LMNA-related DCM with conduction defects is one of the most common forms of inherited dilated cardiomyopathy, second only to DCM associated with mutations in sarcomere protein genes [27, 28], with an estimated 5 to 10% of cases associated with a heterozygous LMNA mutation [29–31]. This evidence concerns the gene LMNA and familial dilated cardiomyopathy.