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

被引：0

作者：

Yao, Atsushi ^{[1
,30
]}

Inuzuka, Ryo ^{[2
]}

Mizuno, Atsushi ^{[3
]}

Iwano, Hiroyuki ^{[4
]}

Tatebe, Shunsuke ^{[5
]}

Tsukamoto, Yasumasa ^{[6
]}

Sakamoto, Ichiro ^{[7
]}

Watanabe, Hiroyuki ^{[8
]}

Fukuda, Nobuyuki ^{[9
]}

Takechi, Fumie ^{[10
]}

Adachi, Shiro ^{[11
]}

Akazawa, Yusuke ^{[12
]}

Kuwahara, Koichiro ^{[13
]}

Dohi, Kaoru ^{[14
]}

Ishizu, Tomoko ^{[15
]}

Miyake, Makoto ^{[16
]}

Koitabashi, Norimichi ^{[17
]}

Hasegawa-Tamba, Saki ^{[18
]}

Sato, Seiichi ^{[19
]}

Fujii, Takanari ^{[20
]}

Ehara, Eiji

Minamino, Tohru ^{[22
]}

Yamada, Hirotsugu ^{[23
]}

Yamashita, Eiji ^{[21
,24
]}

Kawamatsu, Naoto ^{[25
]}

Masuda, Keita ^{[26
]}

Soma, Katsura ^{[27
]}

Shiraishi, Isao ^{[28
]}

Nagai, Ryozo ^{[29
]}

Niwa, Koichiro ^{[3
]}

JNCVD-ACHD Investigators

机构：

[1] Univ Tokyo, Div Hlth Serv Promot, Bunkyo ku, Tokyo, Japan

[2] Univ Tokyo Hosp, Pediat Dept, Bunkyo Ku, Tokyo, Japan

[3] St Lukes Int Hosp, Dept Cardiol, Choo Ku, Tokyo, Japan

[4] Hokkaido Univ, Dept Cardiovasc Med, Grad Sch Med, Sapporo, Hokkaido, Japan

[5] Tohoku Univ, Dept Cardiovasc Med, Grad Sch Med, Sendai, Miyagi, Japan

[6] Osaka Univ, Dept Cardiovasc Med, Grad Sch Med, Suita, Osaka, Japan

[7] Kyushu Univ, Dept Cardiovasc Med, Fukuoka, Fukuoka, Japan

[8] Akita Univ, Dept Cardiovasc Med, Grad Sch Med, Akita, Akita, Japan

[9] Univ Toyama, Dept Internal Med 2, Toyama, Toyama, Japan

[10] Chiba Cerebral & Cardiovasc Ctr, Dept Pediat, Ichihara, Chiba, Japan

[11] Nagoya Univ Hosp, Dept Cardiol, Nagoya, Aichi, Japan

[12] Ehime Univ, Grad Sch Med, Dept Cardiol Pulmonol Hypertens & Nephrol, Toonn, Ehime, Japan

[13] Shinshu Univ, Dept Cardiovasc Med, Sch Med, Matsumoto, Nagano, Japan

[14] Mie Univ, Dept Cardiol & Nephrol, Grad Sch Med, Tsu, Mie, Japan

[15] Univ Tsukuba, Div Cardiol, Tsukuba, Ibaragi, Japan

[16] Tenri Hosp, Dept Cardiol, Tenri, Nara, Japan

[17] Gunma Univ, Dept Cardiovasc Med, Grad Sch Med, Maebashi, Gunma, Japan

[18] Saitama Med Univ, Int Med Ctr, Hidaka, Saitama, Japan

[19] Okinawa Prefectural Nanbu Med Ctr, Div Pediat Cardiol, Haebaru, Okinawa, Japan

[20] Showa Univ Hosp, Pediat Heart Dis & Adult Congenital Heart Dis Ctr, Shinagawa Ku, Tokyo, Japan

[21] Osaka City Gen Hosp, Dept Pediat Cardiol, Osaka, Osaka, Japan

[22] Juntendo Univ, Dept Cardiovasc Biol & Med, Grad Sch Med, Bunkyo Ku, Tokyo, Japan

[23] Tokushima Univ, Dept Community Med Cardiol, Grad Sch Biomed Sci, Tokushima, Tokushima, Japan

[24] Gunma Prefectural Cardiovasc Ctr, Div Cardiol, Maebashi, Gunma, Japan

[25] Mito Saiseikai Gen Hosp, Dept Cardiol, Mito, Ibaragi, Japan

[26] Yokohama Rosai Hosp, Dept Cardiol, Yokohama, Kanagawa, Japan

[27] Univ Tokyo Hosp, Cardiovasc Dept, Bunkyo ku, Tokyo, Japan

[28] Natl Cerebral & Cardiovasc Ctr, Dept Pediat Cardiol, Suita, Osaka, Japan

[29] Jichi Med Univ, Shimotsuke, Tochigi, Japan

[30] Univ Tokyo, Div Hlth Serv Promot, 7-3-1 Hongo,Bunkyo Ku, Tokyo 1130033, Japan

来源：

JOURNAL OF CARDIOLOGY | 2022年 / 80卷 / 06期

关键词：

JNCVD-ACHD; Lifelong care; Adult congenital heart disease; SEPTAL-DEFECT; PREVALENCE;

D O I：

10.1016/j?cc.2022.07.019

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

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.(c) 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/).

引用

页码：525 / 531

页数：7

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