LAUSR

Abstract The current understanding of the genotype-phenotype relationship in arrhythmogenic cardiomyopathy continues to evolve. Initial studies described arrhythmogenic right ventricular cardiomyopathy as a fibrofatty replacement of cardiac myocytes in the right ventricle associated with a high incidence of ventricular arrhythmias. Newer studies suggest a broader phenotypic array of disease driven by distinct genetic mutations. This includes arrhythmogenic left ventricular cardiomyopathy associated with DSP mutations. Herein, we describe an unusual case of an adolescent male with syncope and findings of ALVC secondary to DSP mutation.