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Presentation and Outcome of Arrhythmic Mitral Valve Prolapse
Quick Takes
- Mitral valve prolapse (MVP) was once thought of as a benign entity, but reports in the last several years have renewed attention to the risk of sudden cardiac death from MVP.
- This large cohort of patients identifies phenotypic variables associated with excess mortality and reduced event-free survival.
Study Questions:
What is the prevalence of ventricular arrhythmias in patients with mitral valve prolapse (MVP), and what clinical predictors are associated with long-term outcomes?
Methods:
The study cohort was comprised of 595 patients who were first diagnosed with isolated MVP between 2003-2011; 47% were female, average age was 65 ± 16 years. All underwent comprehensive clinical, echocardiographic, and arrhythmia (Holter, electrocardiogram [ECG]) evaluation. The primary outcome was overall survival; the secondary outcome was event-free survival (mortality, implantable cardioverter-defibrillator [ICD] placement, catheter ablation). Arrhythmia burden was categorized as no/trivial (premature ventricular contraction [PVC] or PVC burden <5%), mild (PVC burden ≥5% and/or ventricular tachycardia [VT] <120 bpm), moderate (VT at 120-179 bpm), or severe (VT ≥180 bpm and/or previous ICD).
Results:
Mild ventricular arrhythmia or worse was seen in 43%; 27% in the moderate and 9% in the severe groups. Male sex, bileaflet prolapse, marked leaflet redundancy, mitral annulus disjunction (MAD), larger left atrium and left ventricular end-systolic diameter, and T-wave inversion/ST-segment depression on ECG were significantly associated with any ventricular arrhythmia. Severe ventricular arrhythmia was independently associated with MAD, leaflet redundancy, and T-wave inversion/ST-segment depression on ECG. Overall mortality after arrhythmia diagnosis was strongly associated with arrhythmia severity: severe arrhythmia was associated with significantly higher excess mortality, defibrillator implantation, and VT ablation.
Conclusions:
MVP was associated with a high prevalence of ventricular arrhythmias, but severe burdens were infrequent; MAD, leaflet redundancy, and ECG repolarization changes were independently associated with severe arrhythmia; overall survival and event-free survival were also significantly associated with severe arrhythmia.
Perspective:
In this large retrospective cohort of MVP patients, MAD, marked leaflet redundancy, and T-wave inversion/ST-segment depression were important markers for adverse outcomes. Female sex, which has been suggested as a risk factor for arrhythmic death in MVP, was not independently associated with arrhythmia. The authors also did not see an association between sudden cardiac death in the young and MVP, although the average age of the subjects was over 60 years. Limitations include short ECG monitoring time, lack of cardiac magnetic resonance imaging (cMRI), and limited data on context and causes of sudden cardiac death (e.g., during exercise). Patients with severe arrhythmia and MVP need to be followed closely on an individual level and studied further with prospective trials on a collective level. Future studies will ideally include a wide range of ages and cMRI for scar and anatomic quantification.
Clinical Topics: Arrhythmias and Clinical EP, Noninvasive Imaging, Prevention, Implantable Devices, EP Basic Science, SCD/Ventricular Arrhythmias, Atrial Fibrillation/Supraventricular Arrhythmias, Echocardiography/Ultrasound, Magnetic Resonance Imaging
Keywords: Arrhythmias, Cardiac, Catheter Ablation, Death, Sudden, Cardiac, Defibrillators, Implantable, Diagnostic Imaging, Echocardiography, Electrocardiography, Ambulatory, Magnetic Resonance Imaging, Mitral Valve Prolapse, Risk Factors, Secondary Prevention, Tachycardia, Ventricular, Ventricular Premature Complexes
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