Presentation and Outcome of Arrhythmic Mitral Valve Prolapse

Essayagh, Benjamin; Sabbag, Avi; Antoine, Clémence; Benfari, Giovanni; Yang, Li-Tan; Maalouf, Joseph F.; Asirvatham, Samuel J.; Michelena, Héctor I.; Enríquez-Sarano, Maurice

doi:10.1016/j.jacc.2020.06.029

Cited by 137 publications

(212 citation statements)

References 37 publications

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“…Mitral annulus disjunction has been proposed to be associated with VA, but particularly when ≥5 mm, and in patients without significant MR [10]. Our patient also presented, soon after hospitalization and until operation, T wave inversion in the inferior ECG leads, which was reported as highly prevalent in patients with MVP and cardiac arrest [3], and is strongly associated with VA [7]. Interestingly, these ECG abnormalities disappeared soon after mitral valve repair, suggesting that LV volume overload reduction or the relief of the mitral valve apparatus stretching after operation can normalize these ECG changes, as also reported by Alqarawi et al [11].…”

Section: Risk Stratification For Ventricular Arrhythmias In Mvp Patientssupporting

confidence: 52%

“…Before discharge, genetic counseling was performed according to standard practice including whole exome sequencing. Firstly, 522 genes associated with cardiovascular development and disease were evaluated, with special attention to the genes know to be associated with mitral valve prolapse (Dachsous Cadherin-Related 1(DCHS1), Filamin A (FLNA), Phospholipase D1 (PLD1), and DAZ interacting protein 1(DZIP1) [7]. Furthermore, family screening was advised, and his sister also underwent echocardiography (which revealed also a Barlow's MVP with mild MR) and genetic testing.…”

Section: Case Reportmentioning

confidence: 99%

“…However, some patients, usually young and without any significant comorbidities, experience malignant ventricular arrhythmias (VA) and sudden cardiac death (SCD), estimated in 0.2-0.4% per year, and up to 1.8% per year in the presence of a flail leaflet [1,2]. Several studies have identified some risk factors associated with malignant arrhythmias and SCD in MVP patients, mainly (i) female sex, (ii) bi-leaflet prolapse, (iii) mitral annulus abnormalities such as dilatation and disjunction (defined as a separation between the left atrial (LA) wall at the level of the valve junction with the LV free wall), (iv) electrocardiographic repolarization abnormalities specifically on the inferolateral leads [3][4][5], (v) frequent and complex premature ventricular contractions (PVCs), and (vi) presence of myocardial fibrosis at the level of the papillary muscles [6,7]. However, it is still largely unknown how to risk-stratify these patients and no specific recommendations have been proposed to help the clinical decision-making.…”

Section: Introductionmentioning

confidence: 99%

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Malignant Arrhythmic Mitral Valve Prolapse: A Continuum of Clinical Challenges from Diagnosis to Risk Stratification and Patient Management

Yedidya

Wijngaarden

Marsan

2020

JCDD

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Mitral valve prolapse (MVP) is a common valvular disease, which may remain a benign condition for a long period of time. However, some patients experience malignant ventricular arrhythmias and sudden cardiac death (SCD). It is still largely unknown how to risk-stratify these patients, and no specific recommendations have been proposed to help the clinical decision-making. We present the case of a young man whose first clinical presentation was an out-of-hospital cardiac arrest and was subsequently diagnosed with MVP. We highlighted the possible risk factors for SCD and the challenges in the clinical management of these patients.

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Section: Risk Stratification For Ventricular Arrhythmias In Mvp Patientssupporting

confidence: 52%

Section: Case Reportmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Malignant Arrhythmic Mitral Valve Prolapse: A Continuum of Clinical Challenges from Diagnosis to Risk Stratification and Patient Management

Yedidya

Wijngaarden

Marsan

2020

JCDD

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“…Considering only the patients with MVP but without MR, MAD remained a predictor of the frequency of premature ventricular complexes (PVCs) and NSVT [50], suggesting that it might be independent from MR. This finding is supported by a recent work by Essayagh and colleagues who found that in a multivariate analysis severe MAD was associated with a sevenfold increased risk of VA [54]. Although associated with MR and NSVTs, MAD has never been tested as an independent risk factor for SCD-large studies using hard-endpoints will give the last word on its relevance for SCD.…”

Section: Mitral Valve Annulus Alterationsmentioning

confidence: 59%

“…Successively, the work of Marks and colleagues defined the patients with MVP and leaflet redundancy as "classic" MVP, while those patients without were termed "non-classic" MVP [52]. Importantly, "classic" MVP has also been demonstrated to be an independent predictor of VA in an Italian cohort [53], a finding later confirmed in larger cohorts [54]; with the incidence of complex VA shown to correlate positively with the degree of MVP [55].…”

