Mitral Valve Prolapse Syndrome Research Articles

Arrhythmic Mitral Valve Prolapse and Sports Activity: Pathophysiology, Risk Stratification, and Sports Eligibility Assessment.

Although mitral valve prolapse (MVP) is the most prevalent valvular abnormality in Western countries and generally carries a good prognosis, a small subset of patients is exposed to a significant risk of malignant ventricular arrhythmias (VAs) and sudden cardiac death (SCD), the so-called arrhythmic MVP (AMVP) syndrome. Recent work has emphasized phenotypical risk features of severe AMVP and clarified its pathophysiology. However, the appropriate assessment and risk stratification of patients with suspected AMVP remains a clinical conundrum, with the possibility of both overestimating and underestimating the risk of malignant VAs, with the inappropriate use of advanced imaging and invasive electrophysiology study on one hand, and the catastrophic occurrence of SCD on the other. Furthermore, the sports eligibility assessment of athletes with AMVP remains ill defined, especially in the grey zone of intermediate arrhythmic risk. The definition, epidemiology, pathophysiology, risk stratification, and treatment of AMVP are covered in the present review. Considering recent guidelines and expert consensus statements, we propose a comprehensive pathway to facilitate appropriate counseling concerning the practice of competitive/leisure-time sports, envisioning shared decision making and the multidisciplinary "sports heart team" evaluation of borderline cases. Our final aim is to encourage an active lifestyle without compromising patients' safety.

Mitral valve prolapse syndrome

Mitral valve prolapse is a common valvular defect. In its overt clinical form, the condition known as mitral valve leaflet prolapse syndrome, or also known as Barlow's syndrome or flaccid valve syndrome, is a significant clinical problem, worsening the quality of life of patients, and in its severe forms is a risk factor for diseases such as congestive heart failure, endocarditis or cardiac arrhythmias, or even lead to death from sudden cardiac death. The development of methods, as well as the increasing general availability of echocardiography methods, makes a specific diagnosis early enough to allow the right treatment strategy to be chosen. Thanks to reliable reports in recent years, it has been possible to identify a group of patients whose cardiovascular risk predisposes them to a more severe course of the disease and thus these patients require special observation and attention in the process of diagnosis and treatment. Recent years have allowed the development of a new method of interventional treatment in patients burdened with multimorbidity for whom conventional surgery would be associated with a significant risk of worsening of the general condition.

Clinical genetic testing in arrhythmogenic mitral valve prolapse syndrome highlights a potential overlap with familial dilated cardiomyopathy

Abstract Background/Introduction Arrhythmogenic mitral valve prolapse syndrome (AMVPS) is a female-predominant sudden cardiac death (SCD)-predisposing disorder characterized clinically by myxomatous mitral valve prolapse (MVP), inferolateral T-wave inversions and complex ventricular ectopy. However, insights into the pathogenesis and potential genetic basis of AMVPS are currently lacking. Purpose Considering recent case reports that suggest a potential role for genetic cardiomyopathies in the pathogenesis of AMVPS, we sought to determine the yield of pathogenic/likely pathogenic (P/LP) genetic variants in strong evidence cardiomyopathy-susceptibility genes amongst AMVPS patients who underwent clinical genetic testing. Methods In this retrospective study, the medical records of 4,907 patients referred to our cardiovascular genetics clinic was used to identify those patients with a clinical phenotype consistent with AMVPS. After exclusion of individuals with severe mitral valve regurgitation, pertinent clinical, electrocardiographic, imaging and genetic testing data was extracted from the electronic record. For those AMVPS that underwent a form of genetic testing that encompassed 23 Clinical Genome Resource-defined strong/definitive evidence cardiomyopathy-susceptibility genes, ultra-rare (minor allele frequency ≤ 0.00005 in the Genome Aggregation Database [n=141,456 individuals]) non-synonymous variants identified within these genes were adjudicated independently as P, LP or variant of uncertain significance with the assistance of Varsome. The frequency of P/LP variants was then compared to a previously published cohort of 973 ostensibly healthy control exomes. Results Overall, 56 unrelated patients with suspected AMVPS were identified (82% female; average age at presentation of 31 ± 13 years; 32% with a family history of MVP and/or SCD). Of these, 24/56 (43%) underwent a form of clinical genetic testing that encompassed the pre-defined panel of cardiomyopathy-susceptibility genes. In comparison to control exomes (n=973), P/LP variants in strong/definitive evidence cardiomyopathy-susceptibility genes were over-represented in AMVPS patients [4/24 (16.7%) vs 20/973 (2.1%); p=0.002]. Interestingly, this enrichment was driven in large part by an excess number of A-band-localizing P/LP truncating variants in TTN-encoded titin in AMVPS cases [3/24 (12.5%) versus 4/973 (0.4%); p=0.0004]. Conclusion This study provides further evidence that a subset of AMVPS may arise secondary to a "two-hit" model whereby an underlying cardiomyopathic genetic substrate is exacerbated by the mechanical force generated by genetically unrelated myxomatous MVP resulting in maladaptive and ventricular arrhythmia-predisposing fibrosis within the inferobasal left ventricle and posteromedial papillary muscles. Larger studies are needed to better understand the potential role of genetic testing in the diagnosis, risk-stratification and management of patients with suspected AMVPS.

