■具有复杂的单心室解剖结构并伴有大动脉转位和全身性流出道阻塞(SV-TGA-SOO)的患者进行各种初始缓解,最终目标是Fontan循环。我们用多种技术检查纵向经验,包括姑息性动脉转换手术(PASO)后公布的最大队列,描述结果和决策因素。
■对1995年至2022年在单一机构接受了初次手术缓解的SV-TGA-SOO新生儿进行了回顾性审查。
■总共,71例患有SV-TGA-SOO的新生儿在中位年龄为7天(四分位数范围,6-10)通过pASO(n=23),肺动脉带(PAB)有或没有弓修复(n=25),或改良的Norwood与Damus-Kaye-Stansel主动脉肺动脉融合(n=23)。单心室病理包括双入口左心室(n=37,52%),三尖瓣闭锁(n=27,38%),和其他(n=7,10%)。所有死亡率(n=5,7%)发生在PAB(n=3)和Norwood(n=2)之后的第一个期间。PAB组的主动脉下梗阻通过手术切除治疗(n=10,在初次手术时为7)和/或延迟的主动脉肺动脉合并(n=13,52%)。2例pASO患者(9%)术后早期冠状动脉并发症,1需要手术翻修。幸存者的中位随访时间为10.4年(四分位间距,4.5-16.6年)。将患者与最初的姑息类型进行比较,显着差异包括球室孔的大小,初始操作时的重量,操作持续时间,术后住院时间,第二阶段缓和的时间,多次肺动脉再介入,和左肺动脉介入治疗。总生存率无显著差异,丰坦完成,在第一个间期无再干预生存,无肺动脉再介入生存,长期的系统瓣膜能力,或心室功能障碍。
■通过pASO对SV-TGA-SOO进行新生儿缓解后,可实现出色的中长期结果,PAB,修改了诺伍德,具有相当的存活率和Fontan完成。初始缓解策略应个体化,以通过确保主动脉下通路通畅和可触及的肺动脉来优化成功Fontan的解剖学和生理学。pASO是考虑这些异质性病变的合理策略。
UNASSIGNED: Patients with complex single-ventricle anatomy with transposed great arteries and systemic outflow obstruction (SV-TGA-SOO) undergo varied initial palliation with ultimate goal of Fontan circulation. We examine a longitudinal experience with multiple techniques, including the largest published cohort following palliative arterial switch operation (pASO), to describe outcomes and decision-making factors.
UNASSIGNED: Neonates with SV-TGA-SOO who underwent initial surgical palliation from 1995 to 2022 at a single institution were retrospectively reviewed.
UNASSIGNED: In total, 71 neonates with SV-TGA-SOO underwent index surgical palliation at a median age of 7 days (interquartile range, 6-10) by pASO (n = 23), pulmonary artery band (PAB) with or without arch repair (n = 25), or modified Norwood with Damus-Kaye-Stansel aortopulmonary amalgamation (n = 23). Single-ventricle pathology included double-inlet left ventricle (n = 37, 52%), tricuspid atresia (n = 27, 38%), and others (n = 7, 10%). All mortalities (n = 5, 7%) occurred in the first interstage period after PAB (n = 3) and Norwood (n = 2). Subaortic obstruction in the PAB group was addressed by operative resection (n = 10 total, 7 at index operation) and/or delayed aortopulmonary amalgamation (n = 13, 52%). Two patients with pASO (9%) had early postoperative coronary complications, 1 requiring operative revision. Median follow-up for survivors was 10.4 years (interquartile range, 4.5-16.6 years). Comparing patients by their initial palliation type, notable significant differences included size of bulboventricular foramen, weight at initial operation, operation duration, postoperative length of stay, time to second-stage palliation, multiple pulmonary artery reinterventions, and left pulmonary artery interventions. There were no significant differences in overall survival, Fontan completion, reintervention-free survival in the first interstage period, pulmonary artery reintervention-free survival, long-term systemic valve competency, or ventricular dysfunction.
UNASSIGNED: Excellent mid- to long-term outcomes are achievable following neonatal palliation for SV-TGA-SOO via pASO, PAB, and modified Norwood, with comparable survival and Fontan completion. Initial palliation strategy should be individualized to optimize anatomy and physiology for successful Fontan by ensuring an unobstructed subaortic pathway and accessible pulmonary arteries. pASO is a reasonable strategy to consider for these heterogeneous lesions.