| Item type |
学術雑誌論文 / Journal Article(1) |
| 公開日 |
2023-01-30 |
| タイトル |
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|
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 |
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|
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 |
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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
Inuzuka, Ryo
Mizuno, Atsushi
Iwano, Hiroyuki
Tatebe, Shunsuke
Tsukamoto, Yasumasa
Sakamoto, Ichiro
Watanabe, Hiroyuki
Fukuda, Nobuyuki
Fumie, Takechi
Adachi, Shiro
Akazawa, Yusuke
Kuwahara, Koichiro
Dohi, Kaoru
Ishizu, Tomoko
Miyake, Makoto
Koitabashi, Norimichi
Hasegawa-Tamba, Saki
Sato Seiichi
Fujii, Takanari
Ehara, Eiji
Minamino, Tohru
Yamada, Hirotsugu
Yamashita, Eiji
Kawamatsu, Naoto
Masuda, Keita
Soma, Katsura
Shiraishi, Isao
Nagai Ryozo
Niwa, Koichiro
|
| 内容記述 |
|
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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
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| 収録物識別子 |
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|
収録物識別子タイプ |
ISSN |
|
収録物識別子 |
09145087 |
| 出版者 |
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|
出版者 |
Japanese College of Cardiology (Nippon-Sinzobyo-Gakkai) |
| 関連情報 |
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|
関連タイプ |
isIdenticalTo |
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|
識別子タイプ |
DOI |
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|
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/). |
iA_2022_22.pdf (679.6 kB)
