Abstract 12895: Characteristics and Outcome of Ventricular Tachycardia in Advanced Fabry Disease Cardiomyopathy
Introduction: Ventricular tachycardia (VT) are suspected the leading cause of premature death in patients with advanced Fabry cardiomyopathy. However, origin and outcome of respective tachyarrhythmic events have yet not been comprehensively investigated.
Methods: All patients (n=261) in care at the Fabry Center for Interdisciplinary Therapy (FAZIT) Würzburg, Germany, with history of (n=17) or demand for (n=1) implantable cardioverter defibrillator (ICD) device therapy for primary or secondary prevention of ventricular tachyarrhythmic events due to advanced Fabry cardiomyopathy underwent comprehensive clinical investigations including analysis of 12-lead ECG, 24-hour Holter ECG, and ICD Holter chart reviews for documented VT episodes with special focus on characteristics and outcome of respective events.
Results: Study subjects were mainly of male gender (14/18, 78%) and middle aged (55±10 years, range 33-74) at point of ICD demand. In total, 11/18 patients (61%) suffered from at least one episode of VT. All documented episodes were sustained monomorphic ventricular tachycardia. Most 12-lead ECG documented VT events (n=7) were self-terminated (n=6, 86%). Five subjects were in need of anti-tachycardia overdrive pacing and four of shocks for termination of respective VT events when followed over time. Analyzing VT episodes documented in 12-lead ECG showed a correlation of VT episode origination from the area of myocardial replacement fibrosis, mostly from the postero-lateral wall segments of the left ventricle. Radiofrequency catheter ablation was performed in three patients (17%), of whom two still suffered from VT episodes afterwards and both finally died due to end-stage heart failure complications. Three other subjects from this group also died due to cardiac events (in total 4/18, 22%), whereas one (6%) due to Fabry-associated stroke.
Conclusions: VT is common in advanced Fabry disease. As all documented VT episodes were monomorphic, radiofrequency catheter ablation might represent a yet underestimated therapy option besides ICD device therapy. However, due to end-stage heart failure associated complications, cardiac mortality remains a substantial factor questioning the clinical impact and benefit in end-stage Fabry disease.
- Ventricular tachycardia
- Ablation, radiofrequency
- Implantable cardiovertor defibrillator
- Heart failure
Author Disclosures: D. Oder: Other; Modest; Travel assistence by Genzyme Corporation, Cambridge, Massachusetts, and Shire Plc., Dublin, Ireland. D. Liu: None. K. Hu: None. T. Salinger: None. F. Weidemann: Consultant/Advisory Board; Modest; Genzyme Corporation, Cambridge, Massachusetts, and Shire Plc., Dublin, Ireland. G. Ertl: None. C. Wanner: Consultant/Advisory Board; Modest; Genzyme Corporation, Cambridge, Massachusetts, and Shire Plc., Dublin, Ireland. P. Nordbeck: Research Grant; Significant; Federal Ministry of Education and Research of the Federal Republic of Germany (BMBF 01EO1504, MO2). Consultant/Advisory Board; Modest; Genzyme Corporation, Cambridge, Massachusetts, and Shire Plc., Dublin, Ireland.
- © 2016 by American Heart Association, Inc.