Abnormal Progenitor Cell Differentiation and Cardiomyocyte Proliferation in Hypoplastic Right Heart Syndrome.

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]