Prevalence of Mitral Annulus Disjunction and Mitral Valve Prolapse in Patients With Idiopathic Ventricular Fibrillation

Groeneveld, Sanne A.; Kirkels, Feddo P.; Evertz, Reinder; Haugaa, Kristina H.; Postema, Pieter G.; Prakken, Niek H J; Teske, Arco J.; Wilde, Arthur A.M.; Velthuis, Birgitta K.; Nijveldt, Robin; Hassink, Rutger J.

doi:10.1161/jaha.121.025364

Cited by 17 publications

(18 citation statements)

References 40 publications

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“…bileaflet MVP and MAD, which are more prevalent in women. 45 Indeed, in addition to greater global ECV%, women with arrhythmic MVP in our study exhibited greater “regionalized” interstitial fibrosis, specifically higher ECV% in the mid inferolateral LV, which was not observed in men. Altogether, these findings support the role of sex-specific factors in the development of myocardial fibrosis and subsequent risk of ventricular arrhythmia in MVP.…”

Section: Discussionmentioning

confidence: 40%

Interstitial Fibrosis and Arrhythmic Mitral Valve Prolapse: Unravelling Sex-Based Differences

Tastet,

Dixit,

Nguyen

et al. 2024

Preprint

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Background: Interstitial fibrosis as quantified by cardiac magnetic resonance (CMR) has been demonstrated in arrhythmic mitral valve prolapse (MVP), a condition with known female predominance. However, prior studies included only MVP cases with significant mitral regurgitation (MR) or mitral annular disjunction (MAD). We sought to evaluate the association between interstitial fibrosis and complex ventricular ectopy (ComVE) in MVPs unselected for MAD or severe MR, and to investigate the contribution of sex to this association. Methods: We performed contrast CMR in consecutive individuals with MVP between 2020 and 2022. Extracellular volume fraction (ECV%), a surrogate marker for interstitial fibrosis, was quantified using T1 mapping. Replacement fibrosis was assessed using late gadolinium enhancement (LGE). ComVE, defined as frequent premature ventricular contractions and/or non-sustained/sustained ventricular tachycardia (VT), was detected using ambulatory ECG monitoring. Results: We identified 59 MVP cases without severe MR (49% women, 80% with mild or less MR) and available ECV% measurement. Among these, 23 (39%) had ComVE, including a case of aborted ventricular fibrillation (VF) and one with sudden arrhythmic death, both females. Global ECV% was significantly greater in ComVE versus non-ComVE (31%[27-33] vs 27%[23-30], p=0.002). In MVP-ComVE, higher segmental ECV% was not limited to the inferolateral/inferior LV wall, but was also demonstrated in atypical segments including the anterior/anterolateral wall (p<0.05). The association between ComVE and ECV% was driven by female sex (32%[30-33] vs 28%[26-30], p=0.003 in females; 31%[25-33] vs 26%[23-30], p=0.22 in males). ECV% remained independently associated with an increased risk of ComVE, including VT/VF, after adjustment for cardiovascular risk factors, MAD, and LGE (p<0.01). Conclusion: In MVP without significant MR, interstitial fibrosis by CMR is associated with an increased risk of ComVE, suggesting a primary myopathic process. The stronger association between interstitial fibrosis and ComVE in females may explain why severe arrhythmic complications are more prevalent among women.

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Section: Discussionmentioning

confidence: 40%

Interstitial Fibrosis and Arrhythmic Mitral Valve Prolapse: Unravelling Sex-Based Differences

Tastet,

Dixit,

Nguyen

et al. 2024

Preprint

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“…However, MAD by itself is a significant risk factor of arrhythmia. Groeneveld et al [47] research also support that hypothesis. In their study, they included 72 subjects with an IVF and 72 healthy controls.…”

