Author + information
- Received April 6, 2011
- Revision received June 9, 2011
- Accepted June 27, 2011
- Published online September 27, 2011.
- Aditya Bhonsale, MD,
- Cynthia A. James, PhD,
- Crystal Tichnell, MS,
- Brittney Murray, MS,
- Dmitri Gagarin, MD,
- Binu Philips, MD,
- Darshan Dalal, MD,
- Ryan Tedford, MD,
- Stuart D. Russell, MD,
- Theodore Abraham, MD,
- Harikrishna Tandri, MD,
- Daniel P. Judge, MD and
- Hugh Calkins, MD⁎ ()
- ↵⁎Reprint requests and correspondence:
Dr. Hugh Calkins, Carnegie 530, The Johns Hopkins Hospital, 600 North Wolfe Street, Baltimore, Maryland 21287
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.
Funding for this study has been received from the National Heart, Lung, and Blood Institute (K23HL093350 to Dr. Tandri), the St. Jude Medical Foundation, Medtronic Inc., and Boston Scientific Corp. The Johns Hopkins ARVD/C Program is supported by the Bogle Foundation, the Healing Hearts Foundation, the Campanella family, and Wilmerding Endowments, and the Dr. Francis P. Chiaramonte Private Foundation. Dr. Dalal is an employee of Genentech Inc. Dr. Tedford has received a Fellow's Travel Grant from Medtronic. Dr. Calkins receives research support from Boston Scientific Corp., Medtronic Inc., and St. Jude Medical Foundation and is a consultant for Medtronic Inc. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.
- Received April 6, 2011.
- Revision received June 9, 2011.
- Accepted June 27, 2011.
- American College of Cardiology Foundation