Advantages of a Subcutaneous Implantable Cardioverter-Defibrillator in LAMP2 Hypertrophic Cardiomyopathy


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Address for correspondence: Clifford J. Garratt, D.M., F.R.C.P., Manchester Heart Centre, Manchester Royal Infirmary, Manchester M13 9WL, UK. Fax: +44-161-276-7956; E-mail:

S-ICD in Danon Disease

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.