Mitral valve prolapse and sudden death: a systematic review

Abstract The Pickelhaube Sign is today recognized as a novel Echocardiographic Risk Marker for Malignant Mitral Valve Prolapse Syndrome. Mitral Valve Prolapse (MVP) has long been recognized to be a relatively common valve abnormality in the general population. Patients with relatively non-specific symptoms and asymptomatic athletes who have MVP still represent an important clinical conundrum for any physician involved in preventive medicine and sports screening. Although cardiac arrhythmias and/or cardiac death are an undesirable problem in MVP patients, when these subjects were studied with Holter Electrocardiogram (ECG) monitoring a prevalence of ventricular arrhythmias up to 34% was observed, with premature ventricular contractions as the most common pattern (66% of cases). At this regard a paper by Anders et al. described a series of cases that suggest that even clinically considered benign cases of MVP in young adults may cause sudden and unexpected death. However, cardiac arrest and Sudden Arrhythmic Cardiac Death (SCD) resulted in rare events only in patients with MVP based on data from a community study. A middle-aged athletic male who has been practicing competitive cycling for about 20 years came to our Sports Medicine Centre to undergo screening of sports preparation for competitive cycling and the related renewal of certification for participation in sports competitions. This athlete was always considered suitable in previous competitive fitness assessments performed in other sports medicine centers. His family history was unremarkable, as well as his recent and remote pathological anamnesis. The physical examination revealed a 3/6 regurgitation heart murmur with a click in the mid late systole. Previous echocardiographic examinations revealed a MVP which was considered benign with mild not relevant mitral regurgitation. He did not complain of symptoms such as dyspnoea or heart palpitations during physical activity. The resting ECG showed negative T waves in the inferior limb leads, and the stress test showed sporadic premature ventricular beats (a couple) with right bundle branch block morphology. An echocardiogram confirmed the presence of a classic mitral valve prolapse with billowing of both mitral leaflets, associated with a mild to moderate valve regurgitation. The TDI exam at the level of the lateral mitral annulus showed a high-velocity mid-systolic spike like a Pickelhaube sign, i.e. spiked German military helmet morphology. Consequently, an in-depth diagnostic imaging with cardiac magnetic resonance imaging was proposed, but the athlete refused it, both because he was totally asymptomatic and above all because he would be forced to pay a considerable amount of money as the examination is not guaranteed by the Italian National Health Service. In conclusion, the athlete remained sub judice as for competitive suitability, Finally, the question is: does MVP really cause sudden death? Is it enough to detect the Pickelhaube signal by echocardiography to stop this athlete? Let us bear in mind that this athlete was asymptomatic, and he had not had any trouble during exercise and maximal effort for many years. Why must we declare him unsuitable to do competitive sports?

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Mitral valve prolapse syndrome as cause of sudden death in young adults

Forensic Science International ◽

10.1016/j.forsciint.2006.10.011 ◽

2007 ◽

Vol 171 (2-3) ◽

pp. 127-130 ◽

Cited By ~ 19

Author(s):

Sven Anders ◽

Samir Said ◽

Friedrich Schulz ◽

Klaus Püschel

Keyword(s):

Young Adults ◽

Mitral Valve ◽

Sudden Death ◽

Mitral Valve Prolapse

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Sudden Death in Mitral Valve Prolapse

Journal of Forensic Sciences ◽

10.1520/jfs12439j ◽

1988 ◽

Vol 33 (1) ◽

pp. 12439J ◽

Cited By ~ 11

Author(s):

Diane M. Scala-Barnett ◽

Edmund R. Donoghue

Keyword(s):

Mitral Valve ◽

Sudden Death ◽

Mitral Valve Prolapse

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Relation of sudden death in pure mitral regurgitation, with and without mitral valve prolapse, to repetitive ventricular arrhythmias and right and left ventricular ejection fractions

The American Journal of Cardiology ◽

10.1016/0002-9149(87)90261-x ◽

1987 ◽

Vol 60 (4) ◽

pp. 397-399 ◽

Cited By ~ 45

Author(s):

Paul Kligfield ◽

Clare Hochreiter ◽

Nathaniel Niles ◽

Richard B. Devereux ◽

Jeffrey S. Borer

Keyword(s):

Mitral Valve ◽

Sudden Death ◽

Mitral Regurgitation ◽

Mitral Valve Prolapse ◽

Ventricular Arrhythmias ◽

Left Ventricular ◽

Ventricular Ejection ◽

Left Ventricular Ejection

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Mitral valve prolapse and sudden cardiac death: a systematic review and meta-analysis

Heart ◽

10.1136/heartjnl-2017-312932 ◽

2018 ◽

Vol 105 (2) ◽

pp. 144-151 ◽

Cited By ~ 22

Author(s):

Chrishan J Nalliah ◽

Rajiv Mahajan ◽

Adrian D Elliott ◽

Haris Haqqani ◽

Dennis H Lau ◽

...

