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

2020 ◽  
Vol 8 (1) ◽  
pp. 2
Author(s):  
Idit Yedidya ◽  
Aniek L. van Wijngaarden ◽  
Nina Ajmone Marsan
Keyword(s):  
Mitral Valve ◽  
Mitral Valve Prolapse ◽  
Ventricular Arrhythmias ◽  
Clinical Decision Making ◽  
Clinical Presentation ◽  
Clinical Decision ◽  
Benign Condition ◽  
Long Period ◽  
Patients Experience ◽  
Hospital Cardiac Arrest

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.

scholarly journals Mitral valve prolapse with ventricular arrhythmias: does it carries a worse prognosis?

2021 ◽  
Vol 23 (Supplement_E) ◽  
pp. E77-E82
Author(s):  
Gloria Vassilikì Coutsoumbas ◽  
Giuseppe Di Pasquale
Keyword(s):  
Mitral Valve ◽  
Mitral Valve Prolapse ◽  
Ventricular Arrhythmias ◽  
Large Population ◽  
Therapeutic Interventions ◽  
Bundle Branch Block ◽  
Benign Condition ◽  
History Of ◽  
Ectopic Beats ◽  
Repolarization Abnormalities

Abstract The association of mitral valve prolapse (MVP) with ventricular arrhythmias has long been known and has generally been considered a benign condition. In recent years, however, a small but not negligible risk of malignant ventricular arrhythmias and sudden cardiac death has been documented in the large population of subjects with MVP. The main predictors of major arrhythmic risk identified so far include history of syncope, ventricular repolarization abnormalities in the inferior-lateral electrocardiogram leads, right bundle branch block morphology of ventricular ectopic beats, finding of areas of myocardial fibrosis on cardiac magnetic resonance, and mitral annular disjunction (MAD) on echocardiogram, as well as a possible pro-arrhythmic genetic substrate. The stratification of arrhythmic risk, with the active search for red flags and in particular of MAD, is important to identify patients with the malignant arrhythmic variant of MVP in whom to implement closer surveillance and possible therapeutic interventions.

scholarly journals Mechanistic insights of the left ventricle structure and fibrosis in the arrhythmogenic mitral valve prolapse

2018 ◽  
Vol 2018 (1) ◽  
Author(s):  
Leticia Fernández-Friera ◽  
Rafael Salguero ◽  
Luca Vannini ◽  
Ana Fidalgo Argüelles ◽  
Fernando Arribas ◽  
...  
Keyword(s):  
Sudden Cardiac Death ◽  
Mitral Valve ◽  
Mitral Valve Prolapse ◽  
Cardiac Death ◽  
Ventricular Arrhythmias ◽  
Imaging Techniques ◽  
Current Evidence ◽  
Special Focus ◽  
Benign Condition ◽  
Life Threatening

Mitral valve prolapse (MVP) is a common and benign condition. However, some anatomic forms have been recently associated with life-threatening ventricular arrhythmias and sudden cardiac death. Imaging MVP holds the promise of individualized MVP risk assessment. Noninvasive imaging techniques available today are playing an increasingly important role in the diagnosis, prognosis and monitoring of MVP. In this article, we will review the current evidence on arrhythmogenic MVP, with special focus on the utility of echocardiography and CMR for identifying benign and ‘‘malignant’’ forms of MVP. The clinical relevance of this manuscript lies in the value of imaging technology to improve MVP risk prediction, including those arrhythmic-MVP cases with a higher risk of sudden cardiac death. 

Mitral Valve Prolapse and Athletic Participation in Children and Adolescents

PEDIATRICS ◽  
1995 ◽  
Vol 95 (5) ◽  
pp. 789-790
Author(s):  
Keyword(s):  
Mitral Valve ◽  
Children And Adolescents ◽  
Mitral Valve Prolapse ◽  
Holter Monitoring ◽  
Premature Ventricular Contractions ◽  
Benign Condition ◽  
Migraine Headaches ◽  
Asymptomatic Patients ◽  
Vascular Accidents

Mitral valve prolapse (MVP) is generally a benign condition characterized by the protrusion of the mitral valve leaflets into the left atrium during systole. The prevalence of MVP in individuals under the age of 18 years is estimated to be 5% but is higher in those with Marfan's syndrome and other collagen vascular disorders.1 A midsystolic nonejection click with or without a late systolic murmur is the auscultatory hallmark of this syndrome. The diagnosis of MVP in children and adolescents should be based primarily on auscultatory findings and not on minor echocardiographic findings.1 The prognosis in children and adolescents with isolated MVP appears to be excellent and complications are rare. In 553 children, aged 15 days to 18 years, who were involved in studies with a follow-up period of 6 to 9 years, the following were reported: subacute bacterial endocarditis (one case), cerebral vascular accidents (two cases), migraine headaches (four cases), and chest pain (12 cases).2,3 Only four cases of sudden death have been reported in patients younger than 20 years of age.1-4 In a study of 103 patients with MVP, 16% were found to have premature ventricular beats during exercise electrocardiography (ECG) (exercise test).3 Thirty-eight percent were found to have premature ventricular contractions (PVCs) on 24-hour ECG (Holter) monitoring. This study, however, does not report the true prevalence of dysrhythmias because all these subjects had been referred to a pediatric cardiologist for evaluation. It is likely that these reported numbers are high because asymptomatic patients are less often referred.