Mitral Annular Disjunction: Associated Pathologies and Clinical Consequences.

To provide an overview of mitral annular disjunction (MAD) and to discuss important challenges in diagnosis and management of MAD. MAD has regained interest in the context of sudden cardiac death (SCD) in patients with mitral valve prolapse (MVP), coined as the "arrhythmic" MVP syndrome. In addition, MAD in isolation was recently suggested to be associated with severe arrhythmia and SCD. There is a lack of consensus on the definition of MAD and the imaging modality to be used for diagnosing MAD, and the therapeutic implications of MAD remain uncertain. Furthermore, the exact mechanism underlying the association of MAD with SCD remains largely unexplored.

Hereditary Haemorrhagic Telangiectasia with Mitral Valve Prolapse and Straight Back Syndrome: A Rare Association

Hereditary Haemorrhagic Telangiectasia with Mitral Valve Prolapse and Straight Back Syndrome: A Rare Association

Abstract 12105: Sudden Death Associated With Pregnancy

Introduction: Sudden and unexpected deaths associated with pregnancy are rare but devastating occurrences in society. There is a significant lack of prospective, population-based data in the US regarding etiologies and clinical manifestation. Hypothesis: Prospective community-wide analyses can inform the assessment of burden as well as prevention strategies for sudden deaths associated with pregnancy. Methods: From the ongoing prospective, Oregon Sudden Unexpected Death Study (ORSUDS, Portland OR catchment pop. 1 M, 2002-2021) and the Prediction of Sudden Death in Multi-Ethnic Communities (PRESTO, Ventura CA catchment pop 850K, 2015-2021), we performed a detailed evaluation of all sudden death cases that occurred within 1 year of pregnancy; including lifetime clinical history, SCA presentation and resuscitation outcome. Results: A total of 381 (46 in PRESTO and 335 in ORSUDS) sudden deaths occurred in females aged 15 to 49 years, and 17 of these (4.5%) occurred within a year of pregnancy. Pregnancy-related cases were between 21 and 39 years of age and included 7 non-Hispanic whites, 5 Hispanic/Latinos, 1 black, 1 Native American and 3 subjects of unknown race/ethnicity. Sudden cardiac arrest occurred while pregnant (6), during delivery (2), within 3 months post-partum (5) and a miscarriage (1), and between 3 and 9 months post-delivery (3). Only witnessed cases survived (4/17, 24%) and presented with both shockable rhythm (2) and non-shockable rhythm (2). Presumptive cause of death included 10 cardiac etiologies including presumed sudden arrhythmic death (4), coronary artery disease (2), left ventricular hypertrophy (1), severe mitral valve prolapse (1), myocardial fibrosis (1) and long QT syndrome (1). Non cardiac causes included drug overdose (2), obstetric thrombo-embolism (2), cerebro-vascular accident (2), and status asthmaticus (1). Conclusions: Our findings suggest that pre-existing heart disease is likely to be the most important risk factor for sudden unexpected death associated with pregnancy, and warrants further detailed investigation.

