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Endocardium in Hypoplastic Left Heart Syndrome: Implications from In Vitro Study
Zhiyun Yu1,2,3, Ziyi Liu1,2, Vidhya Ravichandran1,2
1Perinatal Institute, Division of Pulmonary Biology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH 45229, USA.
Insights
This review explores how induced pluripotent stem cells (iPSCs) advance understanding of endocardial pathology in hypoplastic left heart syndrome (HLHS). It highlights cellular phenotypes and molecular mechanisms, offering insights into heart development defects.
Area of Science:
- Cardiovascular Biology
- Developmental Biology
- Stem Cell Research
Background:
- The endocardium, lining the heart ventricle, is crucial for valve formation but its role in hypoplastic left heart syndrome (HLHS) is not fully understood.
- Abnormalities like endocardial fibroelastosis (EFE) and valve malformations are linked to HLHS, yet molecular mechanisms in endocardial cells remain understudied.
Purpose of the Study:
- To review current in vitro studies utilizing induced pluripotent stem cells (iPSCs) to investigate endocardial pathology in HLHS.
- To emphasize novel findings on cellular phenotypes and molecular mechanisms contributing to HLHS endocardial defects.
Main Methods:
- Review of existing literature on in vitro studies of HLHS using iPSC technology.
- Analysis of research focusing on endocardial cell phenotypes and molecular pathways in HLHS models.
Main Results:
- iPSC-based models offer a powerful platform for studying genetic diseases like HLHS.
- These models reveal specific cellular phenotypes and molecular alterations in endocardial cells relevant to HLHS pathology.
- Current research is beginning to elucidate the molecular underpinnings of endocardial dysfunction in HLHS.
Conclusions:
- In vitro iPSC studies are critical for dissecting endocardial pathology in HLHS.
- Further research is needed to refine these models for better recapitulation of in vivo endocardial phenotypes.
- Understanding endocardial cell behavior is key to developing future therapeutic strategies for HLHS.
Abstract:
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.
