Jacobs M L, Rychik J, Murphy J D, Nicolson S C, Steven J M, Norwood W I
Division of Cardiothoracic Surgery, Children's Hospital of Philadelphia, PA 19104, USA.
J Thorac Cardiovasc Surg. 1995 Nov;110(5):1555-61; discussion 1561-2. doi: 10.1016/S0022-5223(95)70079-X.
Norwood's operation provides satisfactory palliation for neonates with hypoplastic left heart syndrome. The dominant physiologic features of hypoplastic left heart syndrome, ductal dependency of the systemic circulation and parallel pulmonary and systemic circulations, are shared by a multitude of other less common congenital heart malformations. Theoretically, these should be equally amenable to palliation by Norwood's operation. Between January 1990 and June 1994, 60 neonates with malformations other than hypoplastic left heart syndrome underwent initial surgical palliation by Norwood's procedure. Diagnoses included single left ventricle with levo-transposition of the great arteries (12); critical aortic stenosis (8); complex double-outlet right ventricle (8); interrupted aortic arch with ventricular septal defect and subaortic stenosis (7); ventricular septal defect, subaortic stenosis, and coarctation of the aorta (7); aortic atresia with large ventricular septal defect (6); tricuspid atresia with transposition of the great arteries (6); heterotaxy syndrome with subaortic obstruction (3); and other (3). There were 10 hospital deaths and 50 survivors (83% survival). After the introduction of inspired carbon dioxide therapy into the postoperative management protocol (1991), 42 of 47 patients survived (89% survival). Mortality was independent of diagnosis and essentially the same as that for hypoplastic left heart syndrome. With minor technical modifications, Norwood's operation provides satisfactory initial palliation for a wide variety of malformations characterized by ductal dependency of the systemic circulation in anticipation of either a Fontan procedure or a biventricular repair.
诺伍德手术为患有左心发育不全综合征的新生儿提供了令人满意的姑息治疗。左心发育不全综合征的主要生理特征，即体循环的导管依赖性以及肺循环和体循环并行，在许多其他较不常见的先天性心脏畸形中也存在。从理论上讲，这些畸形同样适合通过诺伍德手术进行姑息治疗。1990年1月至1994年6月期间，60例患有除左心发育不全综合征以外畸形的新生儿接受了诺伍德手术进行初始手术姑息治疗。诊断包括左心室单心室合并大动脉左旋位（12例）；严重主动脉狭窄（8例）；复杂的右心室双出口（8例）；主动脉弓中断合并室间隔缺损和主动脉瓣下狭窄（7例）；室间隔缺损、主动脉瓣下狭窄和主动脉缩窄（7例）；主动脉闭锁合并大型室间隔缺损（6例）；三尖瓣闭锁合并大动脉转位（6例）；内脏异位综合征合并主动脉瓣下梗阻（3例）；以及其他（3例）。有10例住院死亡，50例存活（存活率83%）。在术后管理方案中引入吸入二氧化碳治疗（1991年）后，47例患者中有42例存活（存活率89%）。死亡率与诊断无关，与左心发育不全综合征的死亡率基本相同。通过微小的技术改进，诺伍德手术为各种以体循环导管依赖性为特征的畸形提供了令人满意的初始姑息治疗，为后续的Fontan手术或双心室修复做准备。
