Disease-specific aspects of management of cardiac arrhythmias in patients with muscular dystrophies

Cardiac arrhythmias are common in patients with various types of muscular dystrophies. The pathophysiological mechanisms of arrhythmias are complex and related to direct involvement of the conduction system and to the development of cardiomyopathy. The occurrence of atrio-ventricular conduction abnormalities and ventricular arrhythmias are associated with increased risk of sudden cardiac death. The threshold for device therapy ( cardiac pacemaker, implantable cardioverter defibrillator) is relatively low according to current guidelines due to the risk of rapid progression of the disease. Atrial arrhythmias carry high risk of stroke and anticoagulation should be considered even in young patients without coexisting risk factors for stroke as estimated by the CHA2DS2-VASc score. Patients with muscular dystrophies should be under regular cardiology follow up even in the absence of symptoms. Early detection of cardiac involvement is crucial. The management of patients with muscular dystrophies requires disease-specific and multidisciplinary approach due to the multi-organ involvement.