Hypoplastic Left Heart Syndrome Current Considerations and Expectations
Hypoplastic Left Heart Syndrome Current Considerations and Expectations
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DOI：
10.1016/j.jacc.2011.09.022
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发表时间：
2012-01-03
影响因子：
24
通讯作者：
Martin, Gerard R.
中科院分区：
文献类型：
--
作者：
Feinstein, Jeffrey A.;Benson, D. Woodrow;Martin, Gerard R.
在最近的时代，没有先天性心脏病经历了更戏剧性的变化，在诊断方法，管理和结果比发育不良的左心综合征（HLHS）。在此期间，5岁（包括Fontan）的存活率范围为50%至69%，但目前的预期是，今天出生的HLHS新生儿中有70%可能达到成年。虽然HLHS的3阶段治疗方法现在是有充分依据的，但各中心之间存在显著差异。在这篇白色论文中，我们介绍了我们在护理阶段对HLHS的理解和治疗的最新技术水平：1）前期I：胎儿和新生儿评估和管理; 2）阶段I：围手术期护理、阶段间监测和管理策略; 3）阶段II：手术; 4）阶段III：Fontan手术; 5）长期随访。围绕HLHS的遗传学，发育结果和生活质量的问题，除了许多其他考虑因素照顾这组复杂的患者。
In the recent era, no congenital heart defect has undergone a more dramatic change in diagnostic approach, management, and outcomes than hypoplastic left heart syndrome (HLHS). During this time, survival to the age of 5 years (including Fontan) has ranged from 50% to 69%, but current expectations are that 70% of newborns born today with HLHS may reach adulthood. Although the 3-stage treatment approach to HLHS is now well founded, there is significant variation among centers. In this white paper, we present the current state of the art in our understanding and treatment of HLHS during the stages of care: 1) pre-Stage I: fetal and neonatal assessment and management; 2) Stage I: perioperative care, interstage monitoring, and management strategies; 3) Stage II: surgeries; 4) Stage III: Fontan surgery; and 5) long-term follow-up. Issues surrounding the genetics of HLHS, developmental outcomes, and quality of life are addressed in addition to the many other considerations for caring for this group of complex patients.