scholarly journals P5440Mitral annulus disjunction is associated with severe ventricular arrhythmias independently of mitral valve prolapse

2018 ◽  
Vol 39 (suppl_1) ◽  
Author(s):  
L A Dejgaard ◽  
E T Skjolsvik ◽  
O H Lie ◽  
M Ribe ◽  
M K Stokke ◽  
...  
Keyword(s):  
Mitral Valve ◽  
Mitral Valve Prolapse ◽  
Ventricular Arrhythmias

scholarly journals Long-term outcomes of ablation for ventricular arrhythmias in mitral valve prolapse

2020 ◽  
Author(s):  
Paul J. Marano ◽  
Lisa J. Lim ◽  
Jose M. Sanchez ◽  
Raza Alvi ◽  
Gregory Nah ◽  
...  
Keyword(s):  
Mitral Valve ◽  
Mitral Valve Prolapse ◽  
Ventricular Arrhythmias ◽  
Long Term Outcomes

scholarly journals Mitral valve prolapse and electrolyte abnormality: a dangerous combination for ventricular arrhythmias

2014 ◽  
Vol 2014 (may14 1) ◽  
pp. bcr2014205055-bcr2014205055 ◽  
Author(s):  
A. R. Rajani ◽  
V. Murugesan ◽  
F. O. Baslaib ◽  
M. A. Rafiq
Keyword(s):  
Mitral Valve ◽  
Mitral Valve Prolapse ◽  
Ventricular Arrhythmias ◽  
Electrolyte Abnormality

P4172Prevalence and potential genetic determinants of young sudden unexplained death victims with suspected arrhythmogenic mitral valve prolapse

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
J Giudicessi ◽  
D Tester ◽  
M Ackerman
Keyword(s):  
Mitral Valve ◽  
Mitral Valve Prolapse ◽  
Molecular Mechanisms ◽  
Age At Death ◽  
Genetic Determinants ◽  
Contributing Factors ◽  
Benign Condition ◽  
Sudden Unexplained Death ◽  
Unexplained Death ◽  
Whole Exome

Abstract Background Mitral valve prolapse (MVP) is largely considered a benign condition. Recently, arrhythmogenic MVP, a sudden cardiac death (SCD)-predisposing condition characterized clinically by frequent/complex ventricular arrhythmias likely secondary to prolapsing leaflet-induced fibrosis of the left ventricle (LV), has emerged as an underappreciated cause of sudden unexplained death in the young (SUDY). Objective To determine the prevalence and potential genetic underpinnings of suspected arrhythmogenic MVP in a referral cohort of SUDY cases. Methods In this retrospective necropsy study, the medical records, autopsy reports, and whole exome molecular autopsy (WEMA) results for 77 SUDY victims (27 females; average age at death 20.6±8.9 years) were reviewed. Evidence of myxomatous MVP and concomitant LV pathology were noted. Variants detected in the pre-specified 164 WEMA gene panel with a frequency <0.001 in public exomes were classified using the 2015 American College of Medical Genetics (ACMG) guidelines. Results Overall, 6/77 (7.8%; 2 females; average age at death 20.7±6.9 years) SUDY cases had MVP as the lone abnormal post-mortem finding. The majority had bileaflet involvement (5/6; 83%) and microscopic LV fibrosis (5/6; 83%). Two SUDY cases (33%) were diagnosed with MVP by echocardiography prior to death. No gross hypertrophy, myocyte disarray, or fibrofatty replacement was noted. Unexpectedly, an ACMG pathogenic/likely pathogenic (P/LP) was more likely to be detected in SUDY cases with MVP than those without [3/6 (50%) vs 9/71 (13%); p<0.05]. Interestingly, the three variants identified in MVP-positive SUDY cases localized to genes associated previously with a cardiomyopathy/channelopathy predisposition (p.E1518fsX25-DMD, p.S285N-RYR2, and p.R109X-TTN). Conclusion This WEMA series provides additional evidence that the combination of bileaflet MVP and microscopic LV fibrosis underlies an unexpected number of SUDY cases. Whether P/LP variants in cardiomyopathy/channelopathy-susceptibility genes function as contributing factors or if unrelated, but poorly understood, molecular mechanisms account for the distinct cardiomyopathy-like phenotype observed in arrhythmogenic MVP requires further investigation.

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