Osawa Takumi, Machino-Ohtsuka Tomoko, Numata Ruriko, Kuraoka Ayako, Saji Mike, Kanaoka Koshiro, Sumita Yoko, Kawamatsu Naoto, Kato Hideyuki, Hiramatsu Yuji, Ishizu Tomoko
Department of Cardiology, Institute of Medicine, University of Tsukuba.
Department of Cardiology, Fukuoka Children's Hospital.
Circ J. 2025 Jun 25;89(7):946-956. doi: 10.1253/circj.CJ-24-0843. Epub 2025 May 25.
Adult congenital heart disease (ACHD) patients often require additional interventions or surgeries in adulthood, presenting new clinical challenges. However, clinical research on the current status and outcomes of cardiac procedures in ACHD patients remains limited.
We analyzed the Japanese Registry of All Cardiac and Vascular Diseases-Diagnosis Procedure Combination (JROAD-DPC) database between April 2013 and March 2021. Patients with ACHD (aged >15 years) who underwent major cardiac surgery and transcatheter procedures were included. We assessed clinical background, treatment, length of hospital stay, and in-hospital mortality. In all, 22,490 patients with ACHD (median age 56 years [interquartile range 36-69 years], 51.1% female) were enrolled. Emergency hospitalizations and in-hospital deaths were observed in 3.7% and 1.1% of cases, respectively. Congenital heart operations with high in-hospital mortality (>5.0%) included aortic arch repair, systemic-to-pulmonary artery shunts, cardiac tumor resection, coronary artery bypass grafting, 3-valve replacement, and ventricular assist device implantation. Although stent graft procedures had the highest in-hospital mortality rate (2.6%), other transcatheter procedures, such as transcatheter patent ductus arteriosus closure, atrial septal defect closure, and catheter ablation, had in-hospital mortality rates of <1.0%.
This study provides fundamental insights into the current clinical characteristics and outcomes associated with procedures in patients with ACHD. The in-hospital mortality rates for both cardiac surgery and transcatheter procedures in Japanese ACHD patients were low, demonstrating acceptable outcomes.
成人先天性心脏病（ACHD）患者在成年期常需要额外的干预措施或手术，这带来了新的临床挑战。然而，关于ACHD患者心脏手术现状及结果的临床研究仍然有限。
我们分析了2013年4月至2021年3月期间的日本全心脏和血管疾病诊断程序组合登记处（JROAD-DPC）数据库。纳入接受心脏大手术和经导管手术的ACHD患者（年龄>15岁）。我们评估了临床背景、治疗情况、住院时间和住院死亡率。总共纳入了22490例ACHD患者（中位年龄56岁[四分位间距36 - 69岁]，女性占51.1%）。分别有3.7%的病例出现急诊住院情况，1.1%的病例出现住院死亡。住院死亡率高（>5.0%）的先天性心脏手术包括主动脉弓修复、体肺分流术、心脏肿瘤切除术、冠状动脉旁路移植术、三尖瓣置换术和心室辅助装置植入术。尽管覆膜支架手术的住院死亡率最高（2.6%），但其他经导管手术，如经导管动脉导管未闭封堵术、房间隔缺损封堵术和导管消融术，住院死亡率<1.0%。
本研究为ACHD患者手术相关的当前临床特征和结果提供了基本见解。日本ACHD患者心脏手术和经导管手术的住院死亡率均较低，显示出可接受的结果。