Mitral Valve Prolapse and Mitral Annular Disjunction Arrhythmic Syndromes: Diagnosis, Risk Stratification and Management

Although mitral valve prolapse (MVP) is usually considered a benign clinical condition, it has been linked with ventricular arrhythmias and sudden cardiac death in patients with a certain “arrhythmic” phenotype, raising awareness and mandating a specific risk stratification protocol. Mitral annular disjunction (MAD) is considered a “red flag” in malignant MVP syndrome along with bileaflet myxomatous prolapse, female gender, negative or biphasic T waves in the inferior leads, fibrosis in the papillary muscles or inferobasal wall detected by cardiac magnetic resonance imaging and complex arrhythmias of right bundle branch morphology. MAD seems to play a critical role in the chain of morphofunctional abnormalities which lead to increased mechanical stretch and subsequent fibrosis mainly in the papillary muscles, forming the vulnerable anatomic substrate prone to arrhythmogenesis, and associated with long-term severe ventricular arrhythmias. Arrhythmogenesis in MVP/MAD patients is not fully understood but a combination between a substrate and a trigger has been established with premature ventricular contraction triggered ventricular fibrillation being the main mechanism of sudden cardiac death (SCD). Certain characteristics mostly recognized by non-invasive imaging modalities serve as risk factors and can be used to diagnose and identify high risk patients with MAD, while treatment options include catheter ablation, device therapy and surgical intervention. This review focuses on the clinical presentation, the arrhythmogenic substrate, and the incidence of ventricular arrhythmias and SCD in MAD population. The current risk stratification tools in MAD arrhythmogenic entity are discussed.

Mitral and tricuspid annulus disjunction frequently coexist

Abstract Background Mitral annulus disjunction (MAD) is an abnormal atrial displacement of the mitral annulus, frequently found in patients with high-risk arrhythmogenic mitral valve prolapse syndrome. It is unknown whether the annulus disjunction extends to the right side of the heart as tricuspid annulus disjunction (TAD), and whether it is associated with right ventricular electrical instability. Purpose We aimed to explore the presence of TAD, and if extended annulus disjunction was associated with ventricular arrhythmias. Methods We included patients with previously described MAD assessed by cardiac magnetic resonance imaging (CMR) in an ambispective cohort study. MAD and TAD was defined as ≥1 mm separation between the respective atrial wall-valve leaflet junction and the top of the ventricular myocardium. TAD was assessed in the lateral and inferior right ventricular free wall by means of the 4-chamber and right ventricular 2-chamber views, respectively. MAD circumference was assessed by a CMR study protocol with six left ventricular long axis views separated by 30 degrees. Mitral valve prolapse was defined as ≥2 mm superior displacement of any part of the mitral leaflets beyond the mitral annulus. Ventricular arrhythmias were defined as aborted cardiac arrest or non-sustained/sustained ventricular tachycardias recorded by electrocardiogram (ECG), stress ECG or Holter monitoring. Results We included 92 patients with MAD (62% female, age 47±16 years, 71% mitral valve prolapse). TAD was found in 48 (52%) patients, both in the lateral (n=40, 83%) and inferior (n=30, 63%) right ventricular free wall. Patients with TAD were older (age 51±16 years vs. 43±14 years, p=0.01), had greater MAD circumference (168±56° vs. 117±62°, p=0.001) and greater MAD distance (9.2±2.9 mm vs. 6.4±2.8 mm, p<0.001). Additionally, patients with TAD had more frequently mitral valve prolapse (40 patients [85%] vs. 25 patients [57%], p=0.003), whereas similar frequency of bileaflet prolapse (17 patients [39%] vs. 10 patients [39%], p=0.99). Ventricular arrhythmias had occurred in 38 (41%) patients, who were younger (age 40±14 years vs. 52±15 years, p<0.001) and had less frequently TAD (14 patients [37%] vs. 34 patients [63%], p=0.01; univariate odds ratio 0.34 [0.15–0.81], p=0.02). However, TAD was not associated with ventricular arrhythmias when adjusted for age (multivariate odds ratio 0.46 [0.18–1.15], p=0.10). Conclusions TAD by CMR was highly prevalent in patients with MAD and was a marker of severe annulus disjunction and mitral valve prolapse. TAD was not associated with more ventricular arrhythmias. This novel marker warrants further research to explore the clinical implications of right-sided annulus disjunction. Funding Acknowledgement Type of funding sources: Public grant(s) – National budget only. Main funding source(s): Norwegian Research Council

