Uncompensated mitochondrial oxidative stress underlies heart failure in an iPSC-derived model of congenital heart disease

Xu et al. demonstrated feasibility of modeling early heart failure using induced pluripotent stemcell-derived cardiomyocytes from patients with HLHS. They observed that heart failure is linked to increased apoptosis, mitochondrial dysfunction, redox, and endoplasmic reticulum stress, all of which were rescued by sildenafil or TUDCA, suggesting potential for therapy.