Cardiovascular manifestations of myotonic dystrophy

Abstract

The myotonic dystrophies type 1 and type 2 are the most frequently inherited neuromuscular diseases of adult life. They are progressive multisystem disorders and predominantly affect the conduction system of the heart. Tachy and bradyarrhythmias are the predominant cardiac manifestation and are associated with up to 30% of all cause mortality in DM. Routine cardiac screening is recommended in all patients with a minimum of a yearly ECG, ECHO and 24 h Holter monitoring. In our practice we recommend electrophysiological studies when there are any abnormalities on the ECG. Future work will aim to define criteria for implantation of pacemakers and implantable cardioverter defibrillators.