Objective This tertiary centre study aims to identify factors associated with adverse outcomes in adult survivors with total cavopulmonary connection (TCPC) Fontan palliation for single ventricle. Methods This retrospective review of medical records identified adult (≥18 years) survivors of TCPC Fontan palliation who were followed at a single tertiary centre between 1 January 2000 and 1 July 2019. Adverse outcomes were defined as arrhythmia, pacemaker/implantable cardioverter defibrillator placement, liver cirrhosis, protein losing enteropathy, hospitalisation for heart failure, thromboembolic complication and/or death. Results 160 adult TCPC patients met the inclusion criteria: 117 (73.1%) extracardiac and 43 (26.9%) lateral tunnel. The median (IQR) duration of follow-up since TCPC palliation was 17.5 (11.8–21.3) years. An adverse outcome occurred in 87 (54.4%) patients. Adverse outcome-free survival rates at 10, 20 and 25 years post TCPC were 89% (95% CI 82% to 93%), 60% (95% CI 50% to 69%) and 24% (95% CI 15% to 35%), respectively. On multivariate analysis, extracardiac Fontan (HR 2.21, 95% CI 1.20 to 4.08, p=0.011) was observed to be an independent risk factor for adverse outcomes after adjusting for age, race, morphology of the systemic ventricle and history of fenestration. Conclusions In this single-centre retrospective study of adult survivors of TCPC palliation, extracardiac Fontan was associated with an increased hazard for adverse outcomes. This finding could guide clinicians in developing risk modification strategies and management decisions to improve long-term outcomes in these patients. Data are available upon reasonable request. The data used in these analyses include potentially identifying participant information and therefore are not publicly available due to legal and ethical restrictions. Please contact the corresponding author to obtain access to de-identified data.

中文翻译：

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单心室全腔肺 Fontan 姑息治疗成年幸存者的结局

目的 本次三级中心研究旨在确定与单心室全腔肺连接 (TCPC) Fontan 姑息治疗成年幸存者不良结局相关的因素。方肝硬化、蛋白质丢失性肠病、因心力衰竭住院、血栓栓塞并发症和/或死亡。结果 160 名成年 TCPC 患者符合纳入标准：117 名（73.1%）心外患者和 43 名（26.9%）侧管患者。自 TCPC 姑息治疗后的中位 (IQR) 随访持续时间为 17.5 (11.8–21.3) 年。87 名 (54.4%) 患者出现不良结局。TCPC 后 10、20 和 25 年的无不良结果生存率为 89%（95% CI 82% 至 93%）、60%（95% CI 50% 至 69%）和 24%（95% CI 15%）到 35%），分别。在多变量分析中，在调整年龄、种族、全身心室形态和开窗病史后，观察到心外 Fontan（HR 2.21，95% CI 1.20 至 4.08，p=0.011）是不良结局的独立危险因素。结论 在这项针对 TCPC 姑息治疗成年幸存者的单中心回顾性研究中，心外 Fontan 与不良结局风险增加相关。这一发现可以指导临床医生制定风险修正策略和管理决策，以改善这些患者的长期结果。可根据合理要求提供数据。这些分析中使用的数据包括可能识别参与者的信息，因此由于法律和道德限制，无法公开获得。请联系相应的作者以获得对去标识化数据的访问权限。