目的 研究跨肺动脉瓣环补片法洛四联症(TOF)一期根治术患者术后远期疗效.方法 回顾性分析1985年3月至1989年12月单中心TOF根治术采用跨瓣环补片(TAP)并痊愈出院的患儿89例,79例资料完整,随访率88.8％.分析此79例患儿的生存率、免除再干预率和术后远期的心脏结构异常、功能异常及再干预情况；对可能与远期不良事件的发生相关的围术期因素进行Cox危险因素分析.结果 79例跨肺动脉瓣环补片TOF一期根治术患儿中随访死亡15例(19.0％),心力衰竭和猝死为首要死因.远期再干预5例(6.3％),主要病因是心律失常、右心室流出道残留狭窄和心室水平残余分流.患儿5、10、20、25年的生存率和免除再干预率分别为(88.5±3.6)％、(83.1±4.3)％、(80.2±4.6)％、(80.2±4.6)％和(98.6±1.3)％、(94.0±2.9)％、(92.3±3.3)％、(92.3±3.3)％.手术年龄≤1岁(RR =6.7,P=0.001)是影响术后远期随访不良事件发生的独立危险因素；TAP±单瓣对患儿远期生存率、免除再干预率和免除远期不良事件发生率没有影响.结论 TOF患儿TAP一期根治术后远期会出现患儿心脏结构及功能异常；手术年龄≤1岁的患儿,远期随访不良事件发生率可能更高.%Objective To evaluate the long-term outcomes of right ventricular outflow tract patching in total repair of tetralogy of Fallot.Methods Between Mar.1985 and Nov.1989,a total of 89 patients underwent total repair for tetralogy of Fallot. Altogether 79 patients completed the revisit,revisit ( 88.8％ ). Kaplan-Meier method with the log-rank test and Cox proportional hazards regression analysis were used to analysis the lone-term survival,freedom from re-intervention and freedom from long-term adverse events.Results There were 15 late deaths (19.0％ ) and the main causes of death were heart failure and sudden death. Re-intervention was required in 5 patients (6.3％ ) and the most common causes of re-intervention were arrhythmia,RVOT re-obstruction and residual ventricular septal defects. The overall survival rate was ( 88.5± 3.6)％, ( 83.1 ± 4.3 ) ％, ( 80.2 ± 4.6)％,(80.2±4.6) ％ at 5, 10,20 and 25 years; freedom from re-intervention rate was ( 98.6 ± 1.3 ) ％, ( 94.0 ±2.9)％, (92.3 ±3.3) ％ and (92.3± 3.3)％ at 5, 10,20 and 25 years. Age≤l year( RR = 6.7,P = 0.001)was the risk factor for long-term adverse events.The use of a monocusp in patients who underwent trans-annular repair showed no benefit for lone-term survival,avoidance of re-intervention and long-term adverse events.Conclusion The long-term outcome of right ventricular outflow tract patching in total repair for tetralogy of Fallot is not satisfactory; age is a risk factor of long-term adverse events.
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