Hansen D D, Hickey P R
Anesth Analg. 1986 Feb;65(2):127-32.
The anesthetic management of the first stage palliative surgical repair of hypoplastic left heart syndrome with a high-dose fentanyl (50 micrograms/kg)-pancuronium (0.14 mg/kg) anesthetic technique is described in 30 neonates. Previously this severe form of congenital heart disease carried a 100% rate of mortality. The pathophysiology that leads to this mortality is explained and related to the surgical procedure utilized for palliation. The major problem encountered intraoperatively was control of pulmonary blood flow. Management of the effect of anesthesia and intraoperative events on pulmonary blood flow was important because the rapid flux in pulmonary vascular resistance during the postnatal and perioperative period is the major destabilizing factor in these neonates. Ventricular fibrillation with surgical manipulation was a minor problem in the present series but was a major problem in an earlier series where a low dose narcotic-nitrous oxide technique was used. Ventricular fibrillation probably occurred due to the relative coronary insufficiency resulting from the anatomy present in this syndrome.
本文描述了采用高剂量芬太尼（50微克/千克）-潘库溴铵（0.14毫克/千克）麻醉技术对30例新生儿进行左心发育不全综合征一期姑息性手术修复的麻醉管理。此前，这种严重的先天性心脏病死亡率为100%。文中解释了导致这种死亡率的病理生理学，并将其与用于姑息治疗的手术操作相关联。术中遇到的主要问题是控制肺血流量。麻醉效果及术中事件对肺血流量的管理很重要，因为出生后及围手术期肺血管阻力的快速变化是这些新生儿的主要不稳定因素。在本系列中，手术操作引发的心室颤动是一个小问题，但在早期采用低剂量麻醉药-氧化亚氮技术的系列中却是一个大问题。心室颤动可能是由于该综合征的解剖结构导致相对冠状动脉供血不足所致。
