Salve Gananjay G, Datar Gauri M, Perumal Gopinath, Singh Aakansha Ajay Vir, Ayer Julian G, Roberts Philip, Sholler Gary F, Cole Andrew D, Pigott Nick, Loughran-Fowlds Alison, Weatherall Andrew, Alahakoon T Indika, Orr Yishay, Nicholson Ian A, Winlaw David S
1 Heart Centre for Children, The Children's Hospital at Westmead, Westmead, New South Wales, Australia.
2 Faculty of Medicine and Health, The University of Sydney, Sydney, New South Wales, Australia.
World J Pediatr Congenit Heart Surg. 2019 Jul;10(4):475-484. doi: 10.1177/2150135119852319.
Management of hypoplastic left heart syndrome (HLHS) presents many challenges. We describe our institutional outcomes for management of patients with HLHS over the past 12 years and highlight our strategy for those with highly restrictive/intact interatrial septum (R/I-IAS).
Eighty-eight neonates with HLHS underwent surgical treatment, divided equally into Era-I (n = 44, April 2006 to February 2013) and Era-II (n = 44, March 2013 to June 2018). Up to 2013, all patients with R/I-IAS were delivered at an adjacent adult hospital and then moved to our hospital for intensive care and management. From 2014, these patients were delivered at a co-located theatre in our hospital with immediate atrial septectomy. The hybrid approach was occasionally used with preference for the Norwood procedure for suitable candidates.
One-year survival after Norwood procedure was 62.5% and 80% for Era-I and Era-II ( = not significant (ns)), respectively, and 41% of patients were categorized as high risk using conventional criteria. Survival at 1 year differed significantly between high-risk and standard-risk patients ( = 0.01). For high-risk patients, survival increased from 42% to 65% between eras ( = ns). In the R/I-IAS subgroup (n = 15), 11 underwent Norwood procedure after emergency atrial septectomy. Of these, seven born at the adjacent adult hospital had 40% survival to stage II versus 60% for the four born at the colocated theatre. Delivery in a colocated theatre reduced the birth-to-cardiopulmonary bypass median time from 445 (150-660) to 62 (52-71) minutes.
Reported surgical outcomes are comparable to multicenter reports and international databases. Proactive management for risk factors such as R/I-IAS may contribute to improved overall outcomes.
左心发育不全综合征（HLHS）的治疗面临诸多挑战。我们描述了过去12年我院对HLHS患者的治疗结果，并重点介绍了针对高度限制性/完整房间隔（R/I-IAS）患者的治疗策略。
88例HLHS新生儿接受了手术治疗，平均分为I期（n = 44，2006年4月至2013年2月）和II期（n = 44，2013年3月至2018年6月）。到2013年为止，所有R/I-IAS患者均在相邻的成人医院分娩，然后转至我院进行重症监护和治疗。从2014年起，这些患者在我院同址的手术室分娩并立即进行房间隔切除术。对于合适的患者，偶尔采用杂交手术方式，优先选择诺伍德手术。
诺伍德手术后I期和II期的1年生存率分别为62.5%和80%（无显著差异），41%的患者根据传统标准被归类为高危患者。高危和标准风险患者的1年生存率有显著差异（P = 0.01）。对于高危患者，不同时期的生存率从42%提高到了65%（无显著差异）。在R/I-IAS亚组（n = 15）中，11例在急诊房间隔切除术后接受了诺伍德手术。其中，在相邻成人医院出生的7例患者中，40%存活至II期，而在同址手术室出生的4例患者中这一比例为60%。在同址手术室分娩使出生至体外循环的中位时间从445（150 - 660）分钟缩短至62（52 - 71）分钟。
报告的手术结果与多中心报告和国际数据库相当。对R/I-IAS等危险因素进行积极管理可能有助于改善总体治疗效果。
