Abnormal Progenitor Cell Differentiation and Cardiomyocyte Proliferation in Hypoplastic Right Heart Syndrome.
- Resource Type
- Article
- Authors
- Yu, Yang; Wang, Cankun; Ye, Shiqiao; Xu, Zhaohui; Lin, Hui; Texter, Karen; Shukla, Vasudha; Ghadiali, Samir; Ma, Qin; Garg, Vidu; Zhao, Ming-Tao
- Source
- Circulation. 3/12/2024, Vol. 149 Issue 11, p888-891. 4p.
- Subject
- *HYPOPLASTIC left heart syndrome
*PROGENITOR cells
*CELL differentiation
*HEART
*NON-coding RNA
*HEART septum
*INDUCED pluripotent stem cells
PULMONARY atresia
- Language
- ISSN
- 0009-7322
This article explores the pathogenesis of ventricular hypoplasia in pulmonary atresia with intact ventricular septum (PA-IVS), a type of hypoplastic right heart syndrome. The study utilizes patient-specific induced pluripotent stem cells (iPSCs) and single-cell RNA sequencing to investigate the differentiation and proliferation of cardiomyocytes in PA-IVS. The findings suggest that impaired cardiomyocyte proliferation and abnormal lineage differentiation may contribute to the development of right ventricle hypoplasia in PA-IVS. Additionally, metabolic dysfunction and elevated mitochondrial activity are observed in PA-IVS iPSC-derived cardiomyocytes. Further research using 3-dimensional iPSC-derived cardiac organoids is recommended to better understand the specific defects in hypoplastic right heart syndrome. [Extracted from the article]