Arrhythmia-related workup in hereditary myopathies

作者：Finsterer Josef^*; Stoellberger Claudia; Keller Hans

来源：Journal of Electrocardiology, 2012, 45(4): 376-384.

DOI：10.1016/j.jelectrocard.2012.02.003

• Heart rhythm
• Arrhythmias
• Cardiac conduction defects
• Impulse generation or propagation defects
• Electrocardiogram
• Holter
• Loop recorder
• ECG recording

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.

• 出版日期2012-8