A Case of Fabry Disease with HOCM Like Heart Lesion that Intraventricular Pressure Gradient Decreased by Dual Chamber Pacing

The case of a 57-year-old man, who had a brother suddenly died at 62 years of age, admitted to other hospital with palpitation. Because his Holter ECG showed non-sustained ventricular tachycardia, and ultrasound cardiography showed left ventricular hypertrophy with normal cardiac function, he was introduced to our hospital for further cardiac examination of hypertrophic cardiomyopathy. Although his coronary angiography showed no organic stenosis, and his magnetic resonance imaging demonstrated asymmetrical septal hypertrophy and delayed enhancement on inferolateral wall of left ventricle compatible with hypertrophic cardiomyopathy, his biopsied tissue from cardiac muscle revealed cardiac Fabry disease. He received enzyme replacement therapy after implantation of implantable cardioverter defibrillator (ICD) for primary prevention. After 7 months from implantation, he had an appropriate ICD therapy for sustained ventricular tachycardia. Primary prevention of ICD implantation for patients with Fabry disease has not been established, while some cases of cardiac Fabry disease show fatal ventricular tachyarrhythmia in their lives. Primary prevention of ICD could be a considerable therapeutic option in some patients with cardiac Fabry disease, especially who demonstrated a family history of sudden death or ventricular tachyarrhythmias.