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.
Luethje
Lars G C
LG
Department of Cardiology and Pneumology, Georg-August-University, Goettingen, Germany. LarsLuethje@t-online.de
Boennemann
Carsten
C
Goldfarb
Lev
L
Goebel
Hans H
HH
Halle
Martin
M
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
D003893:Desmin