Keyword(s):

Risk Factors ◽

Systematic Review ◽

Sudden Cardiac Death ◽

Mitral Valve ◽

Mitral Valve Prolapse ◽

Cardiac Death ◽

Meta Analysis ◽

Ventricular Ectopy ◽

T Wave ◽

Ventricular Fibrosis

ObjectivesMitral valve prolapse (MVP) is commonly observed as a benign finding. However, the literature suggests that it may be associated with sudden cardiac death (SCD). We performed a meta-analysis and systematic review to determine the: (1) prevalence of MVP in the general population; (2) prevalence of MVP in all SCD and unexplained SCD; (3) incidence of SCD in MVP and (4) risk factors for SCD.MethodsThe English medical literature was searched for: (1) MVP community prevalence; (2) MVP prevalence in SCD cohorts; (3) incidence SCD in MVP and (4) SCD risk factors in MVP. Thirty-four studies were identified for inclusion. This study was registered with PROSPERO (CRD42018089502).ResultsThe prevalence of MVP was 1.2% (95% CI 0.5 to 2.0) in community populations. Among SCD victims, the cause of death remained undetermined in 22.1% (95% CI 13.4 to 30.7); of these, MVP was observed in 11.7% (95% CI 5.8 to 19.1). The incidence of SCD in the MVP population was 0.14% (95% CI 0.1 to 0.3) per year. Potential risk factors for SCD include bileaflet prolapse, ventricular fibrosis complex ventricular ectopy and ST-T wave abnormalities.ConclusionThe high prevalence of MVP in cohorts of unexplained SCD despite low population prevalence provides indirect evidence of an association of MVP with SCD. The absolute number of people exposed to the risk of SCD is significant, although the incidence of life-threatening arrhythmic events in the general MVP population remains low. High-risk features include bileaflet prolapse, ventricular fibrosis, ST-T wave abnormalities and frequent complex ventricular ectopy.Trial registrationPROSPERO (CRD42018089502).

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PHENOTYPES OF MITRAL VALVE PROLAPSE AS A CAUSE OF SUDDEN DEATH: A CASE-CONTROL STUDY

Journal of the American College of Cardiology ◽

10.1016/s0735-1097(15)62022-8 ◽

2015 ◽

Vol 65 (10) ◽

pp. A2022

Author(s):

Jerome Hourdain ◽

Marie Annick Clavel ◽

Jean Claude Deharo ◽

Samuel Asirvatham ◽

Michael Ackerman ◽

...

Keyword(s):

Mitral Valve ◽

Sudden Death ◽

Mitral Valve Prolapse ◽

Case Control Study ◽

Case Control ◽

Control Study

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Mitral Valve Prolapse, Ventricular Arrhythmias, and Sudden Death

Circulation ◽

10.1161/circulationaha.118.034075 ◽

2019 ◽

Vol 140 (11) ◽

pp. 952-964 ◽

Cited By ~ 19

Author(s):

Cristina Basso ◽

Sabino Iliceto ◽

Gaetano Thiene ◽

Martina Perazzolo Marra

Keyword(s):

Cardiac Magnetic Resonance ◽

Magnetic Resonance ◽

Mitral Valve ◽

Sudden Death ◽

Mitral Valve Prolapse ◽

Ventricular Arrhythmias ◽

Left Ventricular ◽

Premature Ventricular Beats ◽

Electrophysiologic Studies

Despite a 2% to 3% prevalence of echocardiographically defined mitral valve prolapse (MVP) in the general population, the actual burden, risk stratification, and treatment of the so-called arrhythmic MVP are unknown. The clinical profile is characterized by a patient, usually female, with mostly bileaflet myxomatous disease, mid-systolic click, repolarization abnormalities in the inferior leads, and complex ventricular arrhythmias with polymorphic/right bundle branch block morphology, without significant regurgitation. Among the various pathophysiologic mechanisms of electrical instability, left ventricular fibrosis in the papillary muscles and inferobasal wall, mitral annulus disjunction, and systolic curling have been recently described by pathological and cardiac magnetic resonance studies in sudden death victims and patients with arrhythmic MVP. In addition, premature ventricular beats arising from the Purkinje tissue as ventricular fibrillation triggers have been documented by electrophysiologic studies in MVP patients with aborted sudden death. The genesis of malignant ventricular arrhythmias in MVP probably recognizes the combination of the substrate (regional myocardial hypertrophy and fibrosis, Purkinje fibers) and the trigger (mechanical stretch) eliciting premature ventricular beats because of a primary morphofunctional abnormality of the mitral valve annulus. The main clinical challenge is how to identify patients with arrhythmic MVP (which imaging technique and in which patient) and how to treat them to prevent sudden death. Thus, there is a necessity for prospective multicenter studies focusing on the prognostic role of cardiac magnetic resonance and electrophysiologic studies and on the therapeutic efficacy of targeted catheter ablation and mitral valve surgery in reducing the risk of life-threatening arrhythmias, as well as the role of implantable cardioverter defibrillators for primary prevention.

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