Journal
PACE-PACING AND CLINICAL ELECTROPHYSIOLOGY
Volume 27, Issue 4, Pages 559-560Publisher
BLACKWELL FUTURA PUBLISHING, INC
DOI: 10.1111/j.1540-8159.2004.00484.x
Keywords
desmin related myopathy; cardiomyopathy; ICD
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LUETHJE, L.G.C., ET AL.: Prophylactic Implantable Cardioverter Defibrillator Placement in a Sporadic Desmin Related Myopathy and Cardiomyopathy. Desminopathy is a neuromuscular disorder associated with the accumulation of the protein desmin. This article reports a case of a man with a mutation in the desmin gene suffering from cardiomyopathy and skeletal myopathy. This patient underwent implantable cardioverter defibrillator (ICD) implantation for prognostic considerations and subsequently developed a sustained ventricular tachycardia (SVT). While nonsustained VTs (NSVT) have previously been reported, this is the first time that a SVT could be seen in a patient with this disease.
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