6533b858fe1ef96bd12b5b18

RESEARCH PRODUCT

Prophylactic Implantable Cardioverter Defibrillator Placement in a Sporadic Desmin Related Myopathy and Cardiomyopathy

Martin HalleLars G. C. LuethjeLev G. GoldfarbCarsten BoennemannHans H. Goebel

subject

Malemedicine.medical_specialtyAdolescentmedicine.medical_treatmentCardiomyopathyDiseaseDesminInternal medicineHumansMedicineDESMIN-RELATED MYOPATHYbusiness.industryImplantable Cardioverter-Defibrillator PlacementGeneral MedicineImplantable cardioverter-defibrillatormedicine.diseaseSkeletal myopathyDefibrillators ImplantableSustained ventricular tachycardiaMutationTachycardia VentricularCardiologyDesminCardiomyopathiesCardiology and Cardiovascular MedicinebusinessMyopathies Structural Congenital

description

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.

yearjournalcountryeditionlanguage
2004-04-14Pacing and Clinical Electrophysiology
https://doi.org/10.1111/j.1540-8159.2004.00484.x