Decreased Contraction Rate, Altered Calcium Transients, and Increased Proliferation seen in Patient-specific iPSC-CMs Modeling Ebstein's Anomaly and Left Ventricular Noncompaction.

These discoveries suggest that EA/LVNC characteristics or pathogenesis may result from decreased contractile ability, altered calcium transients, and cell cycle dysregulation. Through CRISPR-Cas9 correction and introduction of the KLHL26 variant in the daughter lines, we will build upon this understanding to inform exploration of critical clinical targets for EA/LVNC and related forms of cardiomyopathy and heart failure.