动脉调转术治疗合并肺动脉高压的室间隔缺损型心室大动脉连接异常患儿的手术适应证探讨  

Indications of arterial switch operation for complex congenital heart defect with severe pulmonary hypertension and ventriculoarterial discordant connection

作　　者：刘迎龙[1] 胡盛寿[1] 沈向东[1] 李守军[1] 王旭[1] 闫军[1] 崔彬[1] 

机构地区：[1]中国医学科学院中国协和医科大学阜外心血管病医院心血管病研究所小儿心脏外科中心,北京100037

出　　处：《中华医学杂志》2006年第1期23-25,共3页National Medical Journal of China

摘　　要：目的 总结动脉调转术（ASO）治疗年龄6～84个月、合并中～重度肺动脉高压（PH）的室间隔缺损（VSD）型心室大动脉连接异常复杂先天性心脏病患儿的手术疗效，探讨适当的手术适应证。方法 2000年1月至2004年12月，对23例年龄6～84个月、中～重度PH合并VSD的大动脉转位（TGA）和右室双出口（肺动脉瓣下室间隔缺损，Taussig-Bing畸形）患儿行ASO，其中男15例，女8例，年龄7～84（22±21）个月，肺动脉压力24～80（44±14）mmHg（1mmHg=0．133kPa），肺血管阻力（PRA）49、2～1261．9dyn.s．cm^-5。合并主要畸形：左室流出道狭窄3例，肺动脉瓣下隔膜1例，主动脉瓣下隔膜1例。手术在全麻、低温（18℃～22℃）、低流量（50mJ·kg^-1·min^-1）体外循环下完成ASO，合并畸形同期矫治。对合并中～重度肺动脉高压的患儿，围手术期给予α受体阻滞剂或持续吸入低流量一氧化氮（NO）等治疗，改善肺血管压力；连续监测并控制肺动脉压、左房压、右房压。结果 23例患儿病死率13％，3例死亡患儿死亡原因均与术前肺血管压力和阻力增高无明显关系。余患儿虽然术后呼吸机辅助时间及重症监护时间明显延长，但术后肺动脉压力均明显下降（23mmHg±9mmHg，t=13．28，P=0．000），痊愈出院。结论 对年龄6～84个月，合并VSD并发中～重度PH的VSD型TGA和Taussig-Bing畸形患儿，如一般状况良好，紫绀不严重（经皮血氧饱和度〉60％），X线胸片示两肺血较多，心导管检查显示肺血管阻力仍在可接受的范围（PRA〈1000dyn．s．cm^-5），采用ASO仍可取得满意的效果，扩大了ASO的手术适应证。Objective To summarize the experience in arterial switch operation （ASO） for complex congenital heart defect （CHD） with severe pulmonary hypertension （PH） and ventriculoarterial discordant connection in children older than 6 months. Methods Twenty-three consecutive pediatric patients suffering from severe CHD, such as transposition of great arteries （TGA）, Taussing-Bing anomaly, etc, with moderate to severe PH, 15 male and 8 female, aged 7-84 months, with the body weight of 4.5-20.0kg, pulmonary pressure of 24.0-80.0 mmHg, and pulmonary resistance of 49.2-1261.9 dyn·s· cm^-5, and with different complication such as left ventricular outflow tract obstruction （3 cases ）, subpulmonary membrane （1 case）, and subaortic membrane （1 case）, underwent ASO under general arresthesia and extracorporeal circulation with low temperature （18℃ -22℃ ） and low volume blood flow （50 ml·kg^-1· min^-1 ）. The complicated anomalies were corrected simultaneously. Nitric oxide and or-blocker were used to the children with PH during the peri-operational period. Results Twenty children survived, and were cured and discharged with an obvious improvement of heart function, only one of which had sequel of coma due to cerebral anoxia. After operation the pulmonary pressure decreased to 23 mmHg±9 mm Hg, significantly lower than that before operation （P = 0.000）. Physical activities increased obviously. Three children died post-operatively of the reasons un-related to the pre-operative PH and pulmonary arterial resistance, with a mortality of13.04%. Follow-up of 14 ± 8 months showed no death and complication. Conclusion ASO can still realize satisfactory early results for the children older than 6 months suffering from complex CHD with moderate to severe PH and ventrieuloarterial discordant connection provided they are in a rather good condition without severe eyanosis （ SaO2 〉 60% ） and with the pulmonary arterial resistance being acceptable （ pressure of right atrium 〈 1000 dyn·s·cm

关 键 词：心脏缺损 先天性 大血管错位 右室双出口 心脏外科手术 肺动脉高压 

分 类 号：R726.5[医药卫生—儿科]