Author + information
- Received April 30, 2018
- Accepted May 3, 2018
- Published online October 15, 2018.
- Jeremy P. Moore, MD, MSa,∗ (, )
- Kevin M. Shannon, MDa,
- Roberto G. Gallotti, MDa,
- Christopher J. McLeod, MBBSb,
- Anca Chiriac, MDb,
- Edward P. Walsh, MDc,
- Narayanswami Sreeram, MD, PhDd,
- Akash R. Patel, MDe,
- Natasja M. De Groot, MD, PhDf,
- Johannes von Alvensleben, MDg,
- Seshadri Balaji, MBBS, PhDh,
- David S. Frankel, MDi,
- Christina Y. Miyake, MD, MSj,
- James C. Perry, MDk and
- Kalyanam Shivkumar, MD, PhDl
- aDivision of Cardiology, Ahmanson/UCLA Adult Congenital Heart Disease Center, UCLA Medical Center, University of California, Los Angeles, Los Angeles, California
- bDepartment of Cardiovascular Diseases, Mayo Clinic Florida, Jacksonville, Florida
- cDepartment of Cardiology, Boston Children's Hospital, Boston, Massachusetts
- dDepartment of Pediatrics, University Hospital of Cologne, Cologne, Germany
- eDepartment of Pediatrics, University of California San Francisco, Benioff Children's Hospital, San Francisco, California
- fDepartment of Cardiology, Erasmus University Center, Rotterdam, the Netherlands
- gDivision of Cardiology, Heart Institute, Children’s Hospital Colorado, University of Colorado, Aurora, Colorado
- hDivision of Cardiology, Department of Pediatrics, Oregon Health and Science University, Portland, Oregon
- iElectrophysiology Section, Cardiovascular Division, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania
- jLillie Frank Abercrombie Section of Pediatric Cardiology, Texas Children's Hospital, Houston, Texas
- kDivision of Cardiology, Department of Pediatrics, Rady Children’s Hospital, University of California-San Diego, San Diego, California
- lArrhythmia Center, Department of Cardiology, University of California, Los Angeles, Los Angeles, California
- ↵∗Address for correspondence:
Dr. Jeremy P. Moore, Division of Cardiology, Ahmanson/UCLA Adult Congenital Heart Disease Center, University of California Los Angeles, UCLA Medical Center, 100 Medical Plaza Drive, Suite 770, Los Angeles, California 90095.
Objectives The purpose of this study was to determine the ventricular arrhythmia (VA) substrates in patients with unoperated and post-surgical Ebstein's Anomaly (EA).
Background EA is associated with variable atrialization of the right ventricle and a propensity for VA and sudden death. There are scant data on catheter ablation for VA in this population.
Methods This was a retrospective study involving 11 congenital heart disease centers.
Results A total of 24 patients (median age 17 [interquartile range (IQR): 11 to 37] years; age range 1 to 68 years; 42% men) with EA undergoing catheter ablation were identified. Prior tricuspid valve (TV) surgery had been performed in 12 (50%). Presenting symptoms were palpitations in 15, syncope in 4, aborted cardiac arrest in 4, and none in 1. At procedure, 28 VA substrates were encountered and 25 were completely characterized (median 1 per patient; cycle length 305 [IQR: 268 to 400] ms). In 3 cases, premature ventricular contraction (PVC) foci were targeted (1 with a history of PVC-induced ventricular fibrillation). VA mechanisms were focal in 15 and macro–re-entrant in 10, and did not differ significantly between those with and those without prior TV surgery (p = 0.7). Focal VAs predominantly localized to the atrialized right ventricle ARV in unoperated patients and to diseased myocardium or Purkinje tissue after TV surgery. Macro–re-entry was related to isolated scar or split potentials in the ARV in unoperated patients, and larger, more diffuse scar after TV surgery. Complete success was achieved in 22 (92%). There were 2 of 13 complications in patients <18 years of age and none in patients >18 years of age. There was a single recurrence over a median follow-up of 3.4 years.
Conclusions VA in EA may be either focal or macro–re-entrant. In the absence of surgery, substrates chiefly involve the ARV. After surgery, focal VA involves injured myocardium or Purkinje tissue and re-entrant ventricular tachycardia is related to post-surgical scar. Catheter ablation is a reasonable therapeutic approach for these patients.
- catheter ablation
- congenital heart disease
- Ebstein’s anomaly
- sudden cardiac death
- ventricular tachycardia
Dr. Shivkumar is supported by National Institutes of Health Grant Nos. HL084261 and OT2OD023848. 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 April 30, 2018.
- Accepted May 3, 2018.
- 2018 American College of Cardiology Foundation
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