左心发育不良综合征患儿行经典Norwood I期手术围手术期管理早期经验总结

Early experience on the standard Norwood stage Ⅰ procedure for hypoplastic left heart syndrome

目的回顾性分析开展Norwood手术的早期围手术期管理经验。方法2010年6月至2014年8月，5例左心发育不良综合征患儿行经典NorwoodI期手术。患儿均为男性，体质量2．57—3．50kg，中位体质量3．13kg；手术时为出生后29—75天，中位年龄36天。第1、2、3例遵循传统围术期管理策略，术前经静脉给予前列腺素E1，呼吸机辅助通气，因血流动力学状态进行性恶化而行抢救性急诊手术；第4、5例患儿术前除必须检查无其他医疗干预，行限时性急诊手术。5例均在深低温停循环下行经典Norwood手术，第1、2、4、5例采用改良BT分流，第3例采用Sano分流。结果第3例患儿术后因重度低氧血症、酸中毒死亡；第4例体外循环结束后因心肌收缩无力死亡。第1例I期术后12个月在我院行Ⅱ期双向Glenn手术，术后心功能I级、血氧饱和度0．90-0．95，Ⅱ期术后3个月因外伤死亡；第2例术后3个月复查，心功能I级，末梢血氧饱和度可维持在0．78—0．83，随后失访；第5例出院后3个月随访，心功能I级，MBTS血流通畅，末梢血氧饱和度0．84，拟在评估后行Ⅱ期双向Glenn或半Fon—tan术。结论经典NorwoodI期手术的成功要求多学科的充分合作。各心脏中心的经验分享有利于快速积累围术期管理经验，减少手术死亡及并发症发生率。

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 analyse the perio- perative management of Norwood Stage [ . Methods Between June 2010 and August 2014, totally 5 small infants with HLHS underwent the standard Norwood Stage I procedure. They were all boys. Age at surgeries ranged from 29 to 75 days~ and weight from 2.57 - 3.50 kg with median of 3.13 kg. Case 1,2 and 3 received standard medical regimen after accessing NICU, which included intravenous prostaglandin E1 and mechanical ventilation. The 3 infants underwent emergent operations because of unstable hemodynamics. Case 4 and 5 received no medical intervention before the urgent surgeries. All 5 eases underwent the standard Norwood Stage I proeedure under deep hypothermie circulatory arrest, including 4 eases of modified Blalock-Taus- sig shunt （MBTS） and 1 ease of Sano shunt. Results The ease with Sano shunt died from severe hypoxemia and persistent aei- dosis 32 hours after the operation, another ease died from low cardiac output syndrome after cardiopulmonary bypass. The first ease underwent bidirectional Glenn procedure 12 months after Norwood Stage I, the postoperative heart function was NYHA I and the oxygen saturation was 0.90 -0.95 in room air, but he died from accidental brain injury 3 months after stage ~. The second ease was followed up 3 months after stage I procedure with NYHA I and oxygen saturation of 0.78 -0.83 in room air, and lost the follow-up after. The fifth case was followed up 3 months after stage I procedure with NYHA I , confluent MBTS and oxygen saturation of 0.84, the patient is being followed up and waiting for further evaluation for stage 11 procedures. Con- clusion The standard Norwood Stage I procedure is a complex procedure, which demands muhidisplinary cooperation, to pal- liatively correct HLHS. Sharing expericences 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.