Sedmera David
Institute of Anatomy, First Faculty of Medicine, Charles University, 128 00 Prague, Czech Republic.
Laboratory of Developmental Cardiology, Institute of Physiology, Czech Academy of Sciences, 142 20 Prague, Czech Republic.
J Cardiovasc Dev Dis. 2022 Apr 11;9(4):113. doi: 10.3390/jcdd9040113.
Hypoplastic left heart syndrome (HLHS) is a rare but deadly form of human congenital heart disease, most likely of diverse etiologies. Hemodynamic alterations such as those resulting from premature foramen ovale closure or aortic stenosis are among the possible pathways.
The information gained from studies performed in the chick model of HLHS is reviewed. Altered hemodynamics leads to a decrease in myocyte proliferation causing hypoplasia of the left heart structures and their functional changes.
Although the chick phenocopy of HLHS caused by left atrial ligation is certainly not representative of all the possible etiologies, it provides many useful hints regarding the plasticity of the genetically normal developing myocardium under altered hemodynamic loading leading to the HLHS phenotype, and even suggestions on some potential strategies for prenatal repair.
左心发育不全综合征（HLHS）是一种罕见但致命的人类先天性心脏病，病因很可能多种多样。诸如卵圆孔过早闭合或主动脉狭窄导致的血流动力学改变是可能的发病途径之一。
回顾在HLHS鸡模型中进行的研究所获得的信息。血流动力学改变导致心肌细胞增殖减少，引起左心结构发育不全及其功能变化。
尽管由左心房结扎引起的HLHS鸡表型肯定不能代表所有可能的病因，但它为基因正常发育的心肌在导致HLHS表型的血流动力学负荷改变下的可塑性提供了许多有用的线索，甚至对一些产前修复的潜在策略也有启示。
