title: Prophylactic implantable cardioverter defibrillator placement in a sporadic desmin related myopathy and cardiomyopathy.
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.
authors.affiliation: Department of Cardiology and Pneumology, Georg-August-University, Goettingen, Germany. LarsLuethje@t-online.de
authors.forename: Lars G C
authors.initials: LG
authors.lastname: Luethje
authors.forename: Carsten
authors.initials: C
authors.lastname: Boennemann
authors.forename: Lev
authors.initials: L
authors.lastname: Goldfarb
authors.forename: Hans H
authors.initials: HH
authors.lastname: Goebel
authors.forename: Martin
authors.initials: M
authors.lastname: Halle
mesh_terms: D000293:Adolescent; D009202:Cardiomyopathies / Q000150:complications*; D017147:Defibrillators, Implantable; D003893:Desmin / Q000235:genetics*; D006801:Humans; D008297:Male; D009154:Mutation; D020914:Myopathies, Structural, Congenital / Q000150:complications*; D017180:Tachycardia, Ventricular / Q000209:etiology / Q000628:therapy
chemical_list: D003893:Desmin