Pediatric Cardiology and Cardiac Arrhythmia Unit, Department of Pediatric Cardiology and Cardiac Surgery, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.
Cardiac Surgery Unit, Department of Pediatric Cardiology and Cardiac Surgery, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.
Eur J Cardiothorac Surg. 2022 Jun 15;62(1). doi: 10.1093/ejcts/ezac301.
We sought to evaluate the long-term survival and risk factors for morbidity and mortality in our cohort of patients after extracardiac (EC) Fontan.
We collected and analysed follow-up data until September 2020 for all patients who underwent EC Fontan operation at our institution from November 1988 to November 2015.
Out of 406 patients treated with EC Fontan at a mean age of 5.4 ± 4.5 years, 372 (91.6%) were discharged home; 15% were lost to follow-up. The median follow-up was 14.6 years (interquartile range 8.7-20.3). Two hundred patients (54%) had a good long-term outcome, with an event-free long-term survival, but late adverse events of any kind needing interventional or surgical reoperations were reported in 128 patients (34%). Forty-four patients (12%) developed late Fontan failure. Thirty-seven survivors (10%) had late arrhythmias, with the need for pacemaker implantation. Obstruction of the cavopulmonary pathway occurred in 49 patients (13%), but just 8 patients needed conduit surgical replacement. At 32 years, the survival probability was 84%. The risk of orthotopic heart transplant, considering death as a competing event (showed as cumulative incidence), was 12.5%. A preoperative diagnosis of hypoplastic left heart syndrome was an independent risk factor for the need for heart transplant.
The EC Fontan shows satisfactory long-term survival and low incidence of adverse events and late failure. Conduit replacement is rare, and its longevity may not represent a substantial issue in these patients. However, as with other technical variants, the need for reoperations during long-term follow-up is a significant challenge.
我们旨在评估我们的心脏外（EC）Fontan 术后患者队列的长期生存率以及发病率和死亡率的相关风险因素。
我们收集并分析了 1988 年 11 月至 2015 年 11 月期间在我院接受 EC Fontan 手术的所有患者的随访数据，直至 2020 年 9 月。
在接受 EC Fontan 治疗的 406 例患者中，平均年龄为 5.4±4.5 岁，其中 372 例（91.6%）出院回家；15%的患者失访。中位随访时间为 14.6 年（四分位间距 8.7-20.3）。200 例（54%）患者有良好的长期预后，无事件长期生存率，但有 128 例（34%）患者报告了任何类型的晚期不良事件，需要介入或手术再次手术。44 例（12%）患者出现晚期 Fontan 衰竭。37 例存活者（10%）发生晚期心律失常，需要植入起搏器。49 例（13%）患者发生腔肺静脉通道阻塞，但仅 8 例患者需要更换导管。32 年后，生存率为 84%。考虑到死亡作为竞争事件（表现为累积发生率），同种异体心脏移植的风险为 12.5%。术前诊断为左心发育不全综合征是需要心脏移植的独立危险因素。
EC Fontan 显示出令人满意的长期生存率和较低的不良事件及晚期衰竭发生率。很少需要更换导管，其长期寿命在这些患者中可能不是一个实质性问题。然而，与其他技术变异一样，在长期随访期间需要再次手术是一个重大挑战。
