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Published on: February 28, 2012
Outcomes in Patients With Mitral Annular Disjunction and an Implantable-Cardioverter Defibrillator
Fatima M Ezzeddine1, Jee Won Jennifer Shin2, Konstantinos C Siontis1
1Department of Cardiovascular Medicine, Mayo Clinic, Rochester, Minnesota, USA.
Insights
Mitral annular disjunction (MAD) patients with implantable cardioverter-defibrillators (ICDs) frequently receive appropriate therapies, particularly for polymorphic ventricular tachycardia or fibrillation. History of sudden cardiac arrest and low ejection fraction predict these therapies.
Area of Science:
- Cardiology
- Electrophysiology
- Medical Devices
Background:
- Mitral annular disjunction (MAD) is associated with increased risk of life-threatening ventricular arrhythmias and sudden cardiac death.
- Implantable cardioverter-defibrillators (ICDs) are often implanted in these patients, but their outcomes are not well-established.
Purpose of the Study:
- To evaluate ICD implantation outcomes in patients with MAD.
- To identify predictors of appropriate ICD therapies in this population.
Main Methods:
- Retrospective analysis of 49 patients with MAD who underwent ICD implantation.
- Collection of clinical, electrocardiographic, cardiac imaging, and device therapy data.
- Assessment of appropriate and inappropriate ICD therapies during follow-up.
Main Results:
- Nearly half (47%) of patients received ICD therapies, with 37% experiencing appropriate therapies.
- Appropriate therapies were more common in secondary prevention (44%) versus primary prevention (15%) indications.
- History of sudden cardiac arrest and low left ventricular ejection fraction were predictors of appropriate ICD therapies.
Conclusions:
- Appropriate ICD therapies are common in patients with MAD.
- Polymorphic ventricular tachycardia/ventricular fibrillation were the most frequent indications for appropriate ICD therapies.
- Further research is needed to understand arrhythmia mechanisms and improve risk stratification in MAD.
Background:
Mitral annular disjunction (MAD) carries an increased risk of complex ventricular arrhythmias, which can lead to sudden cardiac death. Many of these patients undergo implantable cardioverter defibrillator (ICD) implantation, but their ICD outcomes are not known.
Objective:
The aim of this study was to assess the outcomes of ICD implantation and the predictors of appropriate ICD therapies in patients with MAD.
Methods:
The study included patients with MAD who underwent ICD implantation. Clinical, electrocardiographic, cardiac imaging, and device therapy data were collected.
Results:
Forty-nine patients with MAD and ICD were included. Median age was 49 (21) years, and 29 (59%) were female. 13 (27%) patients underwent ICD implantation for primary prevention and 36 (73%) patients for secondary prevention. Over a median follow-up of 27.3 (35.3) months, 23 (47%) patients received ICD therapies. 18 (37%) patients had appropriate ICD therapies, and 5 (10%) patients had inappropriate ICD shocks. Median time to first appropriate therapy was 22.3 (63.3) months. In patients with a secondary prevention ICD indication, the rate of appropriate ICD therapies was 44%, while in patients with a primary prevention ICD indication, it was 15%. Among patients with appropriate ICD therapies, the first therapies were delivered for monomorphic ventricular tachycardia (VT) in 7 (39%) patients and polymorphic VT or ventricular fibrillation (VF) in 11 (61%) patients. Patients with appropriate ICD therapies were more likely to have a history of SCA (p = 0.003) and/or low left ventricular ejection fraction (LVEF) (p = 0.022) before ICD implantation as compared to patients without appropriate ICD therapies.
Conclusions:
In our cohort of patients with MAD and ICD, appropriate ICD therapies were common. Most appropriate ICD therapies were delivered for polymorphic VT or VF. Larger studies are needed to elucidate the mechanisms of VAs and refine risk stratification in MAD.
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