Ono Masamichi, Boethig Dietmar, Goerler Heidi, Lange Melanie, Westhoff-Bleck Mechthild, Breymann Thomas
Division of Thoracic and Cardiovascular Surgery, Hannover Medical School, Hannover, Germany.
Eur J Cardiothorac Surg. 2006 Dec;30(6):923-9. doi: 10.1016/j.ejcts.2006.08.025. Epub 2006 Oct 30.
The Fontan operation has been proposed as definitive palliation for an increasing variety of hearts with complex univentricular anatomy, but late morbidity after Fontan operation is still a matter of concern. This retrospective study evaluates the late outcome in patients with Fontan circulation.
We included 121 consecutive patients that underwent Fontan operation between 1984 and 2004. Modifications of the Fontan operation included atriopulmonary anastomosis (APA; n = 28), total cavopulmonary connection (TCPC; n = 63), and fenestrated TCPC (f-TCPC; n = 30). Mean age was 5.8+/-5.5 years. Post operative mortality, morbidity, hemodynamics, and somatic development were analyzed.
Actuarial survival was 87% at 20 years after Fontan operation. There were 10 early deaths, 5 late deaths, and 2 takedowns followed by successful conversion and heart transplantation. Among 108 early-survivors with Fontan circulation, 19 underwent reoperation, including 3 conversions of APA to TCPC. Freedom from reoperation was 76% at 20 years. Freedom from intervention was 34% at 20 years. Freedom from tachyarrhythmia or pacemaker implantation was 23% and 77%, respectively at 20 years. Heterotaxy and atrioventricular valve anomaly were risk factors for late failure and tachyarrhythmias. Patients with fenestrated TCPC had reduced incidence of late tachyarrhythmias, and patients with APA who developed collaterals showed low incidence of late tachyarrythmia. Postoperative sinus node dysfunction or tachyarrhythmias was associated with significantly lower cardiac index. Somatic development was gradually compensated after Fontan operation. Weight normalized completely 15 years postoperatively.
Long-term survival after Fontan procedure is encouraging, but late morbidity remains suboptimal. During follow-up, emerging complications should be managed by surgical and interventional procedures. Fenestration in Fontan circulation provided better cardiac output and lower incidence of late tachyarrhythmias, suggesting a benefit of fenestration for late outcome.
对于越来越多具有复杂单心室解剖结构的心脏，Fontan手术已被提议作为最终的姑息治疗方法，但Fontan手术后的晚期发病率仍是一个令人担忧的问题。这项回顾性研究评估了接受Fontan循环的患者的晚期结局。
我们纳入了1984年至2004年间连续接受Fontan手术的121例患者。Fontan手术的改良包括心房肺吻合术（APA；n = 28）、全腔肺连接术（TCPC；n = 63）和开窗TCPC（f-TCPC；n = 30）。平均年龄为5.8±5.5岁。分析了术后死亡率、发病率、血流动力学和身体发育情况。
Fontan手术后20年的精算生存率为87%。有10例早期死亡、5例晚期死亡，2例拆除手术，随后成功转换和心脏移植。在108例具有Fontan循环的早期幸存者中，19例接受了再次手术，包括3例将APA转换为TCPC。20年时无需再次手术的比例为76%。20年时无需干预的比例为34%。20年时无室上性心律失常或起搏器植入的比例分别为23%和77%。内脏异位和房室瓣异常是晚期衰竭和室上性心律失常的危险因素。开窗TCPC患者晚期室上性心律失常的发生率降低，发生侧支循环的APA患者晚期室上性心律失常的发生率较低。术后窦房结功能障碍或室上性心律失常与心脏指数显著降低有关。Fontan手术后身体发育逐渐得到代偿。术后15年体重完全恢复正常。
Fontan手术后的长期生存率令人鼓舞，但晚期发病率仍不理想。在随访期间，新出现的并发症应通过手术和介入程序进行处理。Fontan循环中的开窗术可提供更好的心输出量和更低的晚期室上性心律失常发生率，表明开窗术对晚期结局有益。
