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Updated: Sep 20, 2025

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Published on: May 2, 2025
Epidemiologic study of patients after Fontan operation based on Medical Aid Program for Chronic Pediatric Diseases of
Takanari Fujii1, Akinori Moriichi2, Naoko Kakee2
1Pediatric Heart Disease and Adult Congenital Heart Disease Center, Showa University Hospital, Tokyo, Japan.
Insights
This study analyzed nationwide data of pediatric patients who underwent the Fontan procedure in Japan. It clarifies the current status and outcomes for young individuals with single-ventricle physiology, aiding disease management.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease Epidemiology
Background:
- Nationwide data on single-ventricle physiology patients are scarce.
- The Medical Aid Program for Chronic Pediatric Diseases of Specified Categories in Japan supports children under 20 with chronic conditions.
Purpose of the Study:
- To investigate the epidemiology and medium- to long-term outcomes of patients following a Fontan procedure using registry data.
- To examine the utility of this epidemiologic investigation for identifying real-world objectives and clinical applications.
Main Methods:
- Utilized the database of the Medical Aid Program for Chronic Pediatric Diseases of Specified Categories.
- Identified 2862 patients who underwent a Fontan operation from 18,589 registered patients with chronic heart disease.
- Evaluated symptoms, treatment, and somatic growth data.
Main Results:
- Clarified nationwide data on the current status of post-Fontan patients under 20 years of age in Japan.
- Provided insights into the comprehensive status of pediatric patients with chronic heart disease.
Conclusions:
- The study elucidated the current status of post-Fontan patients under 20 years in Japan.
- Data analysis contributed to improved disease management for patients with single-ventricle physiology.
Abstract:
Nationwide registry data of patients with single-ventricle physiology have been rarely reported. The Medical Aid Program for Chronic Pediatric Diseases of Specified Categories (Japan) has contributed to the financial support of medical expense for patients younger than 20 years with chronic paediatric disease, and almost all children in Japan who require disease-specific treatment voluntarily apply to this programme. The epidemiology and medium- to long-term outcome of patients following a Fontan procedure were investigated using the database. The usefulness of this epidemiologic investigation in identifying real-world objectives and clinical applications was also examined. A total of 2862 patients who underwent a Fontan operation were identified from 18,589 patients with chronic heart disease registered to the medical aid programme. The details of symptoms, treatment, and somatic growth were evaluated, from which we were able to clarify the nationwide data regarding the current status of post-Fontan patients younger than 20 years. This study elucidated the current status of post-Fontan patients under 20 years of age in Japan. Data analysis of the Medical Aid Program for Chronic Pediatric Diseases of Specified Categories cohort provided useful information towards understanding the comprehensive status of patients with chronic heart disease and contributed to improved disease management.
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