Prophylactic implantable cardioverter defibrillator placement in a sporadic desmin related myopathy and cardiomyopathy |
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Authors: | Luethje Lars G C Boennemann Carsten Goldfarb Lev Goebel Hans H Halle Martin |
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Institution: | Department of Cardiology and Pneumology, Georg-August-University, Goettingen, Germany. LarsLuethje@t-online.de |
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Abstract: | 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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Keywords: | desmin related myopathy cardiomyopathy ICD |
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