日本小児循環器学会雑誌 Pediatric Cardiology and Cardiac Surgery

Background: Noncompaction cardiomyopathy is characterized by a left ventricle with a prominent trabecular meshwork. The clinical courses of the growing number of pediatric cases with noncompaction cardiomyopathy remain only partially elucidated.

Methods: We enrolled patients with cardiomyopathies from the database of Specific Pediatric Chronic Diseases in Japan. Questionnaires were sent to institutions throughout Japan to obtain details about the diagnosis, management, and prognosis of these patients.

Results: We enrolled 1,360 patients and obtained clinical data for 470, among whom 46 had noncompaction cardiomyopathy (29 males, 17 females; median age: 0.3 years). Among these patients, 22 displayed symptoms at diagnosis, 4 were detected by school medical screening, and 2 were detected by infant medical screening. Symptomatically, 27 patients had heart failure, 9 had arrhythmia, and 1 had embolism, and almost all the patients received medical therapy (e.g., inotropes, diuretics, antiplatelets, anticoagulants, vasodilators, or β-blockers). Based on the school-life guidance and management in Japan, patients were classified as follows: E-sports club activities allowed (19.5%), E-sports club activities prohibited (13.0%), D (23.9%), C (4.3%), and B (2.1%).

Conclusions: It is imperative that we continue to survey and assess cases of specific pediatric chronic disease associated with cardiomyopathy for inclusion in the repository.