合并肺动脉高压的右心室双出口肺动脉环缩术后Ⅱ期手术治疗效果分析  

Staged operation of double-outlet right ventricular with pulmonary hypertension

作　　者：毛俊[1] 范祥明[1] 许耀强[1] 李刚[1] 段蔚然 樊星[1] 苏俊武[1] MAO Jun;FAN Xiangming;XU Yaoqiang;LI Gang;DUAN Weiran;FAN Xing;SU Junwu(Department of Paediatric Cardiology,Beijing Anzhen Hospital,Capital Medical University,Beijing Institute of Heart,Lung and Blood Vessel Diseases,Beijing 100029,China)

机构地区：[1]首都医科大学附属北京安贞医院-北京市心肺血管疾病研究所小儿心脏中心,100029

出　　处：《心肺血管病杂志》2020年第5期518-521,共4页Journal of Cardiovascular and Pulmonary Diseases

摘　　要：目的:总结合并肺动脉高压的右心室双出口(DORV)患者Ⅰ期行肺动脉环缩术后,Ⅱ期手术的结果。方法:入选既往在本中心接受肺动脉环缩术的合并肺动脉高压的DORV患者共15例,男性12例,女性3例,年龄(5.3±2.1)岁,体质量(17.2±4.0)kg。术前经皮血氧饱和度(78.3±3.2)%。11例(73%)在体外循环下行双心室矫治术,其中2例行心室内隧道补片,9例行心室内隧道+右心室流出道扩大补片术。术中均拆除原肺动脉环缩带。同期矫治合并畸形。4例(27%)在非体外循环下行右侧双向Glenn术,术中均结扎奇静脉。结果:11例双心室矫治患者中,死亡1例,原因为术后左室流出道狭窄、低心排综合征。室间隔缺损残余分流1例。余无严重围术期并发症发生。4例行单心室类手术(Glenn)患者均未出现围术期死亡或严重并发症。结论:对于无法接受Ⅰ期根治的合并肺动脉高压的DORV患者,可采用Ⅰ期先行肺动脉环缩术,择期行Ⅱ期手术的策略。Objective:To summarize the result of stage Ⅱ procedure in patients with Double-outlet right ventricular(DORV)and pulmonary hypertension.Methods:15 patients(12 males and 3 females)with DORV and pulmonary hypertension,who accepted pulmonary artery banding were enrolled.Age and weight were(5.3±2.1)years old and(17.2±4.0)kg.Oxygen saturation were(78.3±3.2)%.11 patients(73%)underwent biventricular repair under cardiopulmonary bypass.Among them,2 patients underwent left ventricle to aorta baffle pathway and 9 patients underwent left ventricle to aorta baffle pathway and RVOTO procedure.The pulmonary artery banding was removed during the operation.Other malformations were corrected at the same time.4 cases(27%)underwent Glenn procedure,and azygos vein was ligated during the operation.Results:Among 11 patients with biventricular correction,1 died due to left ventricular outflow tract stenosis and low cardiac output syndrome,1 had residual shunt of ventricular septal defect.No other serious perioperative complications occurred.No perioperative death or serious complications occurred in 4 patients underwent Glenn procedure.Conclusions:For patients with DORV and pulmonary hypertension who can not accept biventricular cor-rection,the strategy of staged operation can be adopted.

关 键 词：先天性心脏病 右心室双出口 肺动脉高压 肺动脉环缩术 分期手术 

分 类 号：R54[医药卫生—心血管疾病]