Status of adult outpatients with congenital heart disease in Japan: The Japanese Network of Cardiovascular Departments for Adult Congenital Heart Disease Registry
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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日本成人先天性心脏病门诊患者现状：日本成人先天性心脏病登记心血管科网络
DOI：
10.1016/j.jjcc.2022.07.019
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发表时间：
2022
影响因子：
2.5
通讯作者：
Naga
中科院分区：
文献类型：
--
作者：
Yao Atsushi;Inuzuka Ryo;Mizuno Atsushi;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;Naga
日本成人先天性心脏病心血管科网络（JNCVD-ACHD）成立于2011年，旨在为成人先天性心脏病患者（ACHD患者）提供终身护理。方法和结果从2011年到2019年，JNCVD-ACHD在日本47个县中的32个县注册了54家为ACHD患者提供专业护理的机构。该登记处收集了来自50个机构的24，048名患者的疾病概况数据以及来自24个机构的9743名患者的患者特征数据。最常见的ACHD是房间隔缺损（20.5%）、室间隔缺损（20.5%）、法洛四联症（12.9%）和单心室（UVH）/单心室（SV; 6.6%）。未经双心室修复的ACHD患者占总人口的37.0%。还检查了严重的解剖学和/或病理生理学疾病，如肺动脉高压（3.0%），包括艾森曼格综合征（1.2%）、双室循环下的系统性右心室（sRV-2 VC; 2.8%）和Fontan生理学（6.0%）。sRV-2 VC病例包括未经解剖修复的先天性矫正性大动脉转位（61.9%）和接受心房转换手术的大动脉转位（38.1%）。Fontan生理学的主要病因（86.4%）是UVH/SV。此外，发育/染色体/遗传性疾病是异位综合征（无脾，0.9%;多脾，0.7%），21三体（4.0%），22q11.2缺失（0.9%），特纳综合征（0.2%），马凡综合征（1.1%）。对于理想的ACHD护理，JNCVD-ACHD的预期目标是创建本地网络，并为多中心临床试验提供资源，以支持循证实践。
BackgroundThe 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 resultsFrom 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 %).ConclusionsAlthough 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.