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  1. 20 医学系研究科・医学部
  2. 20A 学術誌論文
  3. 20A1 雑誌掲載論文

Status of adult outpatients with congenital heart disease in Japan: The Japanese Network of Cardiovascular Departments for Adult Congenital Heart Disease Registry

http://hdl.handle.net/10295/00006148
http://hdl.handle.net/10295/00006148
d2e0eef3-5a2d-4cc3-abc8-e46612360cce
名前 / ファイル ライセンス アクション
iA_2022_22.pdf iA_2022_22.pdf (679.6 kB)
Item type 学術雑誌論文 / Journal Article(1)
公開日 2023-01-30
タイトル
タイトル Status of adult outpatients with congenital heart disease in Japan: The Japanese Network of Cardiovascular Departments for Adult Congenital Heart Disease Registry
言語 en
言語
言語 eng
主題
主題Scheme Other
主題 JNCVD-ACHD
主題
主題Scheme Other
主題 Lifelong care
主題
主題Scheme Other
主題 Adult congenital heart disease
資源タイプ
資源タイプ識別子 http://purl.org/coar/resource_type/c_6501
資源タイプ journal article
アクセス権
アクセス権 open access
アクセス権URI http://purl.org/coar/access_right/c_abf2
作成者 Yao, Atsushi

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en Yao, Atsushi

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Inuzuka, Ryo

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Mizuno, Atsushi

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Iwano, Hiroyuki

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Tatebe, Shunsuke

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Tsukamoto, Yasumasa

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Sakamoto, Ichiro

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Watanabe, Hiroyuki

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Fukuda, Nobuyuki

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Fumie, Takechi

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Adachi, Shiro

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Akazawa, Yusuke

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Kuwahara, Koichiro

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Dohi, Kaoru

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Ishizu, Tomoko

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Miyake, Makoto

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Koitabashi, Norimichi

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Hasegawa-Tamba, Saki

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Sato Seiichi

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Fujii, Takanari

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Ehara, Eiji

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Minamino, Tohru

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Yamada, Hirotsugu

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Yamashita, Eiji

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Kawamatsu, Naoto

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Masuda, Keita

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Soma, Katsura

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Shiraishi, Isao

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Nagai Ryozo

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Niwa, Koichiro

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内容記述
内容記述タイプ Abstract
内容記述 Background
The Japanese Network of Cardiovascular Departments for Adult Congenital Heart Disease (JNCVD-ACHD) was founded in 2011 for the lifelong care of adult patients with congenital heart disease (ACHD patients). This network maintains the first Japanese ACHD registry.
Methods and results
From 2011 to 2019, the JNCVD-ACHD registered 54 institutions providing specialized care for ACHD patients in 32 of the 47 prefectures in Japan. The registry collected data on the disease profile for 24,048 patients from 50 institutions and the patient characteristics for 9743 patients from 24 institutions. The most common ACHDs were atrial septal defect (20.5 %), ventricular septal defect (20.5 %), tetralogy of Fallot (12.9 %), and univentricular heart (UVH)/single ventricle (SV; 6.6 %). ACHD patients without biventricular repair accounted for 37.0 % of the population. Also examined were the serious anatomical and/or pathophysiological disorders such as pulmonary arterial hypertension (3.0 %) including Eisenmenger syndrome (1.2 %), systemic right ventricle under biventricular circulation (sRV-2VC; 2.8 %), and Fontan physiology (6.0 %). The sRV-2VC cases comprised congenitally corrected transposition of the great arteries without anatomical repair (61.9 %) and transposition of the great arteries with atrial switching surgery (38.1 %). The primary etiology (86.4 %) for Fontan physiology was UVH/SV. In addition, developmental/chromosomal/genetic disorders were heterotaxy syndromes (asplenia, 0.9 %; polysplenia, 0.7 %), trisomy 21 (4.0 %), 22q11.2 deletion (0.9 %), Turner syndrome (0.2 %), and Marfan syndrome (1.1 %).
Conclusions
Although the specific management of ACHD has systematically progressed in Japan, this approach is still evolving. For ideal ACHD care, the prospective goals for the JNCVD-ACHD are to create local networks and provide a resource for multicenter clinical trials to support evidence-based practice.
言語 en
出版タイプ
出版タイプ VoR
出版タイプResource http://purl.org/coar/version/c_970fb48d4fbd8a85
書誌情報 en : Journal of Cardiology

巻 80, 号 6, p. 525-531, 発行日 2022
収録物識別子
収録物識別子タイプ ISSN
収録物識別子 09145087
出版者
出版者 Japanese College of Cardiology (Nippon-Sinzobyo-Gakkai)
関連情報
関連タイプ isIdenticalTo
識別子タイプ DOI
関連識別子 https://doi.org/10.1016/j.jjcc.2022.07.019
権利情報
権利情報 © 2022 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
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