Journal article
Research priorities in Fontan-associated liver disease
Current opinion in organ transplantation, Vol.25(5), pp.489-495
10/01/2020
DOI: 10.1097/MOT.0000000000000803
PMID: 32833705
Abstract
Purpose of review Fontan-associated liver disease (FALD) is an emerging condition in patients who have undergone surgical correction of univentricular congenital heart disease. There is little known about the epidemiology of FALD, including risk factors for end-organ failure or hepatocellular carcinoma nor a consensus on surveillance guidelines. Furthermore, there is a need to understand the role of heart versus combined heart-liver transplantation in this population. Research is limited by systemic barriers hindering the ability to track longitudinal FALD outcomes. Recent findings Nearly all patients post-Fontan develop histological features of FALD as a function of time post-Fontan, regardless of Fontan hemodynamics. In cases of end-organ disease, single-center studies have shown promising outcomes of combined heart-liver transplant in this population, with decreased rates of acute rejection. However, despite the burden of disease, it is not currently possible to identify the population of patients with FALD using existing clinical databases and registries due to a lack of diagnostic codes. Strategies proposed to address barriers to understanding FALD include developing appropriate diagnostic and transplant-related codes for existing registries. Efforts should also be targeted at initiating prospective studies to understand recognized comorbidities related to Fontan physiology, guided by a team of multidisciplinary subspecialists.
Details
- Title: Subtitle
- Research priorities in Fontan-associated liver disease
- Creators
- Sara Khan - Univ Southern Calif, Keck Sch Med, Los Angeles, CA 90007 USAHassan Aziz - University of Southern CaliforniaJuliet Emamaullee - University of Southern California
- Resource Type
- Journal article
- Publication Details
- Current opinion in organ transplantation, Vol.25(5), pp.489-495
- Publisher
- Lippincott Williams & Wilkins
- DOI
- 10.1097/MOT.0000000000000803
- PMID
- 32833705
- ISSN
- 1087-2418
- eISSN
- 1531-7013
- Number of pages
- 7
- Grant note
- R01 HL141857-01 / National Heart, Lung, and Blood Institute of the National Institutes of Health; United States Department of Health & Human Services; National Institutes of Health (NIH) - USA; NIH National Heart Lung & Blood Institute (NHLBI)
- Language
- English
- Date published
- 10/01/2020
- Academic Unit
- Surgery
- Record Identifier
- 9984701551102771
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