Autoimmune Myocarditis and Arrhythmogenic Mitral Valve Prolapse: An Unexpected Overlap Syndrome

Background: both myocarditis and mitral valve prolapse (MVP) are known uncommon causes of ventricular arrhythmias in young patients. Aim: to report the first clinical case of endomyocardial biopsy (EMB)-proven autoimmune myocarditis and associated arrhythmogenic MVP in a patient with recurrent ventricular fibrillation (VF) episodes. Methods: myocarditis was diagnosed both by cardiac magnetic resonance (CMR) and EMB. Arrhythmogenic MVP was documented by transthoracic echocardiogram, CMR, and electroanatomical mapping of the trigger premature ventricular contractions (PVCs). Results: a 22-year-old woman underwent immunosuppressive therapy after EMB-proven diagnosis of autoimmune myocarditis with VF onset and early implantable cardioverter defibrillator (ICD) placement. Three years later, she experienced two VF recurrences and persistent PVCs, despite no signs of myocarditis recurrence. An echocardiogram revealed bileaflet MVP with high arrhythmic risk features. Finally, electroanatomical mapping and ablation of the trigger PVC were successfully performed. Conclusion: in patients with recurrent VF episodes despite evidence-based medical treatment for myocarditis, MVP should be considered as an alternative arrhythmogenic substrate, and warrants early ICD implant and PVC-targeted therapy.

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Parameters associated with ventricular arrhythmias in mitral valve prolapse with significant regurgitation

Heart ◽

10.1136/heartjnl-2020-317451 ◽

2020 ◽

pp. heartjnl-2020-317451 ◽

Cited By ~ 2

Author(s):

Aniek L van Wijngaarden ◽

Marta de Riva ◽

Yasmine Lisanne Hiemstra ◽

Pieter van der Bijl ◽

Federico Fortuni ◽

...

Keyword(s):

Mitral Valve ◽

Mitral Valve Prolapse ◽

Ventricular Arrhythmias ◽

Global Longitudinal Strain ◽

Left Ventricular ◽

Premature Ventricular Contractions ◽

Improve Risk Stratification ◽

Mechanical Dispersion ◽

Barlow’S Disease ◽

Echocardiographic Parameters

ObjectiveMitral valve prolapse (MVP) has been associated with ventricular arrhythmias (VA), but little is known about VA in patients with significant primary mitral regurgitation (MR). Our aim was to describe the prevalence of symptomatic VA in patients with MVP (fibro-elastic deficiency or Barlow’s disease) referred for mitral valve (MV) surgery because of moderate-to-severe MR, and to identify clinical, electrocardiographic, standard and advanced echocardiographic parameters associated with VA.Methods610 consecutive patients (64±12 years, 36% female) were included. Symptomatic VA was defined as symptomatic and frequent premature ventricular contractions (PVC, Lown grade ≥2), non-sustained or sustained ventricular tachycardia (VT) or ventricular fibrillation (VF) without ischaemic aetiology.ResultsA total of 67 (11%) patients showed symptomatic VA, of which 3 (4%) had VF, 3 (4%) sustained VT, 27 (40%) non-sustained VT and 34 (51%) frequent PVCs. Patients with VA were significantly younger, more often female and showed T-wave inversions; furthermore, they showed significant MV morphofunctional abnormalities, such as mitral annular disjunction (39% vs 20%, p<0.001), and dilatation (annular diameter 37±5 mm vs 33±6 mm, p<0.001), lower global longitudinal strain (GLS 20.9±3.1% vs 22.0±3.6%, p=0.032) and prolonged mechanical dispersion (45±12 ms vs 38±14 ms, p=0.003) as compared with patients without VA. Female sex, increased MV annular diameter, lower GLS and prolonged mechanical dispersion were identified as independent associates of symptomatic VA.ConclusionIn patients with MVP with moderate-to-severe MR, symptomatic VA are relatively frequent and associated with significant MV annular abnormalities, subtle left ventricular function impairment and heterogeneous contraction. Assessment of these parameters might help decision-making over further diagnostic analyses and improve risk-stratification.

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Echocardiographic and biochemical predictors of ventricular arrhythmias in young patients with mitral valve prolapse

European Heart Journal ◽

10.1093/eurheartj/eht310.p4747 ◽

2013 ◽

Vol 34 (suppl 1) ◽

pp. P4747-P4747

Author(s):

E. Malev ◽

S. Reeva ◽

E. Timofeev ◽

A. Pshepiy ◽

A. Korshunova ◽

...

Keyword(s):

Mitral Valve ◽

Mitral Valve Prolapse ◽

Ventricular Arrhythmias ◽

Young Patients

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P559 Mitral valve prolapse and atrial septal aneurysm in young patients with stroke and normal carotid arteries

European Heart Journal ◽

10.1016/s0195-668x(03)93997-2 ◽

2003 ◽

Vol 24 (5) ◽

pp. 92

Author(s):

A MATTIOLI

Keyword(s):

Mitral Valve ◽

Mitral Valve Prolapse ◽

Carotid Arteries ◽

Young Patients ◽

Atrial Septal Aneurysm

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407 Is Pickelhaube Sign really the hallmark of arrhythmogenic MVP in athletes? And does MVP really cause sudden death? A case report

European Heart Journal Supplements ◽

10.1093/eurheartj/suaa206 ◽

2020 ◽

Vol 22 (Supplement_N) ◽

pp. N131-N131

Author(s):

Massimo Bolognesi

Keyword(s):

Mitral Valve ◽

Sudden Death ◽

Mitral Valve Prolapse ◽

Sports Medicine ◽

Cardiac Death ◽

Stress Test ◽

Mitral Annulus ◽

Unexpected Death ◽

Premature Ventricular Contractions ◽

Military Helmet

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

Journal of Cardiovascular Development and Disease ◽

10.3390/jcdd8010002 ◽

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.

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Ventricular arrhythmias in mitral valve prolapse syndrome and their relationship with electrocardiographic repolarization parameters

Northern Clinics of Istanbul ◽

10.14744/nci.2021.12058 ◽

2021 ◽

Author(s):

Berat Engin

Keyword(s):

Mitral Valve ◽

Mitral Valve Prolapse ◽

Ventricular Arrhythmias

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Mitral Valve Prolapse and Athletic Participation in Children and Adolescents

PEDIATRICS ◽

10.1542/peds.95.5.789 ◽

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.

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