Deciphering DSC2 arrhythmogenic cardiomyopathy electrical instability: From ion channels to ECG and tailored drug therapy.

Adrien MoreauJean-Baptiste ReisqsHelene Delanoe-AyariMarion PierreAlexandre JaninAntoine DeliniereFrancis BessièreAlbano C MeliAzzouz CharrabiEstele LafontCamille VallaDelphine BauerElodie MorelVincent GacheGilles MillatXavier NissanAdele FaucherreChris JoplingSylvain RichardAlexandre MejatPhilippe Chevalier

Published in: Clinical and translational medicine (2022)

Thorough analysis of hiPSC-CM derived from the DSC2 patient revealed abnormal repolarization dynamics, prompting the discovery of a short QT interval in some ACM patients. Overall, these results confirm a myogenic origin of ACM electrical instability and provide a rationale for prescribing class 1 and 3 AADs in ACM patients with increased ventricular repolarization reserve.

Keyphrases

end stage renal disease
heart failure
newly diagnosed
ejection fraction
primary care
chronic kidney disease
small molecule
prognostic factors
left ventricular
drug induced
case report
stem cells
emergency department
single cell
heart rate
bone marrow
smoking cessation
cell therapy