Online ISSN: 2187-2988 Print ISSN: 0911-1794
特定非営利活動法人日本小児循環器学会 Japanese Society of Pediatric Cardiology and Cardiac Surgery
Pediatric Cardiology and Cardiac Surgery 35(3): 172-178 (2019)


小児慢性特定疾病レポジトリーに基づくアンケート結果からみた心筋緻密化障害の臨床像Clinical Features of Noncompaction Cardiomyopathy: A Questionnaire-Based Survey in Japan

1富山大学附属病院周産期母子センターDepartment of Maternity and Perinatal Care Unit, Toyama University Hospital ◇ Toyama, Japan

2富山大学附属病院周産期母子センター小児科Department of Pediatrics, Toyama University Hospital ◇ Toyama, Japan

3国立成育医療研究センター臨床研究センター企画運営部Division of Clinical Research Planning, Department of Development Strategy, the Center for Clinical Research, National Center for Child Health and Development ◇ Tokyo, Japan

4国立成育医療研究センター研究所小児慢性特定疾病情報室Division of Specific Pediatric Chronic Disease Information the Research Institute, National Center for Child Health and Development ◇ Tokyo, Japan

受付日:2019年2月8日Received: February 8, 2019
受理日:2019年5月24日Accepted: May 24, 2019
発行日:2019年9月1日Published: September 1, 2019





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.

Key words: noncompaction cardiomyopathy; repository; specific chronic diseases

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