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

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)
doi:10.9794/jspccs.35.172

原著Original

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

廣野 恵一1，宮尾 成明2，小林 徹3，盛一 享德4，寶田 真也2，岡部 真子2，仲岡 英幸2，小栗 真人2，伊吹 圭二郞2，小澤 綾佳2，市田 蕗子2Keiichi Hirono1, Nariaki Miyao2, Tohru Kobayashi3, Akinori Moriichi4, Shinya Takarada2, Mako Okabe2, Hideyuki Nakaoka2, Masato Oguri2, Keijiro Ibuki2, Sayaka Ozawa2, Fukiko Ichida2

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
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【背景】心筋緻密化障害は未だ臨床像，管理方法，予後など不明な点が多い．

【目的と方法】我が国における心筋緻密化障害の実態を調査するため，2004～2013年の小児慢性特定疾病レポジトリーから心筋症患者をリクルートし，その登録施設へアンケート調査を実施した．

【結果】心筋症は1,360例であった．309施設にアンケートを送付し，116施設（37.5％）から470例の回答を得た．心筋緻密化障害は46例（男女比29対17）で診断年齢は日齢0～16歳（中央値0.3歳）であった．診断の契機は症状（47.8%），学校心臓検診（8.7%），乳幼児健診（4.4%）の順で多く，経過中の症状は心不全（58.7%），不整脈（20.0%），塞栓症（2.2%）であった．治療は強心薬，利尿薬，抗血小板薬，抗凝固薬，末梢血管拡張薬，β遮断薬などが使用されていた．学校生活管理指導はE可（19.5%），E禁（13.0%），D（23.9%），C（4.3%），B（2.1%）となっていた．

【結語】小児慢性特定疾病の登録症例では治療を必要としたより重症例が登録される傾向を有することを念頭に置き，継続した調査と検討が必要であると思われた．

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

© 2019 特定非営利活動法人日本小児循環器学会© 2019 Japanese Society of Pediatric Cardiology and Cardiac Surgery

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This page was last modified on 2019-10-09T17:57:51.000+09:00

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