Cardiovascular disorders in patients with X-linked Emery—Dreifuss progressive muscular dystrophy

The paper gives the results of a clinical observation of 5 patients with genetically verified X-linked Emery—Dreifuss myopathy. Having no cardiovascular complaints, all the patients were found to have cardiac rhythm and conduction disturbances, such as atrial flutter (n=3), unstable supraventricular tachycardia (n=3), Lown grade III or higher premature contraction (n=3), and atrioventricular block (n=4), as evidenced by cardiological examination. Right atrial dilation was a pathognomonic and earliest sign of myocardial damage. The paper discusses the examination, management, and treatment of the patients. Indications for implantation of a pacemaker (implanted in 2 patients) and the advantages of monofocal models in this nosology are substantiated. 

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