title: Advantages of a subcutaneous implantable cardioverter-defibrillator in LAMP2 hypertrophic cardiomyopathy.
abstract: Danon disease is a rare X-linked lysosomal disease causing severe hypertrophic cardiomyopathy (LAMP2 cardiomyopathy) and an extremely poor prognosis in males, with several reported cases of sudden cardiac death despite the use of transvenous implantable cardioverter defibrillators (TV-ICD). We describe a case in which a TV-ICD was unable to defibrillate induced ventricular fibrillation (VF), but a wholly subcutaneous system (S-ICD) was successful in terminating induced VF and spontaneous ventricular tachycardia. These findings have relevance to the selection of device therapy in the management of these individuals and a wider group of young patients with severe hypertrophic cardiomyopathy.
authors.affiliation: Manchester Heart Centre/Institute of Cardiovascular Medicine; Centre for Genetic Medicine, Institute of Human Development, University of Manchester, Manchester Academic Health Science Centre, Manchester, UK.
authors.forename: Aly
authors.initials: A
authors.lastname: Zaki
authors.forename: Amir
authors.initials: A
authors.lastname: Zaidi
authors.forename: William G
authors.initials: WG
authors.lastname: Newman
authors.forename: Clifford J
authors.initials: CJ
authors.lastname: Garratt
mesh_terms: D000293:Adolescent; D002312:Cardiomyopathy, Hypertrophic / Q000175:diagnosis / Q000235:genetics / Q000628:therapy; D017147:Defibrillators, Implantable; D004554:Electric Countershock / Q000379:methods*; D052120:Glycogen Storage Disease Type IIb / Q000175:diagnosis / Q000235:genetics* / Q000628:therapy*; D006801:Humans; D052119:Lysosomal-Associated Membrane Protein 2 / Q000235:genetics*; D008297:Male; D040521:Subcutaneous Tissue
chemical_list: C497811:LAMP2 protein, human; D052119:Lysosomal-Associated Membrane Protein 2
keywords: Danon disease; LAMP2; hypertrophic cardiomyopathy; subcutaneous implantable cardioverter-defibrillator; ventricular tachycardia