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
- aortic stenosis
- mitral valve
- induced pluripotent stem cells
- endothelial cells
- heart failure
- transcatheter aortic valve replacement
- cell cycle arrest
- transcatheter aortic valve implantation
- aortic valve replacement
- cardiac resynchronization therapy
- atrial fibrillation
- pulmonary hypertension
- left ventricular
- pulmonary artery
- cell death
- mass spectrometry
- gene expression
- copy number
- signaling pathway
- case report
- ejection fraction
- case control
- dna methylation
- high resolution