Williams综合征和并先天性心血管畸形手术纠治的麻醉.DOCVIP

Williams综合征合并先天性心血管疾病手术纠治的麻醉管理 白 洁 黄 悦 张瑞冬 张剑蔚 上海交通大学医学院附属上海儿童医学中心麻醉科，上海200127 【摘 要】 目的 探讨Williams综合征合并先心病手术纠治的麻醉管理特点。方法 2008年至2011年，31例Williams综合征患儿行主动脉瓣上狭窄纠治术。术中采用大剂量舒芬太尼复合咪达唑仑，依托咪酯／异丙酚和罗库溴铵的全身麻醉。主动脉开放后静脉输注多巴胺，肾上腺，米力农和硝酸甘油等血管活性药物。体外循环停机后调节凝血功能，并根据血气分析结果补充电解质、维持酸碱平衡。结果 本组病例全部顺利完成手术，无死亡，无麻醉并发症。结论 麻醉前全面细致的评估、选用适当的麻醉药物诱导和维持、合理应用血管活性药物、严密的围术期监测并及时处理是Williams综合征合并先心病纠治术切实可行的麻醉管理方法。 【关键词】Williams综合征；主动脉瓣上狭窄；全身麻醉 Anesthetic management on the children with Williams syndrome undergoing congenital heart surgery BAI Jie, HUANG Yue, ZHANG Rui-dong, ZHANG Jian-wei, (Department of Anesthesiology, Shanghai Childrens Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai 200127, China) 【Abstract】Objectives To discuss the anesthetic management experience on the children with Williams syndrome undergoing congenital heart surgery. Methods 31 cases study from 2008 to 2011 on children with Williams syndrome undergoing aortic stenosis correction of surgery. General anesthesia was applied by high dose sufentanil combined with midazolam, etomidate/propofol and rocuronium. Dopamine, adrenaline, milrinone and nitroglycerin were infused after clamp opening. Results All cases undergo surgery safely without anesthetic complication and death. Conclusions It is the practical anesthetic management measures on children with Williams syndrome undergoing congenital heart surgery by: comprehensive pre-operative evaluation; proper anesthetic induction and maintain; suitable vasopressor agents; strict perioperative monitor and timely treatment. 【Key words】Williams syndrome; supravalvuar aortic stenosis; general anesthesia Williams-Beuren综合征是一组少见的多系统发育障碍征候群。国外报道,其在活产新生儿中的发病率约为1:20,000，典型表现为先天性主动脉瓣上狭窄（supravalvuar aortic stenosis, SVAS）、智力发育迟缓，行为和性格异常，“小精灵”面容，一些患者伴高钙血症，约80%的患者伴远端肺动脉狭窄[1][2]。以左心流出道梗阻性病变、心肌缺血为主的病理生理特点使其成为围术期心脏骤停和儿童猝死的高危因素，麻醉管理特殊且风险高[2]。现将2008至2011年期间31例William-Beuren综合征合并先天性心血管畸形手术纠治的麻醉体会总结如下。 1.资料与方法 1.1一般资料 本组31名患儿，男22例， 女9例；年龄3个月~12岁，平均3岁7个月；体重5~