LAUSR

Introduction Lamin cardiomyopathies are part of a larger spectrum of disorders involvingmutations in the lamin (A/C) gene (LMNA). The LMNA gene codes for A-type lamins, a group of ubiquitous nuclear membrane filament proteins, which provide nuclear stability. Defects in lamin function result in nuclear fragility, altered nuclear protein interaction, mechanotransduction, signaling, and gene expression with significant phenotypic heterogeneity. LMNA mutations underpin neuromuscular and adipose tissue phenotypes such as Emery-Dreifuss muscular dystrophy, familial lipodystrophy, and Charcot-Marie-Tooth disease. Cardiac involvement can result in a characteristic phenotype with familial dilated cardiomyopathy, atrioventricular (AV) block, arrhythmias, and an aggressive clinical course. We present a classic case of a patient that captures the archetypal natural history of LMNA cardiomyopathy, combined with comprehensive electroanatomic biventricular endocardial and epicardial substrate characterization for ventricular tachycardia (VT), complemented with whole-heart macropathology, histopathology, and genetic evaluation.