完全性肺静脉异位引流的解剖变异及外科处理  

Anatomic variations and corresponding surgical techniques of total anomalous pulmonary venous connection

作　　者：朱雄凯[1] 俞建根[1] 马良龙[1] 石卓[1] 张泽伟[1] 李建华[1] 陈自力[1] 

机构地区：[1]浙江大学医学院附属儿童医院心胸外科,杭州310003

出　　处：《中华医学杂志》2011年第30期2099-2102,共4页National Medical Journal of China

基　　金：浙江省自然科学基金（Y2080144）

摘　　要：目的探讨完全性肺静脉异位引流（TAPVC）的解剖变异及相应的手术方式。方法通过对1981年4月至2010年7月143例患儿的临床观察，分析TAPVC的解剖特征。术前诊断主要依据心脏超声检查，部分病例经心导管检查、MRI或三维CT重建确诊。手术方法除典型术式，根据不同解剖亚型采用改良Warden术等不同术式的矫治术。结果143例患儿解剖分型结果中：（1）心上型71例，按照TAPVC汇入上腔静脉的路径细分为4个亚型：A型（左行），65例；B型（右行），3例；C型（双行），2例，左右肺静脉分别于上腔静脉的左右侧汇人；D型（后行），1例，肺静脉于无名静脉与上腔静脉汇合部后方入上腔静脉。（2）心内型58例，分3个亚型：A型，冠状静脉窦型（20例）。B型，右心房型（37例）。C型，窦房混合型（1例）。（3）心下型6例。（4）混合型8例，根据Ujjwal分类法分为：对称型5例；不对称型3例。治疗结果：手术治疗135例，治愈127例（94．1％，127／135）。术后院内死亡8例（5．9％，8／135）。死因包括低心排综合征致脱机困难、术后肺高压危象合并低心排综合征、肺部感染并发呼吸衰竭。9例出现术后低心排综合征、肺水肿，经治痊愈出院。8例患儿无法耐受手术，内科治疗无效死亡。结论TAPVC的解剖亚型并不少见，且变异复杂；正确认识其亚型及采取个体化的手术方案，有助于提高治疗效果。Objective To describe the anatomic variations of total anomalous pulmonary venous connection （TAPVC） and its corresponding surgical techniques. Methods A total of 143 TAPVC subjects were hospitalized from April 1981 to July 2010. Those patients with other complex congenital heart diseases, such as transposition of great artery and single ventricle, were excluded. A pathological diagnosis was made by echocardiography, magnetic resonance imaging, computed tomography, catheterization and intra-operative findings. The specific types of TAPVC were as follows：supra-cardiac （49. 7% , 71/143 ）, cardiac （40. 6%, 58/143）, infra-cardiac （4. 2% , 6/143） and mixed （5.6% , 8/143）. The subtypes were classified by the pathway of common confluence, distribution of pulmonary vein and their orifice site. The techniques of surgical repairs included modified Warden procedure and pulmonary vein transplantation. Results The patients with supra-cardiac type were further divided into 4 subtypes according to the course of vertical veins and their orifice site： right and left veins forming a common confluence, then draining into vertical and innominate veins （ n = 65 ） ; common confluence of pulmonary vein drainage into superior vena cava through a short vertical vein at the right pulmonary hilus （ n = 3 ） ; right and left pulmonary veins separately draining into superior vena cava （ n = 2 ） ; common confluence draining into innominate vein through a right path beside trachea （ n = 1 ）. Cardiac types were further divided into 3 subtypes ： coronary sinus （ n = 20）, right atrium （n = 37） and right atrium ＆ sinus （n = 1 ）. Infra-cardiac type had no subtype. Mixed type was more complex and it was further divided into 3 subtypes ： bilateral ＆ symmetrical connection （ right 2 ＋ left 2, n = 5）; bilateral ＆ asymmetrical connection （3 ＋ 1, n =3）. Surgical repairs were performed on 135 patients. The surgical mortality of TAPVC was 5.9% （8/135）. And there was no late death.

关 键 词：心脏病 先天性 肺静脉 病理状态 解剖学 手术 

分 类 号：R726[医药卫生—儿科]