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左心发育不全综合征:当前的治疗方式与治疗结果

Hypoplastic left heart syndrome: current modalities of treatment and outcomes.

作者信息

Mohanty Smruti Ranjan, Patel Agastya, Kundan Simran, Radhakrishnan Hari Bipin, Rao Suresh Gururaja

机构信息

Department of Pediatric and Congenital Heart Surgery, Kokilaben Dhirubhai Ambani Hospital, Four Bungalows, Andheri (West), Mumbai, 400053 India.

Medical University of Gdańsk, Gdańsk, Poland.

出版信息

Indian J Thorac Cardiovasc Surg. 2021 Jan;37(Suppl 1):26-35. doi: 10.1007/s12055-019-00919-7. Epub 2020 Mar 11.

Abstract

Hypoplastic left heart syndrome is a constellation of malformations which result from the severe underdevelopment of any left-sided cardiac structures. Once considered to be universally fatal, the prognosis for this condition has tremendously improved over the past four decades since the work of William Norwood in the early 1980s. Today, a staged surgical approach is applied for palliating this distinctive cohort of patients, in which they undergo three operative procedures in the first 10 years of their life. Advancements in medical technologies, surgical techniques, and our growing experience in the management of HLHS have made survival into adulthood a possibility. Through this review, we present the different phases of the staged approach with primary focus on stage 1-its modifications, current technique, alternatives, and latest outcomes.

摘要

左心发育不全综合征是一组由于任何左侧心脏结构严重发育不全而导致的畸形。这种疾病曾被认为普遍致命,但自20世纪80年代初威廉·诺伍德开展相关工作以来,在过去的四十年里其预后有了极大改善。如今,一种分期手术方法被用于缓解这类特殊患者群体的病情,他们在生命的头10年里要接受三次手术。医疗技术、手术技巧的进步以及我们在左心发育不全综合征管理方面不断积累的经验,使得患者存活至成年成为可能。通过本综述,我们介绍分期手术方法的不同阶段,主要聚焦于第一阶段——其改良方法、当前技术、替代方案及最新成果。

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Hypoplastic left heart syndrome: current modalities of treatment and outcomes.
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