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目的 分析功能性单心室合并完全性肺静脉异位引流的手术方法及影响临床结果的危险因素.方法 2010～2016年我院44例功能性单心室合并完全性肺静脉异位引流患者行68次手术,其中男34例、女10例,首次手术时年龄312 (77～4 220)d.结果 Ⅰ期手术死亡8例,Ⅱ期手术死亡1例,随访时间1～80个月,随访死亡5例;总体随访生存率68.2％ (30/44).行Glenn手术34例,死亡5例.行Fontan手术9例,死亡2例.结论 功能性单心室合并完全性肺静脉异位引流需根据患者不同的解剖和病理生理特点制订合理的手术策略.分期手术可降低死亡率和并发症发生率.但肺静脉梗阻和心脾综合征可能是影响这类患者预后的危险因素.“,”Objective To explore the surgical management method and outcome of functional univentricular heart with total anomalous pulmonary vein drainage (UVH-TAPVD).Methods We reviewed the surgical procedures and results for 44 UVH-TAPVD patients in our hospital between the year 2010 and 2016.There were 34 males and 10 females.The age of the patients was 312 (77-4 220) d when they accepted the first surgical treatment.Results There were 8 deaths in stage Ⅰ palliation,1 death in stage Ⅱ palliation and 5 deaths during the follow-up.The overall survival rate was 68.2％ (30/44).Glenn operation was undertaken in 34 patients with 5 deaths.Fontan operation was undertaken in 9 patients with 2 deaths.Conclusion Surgical strategies for UVH-TAPVD should be planned according to different anatomical and pathophysiological conditions in different patients.Staged palliations can reduce mortality and morbidity.But pulmonary venous obstruction and heterotaxy syndrome are still risk factors for these patients.