Genetics of Dilated Cardiomyopathy: Risk of Conduction Defects and Sudden Cardiac Death

Dilated cardiomyopathy is familial in at least 40--60% of cases and causal mutations have been identified in more than 40 different genes. Mutations in lamin A/C (LMNA) and desmosomal components appear associated with increased risk of sudden cardiac death, the latter in the context of left-dominant arrhythmogenic cardiomyopathy. Specific clinical features may be valuable in identifying patients with these mutations. Routine sequencing of all the genes implicated in dilated cardiomyopathy may not be cost-effective at present. Targeted mutation screening of LMNA and desmosomal components is recommended and may facilitate prognostication and management.