Evidence map›Paper›PMID 39712062›Full record

ArticleInternational journal of cardiology. Congenital heart disease2022

Clinical complications of liver disease in adults after the Fontan operation.

Bhavesh Patel, Marina Serper, Emily Ruckdeschel, Sara L Partington, Lauren Andrade, Lynda Tobin, Yuli Kim, Maarouf A Hoteit

Abstract read
In one paragraph

Article in International journal of cardiology. Congenital heart disease, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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1 · What the graph read from it

What it found

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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

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3 · Its place in the literature

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4 · The record

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5 · Who and what money

Authors and funding

8 authors.

Bhavesh PatelInternal Medicine and Pediatrics Residency Program, Hospital of the University of Pennsylvania and Children's Hospital of Philadelphia, 3400 Spruce St, Philadelphia, PA, 19104, USA.
Marina SerperDivision of Gastroenterology and Hepatology, Hospital of the University of Pennsylvania, 3400 Spruce St, Philadelphia, PA, 19104, USA.
Emily RuckdeschelDivision of Cardiology, Children's Hospital of Philadelphia, 3401 Civic Center Blvd, Philadelphia, PA, 19104, USA.
Sara L PartingtonDivision of Cardiology, Hospital of the University of Pennsylvania, 3400 Spruce St, Philadelphia, PA, 19104, USA.
Lauren AndradeDivision of Cardiology, Hospital of the University of Pennsylvania, 3400 Spruce St, Philadelphia, PA, 19104, USA.
Lynda TobinDivision of Cardiology, Hospital of the University of Pennsylvania, 3400 Spruce St, Philadelphia, PA, 19104, USA.
Yuli KimDivision of Cardiology, Hospital of the University of Pennsylvania, 3400 Spruce St, Philadelphia, PA, 19104, USA.
Maarouf A HoteitDivision of Gastroenterology and Hepatology, Hospital of the University of Pennsylvania, 3400 Spruce St, Philadelphia, PA, 19104, USA.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

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.

Indexed as

CirrhosisFontanFontan-associated liver disease

Identifiers

PMID39712062
PMCPMC11657246

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.