Iannettoni M D, Bove E L, Mosca R S, Lupinetti F M, Dorostkar P C, Ludomirsky A, Crowley D C, Kulik T J, Rosenthal A
Department of Surgery, University of Michigan Medical School, Ann Arbor.
J Thorac Cardiovasc Surg. 1994 Mar;107(3):934-40.
Between January 1990 and February 1993, 73 patients underwent first-stage reconstruction for hypoplastic left heart syndrome at the University of Michigan Medical Center. During this period, surgical reconstruction remained essentially constant and consisted of a pulmonary artery-to-aorta anastomosis with allograft augmentation of the ascending, transverse, and proximal descending aorta, restriction of pulmonary blood flow with a polytetrafluoroethylene shunt from the innominate artery to the central pulmonary artery confluence, and atrial septectomy. Hospital survival was 62 of 73 patients, 85% (70% confidence limits: 80% to 89%). These results stand in marked contrast to those obtained during the earlier years of our experience from 1986 to 1989 when only 21 of 50 patients (42%, 70% confidence limits: 35% to 49%) survived (p = 0.001). Among the most recent group of patients, only 2 of 7 patients older than 1 month of age at operation survived, whereas 60 of 66 (91%, 70% confidence limits: 87% to 94%) patients younger than 1 month of age survived (p = 0.0001). Anatomic subtype and ascending aortic diameter were not predictive of survival. Actuarial survivals for those patients younger than 1 month of age at the first-stage operation, including hospital deaths and subsequent operative procedures, were 81%, 74%, and 74% at 6 months, 1 year, and 2 years, respectively. These results indicate that survival for patients after first-stage reconstruction for hypoplastic left heart syndrome has significantly improved in recent years. Older age was a strong risk factor, with a hospital survival of 91% for those patients undergoing first-stage palliation within the first month of life. These data have important implications for the type of operative intervention and its timing.
1990年1月至1993年2月期间，73例患者在密歇根大学医学中心接受了左心发育不全综合征的一期重建手术。在此期间，手术重建基本保持不变，包括肺动脉至主动脉吻合术，并使用同种异体移植物扩大升主动脉、横主动脉和降主动脉近端，通过聚四氟乙烯分流管从无名动脉至中央肺动脉汇合处以限制肺血流量，以及房间隔切除术。73例患者中有62例存活出院，存活率为85%（70%可信区间：80%至89%）。这些结果与我们1986年至1989年早期经验中的结果形成了鲜明对比，当时50例患者中只有21例（42%，70%可信区间：35%至49%）存活（p = 0.001）。在最近一组患者中，手术时年龄超过1个月的7例患者中只有2例存活，而66例年龄小于1个月的患者中有60例（91%，70%可信区间：87%至94%）存活（p = 0.0001）。解剖亚型和升主动脉直径并不能预测存活率。在一期手术时年龄小于1个月的患者，包括住院死亡和随后的手术操作，6个月、1年和2年的实际存活率分别为81%、74%和74%。这些结果表明，近年来左心发育不全综合征一期重建术后患者的存活率有了显著提高。年龄较大是一个很强的危险因素，出生后第一个月内接受一期姑息治疗的患者住院存活率为91%。这些数据对手术干预的类型及其时机具有重要意义。
