Arrhythmia-related workup in hereditary myopathies

Background: Arrhythmias determine life expectancy in patients with hereditary myopathies.

Aims: The aim of this study was to summarize recent advances in the diagnosis and management of arrhythmias in hereditary myopathies.

Methods: Literature search via PubMed and inclusion of own experiences were performed.

Results: All types of arrhythmias can be found in patients with hereditary myopathies, but some are more prevalent than others. Arrhythmias reported in myopathies include atrial fibrillation, atrial flutter, sick-sinus syndrome, preexcitation syndromes, atrioventricular conduction delay, intraventricular conduction delay, and ventricular tachycardia. Sudden cardiac death is a common finding in certain myopathies, and patients at risk for ventricular arrhythmias and sudden cardiac death should be identified early enough to implant a cardioverter-defibrillator to prevent a fatal outcome. Myopathies associated with a high risk for arrhythmias include laminopathies, Emery-Dreifuss muscular dystrophy, myotonic dystrophy I, mitochondrial myopathies, fatty-acid oxidation defects, and dystrophinopathies. To detect arrhythmias with high risk for sudden cardiac death, patients require close follow-up investigations or an implantable loop recorder. Documentation of severe arrhythmias requires immediate treatment according to established guidelines.

Conclusions: Patients with certain hereditary myopathies carry an increased risk for developing severe supraventricular or ventricular arrhythmias and for dying of sudden cardiac death. Close follow-up and long-term surveillance of the electrocardiogram may prevent fatal complications of arrhythmias in these patients.