相关实验视频

Updated: May 2, 2026

Echocardiographic Evaluation of Atrial Communications before Transcatheter Closure

Echocardiographic Evaluation of Atrial Communications before Transcatheter Closure

Published on: February 8, 2022

缺血性左心综合征-一个范围审查审查

Consolato M Sergi^1,2

¹Department of Laboratory Medicine, University of Alberta, Edmonton, AB, Canada.

Translational pediatrics

|May 19, 2025

概括

缺血性左心综合征 (HLHS) 是一种复杂的先天性心脏缺陷,具有显著的遗传成分,尽管其确切原因仍然难以捉摸. 对内心干扰的进一步研究可能会为HLHS的发病过程提供新的见解.

科学领域:

心脏病学心脏病学
遗传学是一个遗传学.
发育生物学发展生物学

背景情况:

缺血左心综合征 (HLHS) 影响大约3%的新生儿患有先天性心脏病.
在没有及时的手术干预或心脏移植的情况下,它是患病婴儿死亡的主要原因.
虽然涉及遗传因素,但HLHS的特定遗传原因仅在一小组患者中被确定,这表明病因复杂.

研究的目的:

进行范围审查,以识别,综合和分析关于低质左心综合征 (HLHS) 的现有科学知识.
探索当前研究对HLHS病因和病原学的影响.

主要方法:

在2019年1月1日至2025年2月20日期间,在PubMed/MEDLINE,Scopus,Web of Science和Cochrane数据库中进行了全面的文献搜索.
搜索补充了灰色文献,包括互联网搜索和医学教科书.
研究问题侧重于有关HLHS病因和病原学的基本数据.

主要成果:

在最初识别的1364篇文章中,75篇被选择用于分析,并补充了25篇来自参考文献和灰色文献的其他文章.
HLHS的特点是心脏左侧结构发育不良,右心室占主导地位,使其成为一种复杂的先天性心脏缺陷.
尽管在像C57Bl/6J-b2b635Clo/J (俄俄州) 老鼠这样的模型中存在遗传影响和不完全透的证据,但HLHS的确切遗传原因仍然在很大程度上是未知的,并且在遗传上是异质的.

结论:

HLHS是一种复杂的先天性心脏病,需要进一步调查.
研究了内心脏在心室缺血症进展中的作用,这可能解释了由于血液流量受损而导致的形态变化.
研究结果表明,结合性遗传方法可能是HLHS的基础,支持其复杂遗传学的新范式.

关键词:

低增生性左心脏的左心脏.动物模型动物模型遗传学遗传学遗传学是一个缺氧缺氧是指缺氧的情况.病理学的病理学

更多相关视频

A Modified Sonographic Algorithm for Image Acquisition in Life-Threatening Emergencies in the Critically Ill Newborn

A Modified Sonographic Algorithm for Image Acquisition in Life-Threatening Emergencies in the Critically Ill Newborn

Published on: April 7, 2023

Author Spotlight: Effect of Left Atrial Ligation on Avian Embryonic Hearts and HLHS Implications

Author Spotlight: Effect of Left Atrial Ligation on Avian Embryonic Hearts and HLHS Implications

Published on: June 16, 2023

相关概念视频

Cardiomyopathy III: Hypertrophic Cardiomyopathy

Cardiomyopathy III: Hypertrophic Cardiomyopathy

Hypertrophic cardiomyopathy, or HCM, is an autosomal dominant genetic disorder characterized by asymmetric left ventricular hypertrophy without ventricular dilation. It is more common in men and is typically diagnosed in young, athletic adults.EtiologyHCM is primarily genetic and is caused by mutations in genes encoding sarcomeric proteins. Researchers have identified over 1400 mutations across at least 11 different genes. Among these, the most frequently occurring mutations are found in the...

Cardiomyopathy V: Interprofessional Care

Cardiomyopathy V: Interprofessional Care

Managing cardiomyopathy involves addressing underlying or precipitating causes, treating heart failure with medications, and implementing dietary changes and a balanced exercise and rest regimen.Lifestyle ModificationsCardiomyopathy patients should adopt a low-sodium diet to reduce fluid retention and manage heart failure. A personalized exercise and rest plan helps maintain physical fitness without overstraining the heart. Avoiding alcohol and tobacco is essential to prevent further damage to...