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Prophylactic Implantable Defibrillator in Patients With Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia and No Prior Ventricular Fibrillation or Sustained Ventricular Tachycardia
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BACKGROUND: The role of implantable cardioverter-defibrillator (ICD) in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia and no prior ventricular fibrillation (VF) or sustained ventricular tachycardia is an unsolved issue. 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: One fourth of patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia and no prior sustained ventricular tachycardia or VF had appropriate ICD interventions. Syncope was an important predictor of life-saving ICD intervention and is an indication for ICD. Prophylactic ICD may not be indicated in asymptomatic patients because of their low arrhythmic risk regardless of familial sudden death and programmed ventricular stimulation findings. Programmed ventricular stimulation had a low predictive accuracy for ICD therapy.
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“In general, a history of syncope in patients with structural heart disease or inheritable arrhythmia syndromes is associated with a two- to four-fold increased risk of death, 348,350-353 but varies between specific conditions. 354-356”
From 2018 ESC Guidelines for the diagnosis and management of syncope (2018) · cites this paper as [351]
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“Although limited and diverse, current data suggest that unexplained syncope is a marker of arrhythmic risk in patients with ARVC. 46,351,362,363”
From 2018 ESC Guidelines for the diagnosis and management of syncope (2018) · cites this paper as [351]
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Bouar, R. L., Halbwachs, F., Wiedemann, J.-Y., Nazer, T. E., Bourrelly, N., Jacquemin, L., & Roth, O. (2023) · Springer eBooks · doi:10.1007/978-3-031-35579-0_10
Implantable cardioverter defibrillators for primary prevention in cardiomyopathies
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A new prediction model for sustained ventricular tachycardia in arrhythmogenic cardiomyopathy
Zhang, B., Xie, X., Yu, J., Wu, Y., Zhou, J. P., Li, X., & Yang, B. (2024) · Frontiers in Cardiovascular Medicine · doi:10.3389/fcvm.2024.1477931
Pilichou, K., Basso, C., Corrado, D., & Thiene, G. (2025) · Elsevier eBooks · doi:10.1016/b978-0-702-08231-3.00062-4
Self-citationWhen Substrate Meets Device: ICD Implantation and Therapy across the Spectrum of Cardiomyopathies
Sisti, N., Cardona, A., Lazzari, L., Fortuni, F., Santoro, A., Andreoli, C., Notaristefano, F., & Carreras, G. (2026) · Pacing and Clinical Electrophysiology · doi:10.1111/pace.70149
Metadata
- Authors
- Corrado, D., Calkins, H., Link, M. S., Leoni, L., Favale, S., Bevilacqua, M., Basso, C., Ward, D., Boriani, G., Ricci, R., Piccini, J. P., Dalal, D., Santini, M., Buja, G., Iliceto, S., Estes, N. A. M., Wichter, T., McKenna, W. J., Thiene, G., & Marcus, F. I.
- Affiliations
- Tufts Medical Center (US)
- From the Department of Cardiac, Thoracic, and Vascular Sciences, University of Padua Medical School, Padua, Italy (D.C., L.L., M.B., G.B., S.I.); Department of Cardiology, Johns Hopkins Hospital, Baltimore, Md (H.C., J.P.P., D.D.); Tufts Medical Center, Boston, Mass (M.S.L., N.A.M.E.); Department of Cardiology, University of Bari, Bari, Italy (S.F.); Department of Medical-Diagnostic Sciences and Special Therapies, University of Padua, Padua, Italy (C.B., G.T.); The Heart Hospital, University College...
- ORCID
- Venue
- Circulation
- Published
- 2010-09-21
- Volume
- 122
- Issue
- 12
- Pages
- 1144-1152
- ISSN
- 0009-7322 (ISSN-L)
- 1524-4539
- OpenAlex
- W2000286264
- Cited by
- 306
- References
- 25
- Citations by year
- 2026: 6 · 2025: 5 · 2024: 5 · 2023: 21 · 2022: 12 · 2021: 22 · 2020: 11 · 2019: 22 · 2018: 21 · 2017: 31 · 2016: 21 · 2015: 27 · 2014: 26 · 2013: 19 · 2012: 35
- FWCI
- 16.69
- Type
- journal article
- Language
- en
- Open access
- green
- hdl.handle.net
- Licence
- cc-by
- Locations
- Circulation (publishedVersion)
- PubMed (publishedVersion)
- Archivio istituzionale della ricerca (Alma Mater Studiorum Università di Bologna) (submittedVersion)
- UCL Discovery (University College London) (submittedVersion)
- IRIS UNIMORE (University of Modena and Reggio Emilia) (submittedVersion, cc-by)
- CINECA IRIS Institutional Research Information System (University of Bari Aldo Moro) (submittedVersion)
- Lenus, The Irish Health Repository (Dr Steevens Hospital Library) (submittedVersion)
- Padua Research Archive (University of Padova) (submittedVersion)
- Topics
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiovascular Effects of Exercise
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac pacing and defibrillation studies
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac electrophysiology and arrhythmias
- Keywords
- arrhythmogenic right ventricular cardiomyopathy, implantable cardioverter-defibrillator, prophylactic ICD, syncope, programmed ventricular stimulation, ventricular fibrillation, ventricular flutter, sudden cardiac death, arrhythmia risk prediction, inappropriate shocks, device complications, familial sudden death, young adults
- MeSH terms
- Adolescent, Adult, Female, Follow-Up Studies, Humans, International Cooperation, Male, Middle Aged, Retrospective Studies, Ventricular Fibrillation, Survival Rate, Treatment Outcome, Defibrillators, Implantable, Tachycardia, Ventricular, Arrhythmogenic Right Ventricular Dysplasia, Electrophysiologic Techniques, Cardiac, Kaplan-Meier Estimate, Young Adult
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