Luijten Linda W G, van den Bosch Eva, Duppen Nienke, Tanke Ronald, Roos-Hesselink J, Nijveld Aagje, van Dijk Arie, Bogers Ad J J C, van Domburg Ron, Helbing Willem A
Department of Paediatrics (Cardiology), Erasmus University Medical Center-Sophia Children's Hospital, Rotterdam, the Netherlands Department of Paediatrics (Cardiology), University Medical Center St Radboud, Nijmegen, the Netherlands.
Department of Paediatrics (Cardiology), Erasmus University Medical Center-Sophia Children's Hospital, Rotterdam, the Netherlands Department of Paediatrics (Cardiology), University Medical Center St Radboud, Nijmegen, the Netherlands Department of Radiology, Erasmus University Medical Center, Rotterdam, the Netherlands.
Eur J Cardiothorac Surg. 2015 Mar;47(3):527-34. doi: 10.1093/ejcts/ezu182. Epub 2014 May 6.
The surgical approach to repair of tetralogy of Fallot (ToF) has shifted over the years. We aimed to report the long-term follow-up after ToF repair with the transatrial-transpulmonary approach and to determine predictors of long-term outcomes.
Retrospective analysis of patients operated on in two tertiary referral centres. Primary outcome measures were: death, pulmonary valve replacement (PVR), reintervention for other reasons, internal cardiodefibrillator and/or pacemaker placement. Kaplan-Meier assessment of overall and event-free survival as well as uni- and multivariate analyses of risk factors for outcomes were performed.
Four hundred and fifty-three patients were included. Median age at operation was: 0.6 years (range 0-19.6) and median age at the last follow-up was 14.3 years (range 0.1-42.1). Median age at repair decreased from 1.2 years (range 0.6-5.8) (1970-80) to 0.3 years (range 0-4.7) (2000-12). A transannular patch (TP) was used in 65% of all patients. The use of a TP showed a decline from 89% in the initial years of the cohort to 64% in 2000-12. Early mortality was 1.1% (5 patients) for the entire cohort and late mortality 2.4% (11 patients). Overall survival for the entire cohort was 97.3% (95% CI 95.7-98.8) and 91.8% (95% CI 85.9-97.7) at 10 and 25 years, respectively. For patients with a TP (n = 294) vs non-TP (n = 159), this was 97.2% (95% CI 95.2-99.2) vs 97.5% (95% CI 95.1-99.9) at 10-year and 91.0% (95% CI 83.9-98.1) vs 96.3% (95% CI 93.0-99.6) at 25-year follow-up (P = 0.958). Fifty-two patients underwent PVR, and in 5 a pacemaker was inserted. Event-free survival for TP versus non-TP patients was 80.2% (95 CI% 75.5-84.9) vs 81.7% (95% CI 75.2-88.2) at 10-year and 27.9% (95% CI 17.7-38.1) vs 78.5% (95% CI 71.4-85.6) at 25-year follow-up (P = 0.016). In multivariate analysis, both the use of a TP (HR 1.705, 95% CI 1.023-2.842) and the year of surgical repair of tetralogy of Fallot (HR 1.039, 95% CI 1.006-1.073) were associated with a higher probability of an event.
ToF patients corrected with the transatrial-transpulmonary approach have good long-term survival. PVR is a frequent event at longer follow-up, and other events are limited. The use of a TP is a predictor for poorer event-free outcomes, increasing the risk of the composite endpoint 1.7 times.
多年来，法洛四联症（ToF）的手术修复方法已经发生了转变。我们旨在报告经心房-肺动脉途径修复ToF后的长期随访情况，并确定长期预后的预测因素。
对在两个三级转诊中心接受手术的患者进行回顾性分析。主要结局指标为：死亡、肺动脉瓣置换（PVR）、因其他原因再次干预、植入体内心脏除颤器和/或起搏器。采用Kaplan-Meier法评估总体生存率和无事件生存率，并对结局的危险因素进行单因素和多因素分析。
共纳入453例患者。手术时的中位年龄为：0.6岁（范围0 - 19.6岁），最后一次随访时的中位年龄为14.3岁（范围0.1 - 42.1岁）。修复时的中位年龄从1970 - 1980年的1.2岁（范围0.6 - 5.8岁）降至2000 - 2012年的0.3岁（范围0 - 4.7岁）。所有患者中有65%使用了跨环补片（TP）。TP的使用从队列最初几年的89%降至2000 - 2012年的64%。整个队列的早期死亡率为1.1%（5例患者），晚期死亡率为2.4%（11例患者）。整个队列在10年和25年时的总体生存率分别为97.3%（95%CI 95.7 - 98.8）和91.8%（95%CI 85.9 - 97.7）。对于使用TP的患者（n = 294）与未使用TP的患者（n = 159），10年时分别为97.2%（95%CI 95.2 - 99.2）和97.5%（95%CI 95.1 - 99.9），25年随访时分别为91.0%（95%CI 83.9 - 98.1）和96.3%（95%CI 93.0 - 99.6）（P = 0.958）。52例患者接受了PVR，5例患者植入了起搏器。TP组与非TP组患者的无事件生存率在10年时分别为80.2%（95CI% 75.5 - 84.9）和81.7%（95%CI 75.2 - 88.2），25年随访时分别为27.9%（95%CI 17.7 - 38.1）和78.5%（95%CI 71.4 - 85.6）（P = 0.016）。在多因素分析中，使用TP（HR 1.705，95%CI 1.023 - 2.842）和法洛四联症手术修复年份（HR 1.039，95%CI 1.006 - 1.073）均与事件发生概率较高相关。
经心房-肺动脉途径矫正的ToF患者具有良好的长期生存率。在较长时间的随访中，PVR是常见事件，其他事件较少。使用TP是无事件结局较差的预测因素，使复合终点风险增加1.7倍。
