Objective Given the paucity of long-term outcome data for complex congenital heart disease (CHD), we aimed to describe the treatment pathways and survival for patients who started interventions for functionally univentricular heart (FUH) conditions, excluding hypoplastic left heart syndrome. Methods We performed a retrospective cohort study using all procedure records from the National Congenital Heart Diseases Audit for children born in 2000–2018. The primary outcome was mortality, ascertained from the Office for National Statistics in 2020. Results Of 53 615 patients, 1557 had FUH: 55.9% were boys and 67.4% were of White ethnic groups. The largest diagnostic categories were tricuspid atresia (28.9%), double inlet left ventricle (21.0%) and unbalanced atrioventricular septal defect (AVSD) (15.2%). The ages at staged surgery were: initial palliation 11.5 (IQR 5.5–43.5) days, cavopulmonary shunt 9.2 (IQR 6.0–17.1) months and Fontan 56.2 (IQR 45.5–70.3) months. The median follow-up time was 10.8 (IQR 7.0–14.9) years and the 1, 5 and 10-year survival rates after initial palliation were 83.6% (95% CI 81.7% to 85.4%), 79.4% (95% CI 77.3% to 81.4%) and 77.2% (95% CI 75.0% to 79.2%), respectively. Higher hazards were present for unbalanced AVSD HR 2.75 (95% CI 1.82 to 4.17), atrial isomerism HR 1.75 (95% CI 1.14 to 2.70) and low weight HR 1.65 (95% CI 1.13 to 2.41), critical illness HR 2.30 (95% CI 1.67 to 3.18) or acquired comorbidities HR 2.71 (95% CI 1.82 to 4.04) at initial palliation. Conclusion Although treatment pathways for FUH are complex and variable, nearly 8 out of 10 children survived to 10 years. Longer-term analyses of outcome based on diagnosis (rather than procedure) can inform parents, patients and clinicians, driving practice improvements for complex CHD. Data are available subject to legal data sharing agreements with the data providers (NCHDA, NHS Digital) and data sharing requires ethical approval and CAG approval. Study data are held subject to a data sharing agreements with the data providers. Requests for data sharing would be subject to approvals by the National Cardiac Audit Programme, the Health Quality Improvement Partnership and NHS Digital.

中文翻译：

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英格兰和威尔士干预功能性单心室心脏的队列研究（2000-2018）

目的 鉴于缺乏复杂先​​天性心脏病 (CHD) 的长期结果数据，我们旨在描述开始干预功能性单心室心脏 (FUH) 疾病（不包括左心发育不良综合征）的患者的治疗途径和生存率。方法 我们使用国家先天性心脏病审计的所有程序记录对 2000-2018 年出生的儿童进行了一项回顾性队列研究。主要结果是死亡率，从 2020 年国家统计局确定。结果在 53615 名患者中，1557 名患有 FUH：55.9% 为男孩，67.4% 为白人。最大的诊断类别是三尖瓣闭锁（28.9%）、双入口左心室（21.0%）和不平衡房室间隔缺损（AVSD）（15.2%）。分期手术的年龄是：初始姑息治疗 11.5 (IQR 5.5–43.5) 天，腔静脉分流术 9.2 (IQR 6.0–17.1) 个月和 Fontan 56.2 (IQR 45.5–70.3) 个月。中位随访时间为 10.8 (IQR 7.0–14.9) 年，初始缓解后的 1、5 和 10 年生存率为 83.6%（95% CI 81.7% 至 85.4%）、79.4%（95% CI 77.3 % 至 81.4%) 和 77.2% (95% CI 75.0% 至 79.2%)。不平衡 AVSD HR 2.75（95% CI 1.82 至 4.17）、心房异构 HR 1.75（95% CI 1.14 至 2.70）和低体重 HR 1.65（95% CI 1.13 至 2.41）、危重病 HR 2.30（95 % CI 1.67 至 3.18）或获得性合并症 HR 2.71（95% CI 1.82 至 4.04）在初始缓解时。结论 尽管 FUH 的治疗途径复杂且多变，但 10 名儿童中有近 8 名存活至 10 年。基于诊断（而不是手术）对结果的长期分析可以为父母、患者和临床医生提供信息，从而推动复杂 CHD 的实践改进。根据与数据提供者（NCHDA、NHS Digital）的法律数据共享协议，数据可用，并且数据共享需要道德批准和 CAG 批准。研究数据根据与数据提供者的数据共享协议进行保存。数据共享请求将得到国家心脏审计计划、健康质量改善伙伴关系和 NHS Digital 的批准。研究数据根据与数据提供者的数据共享协议进行保存。数据共享请求将得到国家心脏审计计划、健康质量改善伙伴关系和 NHS Digital 的批准。研究数据根据与数据提供者的数据共享协议进行保存。数据共享请求将得到国家心脏审计计划、健康质量改善伙伴关系和 NHS Digital 的批准。