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Published on: September 13, 2014
Big Data in Transplantation Practice-the Devil Is in the Detail-Fontan-associated Liver Disease
Michelle H Kim1, Ailene Nguyen1, Mary Lo2
1Liver Transplant Center, Division of Abdominal Organ Transplantation, Department of Surgery, Children's Hospital-Los Angeles, Los Angeles, CA.
Insights
Fontan-associated liver disease (FALD) is a growing concern for single-ventricle heart patients. Current data collection methods hinder FALD research, necessitating improved registries for better patient care and understanding.
Area of Science:
- Cardiology
- Hepatology
- Transplantation Science
Background:
- The Fontan procedure improves survival for congenital single-ventricle heart disease, enabling patients to reach adulthood.
- Chronic Fontan hemodynamics lead to secondary noncardiac complications, notably Fontan-associated liver disease (FALD).
- FALD involves progressive hepatic fibrosis, potentially causing cirrhosis, hepatocellular carcinoma, and liver transplant needs.
Purpose of the Study:
- To analyze the epidemiology of Fontan-associated liver disease (FALD).
- To identify challenges in studying FALD using existing administrative and registry data.
- To highlight the need for improved data collection for FALD research.
Main Methods:
- Analysis of administrative datasets by a multidisciplinary group from the American Society for Transplantation.
- Review of ongoing Fontan registries to assess their capacity for capturing FALD-related variables.
Main Results:
- Studying FALD epidemiology was hindered by a lack of specific diagnostic codes for Fontan and FALD.
- Inability to track individual patients across multiple health systems limited data analysis.
- Existing Fontan registries were insufficient for capturing crucial FALD-related information.
Conclusions:
- Studying emerging transplant-related diagnoses like FALD presents significant challenges with current datasets.
- There is a critical need for adaptable registry mechanisms to identify patients with rare or emerging conditions.
- Enhanced data collection strategies are essential for advancing the understanding and management of FALD.
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.