Section: Madmentioning

confidence: 74%

Mitral valve prolapse—arrhythmic faces of the valve disease

Możdżan,

Duraj

et al. 2023

Explor Cardiol

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Mitral valve prolapse (MVP) is a relatively common mitral valvulopathy and the most common cause of isolated primary mitral regurgitation (MR) requiring surgical repair. It affects about 1–3% of the general population. Although MVP is viewed as a benign condition, the association between MVP and sudden cardiac death (SCD) has been proven. Patients with MVP have a three times higher risk of SCD than the general population. The underlying mechanisms and predictors of arrhythmias, which occur in patients with MVP, are still poorly understood. However, some echocardiographic features such as mitral annulus disjunction (MAD), bileaflet MVP (biMVP), and papillary muscle (PM) fibrosis were frequently linked with increased number of arrhythmic events and are referred to as “arrhythmogenic” or “malignant”. Arrhythmogenic MVP (AMVP) has also been associated with other factors such as female sex, polymorphic premature ventricular contraction (PVC), abnormalities of T-waves, and Pickelhaube sign on tissue Doppler tracing of the lateral part of the mitral annulus. Cardiac magnetic resonance (CMR) imaging and speckle tracking echocardiography are new tools showing significant potential for detection of malignant features of AMVP. This paper presents various data coming from electrocardiography (ECG) analysis, echocardiography, and other imaging techniques as well as compilation of the recent studies on the subject of MVP.

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“…Hence, Groeneveld et al re-evaluated CMR images to study the prevalence and morphology of mitral annulus disjunction (MAD) and MVP in a multicenter IVF cohort. Mitral valve abnormalities have been previously associated with UCA [ 67 , 68 ]. MAD is a structural abnormality in which the hinge point of the mitral valve is abnormally placed in the atrium [ 69 ].…”

Section: The Use Of Multiple Imaging Modalitiesmentioning

confidence: 99%

“…The study of Groeneveld et al revealed that inferolateral MAD and MVP were significantly more prevalent in IVF patients compared to healthy controls ( Figure 5 ). The authors recommended that MAD and MVP should deserve specific attention when evaluating UCA patients and showed the value of re-evaluating IVF patients based on the expanded medical knowledge during follow-up [ 67 ].…”

Section: The Use Of Multiple Imaging Modalitiesmentioning

confidence: 99%

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State-of-the-Art Multimodality Imaging in Sudden Cardiac Arrest with Focus on Idiopathic Ventricular Fibrillation: A Review

Verheul

Groeneveld

Kirkels

et al. 2022

JCM

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Idiopathic ventricular fibrillation is a rare cause of sudden cardiac arrest and a diagnosis by exclusion. Unraveling the mechanism of ventricular fibrillation is important for targeted management, and potentially for initiating family screening. Sudden cardiac arrest survivors undergo extensive clinical testing, with a growing role for multimodality imaging, before diagnosing “‘idiopathic” ventricular fibrillation. Multimodality imaging, considered as using multiple imaging modalities as diagnostics, is important for revealing structural myocardial abnormalities in patients with cardiac arrest. This review focuses on combining imaging modalities (echocardiography, cardiac magnetic resonance and computed tomography) and the electrocardiographic characterization of sudden cardiac arrest survivors and discusses the surplus value of multimodality imaging in the diagnostic routing of these patients. We focus on novel insights obtained through electrostructural and/or electromechanical imaging in apparently idiopathic ventricular fibrillation patients, with special attention to non-invasive electrocardiographic imaging.

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Prevalence of Mitral Annulus Disjunction and Mitral Valve Prolapse in Patients With Idiopathic Ventricular Fibrillation

Cited by 17 publications

References 40 publications

Interstitial Fibrosis and Arrhythmic Mitral Valve Prolapse: Unravelling Sex-Based Differences

Interstitial Fibrosis and Arrhythmic Mitral Valve Prolapse: Unravelling Sex-Based Differences

Mitral valve prolapse—arrhythmic faces of the valve disease

State-of-the-Art Multimodality Imaging in Sudden Cardiac Arrest with Focus on Idiopathic Ventricular Fibrillation: A Review

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