Emery-Dreifuss Muscular Dystrophy 1 is associated with high risk of malignant ventricular arrhythmias and end-stage heart failure.
Douglas E CanniePetros SyrrisAlexandros ProtonotariosAthanasios BakalakosJean-François PrunyRaffaello DitarantoCristina Martinez-VeiraJose M Larrañaga-MoreiraKristen MedoFrancisco José Bermúdez-JiménezRabah Ben YaouFrance LeturqAinhoa Robles MezcuaChiara Marini-BettoloEva CabreraChloe ReuterJavier Limeres FreireJosé F Rodríguez-PalomaresLuisa MestroniMatthew R G TaylorVictoria N ParikhEuan A AshleyRoberto Barriales VillaJuan Jiménez JaimezPablo García-PavíaPhilippe CharronElena BiaginiJosé M García PinillaJohn BourkeKonstantinos SavvatisKarim WahbiPerry Mark ElliottPublished in: European heart journal (2023)
Male EMD variant-carriers have a risk of progressive heart failure and ventricular arrhythmias similar to that of male LMNA variant-carriers. Early implantable cardioverter defibrillator implantation and heart failure drug therapy should be considered in male EMD variant-carriers with cardiac disease.