肺静脉内膜剥脱术治疗小儿先天性肺静脉狭窄

目的 分析先天性肺静脉狭窄患儿的临床特点,探讨肺静脉内膜剥脱术治疗小儿先天性肺静脉狭窄的疗效.方法 回顾性分析2008年1月至2009年12月收治的4例先天性肺静脉狭窄患儿的临床资料,其中男3例,女1例,年龄2个月～2岁11个月.均行胸片、心电图和超声心动图检查,其中2例行心脏多排计算机断层扫描检查,以期明确诊断.4例患者共有7支肺静脉狭窄,右上肺静脉3例,右下肺静脉1例,左上肺静脉1例,左下肺静脉2例.狭窄部位均在肺静脉开口处,均合并其他心血管畸形：室间隔缺损3例,房间隔缺损1例,动脉导管未闭2例,主动脉缩窄1例,术前均提示重度肺动脉高压.术前根据病情予强心、利尿、扩血管及抗感染等治疗.完善术前准备后在全身麻醉、胸骨正中切口、深低温、体外循环下行肺静脉内膜剥脱术.结果 术后患儿血流动力学稳定;机械通气时间3.7（2～4）d,重症监护时间4.2（3～5）d;围术期无一例死亡.随访18～26个月,患儿均存活,心功能（纽约心脏协会）Ⅰ级3例、Ⅱ级1例,生长发育均正常.结论 先天性肺静脉狭窄常合并各种心内畸形,超声心动图、心脏多排计算机断层扫描检查对诊断帮助较大.肺静脉内膜剥脱术治疗先天性肺静脉狭窄患儿安全、有效,手术成功率高,近期随访疗效较好,对于婴幼儿先天性肺静脉狭窄不失为一种治疗选择.

Pulmonary vein endarterectomy for children with congenital pulmonary vein stenosis

Objectives To analyse the clinical feature of children with congenital pulmonary vein stenosis,and to explore the effects of pulmonary vein endarterectomy for children with congenital pulmonary vein stenosis.Methods Clinical date of 4 children with congenital pulmonary vein stenosis from January 2008 to December 2009 were retrospectively analyzed.Of which,3 males and 1 female,aged from 2 months to 2 years 11 months.They were underwent chest X-ray,electrocardiography,echocardiography and cardiac multi-slice computed tomography （2 cases） in order to confirm the diagnosis.Totally,7 pulmonary veins were founded stenosis （all in the opening of pulmonary veins） in these 4 children,including right superior pulmonary vein （3 cases）,lower right pulmonary vein （1 case）,left superior pulmonary vein （1 case）,and lower left pulmonary vein （2 cases）.All the 4 children had other cardiovascular malformations.Among them,3 cases were with ventricular septal defect,1 cases was with atrial septal defect,2 cases were with patent ductus arteriosus,and 1 case was with coaretation of the aorta.They were revealed severe pulmonary hypertension in preoperative evaluation.Pulmonary vein endarterectomy were performed under general anesthesia,median sternotomy,deep hypothermia,cardiopulmonary bypass after perfect preparation.Results These 4 children were haemodynamically stable after pulmonary vein endarterectomy,with duration of mechanical ventilation of 3.7 （2-4） d,duration of intensive care of 4.2 （3-5） d,and no deaths perioperatively.They were alive when followed-up 18 to 26 months with cardiac function （New York Heart Association） class Ⅰ （3 cases）-Ⅱ （1 cases）,and normal growth and development.Conclusions Congenital pulmonary vein stenosis is often associated with a variety of cardiac abnormalities.Echocardiography and cardiac multislice computed tomography are helpful in the diagnosis.Pulmonary vein endarterectomy is safe and effective.It remains a potentially therapeutic alternative for c