定义低可塑性左心综合征 (HLHS) 的频谱
Jeffrey P Jacobs1, Mark S Bleiweis1, Meryl S Cohen2
1Division of Cardiovascular Surgery, Departments of Surgery and Pediatrics, Congenital Heart Center, University of Florida, Gainesville, FL, United States of America.
World journal for pediatric & congenital heart surgery
|November 7, 2023
概括
低发性左心综合征 (HLHS) 是一种先天性心脏缺陷的谱,涉及左心脏发育不足. 带有完整腹腔隔膜的HLHS (HLHS+IVS) 和带有腹腔隔膜缺陷的HLHS (HLHS+VSD) 都属于这个定义.
科学领域:
- 心脏病学 心脏病学
- 儿童心脏病学 儿童心脏病学
- 医学诊断 医学诊断 医学诊断
背景情况:
- 缺血性左心综合征 (HLHS) 是一种严重的先天性心脏缺陷.
- 标准化定义对于准确的诊断和分类至关重要.
- 2021年国际儿科和先天性心脏病规范和ICD-11提供了更新的定义.
研究的目的:
- 根据2021年"国际儿科和先天性心脏病规范"和ICD-11的定义,提出左心低质综合征 (HLHS) 的定义.
- 澄清HLHS涵盖了一系列疾病,包括那些心室隔膜完整 (HLHS+IVS) 和心室隔膜缺陷 (HLHS+VSD) 的疾病.
主要方法:
- 审查和综合2021年"国际儿科和先天性心脏病规范"提供的官方定义.
- 分析HLHS的诊断标准,包括特定的心脏形.
- 不同HLHS表型的比较,例如HLHS+IVS和HLHS+VSD.
主要成果:
- HLHS被定义为一系列先天性心血管形,其特点是左心发育不良.
- 关键特征包括左心室的低成形, mitra 和/或大动脉,上升大动脉和大动脉门.
- 尽管存在表型差异,但HLHS+IVS和HLHS+VSD都被认为是HLHS谱的一部分.
结论:
- 提供的定义为HLHS作为缺陷的范围提供了全面的理解.
- 对HLHS表型的准确分类对于临床管理和研究至关重要.
- 该定义确保了全球范围内诊断和报告HLHS病例的一致性.
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