Open AccessBig Data in Transplantation Practice—the Devil Is in the Detail—Fontan-associated Liver Disease
Author(s) -
Michelle H. Kim,
Andrew Loc Nguyen,
Mary Lo,
S. Ram Kumar,
John Bucuvalas,
Earl F. Glynn,
Mark A. Hoffman,
Ryan T. Fischer,
Juliet Emamaullee
Publication year - 2020
Publication title -
transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.45
H-Index - 204
eISSN - 1534-6080
pISSN - 0041-1337
DOI - 10.1097/tp.0000000000003308
Subject(s) - medicine , fontan procedure , transplantation , liver transplantation , lung transplantation , intensive care medicine , heart disease , cirrhosis , liver disease , population , cardiology , environmental health
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 have 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.