单心室合并完全性肺静脉异位引流的外科治疗

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

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。

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 hospi- tal 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 palhation. 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.