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

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. [ABSTRACT FROM AUTHOR]