Ebstein’s Anomaly and Ventricular Arrhythmias

Oct 19, 2018
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Authors:
Moore JP, Shannon KM, Gallotti RG, et al.
Citation:
Catheter Ablation of Ventricular Arrhythmia for Ebstein’s Anomaly in Unoperated and Post-Surgical Patients. JACC Clin Electrophysiol 2018;4:1300-1307.
Summary By:
Martin LaPage, MD, FACC

Study Questions:

What are the ventricular arrhythmia (VA) substrates in Ebstein’s anomaly?

Methods:

This is a retrospective multicenter cohort study of Ebstein’s anomaly patients undergoing electrophysiology study and ablation for VAs.

Results:

The study included 24 patients from 11 centers ranging in age from 1 to 68 years. Focal tachycardia substrates were more common than macro–re-entrant substrates. Unoperated Ebstein’s substrates clustered primarily in the atrialized portion of the right ventricle (RV). Repaired Ebstein’s substrates were more diversely located throughout the ventricle; though focal ventricular tachycardia or premature ventricular contractions from the RV basal septum remained common in this group as well.

Conclusions:

The VA substrate in unrepaired Ebstein’s is most commonly the atrialized RV. Catheter ablation is a reasonable therapy for VAs in Ebstein’s anomaly.

Perspective:

In a great example of the difficulty in studying rare diseases, Moore and colleagues describe 24 patients seen at 11 centers over a 22-year period. Given the evolution of catheter ablation over that time, conclusions about techniques and outcomes cannot be made. Despite the challenge, the authors uncovered useful data on likely substrate locations, which could serve to aid in mapping these arrhythmias more efficiently.

Clinical Topics: Arrhythmias and Clinical EP, Congenital Heart Disease and Pediatric Cardiology, Implantable Devices, EP Basic Science, SCD/Ventricular Arrhythmias, Atrial Fibrillation/Supraventricular Arrhythmias, Congenital Heart Disease, CHD and Pediatrics and Arrhythmias

Keywords: Arrhythmias, Cardiac, Catheter Ablation, Ebstein Anomaly, Electrophysiology, Heart Defects, Congenital, Tachycardia, Ventricular, Ventricular Premature Complexes


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