Differential approach to treating life-threatening arrhythmogenic conditions in patients with X-linked Emery—Dreifuss myopathy
Abstract
The paper gives 2 clinical examples of patients with the same diagnosis of progressive X-linked Emery-Dreifuss muscular dystrophy and the different course of rhythm and conduction disorders, which have determined various approaches to management tactics. There is evidence for a differential approach to pacemaker implantation, by confirming the correctness of management tactics in a long-term follow-up. A 27-year-old patient had no indications for pacemaker implantation. The other patient aged 13 years was found to have indications and underwent implantation; however, thrombi formed on the electrodes and the atrial electrode needed to be further disconnected.
About the Authors
O. S. Groznova
Research Clinical Institute of Pediatrics, N.I. Pirogov Russian National Research Medical University, Moscow; Research Center for Medical Genetics, Russian Academy of Medical Sciences, Moscow
Russian Federation
Russian Federation
G. E. Rudenskaya
Research Clinical Institute of Pediatrics, N.I. Pirogov Russian National Research Medical University, Moscow; Research Center for Medical Genetics, Russian Academy of Medical Sciences, Moscow
Russian Federation
Russian Federation
T. A. Adyan
Research Clinical Institute of Pediatrics, N.I. Pirogov Russian National Research Medical University, Moscow; Research Center for Medical Genetics, Russian Academy of Medical Sciences, Moscow
Russian Federation
Russian Federation
D. A. Kharlamov
Research Clinical Institute of Pediatrics, N.I. Pirogov Russian National Research Medical University, Moscow; Research Center for Medical Genetics, Russian Academy of Medical Sciences, Moscow
Russian Federation
Russian Federation
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Review
For citations:
Groznova O.S., Rudenskaya G.E., Adyan T.A., Kharlamov D.A. Differential approach to treating life-threatening arrhythmogenic conditions in patients with X-linked Emery—Dreifuss myopathy. Rossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics). 2015;60(4):63-68. (In Russ.)
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