Endocardium in Hypoplastic Left Heart Syndrome: Implications from In Vitro Study.
Zhiyun YuZiyi LiuVidhya RavichandranBonny LamiMingxia GuPublished in: Journal of cardiovascular development and disease (2022)
Endocardium lines the inner layer of the heart ventricle and serves as the source of valve endothelial cells and interstitial cells. Previously, endocardium-associated abnormalities in hypoplastic left heart syndrome (HLHS) have been reported, including endocardial fibroelastosis (EFE) and mitral and aortic valve malformation. However, few mechanistic studies have investigated the molecular pathological changes in endocardial cells. Recently, the emergence of a powerful in vitro system-induced pluripotent stem cells (iPSCs)-was applied to study various genetic diseases, including HLHS. This review summarized current in vitro studies in understanding the endocardial pathology in HLHS, emphasizing new findings of the cellular phenotypes and underlying molecular mechanisms. Lastly, a future perspective is provided regarding the better recapitulation of endocardial phenotypes in a dish.
Keyphrases
- aortic valve
- induced apoptosis
- mitral valve
- induced pluripotent stem cells
- aortic stenosis
- endothelial cells
- heart failure
- transcatheter aortic valve replacement
- transcatheter aortic valve implantation
- oxidative stress
- endoplasmic reticulum stress
- cell proliferation
- mass spectrometry
- current status
- coronary artery
- atomic force microscopy