Incidence and predictors of implantable cardioverter-defibrillator therapy in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy undergoing implantable cardioverter-defibrillator implantation for primary prevention
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Objectives The purpose of this study was to define the incidence and predictors of implantable cardioverter-defibrillator (ICD) therapy in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) after placement of an ICD for primary prevention.Background Patients with a diagnosis of ARVD/C often receive an ICD for prevention of sudden cardiac death.Methods Patients (n = 84) from the Johns Hopkins registry with definite or probable ARVD/C who underwent ICD implantation for primary prevention were studied. Detailed phenotypic, genotype, and ICD event information was obtained and appropriate ICD therapies were adjudicated based on intracardiac electrograms.Results Over a mean follow-up of 4.7 ± 3.4 years, appropriate ICD therapy was seen in 40 patients (48%), of whom 16 (19%) received interventions for potentially fatal ventricular fibrillation/flutter episodes. Proband status (p < 0.001), inducibility at electrophysiologic study (p = 0.005), presence of nonsustained ventricular tachycardia (p < 0 .001), and Holter premature ventricular complex count >1,000/24 h (p = 0.024) were identified as significant predictors of appropriate ICD therapy. The 5-year survival free of appropriate ICD therapy for patients with 1, 2, 3, and 4 risk factors was 100%, 83%, 21%, and 15%, respectively. Inducibility at electrophysiologic study (hazard ratio: 4.5, 95% confidence interval: 1.4 to 15, p = 0.013) and nonsustained ventricular tachycardia (hazard ratio: 10.5, 95% confidence interval: 2.4 to 46.2, p = 0.002) remained as significant predictors on multivariable analysis.Conclusions Nearly one-half of the ARVD/C patients with primary prevention ICD implantation experience appropriate ICD interventions. Inducibility at electrophysiologic study and nonsustained ventricular tachycardia are independent strong predictors of appropriate ICD therapy. An increase in ventricular ectopy burden was associated with progressively lower event-free (appropriate ICD interventions) survival. Incremental risk of ventricular arrhythmias and ICD therapy was observed with the presence of multiple risk factors.
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NSVT or PVC burden >1,000/24 hr may predict appropriate ICD discharge, with the latter associated with over threefold higher risk of ICD discharge.
“NSVT or PVC burden .1,000/24hr may predict appropriate ICD discharge, the latter PVC burden associated with over threefold higher risk of ICD discharge”
From 2017 ISHNE-HRS expert consensus statement on ambulatory ECG and external cardiac monitoring/telemetry (2017) · cites this paper as Bhonsale et al., 2011
Current data suggest that unexplained syncope is a marker of arrhythmic risk in patients with arrhythmogenic right ventricular cardiomyopathy.
“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 [363]
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Self-citation
- Authors
- Bhonsale, A., James, C. A., Tichnell, C., Murray, B., Gagarin, D., Philips, B., Dalal, D., Tedford, R., Russell, S. D., Abraham, T., Tandri, H., Judge, D. P., & Calkins, H.
- Affiliations
- Johns Hopkins University (US)
- Corresponding author
- Calkins, Hugh
- ORCID
- Venue
- Journal of the American College of Cardiology
- Publisher
- Elsevier BV
- Published
- 2011-09
- Volume
- 58
- Issue
- 14
- Pages
- 1485-1496
- ISSN
- 0735-1097 (ISSN-L)
- OpenAlex
- W2084801770
- Cited by
- 250
- References
- 24
- Citations by year
- 2026: 2 · 2025: 7 · 2024: 9 · 2023: 15 · 2022: 15 · 2021: 20 · 2020: 7 · 2019: 16 · 2018: 20 · 2017: 43 · 2016: 17 · 2015: 25 · 2014: 22 · 2013: 20 · 2012: 11
- FWCI
- 11.66
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Licence
- Locations
- Journal of the American College of Cardiology (publishedVersion)
- PubMed (publishedVersion)
- Funders
- Medtronic
- 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, primary prevention, appropriate ICD therapy, sudden cardiac death, ventricular fibrillation, non-sustained ventricular tachycardia, risk factors, premature ventricular contractions, survival, intracardiac electrograms
- MeSH terms
- Adolescent, Adult, Child, Female, Follow-Up Studies, Humans, Male, Middle Aged, Predictive Value of Tests, Primary Prevention, Incidence, Defibrillators, Implantable, Arrhythmogenic Right Ventricular Dysplasia, Young Adult, Cardiac Resynchronization Therapy
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