功能性单心室合并完全型肺静脉异位引流的外科治疗和中期随访结果

Surgical treatment and mid-term results of functional single ventricle associated with total anomalous pulmonary venous connection

目的分析功能性单心室(FSV)合并完全型肺静脉异位引流(TAPVC)的外科治疗及中期结果。方法回顾性分析2008~2018年我院12例同时行FSV矫治和TAPVC矫治患者的临床资料和随访结果。男6例、女6例,年龄2.3(1~21)岁。采用Cox回归模型分析患者死亡预后因素,采用Kaplan-Meier法进行生存分析。结果所有患者术后均顺利脱离体外循环,体外循环时间(113.8±42.5)min,心肌阻断时间(57.7±31.7)min。院内死亡3例(3/12,25%),死亡原因为肺动脉高压、肺出血和低氧血症。中位随访时间48.3(1~118)个月,期间死亡4例,均为心功能衰竭。1年和3年总体生存率分别为58.3%(95%CI 27%~80%)和40%(95%CI 13%~65%)。结论FSV合并TAPVC属于临床重症,治疗效果差,总体死亡率高。

Objective To evaluate the mid-term results of surgical treatment for functional single ventricle associated with total anomalous pulmonary venous connection.Methods We reviewed the clinical data and follow-up results of 12 patients in our hospital who underwent both single ventricle series palliation and total anomalous pulmonary venous connection correction from 2008 to 2018.There were 6 males and 6 females at age of 2.3(1-21)years.Univariable and multivariable Cox proportional hazard regression methods were performed.The Kaplan-Meier method was used to estimate the survival rate.Results All patients were successfully removed from extracorporeal circulation.The cardiopulmonary bypass time was 113.8±42.5 min.The myocardial block time was 57.7±31.7 min.There were 3 inhospital deaths and 4 late deaths.The causes of death in hospital were pulmonary hypertension,pulmonary hemorrhage,and hypoxemia.During follow-up,the cause of death was heart failure.The survival rate at 1 year and 3 years was 58.3%(95%CI 27%to 80%)and 40%(95%CI 13%to 65%),respectively.Median follow-up was 48.3 months(range:1 to 118 months).Conclusion Functional single ventricle combined with complete pulmonary venous drainage is a serious clinical disease,with poor therapeutic effect and high overall mortality.