COMBINED HEART AND LIVER TRANSPLANTATION: Edited by Juliet Emamaullee and Eugene DePasqualeResearch priorities in Fontan-associated liver diseaseKhan, Saraa; Aziz, Hassana,b; Emamaullee, Julieta,bAuthor Information aKeck School of Medicine bDepartment of Surgery, University of Southern California, Los Angeles, California, USA Correspondence to Juliet Emamaullee, MD, PhD, FRCSC FACS, Assistant Professor of Clinical Surgery, Department of Surgery, University of Southern California, 1510 San Pablo St, Suite 412, Los Angeles, CA 90033, USA. Tel: +1 323 442 5908; e-mail: [email protected] Current Opinion in Organ Transplantation: October 2020 - Volume 25 - Issue 5 - p 489-495 doi: 10.1097/MOT.0000000000000803 Buy Metrics 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. Summary 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. Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.