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

BackgroundMitral valve prolapse (MVP) is largely considered a benign condition. However, MVP is over-represented consistently in sudden unexplained death in the young (SUDY) cohorts.ObjectiveTo determine the prevalence and potential genetic underpinnings of suspected arrhythmogenic MVP in a referral cohort of SUDY cases.MethodsIn this retrospective study, medical records/autopsy reports and whole exome molecular autopsy (WEMA) results for 77 SUDY victims (27 female; average age at death 20.6 ± 8.9 years) were reviewed for evidence of myxomatous MVP and left ventricle (LV) fibrosis. Variants detected in the prespecified 147 WEMA gene panel with a minor allele frequency ≤ 0.001 in public exomes/genomes were classified using the 2015 American College of Medical Genetics (ACMG) guidelines.ResultsOverall, 6 of 77 (7.8%; 2 female; average age at death 20.7 ± 6.9 years) SUDY cases had MVP as the lone abnormal postmortem finding. The majority had bileaflet involvement (5/6; 83%) and microscopic LV fibrosis (5/6; 83%). In 2 SUDY cases (33%), subjects were diagnosed with MVP by echocardiography prior to death. 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 < .05). Interestingly, the 3 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).ConclusionThis WEMA series provides additional evidence that the combination of MVP and LV fibrosis underlies an unexpected number of SUDY cases. Whether P/LP variants in cardiomyopathy/channelopathy-susceptibility genes contribute to the pathogenesis of arrhythmogenic MVP requires further investigation.

Atrial Septal Aneurysm Associated with Mitral Valve Prolapse Syndrome

A 68-year-old male presented to the out-patient clinic with history of intermittent palpitations and fatigability for past seven months. On cardiovascular system examination, mid-systolic click and pan-systolic murmur grade III/VI were audible at the apex with radiation to the base of heart. Echocardiographic study revealed atrial septal aneurysm (Figure 1, Figure 2, Video 1 and Video 2), along with prolapse of Posterior Mitral Leaflet (PML) into left atrium (Figure 3 and Figure 4).

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

The aim of present study is to compare ventricular and supraventricular arrhythmia incidences in subjects with and without mitral valve prolapse (MVP) syndrome and to examine if an association exists between ventricular arrhythmias and repolarization parameters in patients with MVP syndrome. This cross-sectional study involved 41 subjects with MVP Syndrome and 41 subjects with palpitation but without MVP (control group). All subjects were subjected to lead-electrocardiogram, transthoracic echocardiography, and 24-h Holter monitoring to identify repolarization abnormalities, structural abnormalities, and supraventricular and ventricular arrhythmias. The QRS width, QTC interval, and Tpeak-Tend intervals were measured for each participant. The number of subjects who had premature ventricular contractions (PVCs), couplets, and non-sustained ventricular tachycardia (NSVTs) was significantly higher in the MVP group compared to the control group. Left ventricular end-systolic diameter (LVESD) and left ventricular end-diastolic diameter (LVEDD) and left atrial diameter were also significantly higher in the MVP group than the control group. QRS width and Tpeak-Tend interval were also significantly higher in subjects with MVP than the controls. Correlation analysis showed a positive correlation between the severity of mitral regurgitation (MR) and the number of PVCs and couplets, while there was a significant correlation between left atrium (LA) diameter and the number of the PVCs and NSVTs. Subjects with MVP experience ventricular arrhythmias more often including PVCs, couplets, and NSVTs compared to subjects without MVP. LVESD, LVEDD, LA diameter, QRS width, and Tpeak-Tend interval were increased in MVP subjects than those without MVP. There is an association between the severity of the MR and the frequency of the PVCs, couplets, or NSVTs.

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

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?

