Abstract | BACKGROUND: METHODS AND RESULTS: We studied 106 consecutive patients (62 men and 44 women; age, 35.6±18 years) with arrhythmogenic right ventricular cardiomyopathy/dysplasia who received an ICD based on 1 or more arrhythmic risk factors such as syncope, nonsustained ventricular tachycardia, familial sudden death, and inducibility at programmed ventricular stimulation. During follow-up of 58±35 months, 25 patients (24%) had appropriate ICD interventions and 17 (16%) had shocks for life-threatening VF or ventricular flutter. At 48 months, the actual survival rate was 100% compared with the VF/ ventricular flutter-free survival rate of 77% (log-rank P=0.01). Syncope significantly predicted any appropriate ICD interventions (hazard ratio, 2.94; 95% confidence interval, 1.83 to 4.67; P=0.013) and shocks for VF/ ventricular flutter (hazard ratio, 3.16; 95% confidence interval, 1.39 to 5.63; P=0.005). The positive predictive value of programmed ventricular stimulation was 35% for any appropriate ICD intervention and 20% for shocks for VF/ ventricular flutter, with a negative predictive value of 70% and 74%. None of the 27 asymptomatic patients with isolated familial sudden death had appropriate ICD therapy. Twenty patients (19%) had inappropriate ICD interventions, and 18 (17%) had device-related complications. CONCLUSIONS:
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Authors | Domenico Corrado, Hugh Calkins, Mark S Link, Loira Leoni, Stefano Favale, Michela Bevilacqua, Cristina Basso, Deirdre Ward, Giuseppe Boriani, Renato Ricci, Jonathan P Piccini, Darshan Dalal, Massimo Santini, Gianfranco Buja, Sabino Iliceto, N A Mark Estes 3rd, Thomas Wichter, William J McKenna, Gaetano Thiene, Frank I Marcus |
Journal | Circulation
(Circulation)
Vol. 122
Issue 12
Pg. 1144-52
(Sep 21 2010)
ISSN: 1524-4539 [Electronic] United States |
PMID | 20823389
(Publication Type: Journal Article, Multicenter Study, Research Support, U.S. Gov't, Non-P.H.S.)
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Topics |
- Adolescent
- Adult
- Arrhythmogenic Right Ventricular Dysplasia
(mortality, physiopathology, therapy)
- Defibrillators, Implantable
- Electrophysiologic Techniques, Cardiac
- Female
- Follow-Up Studies
- Humans
- International Cooperation
- Kaplan-Meier Estimate
- Male
- Middle Aged
- Retrospective Studies
- Survival Rate
- Tachycardia, Ventricular
(physiopathology)
- Treatment Outcome
- Ventricular Fibrillation
(physiopathology)
- Young Adult
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