法洛四联症合并肺动脉瓣缺如患者手术矫治的近中期结果

目的总结法洛四联症合并肺动脉瓣缺如（TOF/PV-AB）患者手术矫治的近中期结果,为临床提供借鉴。方法 1996年1月至2009年8月北京阜外心血管病医院对14例TOF/PV-AB患者施行根治手术,其中男5例,女9例;年龄3.4±3.4岁（0.2~11.0岁）;体重12.0±6.3 kg（4~26 kg）。肺动脉处植入带瓣管道4例,采用带单瓣的跨环补片10例;行部分肺动脉壁切除6例,部分肺动脉壁切除加肺动脉壁折叠2例。结果围术期死亡2例（14.3%）,均为低体重小婴儿患者,1例死于低心排血量和呼吸衰竭,另1例死于中枢神经并发症。失访2例,随访10例,随访时间8.3±4.3年（0.6~13.0年）,随访患者均生存;超声心动图检查发现肺动脉瓣功能异常4例,无再次手术者。心功能分级（NYHA）均为Ⅰ~Ⅱ级。结论 TOF/PV-AB外科矫治术的近、中期结果良好,但需密切随诊肺动脉瓣或带瓣管道的功能。

Immediate and Intermediate Outcomes of Correction on Patients with Tetralogy of Fallot and Absent Pulmonary Valve

Objective To summarize the immediate and intermediate outcomes of surgical correction on patients with tetralogy of Fallot and absent pulmonary valve（TOF/PV-AB）.Methods From January 1996 to August 2009,14 patients,including 5 males and 9 females,aged 3.4±3.4 years（0.2-11.0 years） with an average weight of 12.0±6.3 kg（4-26 kg）,underwent complete surgical correction in Beijing Fu Wai Cardiovascular Hospital.The right ventricular outflow tract was reconstructed with valved conduit in 4 patients,and monocusp with transannular patch was used in 10 patients.Six patients underwent pulmonary artery wall reduction,and 2 patients underwent both pulmonary artery plication and wall reduction.Results There were 2（14.3%） peri-operative deaths.Both were low body-weight infants.One died of low cardiac output and respiratory failure,and the other died of central nervous system complications.Ten patients were followed up for an average time of 8.3±4.3 years（0.6-13.0 years）.All patients followed up survived.The echocardiogram found pulmonary valvular dysfunction in 4 patients.The patients＇ cardiac function were classified as New York Heart Association（NYHA） Ⅰ to Ⅱ.There was no late death or reoperation.Conclusion The immediate and intermediate outcomes of surgical correction of TOF/PV-AB are good,but the function of pulmonary valves and conduit should be followed-up closely.