Shuhaiber Jeffrey H, Pigula Frank A
Rhode Island Hospital and Hasbro Children Hospital, The Cardiovascular Institute, Brown Medical School, 2 Dudley Street, MOC 360, Providence, RI 02905, USA.
Pediatr Cardiol. 2013 Jun;34(5):1063-72. doi: 10.1007/s00246-013-0677-5. Epub 2013 Mar 22.
Hypoplasia of the left side of the heart is the most common cause of death from congenital heart disease in the first weeks of life. Once considered a surgically fatal disease, hypoplasia has been successfully palliated for more than 30 years. Although the palliative route is staged by an early differential bypass of the systemic outflow and the venous inflow to the right ventricle, the left ventricle remains anatomically and biologically influential throughout. Given the variation of the left ventricle, contemporary outcomes for different hypoplastic left heart subsets can vary both early after palliation and long term. This review critically examines the contemporary understanding of the structure and function of the hypoplastic ventricle in this syndrome. It also provides insight into future research directions relevant to clinicians and surgeons.
左心发育不全是出生后最初几周内先天性心脏病致死的最常见原因。左心发育不全曾被认为是一种手术无法治愈的致命疾病,但在过去30多年里已成功实施姑息治疗。尽管姑息治疗方案是通过早期将体循环流出道和静脉血流入右心室进行分流来分阶段实施的,但左心室在解剖学和生物学上始终具有影响。鉴于左心室存在变异,不同类型左心发育不全的当代治疗效果在姑息治疗早期和长期都可能有所不同。本综述批判性地审视了目前对该综合征中发育不全心室的结构和功能的认识。它还为临床医生和外科医生提供了与未来研究方向相关的见解。