1642 Mitral annulus disjunction, a rare form of focal arrhythmogenic cardiomyopathy

Abstract Funding Acknowledgements Project no. NVKP_16-1-2016-0017 has been implemented with the support provided from the National Research, Development and Innovation Fund of Hungary The rate of sudden cardiac death in mitral valve prolapse (MVP) patients is roughly twice that observed in the general population. Arrhythmogenic bileaflet MVP syndrome has been recently described and mitral annulus disjunction (MAD) has been found associated with papillary muscle fibrosis and ventricular arrhythmias even without severe mitral regurgitation. An underlying primary structural myocardial disease might be expected and the non-invasive imaging techniques are playing an increasingly important role in the detection of myocardial fibrosis as a focal arrhythmogenic substrate. Standard 2D-, 3D- and speckle tracking echocardiography and cardiac MRI can help to differentiate between the benign and malignant forms of MVP. A 52-year-old man with a history of hypertension was referred for evaluation of frequent palpitations and presyncope. Ambulatory Holter monitoring showed paroxysmal atrial fibrillation and non-sustained ventricular tachycardias. Dilated left ventricle (LVEDD: 65mm, LVESD: 45 mm) and left atrium (LAVi: 52mL/m2), moderately reduced left ventricular (LV) systolic function (LVEF: 42%), and reduced LV global longitudinal strain (GLS: -13.5%) with severely decreased segmental longitudinal strain in the basal inferolateral segment were measured by transthoracic echocardiography. The diastolic function was preserved, however Pickelhaube sign was noticed in the lateral mitral annulus TDI curves. MAD (17 mm) and curling motion of the inferolateral basal segment of the left ventricle was observed. Transesophageal echocardiography showed bileaflet mitral valve prolapse with the involvement of all the six scallops (mitral valve prolapse volume: 10.2 mL). Severe mitral regurgitation (3D vena contracta area: 1cm2) and extremely dilated and dyskinetic mitral annulus (diameters: 55x72x12 mm, area: 34 cm2) was measured by 3D echocardiography. MRI showed transmural late gadolinium enhancement in the underlying myocardium of both papillary muscles and midmyocardial enhancement in the basal inferior, inferolateral and anteroseptal LV segments. During electrophysiology study ventricular fibrillation was easily induced and ICD was placed for primary prevention of sudden cardiac death. The patient was referred to the heart surgeon for mitral valve replacement. This case highlights the importance of early recognition of MAD and arrhythmogenic MVP syndrome using novel non-invasive imaging techniques. The presence of MAD, the curling motion of the inferolateral basal LV segment, the Pickelhaube sign, the decreased LVEF and GLS, the characteristic segmental longitudinal strain pattern and the presence of late enhancement in the papillary muscles and the surrounding myocardium could be warning signs of the malignant form of MVP. Abstract 1642 Figure. CMR images of arrhythmogenic MAD

Abstract 16731: Novel CMR Anatomical Predictors of Mitral Regurgitation Presence and Severity in Mitral Valve Prolapse

Introduction: Mitral valve (MV) prolapse (MVP) is often associated with mitral regurgitation (MR). The determinants of MR in MVP are incompletely understood. Methods: We retrospectively identified 86 consecutive patients with MVP and preserved ejection fraction undergoing CMR with velocity encoding for MR quantification between 2005 and 2017. On 3-chamber cine images we measured, in end-diastole (Figure), end-systole, and their percentage change during the cardiac cycle, the following parameters: basal and mid inferolateral wall thickness, posterior papillary muscle length, annulus diameter, LV diameter and distance from the posterior papillary muscle to anterior and posterior annulus. In addition, we measured anterior and posterior leaflet thickness in diastole and maximal prolapse distance in end-systole. We explored the associations between these parameters and mitral regurgitant fraction (RF) using multivariate linear regression. Results: Median age was 52.6 years and 48% were males. There was posterior MVP in 49 (57%), bileaflet MVP in 35 (41%) patients, and isolated anterior MVP in 2 (2%) patients. MR was present in 73 (85%) and, in these, median RF was 23% (IQR 13-33%). In multivariate analyses, diastolic papillary muscle to posterior annulus distance, maximal prolapse distance, maximal posterior leaflet thickness, and LV basal inferolateral diastolic thickness were independent predictors of mitral RF (p&lt;0.05 for all). Conclusions: Beyond known determinants of MR in MVP such as maximal prolapse and leaflet thickness, we identified novel anatomical predictors of MR severity, namely increased diastolic papillary muscle to posterior annulus distance and LV basal inferolateral thickness. Whether these abnormalities represent secondary changes to MVP (i.e. basal inferolateral “stretch” and compensatory regional LV hypertrophy, respectively) or primary phenotypic features of the MVP syndrome is uncertain.

