Catheter Ablation of Ventricular Arrhythmia for Ebstein's Anomaly in Unoperated and Post-Surgical Patients

Jeremy P Moore; Kevin M Shannon; Roberto G Gallotti; Christopher J McLeod; Anca Chiriac; Edward P Walsh; Narayanswami Sreeram; Akash R Patel; Natasja M De Groot; Johannes von Alvensleben; Seshadri Balaji; David S Frankel; Christina Y Miyake; James C Perry; Kalyanam Shivkumar

doi:10.1016/j.jacep.2018.05.009

Catheter Ablation of Ventricular Arrhythmia for Ebstein's Anomaly in Unoperated and Post-Surgical Patients

JACC Clin Electrophysiol. 2018 Oct;4(10):1300-1307. doi: 10.1016/j.jacep.2018.05.009. Epub 2018 Jun 27.

Authors

Affiliations

¹ Division of Cardiology, Ahmanson/UCLA Adult Congenital Heart Disease Center, UCLA Medical Center, University of California, Los Angeles, Los Angeles, California. Electronic address: jpmoore@mednet.ucla.edu.
² Division of Cardiology, Ahmanson/UCLA Adult Congenital Heart Disease Center, UCLA Medical Center, University of California, Los Angeles, Los Angeles, California.
³ Department of Cardiovascular Diseases, Mayo Clinic Florida, Jacksonville, Florida.
⁴ Department of Cardiology, Boston Children's Hospital, Boston, Massachusetts.
⁵ Department of Pediatrics, University Hospital of Cologne, Cologne, Germany.
⁶ Department of Pediatrics, University of California San Francisco, Benioff Children's Hospital, San Francisco, California.
⁷ Department of Cardiology, Erasmus University Center, Rotterdam, the Netherlands.
⁸ Division of Cardiology, Heart Institute, Children's Hospital Colorado, University of Colorado, Aurora, Colorado.
⁹ Division of Cardiology, Department of Pediatrics, Oregon Health and Science University, Portland, Oregon.
¹⁰ Electrophysiology Section, Cardiovascular Division, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania.
¹¹ Lillie Frank Abercrombie Section of Pediatric Cardiology, Texas Children's Hospital, Houston, Texas.
¹² Division of Cardiology, Department of Pediatrics, Rady Children's Hospital, University of California-San Diego, San Diego, California.
¹³ Arrhythmia Center, Department of Cardiology, University of California, Los Angeles, Los Angeles, California.

PMID: 30336876
DOI: 10.1016/j.jacep.2018.05.009

Abstract

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.

Keywords: Ebstein’s anomaly; catheter ablation; congenital heart disease; sudden cardiac death; ventricular tachycardia.

Publication types

Research Support, N.I.H., Extramural

MeSH terms

Adolescent
Adult
Catheter Ablation* / adverse effects
Catheter Ablation* / methods
Child
Child, Preschool
Ebstein Anomaly* / complications
Ebstein Anomaly* / surgery
Female
Humans
Male
Middle Aged
Postoperative Complications
Retrospective Studies
Tachycardia, Ventricular* / etiology
Tachycardia, Ventricular* / surgery
Young Adult