Role of electrophysiological studies and arrhythmia intervention in repairing Ebstein's anomaly
Abstract
BACKGROUND: Repairing Ebstein's anomaly without correction of associated arrhythmia may result in sudden death. Catheter or surgical ablation is indicated for various symptomatic tachyarrhythmias in Ebstein's anomaly. METHODS: Between October 1973 and October 1997, 30 patients with Ebstein's anomaly underwent surgical repair in our hospital. Tricuspid valve replacement was performed in 13 patients, tricuspid annuloplasty and valvuloplasty in the remaining 17 patients. Preoperative electrophysiological studies were performed in 11 patients after 1980. Concomitant arrhythmia ablation was done in 10 patients: 4 for Wolff-Parkinson-White syndrome, 2 for atrioventricular (AV) nodal reentrant tachycardia, one for ventricular tachycardia and 3 for paroxysmal atrial flutter and fibrillation. RESULTS: No mortality or major morbidity occurred in those patients undergoing arrhythmia intervention. There were 7 deaths in total; 6 died suddenly, and the other died of purulent mediastinitis. None of the 6 sudden deaths underwent preoperative electrophysiological evaluation. The functional recovery was good in all survivals. CONCLUSIONS: We conclude that detailed preoperative electrophysiological evaluation in patients with Ebstein's anomaly is mandatory. Aggressive surgical intervention of the associated arrhythmias in addition to anatomic correction can reduce the sudden death in Ebstein's anomaly.
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Patients who underwent repair of Ebstein anomaly and preoperative EP study with intraoperative ablation of arrhythmia substrate, in a small series, had a lower risk of SCD than patients without arrhythmia intervention.
“Patients who underwent repair of Ebstein anomaly and preoperative EP study with intraoperative ablation of arrhythmia substrate, in a small series, had a lower risk of SCD than patients without arrhythmia intervention.375”
From European Heart Rhythm Association (EHRA) consensus document on the management of supraventricular arrhythmias, endorsed by Heart Rhythm Society (HRS), Asia-Pacific Heart Rhythm Society (APHRS), and Sociedad Latinoamericana de Estimulacion Cardiaca y Electrofisiologia (SOLAECE) (2017) · cites this paper as [375]
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“Given the known increased risk for atrial flutter/IART in the EA population and the difficulties with catheter ablation after CR, our center routinely performs empiric CTI ablation intraoperatively to hopefully limit the possibility of future CTI‐ dependent IART [3–5 , 10 ].”
- Authors
- Huang, C.-J., Chiu, I.-S., Lin, F.-Y., Chen, W.-J., Lin, J.-L., Lo, H.-M., Wu, M.-H., & Chu, S.-H.
- Affiliations
- National Taiwan University Hospital (TW)
- Venue
- The Thoracic and Cardiovascular Surgeon
- Publisher
- Georg Thieme Verlag KG
- Published
- 2000-12
- Volume
- 48
- Issue
- 6
- Pages
- 347-350
- ISSN
- 0171-6425 (ISSN-L)
- 1439-1902
- OpenAlex
- W1983233704
- Cited by
- 45
- References
- 0
- Citations by year
- 2026: 1 · 2025: 2 · 2024: 1 · 2020: 2 · 2018: 2 · 2017: 3 · 2016: 4 · 2015: 7 · 2014: 5 · 2013: 1 · 2012: 1
- FWCI
- 1.60
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- The Thoracic and Cardiovascular Surgeon (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Epidemiology › Congenital Heart Disease Studies
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac Arrhythmias and Treatments
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac electrophysiology and arrhythmias
- Keywords
- Ebstein's anomaly, arrhythmia ablation, sudden death, tricuspid valve replacement, tricuspid annuloplasty, valvuloplasty, Wolff-Parkinson-White syndrome, atrioventricular nodal reentrant tachycardia, paroxysmal atrial flutter, atrial fibrillation, ventricular tachycardia, congenital heart surgery
- MeSH terms
- Adolescent, Adult, Arrhythmias, Cardiac, Child, Ebstein Anomaly, Electrophysiology, Female, Humans, Male, Postoperative Complications, Recurrence, Survival Analysis, Death, Sudden, Cardiac, Treatment Outcome
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