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Tytuł:
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Status of adult outpatients with congenital heart disease in Japan: The Japanese Network of Cardiovascular Departments for Adult Congenital Heart Disease Registry.
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Autorzy:
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Yao A; Division for Health Service Promotion, the University of Tokyo, Bunkyo-ku, Tokyo, Japan. Electronic address: .
Inuzuka R; Pediatric Department, the University of Tokyo Hospital, Bunkyo-ku, Tokyo, Japan.
Mizuno A; Department of Cardiology, St. Luke's International Hospital, Choo-ku, Tokyo, Japan.
Iwano H; Department of Cardiovascular Medicine, Hokkaido University Graduate School of Medicine, Sapporo, Hokkaido, Japan.
Tatebe S; Department of Cardiovascular Medicine, Tohoku University Graduate School of Medicine, Sendai, Miyagi, Japan.
Tsukamoto Y; Department of Cardiovascular Medicine, Osaka University Graduate School of Medicine, Suita, Osaka, Japan.
Sakamoto I; Department of Cardiovascular Medicine, Kyushu University, Fukuoka, Fukuoka, Japan.
Watanabe H; Department of Cardiovascular Medicine, Akita University Graduate School of Medicine, Akita, Akita, Japan.
Fukuda N; Second Department of Internal Medicine, University of Toyama, Toyama, Toyama, Japan.
Takechi F; Department of Pediatrics, Chiba Cerebral and Cardiovascular Center, Ichihara, Chiba, Japan.
Adachi S; Department of Cardiology, Nagoya University Hospital, Nagoya, Aichi, Japan.
Akazawa Y; Department of Cardiology, Pulmonology, Hypertension and Nephrology, Ehime University Graduate School of Medicine, Toonn, Ehime, Japan.
Kuwahara K; Department of Cardiovascular Medicine, Shinshu University School of Medicine, Matsumoto, Nagano, Japan.
Dohi K; Department of Cardiology and Nephrology, Mie University Graduate School of Medicine, Tsu, Mie, Japan.
Ishizu T; Division of Cardiology, University of Tsukuba, Tsukuba, Ibaragi, Japan.
Miyake M; Department of Cardiology, Tenri Hospital, Tenri, Nara, Japan.
Koitabashi N; Department of Cardiovascular Medicine, Gunma University Graduate School of Medicine, Maebashi, Gunma, Japan.
Hasegawa-Tamba S; Saitama Medical University International Medical Center, Hidaka, Saitama, Japan.
Sato S; Division of Pediatric Cardiology, Okinawa Prefectural Nanbu Medical Center & Children's Medical Center, Shimajiri-gun, Okinawa, Japan.
Fujii T; Pediatric Heart Disease and Adult Congenital Heart Disease Center, Showa University Hospital, Shinagawa-ku, Tokyo, Japan.
Ehara E; Department of Pediatric Cardiology, Osaka City General Hospital, Osaka, Osaka, Japan.
Minamino T; Department of Cardiovascular Biology and Medicine, Juntendo University Graduate School of Medicine, Bunkyo-ku, Tokyo, Japan.
Yamada H; Department of Community Medicine for Cardiology, Tokushima University Graduate School of Biomedical Sciences, Tokushima, Tokushima, Japan.
Yamashita E; Division of Cardiology, Gunma Prefectural Cardiovascular Center, Maebashi, Gunma, Japan.
Kawamatsu N; Mito Saiseikai General Hospital, Department of Cardiology, Mito, Ibaragi, Japan.
Masuda K; Department of Cardiology, Yokohama Rosai Hospital, Yokohama, Kanagawa, Japan.
Soma K; Cardiovascular Department, the University of Tokyo Hospital, Bunkyo-ku, Tokyo, Japan.
Shiraishi I; Department of Pediatric Cardiology, National Cerebral and Cardiovascular Center, Suita, Osaka, Japan.
Nagai R; Jichi Medical University, Shimotsuke, Tochigi, Japan.
Niwa K; Department of Cardiology, St. Luke's International Hospital, Choo-ku, Tokyo, Japan.
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Corporate Authors:
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JNCVD-ACHD investigators
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Źródło:
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Journal of cardiology [J Cardiol] 2022 Dec; Vol. 80 (6), pp. 525-531. Date of Electronic Publication: 2022 Aug 19.
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Typ publikacji:
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Multicenter Study; Journal Article; Research Support, Non-U.S. Gov't
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Język:
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English
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Imprint Name(s):
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Publication: Amsterdam : Elsevier
Original Publication: Tokyo : Japanese College Of Cardiology
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MeSH Terms:
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Heart Defects, Congenital*/epidemiology
Heart Defects, Congenital*/surgery
Transposition of Great Vessels*/surgery
Adult ; Humans ; Japan/epidemiology ; Prospective Studies ; Outpatients ; Registries
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Contributed Indexing:
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Keywords: Adult congenital heart disease; JNCVD-ACHD; Lifelong care
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Entry Date(s):
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Date Created: 20220822 Date Completed: 20221020 Latest Revision: 20221221
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Update Code:
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20240104
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DOI:
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10.1016/j.jjcc.2022.07.019
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PMID:
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35995687
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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.
Competing Interests: Declaration of competing interest The authors declare that there is no conflict of interest.
(Copyright © 2022 The Authors. Published by Elsevier Ltd.. All rights reserved.)
