Lee Sang-Yun, Kim Soo-Jin, Lee Chang-Ha, Park Chun Soo, Choi Eun Seok, Ko Hoon, An Hyo Soon, Kang I Seok, Yoon Ja Kyoung, Baek Jae Suk, Lee Jae Young, Song Jinyoung, Lee Joowon, Huh June, Ahn Kyung-Jin, Jung Se Yong, Cha Seul Gi, Kim Yeo Hyang, Lee Youngseok, Cho Sanghoon
Department of Pediatrics, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Korea.
Department of Pediatrics, Bucheon Sejong Hospital, Bucheon, Korea.
Korean Circ J. 2024 Oct;54(10):653-668. doi: 10.4070/kcj.2023.0211. Epub 2024 Jul 8.
This study aimed to analyze the outcomes of Fontan surgery in the Republic of Korea, as there were only a few studies from Asian countries.
The medical records of 1,732 patients who underwent Fontan surgery in 10 cardiac centers were reviewed.
Among them, 1,040 (58.8%) were men. The mean age at Fontan surgery was 4.3±4.2 years, and 395 (22.8%) patients presented with heterotaxy syndrome. According to the types of Fontan surgery, 157 patients underwent atriopulmonary (AP) type; 303, lateral tunnel (LT) type; and 1,266, extracardiac conduit (ECC) type. The overall survival rates were 91.7%, 87.1%, and 74.4% at 10, 20, and 30 years, respectively. The risk factors of early mortality were male, heterotaxy syndrome, AP-type Fontan surgery, high mean pulmonary artery pressure (mPAP) in pre-Fontan cardiac catheterization, and early Fontan surgery year. The risk factors of late mortality were heterotaxy syndrome, genetic disorder, significant atrioventricular valve regurgitation (AVVR) before Fontan surgery, high mPAP in pre-Fontan cardiac catheterization, and no fenestration.
In Asian population with a high incidence of heterotaxy syndrome, the heterotaxy syndrome was identified as the poor prognostic factors for Fontan surgery. The preoperative low mPAP and less AVVR are associated with better early and long-term outcomes of Fontan surgery.
由于亚洲国家仅有少数关于此的研究，本研究旨在分析韩国的Fontan手术结果。
回顾了10个心脏中心1732例行Fontan手术患者的病历。
其中男性1040例（58.8%）。Fontan手术时的平均年龄为4.3±4.2岁，395例（22.8%）患者患有内脏异位综合征。根据Fontan手术类型，157例行心房肺动脉（AP）型；303例行侧隧道（LT）型；1266例行心外管道（ECC）型。10年、20年和30年的总生存率分别为91.7%、87.1%和74.4%。早期死亡的危险因素为男性、内脏异位综合征、AP型Fontan手术、Fontan术前心脏导管检查时平均肺动脉压（mPAP）高以及Fontan手术年份早。晚期死亡的危险因素为内脏异位综合征、遗传疾病、Fontan手术前明显的房室瓣反流（AVVR）、Fontan术前心脏导管检查时mPAP高以及无开窗。
在内脏异位综合征高发的亚洲人群中，内脏异位综合征被确定为Fontan手术预后不良的因素。Fontan手术术前mPAP低和AVVR少与更好的早期和长期结果相关。
