Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, Royal Brompton & Harefield Hospitals, Guy's and St Thomas' NHS Foundation Trust, Sydney Street, SW3 6NP London, UK.
National Heart and Lung Institute, Imperial College London, Guy Scadding Building, Dovehouse Street, SW3 6LY London, UK.
Eur J Prev Cardiol. 2024 Aug 22;31(11):1316-1323. doi: 10.1093/eurjpc/zwae031.
To evaluate the late outcomes of adults (above 35 years) with a Fontan-type circulation, for whom current data on morbidity and mortality are lacking.
Data were collected retrospectively on consecutive patients with Fontan circulation above the age of 35 years followed in three European specialist centres. Overall, 115 Fontan patients were included [median age 35 (range 35-48) years, 47.8% female]. The most common underlying congenital heart disease diagnosis was tricuspid atresia (n = 58, 50.4%), and the age at first Fontan completion was 9.1 (interquartile range 5.0-15.8) years. Almost two-thirds (61.7%) of patients had undergone an atriopulmonary Fontan, and 23.5% had received a total cavopulmonary connection. One-third required repeat surgery or intervention. Most patients (55.9%) were in New York Heart Association functional class II or class I (30.6%), 76 (66.1%) patients had experienced at least one arrhythmia, and eight (7.0%) protein-losing enteropathy. At a median follow-up of 5.0 (2.4-10.3) years, 15 (13.0%) patients were referred for transplantation assessment and 19 (16.5%) patients died, mainly from heart failure (84.2%). Univariable predictors of death or transplantation included lower serum albumin level [hazard ratio (HR) 1.09 per g/L decrease, 95% confidence interval (CI): 1.04-1.15, P = 0.0009], prior heart failure admission (HR 4.28, 95% CI:1.75-10.44, P = 0.001), prior atrial tachycardia or flutter (HR 3.02, 95% CI: 1.23-7.38, P = 0.02), and baseline pulmonary vasodilator therapy (HR 8.59, 95% CI:1.05-70.13, P = 0.04). Lower serum albumin and prior atrial tachycardia or flutter remained significant on bivariable analysis.
Our study highlights the significant morbidity and mortality in older adults with a Fontan-type circulation, emphasizing the need for lifelong specialist surveillance with frequent risk stratification, close monitoring, and early consideration for transplantation assessment.
评估成人（35 岁以上）Fontan 循环的晚期结果，因为目前缺乏有关发病率和死亡率的数据。
本研究回顾性收集了三个欧洲专科中心连续接受 Fontan 循环治疗的 35 岁以上患者的数据。共有 115 例 Fontan 患者入组[中位年龄 35（范围 35-48）岁，47.8%为女性]。最常见的先天性心脏病诊断是三尖瓣闭锁（n=58，50.4%），首次 Fontan 完成的年龄为 9.1（四分位距 5.0-15.8）岁。近三分之二（61.7%）的患者接受了房室连接的 Fontan 手术，23.5%接受了全腔静脉肺动脉连接术。三分之一的患者需要再次手术或介入治疗。大多数患者（55.9%）纽约心脏协会心功能分级为 II 级或 I 级（30.6%），76 例（66.1%）患者至少发生过一次心律失常，8 例（7.0%）患者发生蛋白丢失性肠病。中位随访 5.0（2.4-10.3）年后，15 例（13.0%）患者因需要接受移植评估而转诊，19 例（16.5%）患者死亡，主要死于心力衰竭（84.2%）。单变量分析显示，死亡或移植的预测因素包括血清白蛋白水平较低[每降低 1g/L 的风险比（HR）为 1.09，95%置信区间（CI）：1.04-1.15，P=0.0009]、既往心力衰竭入院（HR 4.28，95%CI：1.75-10.44，P=0.001）、既往房性心动过速或房扑（HR 3.02，95%CI：1.23-7.38，P=0.02）和基线肺血管扩张剂治疗（HR 8.59，95%CI：1.05-70.13，P=0.04）。双变量分析显示，较低的血清白蛋白和既往房性心动过速或房扑仍然是显著的预测因素。
我们的研究强调了成人 Fontan 循环患者的显著发病率和死亡率，强调了需要进行终生的专科监测，进行频繁的风险分层、密切监测，并尽早考虑进行移植评估。
