应用NorwoodⅠ期手术治疗左心发育不良综合征围手术期管理策略的反思  

Reflection of the perioperative managements of Norwood stage Ⅰ procedure for hypoplastic left heart syndrome

作　　者：张程 彭乐[1] 孟保英[1] 王元祥[1] 刘怀普[1] 王鹏程 张青[1] 丁以群[1] Zhang Cheng;Peng Le;Meng Baoying;Wang Yuanxiang;Liu Huaipu;Wang Pengcheng;Zhang Qing;Ding Yiqun(Department of Cardiac Surgery, Shenzhen Children＇ s Hospital, Shenzhen 518038, China)

机构地区：[1]深圳市儿童医院心脏外科,518038

出　　处：《中华胸心血管外科杂志》2018年第6期331-334,共4页Chinese Journal of Thoracic and Cardiovascular Surgery

摘　　要：目的比较左心发育不良综合征经典围手术期管理策略及改进策略，以期获得更理想的治疗方法。方法2010年6月至2017年11月，10例左心发育不良综合征的患婴行经典NorwoodⅠ期手术。按不同手术时间分组。2010年6月至2014年8月5例采用经典围手术期管理策略，为A组，均为男患婴，体质量2．57～3．50kg，出生后29～75天手术，中位时间36天；采用改良BT分流4例，RV-PA分流1例，手术方法包括经典的肺动脉上置入主动脉插管，PTFE补片行升主动脉及主动脉弓重建。2014年8月至2017年11月5例采用修正的围手术期管理策略，为B组，男4例，女1例，体质量2．00-3．10kg，手术时年龄6～22天，中位年龄12天；均采用sidegraft技术行主动脉插管，牛心包补片行升主动脉及主动脉弓重建；术后应用新生JIN育箱、通过调节环境温度调整Qp／Qs，慎用血管活性药物。结果A组术后早期死亡2例；B组1例术后早期死于心包压塞。结论对于左心发育不良综合征患婴，调整围手术期管理策略，寻找适合我国心脏外科医师的治疗方法，可以有效地提高手术成功率。Objective Norwood Stage Ⅰ is the standard procedure to cope with hypoplastic left heart syndrome （ HLHS）, which continues to be the most challenging congenital heart disease. The aim of this study is to retrospectively com- pare the classical perioperative management of Norwood Stage I with the modified strategy. Methods Between June 2010 and November 2017, totally 10 patients with HLHS underwent the standard Norwood Stage I procedure. They are stratified to two tiers： Group A, from June 2010 to August 2014, there were 5 boys. Age at surgeries ranged from 29 to 75 days, and weight 2.57 - 3.50 kg with median of 3.13 kg. Group B, from August 2014 to November 2017, there were 4 boys and 1 girl. Age at surgeries ranged from 6 to 22 days, and weight from 2.0 - 3.1 kg. In Group A, all 5 cases underwent the standard Norwood Stage I procedure under deep hypothermic circulatory arrest, including 4 cases of modified Blalock-Taussig shunt（MBTS） and 1 case of RV-PA shunt. In Group B, all 5 cases adopted side graft technique and RV-PA shunt, aortic arch and ascending aor- ta were reconstructed with treated bovine pericardial patch. Group B used incubators to adjust systemic vascular resistance in- stead of vasodilators. Results Group A＇ s early mortality is 40% ; Group B＇ s early mortality is 20%, 1 case died of tampon- ade. Conclusion The standard Norwood Stage Ⅰ procedure is a complex procedure, which demands multidisplinary coopera- tion, to palliatively correct HLHS. To adjust and find a suitable perioperative managements can improve the results. Sharing experiences on perioperative managements of Norwood Stage I between heart centers in China will be helpful to decrease the mortality and morbidity in relatively short period.

关 键 词：心脏缺损 先天性 左心发育不良综合征 NORWOOD Ⅰ期 围手术期 

分 类 号：R726.5[医药卫生—儿科]