大动脉调转术治疗小儿复杂性先天性心脏病

Arterial switch for congenital heart disease in children

目的回顾总结单个手术组应用大动脉调转术治疗小儿复杂性先天性心脏病(包括完全性大动脉转位和Taussig-Bing心脏畸形)的临床治疗经验。方法应用大动脉调转术治疗小儿复杂性先天性心脏病56例,男37例,女19例,6d^9岁,体质量2.55~18kg,其中完全性大动脉转位患儿40例,Taussig-B ing心脏畸形患儿16例,TGA/IVS15例,TGA/VSD25例,合并房间隔缺损10例,动脉导管未闭32例,肺动脉瓣轻度狭窄8例。全部患者均采用大动脉调转术进行治疗,手术在体外循环(CPB)下进行,冷血停博液或HTK液灌注保护心肌,停机改良超滤。结果本组手术CPB时间为150~259min,主动脉阻断时间为78~163m in。术后早期患儿死亡6例,3例死于术后出血(其中1例系PDA缝线松开),1例术后12h死于肺高压危象,1例术后5d死于肾功能衰竭,1例术后22d死于低心排出量综合征,ICU滞留时间为5~22d,住院时间为14~48d。术后随访3个月~7年,死亡1例,系单支冠状动脉患儿,出院后半年因肺炎心衰死亡,其余49例紫绀症状均消失,活动能力明显增强,生长发育良好,生活质量得到显著改善,心功能Ⅰ级(NYHA分级)。8例肺动脉狭窄患儿术后压差均有下降。结论大动脉调转术是治疗小儿复杂性先天性心脏病(包括完全性大动脉转位和Taussig-Bing心脏畸形)的理想手术方式,效果满意。

Objective To review and sum up the clinical experience of complex congenital heart disease in children （ inchiding complete transposition of great arteries and Tanssig-Bing heart malformation） by arterial switch operation in a single surgical group. Methods Arterial switch operations were performed on 56 children with complex congenital heart disease, including 37 males and 19 females, whose age ranged from 6 days to 9 years and weight from 2.55 to 18 kg. Among them, there were 40 complete transposition of great arteries, 16 Taussig-Bing heart malformations, 15 TGA/IVS , 25 TGA/VSD, associated with 10 atrial septal defect, 32 patent ductus arteriosus and 8 mild pulmonary valve stenosis. All cases were treated with arterial switch operation under CPB. Cold blood cardioplegia or HTK solution was used for myocardial preservation, while modified ultra-filtration was applied. Results The CPB time and aortic clamping time were 150-259 min and 78-163 min respectively. There were six deaths in the early time after the operation, including three cases of hemorrhage （ one case due to releasing of suture of PDA ）, one case died of pulmonary hypertension crisis in the twelfth hour after arterial switch operation, one case died of renal failure in the fifth day, one case died of low cardiac output syndrome in the twenty-second day, postoperative intensive care unit stay was 5-22 days,the hospital stay 14-48 days. During three months to seven years＇ follow-up, one case with single coronary artery died of pneumonia and heart failure in the sixth month after discharge. Cyanosis of the remaining forty-nine cases disappeared, activity greatly increased, growth and development were good and quality of life significantly improved, and heart function was class I（NYHA classification）. Pulmonary pressure difference of eight cases with pulmonary stenosis decreased after surgery. Conclusion The arterial switch operation is an ideal surgical procedure for congenital heart disease in children （including complete transposition of great arteries and Taussig-Bing heart malformation） and satisfactory results can be obtained.