Abstract 14092: Clinical Course and Age Dependent Arrhythmic Risk in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy Associated Desmosomal Mutation Carriers

Background: Presence of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) associated desmosomal mutations is thought to confer a lifelong risk of ventricular arrhythmias. We sought to define the arrhythmic penetrance in these patients and identify the association between age and occurrence of ventricular tachycardia (VT). Methods: The study population included 110 patients (age 2-80 years) belonging to 28 families with ARVD-associated heterozygous desmosomal mutations (PKP 2; 91%) from the Johns Hopkins ARVD registry. Detailed clinical phenotyping including arrhythmic evaluation was performed. Age at occurrence of first documented VT (Non sustained VT/Sustained VT) was used to determine arrhythmia free survival. Results: In our study population (49% males, median age 39 {IQR 28} years), VT was seen in 29 (26%) patients. The median age at the first episode of VT was 29 years (range 14-50, IQR 14 years) with very few patients experiencing VT incidence after 40 years of age. A diagnosis of A...