左心发育不全综合征Ⅰ期Norwood手术的围术期处理

Perioperative treatment for first stage of Norwood procedure for hypoplastic left heart syndrome

目的总结Ⅰ期Norwood手术围术期处理的经验。方法2004年7月～2006年3月,应用Ⅰ期Norwood手术治疗3例患左心发育不全综合征(hypoplastic left heart syndrome,HLHS)及单心室合并左心流出道梗阻的患婴。第1例行升主动脉与主肺动脉侧侧吻合,体-肺分流重建肺循环血流;第2例利用主肺动脉重建升主动脉以及弓部,Core-Tex人造血管连接右心室和肺动脉(Sano Shunt术式)重建肺循环血流;第3例同样应用Sano Shunt术式重建肺循环血流。结果前2例手术取得成功,随访24～34个月,两例均已成功进行Ⅱ期Norwood手术,心功能Ⅰ～Ⅱ级;第3例术后早期死亡,死因是重度低心输出量综合征。结论Norwood手术是治疗左心发育不全综合征和单心室合并左心流出道梗阻疾病的有效治疗方法之一,选择适当的手术方式和术前、术后维持体-肺循环动态平衡是取得良好手术疗效的关键。

Objectives To summarize experience of perioperative treatment for first stage of Norwood procedure. Methods From July 2004 to March 2006, we carried out Norwood procedure （first stage） for 3 patients who suffered from complex congenital heart diseases. The first diagnosis was SV, IAA and PDA in case 1. TGA/VSD, IAA and hypoplastic aortic arch in case 2. HLHS in case 3. Reconstruction of aorta with main pulmonary artery was performed in all patients. Reconstruction of pulmonary circulation with aortic pulmonary shunt in one patient, RV-PA conduit （ Sano shunt） in another two patients. Results One patient died postoperatively. The follow-up was 24 months and 34 months respectively. Two patients received Norwood （second stage） procedure successfully. Cardiac function was rehabilitated to grade Ⅰ- Ⅱ. Conclusions Norwood procedure is the most important method to treat the patients suffering from HLHS or SV with obstruction of left ventricular outflow tract. Proper surgical approach and keeping balance between systemic circulation and pulmonary circulation are the key points for successful operation.