Prevalence and prognostic impact of cardiac resonance abnormalities in myotonic dystrophy patients

Abstract

Myotonic dystrophy (DM) is an autosomal dominant inherited neuromuscular disorder characterized by progressive muscular weakness and multisystem involvement. Cardiac involvement is recorded in about 80 % of cases and often precedes the involvement of skeletal muscle. Cardiovascular magnetic resonance (CMR) has been recently included as a recommended test in the cardiac assessment of DM patients at the time of diagnosis, and subsequently with periodic testing. Our systematic review aims to describe the prevalence and prognosis of disease-related CMR abnormalities in DM patients.

Keywords: Arrhythmias; Atrial fibrillation; Cardiac magnetic resonance; Late gadolinium enhancement; Myotonic dystrophy.