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Abstract Details
Big Data in Transplantation Practice - the Devil Is in the Detail - Fontan-associated Liver Disease
Transplantation. 2020 Jul 2. doi: 10.1097/TP.0000000000003308. Online ahead of print.
Michelle H Kim1, Ailene Nguyen1, Mary Lo2, S Ram Kumar3, John Bucuvalas4, Earl F Glynn5, Mark A Hoffman5, Ryan Fischer6, Juliet Emamaullee17
Author information
1Liver Transplant Center, Children's Hospital-Los Angeles, Los Angeles, CA.
2Department of Preventive Medicine, University of Southern California, Los Angeles, CA.
3Division of Cardiothoracic Surgery, Department of Surgery, University of Southern California, and Heart Institute, Children's Hospital, Los Angeles, both in Los Angeles, CA.
4Division of Pediatric Hepatology, Recanati/Miller Transplantation Institute, Icahn School of Medicine at Mount Sinai, New York, NY.
5Children's Research Institute, Children's Mercy Hospital, Kansas City, MO.
6Department of Gastroenterology, Liver Care Center, Children's Mercy Hospital, Kansas City, MO.
7Liver Transplant Center, University of Southern California, Los Angeles, CA.
Abstract
Background: As a result of the Fontan procedure, the prognosis of congenital single-ventricle heart disease has improved, with many affected children surviving into adulthood. However, the unanticipated consequences of chronic exposure to Fontan hemodynamics has revealed a new set of secondary noncardiac complications. Fontan-associated liver disease (FALD) is characterized by progressive hepatic fibrosis in nearly all patients post-Fontan, with the potential to develop cirrhosis, hepatocellular carcinoma, and the need for liver transplantation. A lack of data regarding FALD-related prognosis makes consideration of indications for and timing of heart alone versus combined heart-liver transplantation challenging.
Methods: A multidisciplinary group within the American Society for Transplantation analyzed several administrative datasets in order to study the epidemiology of FALD.
Results: This approach presented several obstacles, and efforts to characterize FALD were limited by a lack of Fontan- and FALD-specific diagnostic codes and an inability to follow individual patients through multiple health systems. Several ongoing Fontan registries were also reviewed but these do not adequately capture FALD-related variables. Such barriers highlight the need for large-scale data collection in patients post-Fontan to better understand and care for this complex population.
Conclusions: This study emphasizes the challenges of studying emerging transplant-related diagnoses in existing datasets and the need for mechanisms to adapt registries to appropriately identify patients with rare or emerging conditions.