Selection of Waste Lubricant Oil Regenerative Technology Using Entropy-Weighted Risk-Based Fuzzy Axiomatic Design Approach

In the paper we study the possibility of constructing decision graphs with the help of several meta agents. Decision graphs are an extension of the well known decision trees and introduce the possibility of program nodes and cycles in a classification model. A two-leveled evolutionary algorithm for the induction of decision graphs is presented and the principle of classification based on the decision graphs is described. Several agents are used to construct the decision graphs; they are constructed and evolved with the help of automatic programming and evaluated with a universal complexity measure. The developed model is applied to a medical dataset for the classification of patients with mitral valve prolapse syndrome.

Mitral Valve Prolapse and Cardiac Arrest: Defining Malignant Mitral Valve Prolapse Syndrome

Background: In an attempt to better understand malignant mitral valve prolapse (MVP) syndrome, this study aimed to comprehensively evaluate the scientific literature in relation to case reports and series of MVP and cardiac arrest.

Mitral valve prolapse and Marfan syndrome.

Marfan syndrome is a multisystemic genetic condition affecting connective tissue. It carries a reduced life expectancy, largely dependent on cardiovascular complications. More common cardiac manifestations such as aortic dissection and aortic valve incompetence have been widely documented in the literature. Mitral valve prolapse (MVP), however, has remained poorly documented. This article aims at exploring the existing literature on the pathophysiology and diagnosis of MVP in patients with Marfan syndrome, defining its current management and outlining the future developments surrounding it.

Mitral Valve Prolapse

Mitral valve prolapse (MVP) is the most common valvular abnormality, affecting 2.4% of the population. Usually MVP is a benign disease and remains asymptomatic. The diagnosis of MVP is based on clinical presentation, physical examination and echocardiography. Some atypical symptoms that are not correlated with mitral valve function, are described as the MVP syndrome. Potential complications such as infective endocarditis, thromboembolic events, atrial and ventricular arrhythmias, and progressive mitral valve regurgitation may occur. Management should concentrate on adequate guidance of the patients, relief of symptoms and avoidance of complications.

Effects of mitral valve repair on ventricular arrhythmia in patients with mitral valve prolapse syndrome: A report of two cases

Effects of mitral valve repair on ventricular arrhythmia in patients with mitral valve prolapse syndrome: A report of two cases

Correction.

HomeCirculation: Arrhythmia and ElectrophysiologyVol. 9, No. 6Correction Free AccessCorrectionPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessCorrectionPDF/EPUBCorrection Originally published2 Jun 2016https://doi.org/10.1161/HAE.0000000000000018Circulation: Arrhythmia and Electrophysiology. 2016;9:e000018This article corrects the followingSites of Successful Ventricular Fibrillation Ablation in Bileaflet Mitral Valve Prolapse SyndromeIn the article by Syed et al (Syed FF, Ackerman MJ, McLeod CJ, Kapa S, Mulpuru SK, Sriram CS, Cannon BC, Asirvatham SJ, Noseworthy PA. Sites of successful ventricular fibrillation ablation in bileaflet mitral valve prolapse syndrome. Circulation: Arrhythmia and Electrophysiology. 2016;9:e004005, doi: 10.1161/CIRCEP.116.004005), which published April 21, 2016, and appeared in the May 2016 issue of the journal, corrections were needed.The authors apologize for these errors.In Figure 1E, the arrows indicating ablation catheter (Abl) channel and the posterior papillary muscle (PM) were shifted. The figure has been replaced and is shown below.On p 9, right column, line 5, “VF” has been corrected to read “sustained VA”.In Table 3, a missing subheading “Repeat ablation procedure” was added above the row “No. patients”.Download figureDownload PowerPointThese corrections have been made to the current version of the article, which is available at http://circep.ahajournals.org/content/9/5/e004005.full. Previous Back to top Next FiguresReferencesRelatedDetailsRelated articlesSites of Successful Ventricular Fibrillation Ablation in Bileaflet Mitral Valve Prolapse SyndromeFaisal F. Syed, et al. Circulation: Arrhythmia and Electrophysiology. 2016;9 June 2016Vol 9, Issue 6 Advertisement Article InformationMetrics © 2016 American Heart Association, Inc.https://doi.org/10.1161/HAE.0000000000000018PMID: 27255964 Originally publishedJune 2, 2016 PDF download Advertisement