Author + information
- Received October 3, 2016
- Accepted October 20, 2016
- Published online July 17, 2017.
- Federico Migliore, MD, PhD∗ (, )
- Emanuele Bertaglia, MD, PhD,
- Alessandro Zorzi, MD and
- Domenico Corrado, MD, PhD
- ↵∗Address for correspondence:
Dr. Federico Migliore, Department of Cardiac Thoracic and Vascular Sciences, University of Padova Medical School, Via N. Giustiniani 2, 35121 Padova, Italy.
- arrhythmogenic right ventricular cardiomyopathy
- implantable cardioverter-defibrillator
- subcutaneous implantable cardioverter defibrillator
We report the case of a 37-year-old man with a definite diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC) according with the current Task Force Criteria (1) referred to our institution for implantable cardioverter-defibrillator (ICD) for secondary prevention of sudden cardiac death. The basal 12-lead electrocardiogram (ECG) showed normal PR, QRS, and QT interval and negative T waves (NTWs) in the right precordial leads (Figure 1A). To screen the patient as potential candidate to subcutaneous ICD (S-ICD) a pre-implantation QRS-T morphology screening using an ECG simulating the 3 sensing vectors of the S-ICD was performed in both standing and supine position. Two of the 3 sense vectors were appropriate (II and III). The I sense vector was not acceptable because of the high negative amplitude of the T-wave (Figure 1B). The morphology screening was repeated during exercise. During the test NTWs completely normalized (Figure 1C) and the previously appropriate sense vectors were no longer acceptable because of high amplitude of the T waves. Because of the presence of exercise-induced NTWs changes leading to the lack of consistency of an appropriate sense vector both at resting and during exercise, a traditional transvenous single-chamber ICD was implanted.
ARVC is an inherited heart muscle disease potentially leading to sudden cardiac death and typically characterized by ECG repolarization and/or depolarization abnormalities in the right precordial leads (1). To date, there are limited data on S-ICD use in ARVC patients.
Recently, we demonstrated that right precordial NTWs partially or completely revert with exercise in most patients with ARVC, and NTW normalization is unrelated to the clinical phenotype (2). Because the appropriate morphology analysis of surface ECG is the main limitation of S-ICD eligibility, repeat screening during exercise test should be mandatory in patients with inherited cardiomyopathy with repolarization or depolarization abnormalities to evaluate the eligibility for S-ICD indication and avoid inappropriate shocks.
Dr. Migliore is consultant for Boston Scientific. Dr. Bertaglia is consultant for Boston Scientific and St. Jude Medical. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.
All authors attest they are in compliance with human studies committees and animal welfare regulations of the authors’ institutions and Food and Drug Administration guidelines, including patient consent where appropriate. For more information, visit the JACC: Clinical Electrophysiology author instructions page.
- Received October 3, 2016.
- Accepted October 20, 2016.
- 2017 American College of Cardiology Foundation