Fontan patients followed in an adult congenital heart disease center from 2006 to 2019. The primary outcome was clinical complications of liver disease (CCLD) defined as portal hypertension (pHTN)-related ascites, pHTN-related gastrointestinal bleeding, hepatic encephalopathy, or hepatocellular carcinoma. We also explored the composite outcome of CCLD, transplantation, or death.
Results:
Among 182 adult Fontan patients (46% female), median time from Fontan to first liver evaluation was 25.5 years (interquartile range [IQR] 22.3-30.4) with median subsequent follow-up of 4.1 years (IQR 2.5-7.6). There were 14 CCLD, 6 heart-liver and 1 heart transplant, and 16 deaths. The cumulative incidence of CCLD and the composite outcome were 2.6 per 1000-person years and 6.2 per 1000 person-years, respectively. Median time from Fontan to CCLD was 26.8 years (IQR 23.1-31.2), and to the composite outcome 26.6 years (IQR 23.1-30.5). Prior diuretic use was associated with the development of CCLD (hazard ratio [HR] 10.03, 95% confidence interval [CI] 1.25-80.22, P 0.030). Diuretic use (HR 16.47, 95% CI 3.90-69.57, P < 0.001), thrombocytopenia (HR 2.30, 95% CI 1.05-5.01, P 0.036), and cyanosis (HR 5.68, 95% CI 2.47-13.08, P < 0.001) were associated with the composite outcome.
Conclusion:
We identified clinical factors associated with adverse outcomes in FALD that may help guide early recognition of complications and clinical management.
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