单心室合并完全性肺静脉异位引流的外科治疗

目的:探讨单心室合并完全性肺静脉异位引流(TAPVC)的外科治疗效果。方法:2009-05至2011-09,我科共收治单心室合并TAPVC患儿11例,年龄(30.1±21.4)个月,体重(11.0±3.2)kg。例2行改良B-T分流术,例1、3～6、8、9、11行一侧或双侧双向腔肺分流术,例7、10行一期改良Fontan术。例1、6～9、11同期行TAPVC矫治术。结果:例6、10围术期死亡(18.2%,2/11),死亡原因分别为中枢神经系统感染和多器官功能衰竭。存活的患儿随访1～29个月,例4死亡,余患儿氧饱和度改善,未发现残余肺静脉梗阻,例5行二期改良Fontan术并顺利出院。结论:单心室合并TAPVC患儿仍具有较高早期死亡率,术前明确诊断TAPVC并制定合理的个体化治疗方案是提高手术疗效的关键。

Surgical Treatment of Patients With Total Anomalous Pulmonary Venous Connection Undergoing Single Ventricle Series Palliation

Objective:To evaluate the effect on surgical treatment of patients with total anomalous pulmonary venous connection(TAPVC)undergoing single ventricle series palliation. Methods:A total of 11 patients with TAPVC undergoing single ventricle series palliation were surgically treated in our hospital from May 2009 to September 2011.The patients had the mean age of(30.1±21.4)months with the mean body weight of(11.0±3.2)kg.The initial palliative procedures were as modified B-T shunt in 1 patient(NO.1),unilateral or bilateral cavopulmonary shunt in 8 patients(NO.1,3～6,8,9,11),and one-stage modified Fontan operation in 2 patients(NO.7,10).6 patients(NO.1,6～9,11)received TAPVC repair at initial palliation. Results:The peri-operative mortality was 18.2%(NO.6,10,2/11)which was caused by central nervous system infection and multiple organ failure respectively.9 surviving children were followed-up for(1~29)months.1 patient(NO.4)died and the other had significantly improved oxygen saturation without residual pulmonary vein stenosis.1 patient(NO.5)underwent the second stage modified Fontan operation and discharged smoothly. Conclusion:The patients with TAPVC undergoing single ventricle series palliation had higher early mortality.Clear pre-operative diagnosis and accurate individualized treatment were the key points for improving the therapeutic efficacy.