Contemporary Insights into LMNA Cardiomyopathy.

PURPOSE OF REVIEW: This review aims to explore how a diagnosis of LMNA-related cardiomyopathy (LMNA-CM) informs clinical management, focusing on the prevention and management of its complications, through practical clinical strategies.

RECENT FINDINGS: Longitudinal studies have enhanced our understanding of the natural history of LMNA-CM including its arrhythmic and non-arrhythmic complications. A LMNA specific ventricular arrhythmia risk prediction strategy has been integrated into clinical practice guidelines. Although less robust, observational studies are shaping gene-specific strategies for mitigating other complications including atrioventricular block, atrial fibrillation and cardiomyopathy, while novel therapies have been evaluated in clinical trials. LMNA-CM follows an aggressive yet generally stereotyped course. Early recognition of anticipated complications allows for more effective prevention and management in both symptomatic and asymptomatic patients.