Dennis Mark, Moore Ben, Kotchetkova Irina, Pressley Lynne, Cordina Rachael, Celermajer David S
Sydney Medical School, University of Sydney, Sydney, New South Wales, Australia.
Department of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia.
Open Heart. 2017 Mar 1;4(1):e000564. doi: 10.1136/openhrt-2016-000564. eCollection 2017.
Survival of patients with repaired tetralogy of Fallot (rToF) into young adulthood is very good. Concerns exist, however, over long-term morbidity and mortality as these subjects reach middle age. We aimed to assess survival and the prevalence of complications in patients with rToF seen in our Adult Congenital Heart Disease (ACHD) service.
One hundred and sixty-eight consecutive patients with 'simple rToF', aged over 16 years, followed up at our tertiary-level ACHD service in Sydney, Australia since 2000, were included. We documented mortality and analysed the prospectively defined composite end points of (a) 'Serious adverse events', including death, heart failure hospitalisation and/or documented ventricular arrhythmia and (b) 'Adverse events' inclusive of the above and endocarditis, atrial arrhythmia, defibrillator and/or pacemaker implantation.
Mean age at the last follow-up was 34±12 years, and 55% were men. There were 10 (6%) deaths, and 26 patients (16%) experienced a 'serious adverse event'. Fifty-one patients (30%) experienced an 'adverse event' and 29 patients had atrial arrhythmias. One hundred and one (61%) patients had at least one pulmonary valve replacement. By age 40 years, 93% were free of serious adverse events, and 83% were free of any adverse event. By age 50 years, only 56% had not had an adverse event. Older age and history of atrial arrhythmia were predictive of serious adverse events.
Survival into mid-adulthood in patients with rToF is very good; however, a substantial number of survivors have adverse events by the age of 50 years.
法洛四联症修复术后（rToF）患者存活至青年期的情况非常良好。然而，随着这些患者步入中年，人们对其长期发病率和死亡率存在担忧。我们旨在评估在我们成人先天性心脏病（ACHD）科室就诊的rToF患者的生存率及并发症发生率。
纳入自2000年起在澳大利亚悉尼我们的三级ACHD科室接受随访的168例年龄超过16岁的连续性“单纯rToF”患者。我们记录了死亡率，并分析了前瞻性定义的复合终点：（a）“严重不良事件”，包括死亡、心力衰竭住院和/或记录在案的室性心律失常；（b）“不良事件”，包括上述情况以及心内膜炎、房性心律失常、除颤器和/或起搏器植入。
最后一次随访时的平均年龄为34±12岁，55%为男性。有10例（6%）死亡，26例患者（16%）发生了“严重不良事件”。51例患者（30%）发生了“不良事件”，29例患者发生了房性心律失常。101例（61%）患者至少进行过一次肺动脉瓣置换。到40岁时，93%的患者无严重不良事件，83%的患者无任何不良事件。到50岁时，只有56%的患者未发生不良事件。年龄较大和有房性心律失常病史是严重不良事件的预测因素。
rToF患者存活至中年的情况非常良好；然而，相当数量的幸存者在50岁时会出现不良事件。