Section: Mitral Valve Leaflet Alterationsmentioning

confidence: 99%

Arrhythmic Mitral Valve Prolapse: Introducing an Era of Multimodality Imaging-Based Diagnosis and Risk Stratification

et al. 2021

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Mitral valve prolapse is a common cardiac condition, with an estimated prevalence between 1% and 3%. Most patients have a benign course, but ever since its initial description mitral valve prolapse has been associated to sudden cardiac death. Although the causal relationship between mitral valve prolapse and sudden cardiac death has never been clearly demonstrated, different factors have been implicated in arrhythmogenesis in patients with mitral valve prolapse. In this work, we offer a comprehensive overview of the etiology and the genetic background, epidemiology, pathophysiology, and we focus on the state-of-the-art imaging-based diagnosis of mitral valve prolapse. Going beyond the classical, well-described clinical factors, such as young age, female gender and auscultatory findings, we investigate multimodality imaging features, such as alterations of anatomy and function of the mitral valve and its leaflets, the structural and contractile anomalies of the myocardium, all of which have been associated to sudden cardiac death.

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Association of Mitral Annular Disjunction With Cardiovascular Outcomes Among Patients With Marfan Syndrome

et al. 2021

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IMPORTANCE Mitral annular disjunction (MAD) has received particular interest in patients with mitral valve prolapse, ventricular tachycardia, and sudden cardiac death. The clinical significance of MAD for patients with Marfan syndrome (MFS) remains largely unexplored. OBJECTIVE To define the prevalence of MAD and examine its association with cardiovascular outcomes and arrhythmia among patients with MFS. DESIGN, SETTING, AND PARTICIPANTS This retrospective, single-center cohort study included 142 patients with a diagnosis of MFS based on the revised Ghent criteria and a confirmed (likely) pathogenic variant in the FBN1 gene who underwent regular follow-up between January 1, 2004, and December 31, 2019. MAIN OUTCOMES AND MEASURES The presence of MAD was assessed by echocardiography, and the extent of MAD was categorized in tertiles. Patients also underwent resting electrocardiography and 24-hour Holter monitoring. Outcomes included aortic events (aortic dissection or prophylactic aortic surgery), arrhythmic events (defined as sustained ventricular tachycardia or sudden cardiac death), and mitral valve surgery. RESULTS A total of 142 patients (72 female patients [51%]; median age at first examination, 25 years [range, 2-64 years]) were evaluated. Forty-eight patients (34%) had MAD. Patients with MAD had larger aortic root z scores than patients without MAD (4.1 [interquartile range, 2.8-5.7] vs 3.0 [interquartile range, 1.8-4.0]; P < .001) and more often had mitral valve prolapse (34 of 48 [71%] vs 14 of 94 [15%]; P < .001), ventricular ectopy (14 of 33 [42%] vs 15 of 70 [21%]; P = .03), and nonsustained ventricular tachycardia (13 of 33 [39%] vs 12 of 70 [17%]; P = .01). During follow-up, aortic events occurred at similar rates among patients with vs without MAD (15 of 43 [35%] vs 21 of 84 [25%]; P = .24), but patients in the upper MAD tertile (>10 mm) showed a higher occurrence of aortic events compared with patients with MAD of 10 mm or smaller (9 of 15 [60%] vs 6 of 28 [21%]; P = .01). Patients with arrhythmic events (n = 5) and patients requiring mitral valve surgery (n = 7) were observed exclusively in the group displaying MAD.CONCLUSIONS AND RELEVANCE This study suggests that MAD among patients with MFS is associated with the occurrence of arrhythmic events, a higher need for mitral valve intervention, and, among patients with extensive MAD, more aortic events. Cardiac imaging for patients with MFS should consider the assessment of MAD as a potential marker for adverse outcomes.

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Presentation and Outcome of Arrhythmic Mitral Valve Prolapse

Cited by 137 publications

References 37 publications

Malignant Arrhythmic Mitral Valve Prolapse: A Continuum of Clinical Challenges from Diagnosis to Risk Stratification and Patient Management

Malignant Arrhythmic Mitral Valve Prolapse: A Continuum of Clinical Challenges from Diagnosis to Risk Stratification and Patient Management

Arrhythmic Mitral Valve Prolapse: Introducing an Era of Multimodality Imaging-Based Diagnosis and Risk Stratification

Association of Mitral Annular Disjunction With Cardiovascular Outcomes Among Patients With Marfan Syndrome

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