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Sudden death in patients with Ebstein anomaly.
Christine H Attenhofer Jost1, Nicholas Y Tan2, Abdalla Hassan3
1Department of Cardiovascular Disease, Mayo Clinic, 200 1st Street Southwest, Rochester, MN 55905, USA.
European Heart Journal
|January 10, 2018
Summary
Ebstein anomaly (EA) patients face significant sudden death (SD) risks. Ventricular tachycardia, heart failure, and tricuspid valve surgery are key predictors, aiding risk stratification for prevention.
Area of Science:
- Cardiology
- Congenital Heart Disease
- Electrophysiology
Background:
- Ventricular dysfunction is a known risk factor for sudden death (SD).
- Ebstein anomaly (EA) involves right and left heart abnormalities, but SD predictors are undescribed.
- Characterizing SD risk in EA is crucial for patient management.
Purpose of the Study:
- To determine the incidence of sudden death (SD) in Ebstein anomaly (EA) patients.
- To identify clinical and surgical predictors of SD in a large EA cohort.
- To inform risk stratification and preventive strategies for SD in EA.
Main Methods:
- Retrospective review of 968 EA patients over 4 decades at a high-volume institution.
- Kaplan-Meier analysis for SD incidence from birth and post-tricuspid valve (TV) surgery.
- Cox regression to identify multivariable predictors of SD.
Main Results:
- 10-, 50-, and 70-year cumulative incidences of SD from birth were 0.8%, 8.3%, and 14.6%.
- Multivariable predictors of SD included ventricular tachycardia, heart failure, TV surgery, syncope, pulmonic stenosis, and high hemoglobin.
- Post-TV surgery SD was associated with all identified predictors except syncope.
Conclusions:
- Ebstein anomaly patients are at significant risk for sudden death.
- Identifying key clinical predictors like ventricular tachycardia and heart failure aids in risk stratification.
- These findings may guide primary prevention strategies, including implantable cardioverter-defibrillator (ICD) implantation.
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