Big Data in Transplantation Practice-the Devil Is in the Detail-Fontan-associated Liver Disease

Transplantation. 2021 Jan 1;105(1):18-22. doi: 10.1097/TP.0000000000003308.


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 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.

Methods: A multidisciplinary group within the American Society for Transplantation analyzed several administrative datasets 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.

MeSH terms

  • Adolescent
  • Adult
  • Age Factors
  • Aged
  • Aged, 80 and over
  • Big Data*
  • Child
  • Child, Preschool
  • Data Collection
  • Data Mining
  • Databases, Factual
  • Female
  • Fontan Procedure / adverse effects*
  • Heart Defects, Congenital / surgery*
  • Humans
  • Infant
  • Infant, Newborn
  • Liver Diseases / diagnosis
  • Liver Diseases / epidemiology*
  • Liver Diseases / surgery
  • Liver Transplantation
  • Male
  • Middle Aged
  • Risk Assessment
  • Risk Factors
  • Treatment Outcome
  • Young Adult