Author + information
- Received February 22, 2018
- Revision received May 30, 2018
- Accepted June 7, 2018
- Published online September 17, 2018.
- Alan Sugrue, MBBCha,
- Ram K. Rohatgi, MDb,
- Martijn Bos, MD, PhDb,c,
- Vaibhav R. Vaidya, MBBSa,
- Samuel J. Asirvatham, MDa,b,
- Peter A. Noseworthy, MDa and
- Michael J. Ackerman, MD, PhDa,b,c,∗ ()
- aDepartment of Cardiovascular Medicine, Division of Heart Rhythm Services, Mayo Clinic, Rochester, Minnesota
- bDepartment of Pediatric and Adolescent Medicine, Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota
- cDepartment of Molecular Pharmacology & Experimental Therapeutics, Windland Smith Rice Sudden Death Genomics Laboratory, Mayo Clinic, Rochester, Minnesota
- ↵∗Address for correspondence:
Dr. Michael J. Ackerman, Genetic Heart Rhythm Clinic and the Mayo Clinic Windland Smith Rice Sudden Death Genomics Laboratory, Guggenheim 501, Mayo Clinic, 200 First Street Southwest, Rochester, Minnesota 55905.
Objectives This study sought to determine the prevalence of early repolarization pattern (ERP) within a large cohort of patients with long QT syndrome (LQTS) and examine the correlation and clinical significance of ERP with symptomatic status and subsequent risk of breakthrough cardiac events (BCEs).
Background The electrocardiographic ERP is associated with an increased risk of arrhythmic events and sudden cardiac death.
Methods ERP was defined as an end-QRS notch or slur on the downslope of a prominent R-wave with a J point ≥0.1 mV in 2 or more contiguous leads of the 12-lead electrocardiogram, excluding V1 to V3. A patient was considered previously symptomatic if they had a suspected LQTS-triggered cardiac event prior to diagnosis. BCEs were defined as LQTS-attributable syncope/seizures, aborted cardiac arrest, appropriate ventricular fibrillation–terminating implantable cardioverter-defibrillator shocks, and sudden cardiac death following diagnosis and institution of a LQTS-directed treatment program.
Results In this study, 528 patients (57% female) with genotype-confirmed LQTS (283 with LQT1, 193 with LQT2, and 52 with LQT3) were reviewed from which 2,618 electrocardiograms were analyzed over a median follow-up of 6.7 (interquartile range, 3.6 to 10 years) years. Eighty-two (15.5%; female 51%) patients were identified as having ERP; 40 (50%) of these ERP-positive patients showed persistent ERP. One hundred twenty-four patients (23.5%) were classified as previously symptomatic LQTS and 39 (7.2%) experienced a subsequent BCE. ERP was not associated with either symptomatic status (p = 0.62) or BCE (p = 0.61).
Conclusions Although ERP is common in LQTS, this extensive study suggests that the presence of concomitant ERP does not correlate with either those with a history of LQTS-triggered events prior to diagnosis or those with subsequent BCEs from their treated LQTS substrate.
Dr. Ackerman has worked as a consultant and has received equity/royalties from AliveCor, Audentes Therapeutics, Blue Ox Health, Boston Scientific, Gilead Sciences, Invitae, Medtronic, MyoKardia, St. Jude Medicial, and StemoniX. 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 February 22, 2018.
- Revision received May 30, 2018.
- Accepted June 7, 2018.
- 2018 American College of Cardiology Foundation
This article requires a subscription or purchase to view the full text. If you are a subscriber or member, click Login or the Subscribe link (top menu above) to access this article.