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

作者：Atsushi Yao, Ryo Inuzuka, Atsushi Mizuno, Hiroyuki Iwano, Shunsuke Tatebe, Yasumasa Tsukamoto, Ichiro Sakamoto, Hiroyuki Watanabe, Nobuyuki Fukuda, Fumie Takechi, Shiro Adachi, Yusuke Akazawa, Koichiro Kuwahara, Kaoru Dohi, Tomoko Ishizu, Makoto Miyake, Norimichi Koitabashi, Saki Hasegawa-Tamba, Seiichi Sato, Takanari Fujii, Eiji Ehara, Tohru Minamino, Hirotsugu Yamada, Eiji Yamashita, Naoto Kawamatsu, Keita Masuda, Katsura Soma, Isao Shiraishi, Ryozo Nagai, Koichiro Niwa · 发表于：Journal of Cardiology · 年份：2022 · DOI：10.1016/j.jjcc.2022.07.019 · 被引用次数：15 · 研究领域：Congenital Heart Disease Studies、Congenital heart defects research、Tracheal and airway disorders

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 (asplen...