心房动脉双调转术治疗小儿先天性矫正型大动脉转位的围术期管理

Perioperative Management of Congenitally Corrected Transposition of the Great Arteries Treated by Double Switch Operation in Children

目的总结分析采用心房动脉双调转(DS)术治疗小儿先天性矫正型大动脉转位(ccTGA)的围术期管理经验。方法 2004年1月-2009年12月北京阜外心血管病医院共实施DS手术26例,全部为ccTGA患儿。患儿平均年龄为3.0岁(0.5～10.0岁),平均体质量13.0kg(5.0～24.5kg)。根据手术方式,将患儿分为2组,比较围术期管理的特点。16例直接Ⅰ期行DS手术,即主动脉与肺动脉调转术和左心房与右心房转流术(Ⅰ组)。10例为分期手术,先行左心室功能锻炼手术(即肺动脉环缩术),择期再行DS手术(Ⅱ组)。结果Ⅰ组患儿体外循环(CPB)时间和主动脉阻断(AO)时间分别为(343.3±51.5)min和(232.3±56.6)min,Ⅱ组分别为(321.5±88.8)min和(192.1±11.7)min。Ⅰ组患儿无死亡,但发生右心功能衰竭(4例,25%)和心律失常(12例,75%)的概率较高。1例使用CPB膜肺氧合器(ECMO)进行右心功能辅助,7d后成功脱机。Ⅱ组患儿死亡4例(40%),均为左心衰竭死亡,其中3例使用ECMO进行左心功能辅助,1例未能脱机。结论采用DS术Ⅰ期治疗小儿ccTGA,术中右心功能异常多见,应主要预防和治疗肺动脉高压,降低右心后负荷。分期DS术治疗小儿ccTGA应注意降低左心后负荷,避免左心室做功突然增加。ECMO辅助循环可促进心脏功能恢复。

Objective To summarize the experience of perioperative management of congenitally corrected transposition of the great ar- teries （ccTGA） treated by double switch （DS）operation in children. Methods Twenty - six children with ccTGA underwent DS operation at Cardiovascular Institute and Fuwai Hospital from Jan. 2004 to Dec. 2009 were retrospectively studied. The mean age was 3.0 years old （ ranged from 0.5 to 10.0 years old） and mean weight was 13.0 kg （ ranged from 5.0 to 24.5 kg）. Children were divided into 2 groups by the method of operation. The characteristics of perioperative management were compared. Sixteen cases had undergone DS operation which com- bined arterial switch and Senning operation （group I , single- stage operation） and 10 cases received left ventricle training, pulmonary ban- ding,before DS operation（group Ⅱ ,staged operation）. Results The cardopulmonary bypass （CPB） time was （343.3 ±51.5） min and the aortic cross- clamping （AO） time was（ 232.3 ± 56.6） rain in group I ,while in group Ⅱ CPB and AO time were（321.5 ± 88.8） min and （192.1 ± 11.7） min,respectively. There were no deaths in group I ,but right heart failure（4 cases,25% ） and arrhythmia（12 cases,75% ） occurred more frequently. In one child, the extracorporeal circulation membrane oxygenation （ EC MO） support was successfully used for severe right heart dysfunction for a period of 7 days. In group Ⅱ ,4 cases died （40%） because left ventricular failure. Three of these cases received ECMO for left ventricular function support and 1 child failed the trial off. Conclusions Single - stage DS for children with ccTGA may be accompanied by right heart dysfunction more commonly. It is important to prevent and treat pulmonary hypertension to reduce the afterload of right heart. In DS following staged surgical treatment of ccTGA by functional retraining of the left ventricle, the treatment should focus on re- ducing left ventricular afterload and avoiding a sudden increase in left ventricular work. The ECMO circulatory support can promote recovery of cardiac function.