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: Aly Zaki, Amir Zaidi, William G Newman, Clifford J Garratt
• Journal: Journal of cardiovascular electrophysiology (J Cardiovasc Electrophysiol) Vol. 24 Issue 9 Pg. 1051-3 (Sep 2013) ISSN: 1540-8167 [Electronic] United States
• PMID: 23577876 (Publication Type: Case Reports, Journal Article)
• Copyright: © 2013 Wiley Periodicals, Inc.

Chemical References

• LAMP2 protein, human
• Lysosomal-Associated Membrane Protein 2

Topics

• Adolescent
• Cardiomyopathy, Hypertrophic (diagnosis, genetics, therapy)
• Defibrillators, Implantable
• Electric Countershock (methods)
• Glycogen Storage Disease Type IIb (diagnosis, genetics, therapy)
• Humans
• Lysosomal-Associated Membrane Protein 2 (genetics)
• Male
• Subcutaneous Tissue