Cases Of Mitral Valve Prolapse Research Articles

Arrhythmogenic Mitral Valve Prolapse Revisited: A Not Uncommon Cause of Youthful Sudden Death in Athletes and Women

BackgroundSudden deaths (SDs) in young people, including competitive athletes, albeit uncommon, are usually attributable to genetic, congenital or acquired cardiovascular conditions. However, it is under-appreciated that mitral valve prolapse (MVP), a relatively common valvular heart disease, is associated with SD in this youthful population. MethodsForty-three MVP-related SDs were identified from 2 large cardiovascular registries with pathologic, clinical, and demographic findings reported. ResultsEvents occurred in both sexes, but females were unexpectedly common (49%); median age was 22 ± 8 years, and 29 (67%) were engaged in competitive sports, including 17 with preparticipation examination. Of the 43 MVP cases, 21 died suddenly during or just after vigorous exercise, including 6 during organized sports. Sixteen (37%) had been evaluated by a cardiologist, resulting in confirmed MVP diagnosis in 11. Pathologic findings characteristic of MVP included bileaflet myxomatous involvement (in all cases) and areas of interstitial or replacement myocardial fibrosis (in 79%), most evident in posterolateral left ventricular wall. ConclusionsArrhythmogenic myxomatous degeneration (MVP) is an under-recognized cause of SD in young people, including competitive athletes, disproportionally affecting females and requires a high index of clinical suspicion. Frequency of left ventricular fibrosis in these young people with MVP suggests a mechanism for ventricular tachyarrhythmias and SD, relevant to future risk stratification.

Insights into malignant mitral valve degenerative disease from a sudden cardiac death cohort highlighting significant measurement differences from normal.

Mitral valve prolapse (MVP) is an accepted cause of sudden cardiac death (SCD) in most autopsy series. Diagnosis at autopsy relies upon subjective assessment with no established objective pathological criteria. This study set out to establish objective measurements to help pathologists dealing with SCD. We diagnosed 120 (1.5%) cases of MVP in 8108 cases of SCD. We measured the mitral annulus, anterior and posterior leaflets, rough zone and mitral annular disjunction (MAD) in 27 MVP cases and compared them to 54 age- and sex-matched normal mitral valves. Age of death was 39 ± 16 years, with 59 females and 61 males. History of mild MV disease was present in 19 (16%). Eleven (9%) died associated with exertion. Left ventricular hypertrophy was present in nine (15%) females and 10 (16%) males. Both MV leaflets showed thickening and ballooning in all individuals. MVP showed highly significantly increased annular circumference, elongation and thickening of both leaflets as well as increased MAD (all P < 0.001). Left ventricular fibrosis was present in 108 (90%), with interstitial fibrosis in the posterolateral wall and papillary muscle in 88 (81%) and coexisting replacement fibrosis in 40 (37%). This is the largest MVP associated with SCD series highlighting a young cohort with equal representation of males and females. There is involvement of both leaflets with significant annular dilatation, elongation and thickening of both leaflets with MAD. Left ventricular fibrosis explains arrhythmia. Our quantitative measurements should serve as a reference for pathologists assessing post-mortem hearts for MVP.

SCN5A variants as genetic arrhythmias triggers for familial bileaflet mitral valve prolapse

Mitral valve prolapse (MVP) is a common valvulopathy with variable outcomes. Several studies reported MVP as an underestimated cause of life-threatening arrhythmias and sudden cardiac death (SCD), mostly in young adult women. The association of MVP with ventricular arrhythmias as a substrate of SCD has been documented in patients with MVP. Genetic variations in DCHS1, DZIP1, FLNA and PLD1 genes have been associated with MVP. The index case, a 42-year-old woman, was diagnosed with a bileaflet myxomatous MVP after a resuscitated SCD at the age of 27 years. MVP was the only abnormality found to explain the occurrence of life-threatening ventricular arrhythmia. Structural cardiac diseases and Brugada syndrome were excluded. Family screening revealed one symptomatic daughter with a history of two syncopes at 11 years old during physical effort. Her echocardiography showed a bileaflet MVP and a mildly dilated left ventricle. The MVP is complicated by moderate mitral regurgitation (MR). The oldest sister, 23 years old is currently asymptomatic. Her echocardiography revealed a prodromal MVP with a mild holosystolic MR. The father had normal echocardiography. The aim of this study was to identify the likely causative genetic etiology of the familial MVP and particularly the arrhythmias substrate linked to this phenotype. Whole exome sequencing (WES) was performed for the family. WES data were analyzed with a primary focus on genes related to MVP but also on genes implicated in valvular and aortic defects. Using family-based exome sequencing, no variants were found in known MVP genes. However, we identified two missense variants in the SCN5A gene. A rare variant SCN5A: p.Ala572Asp and the functional modifying SCN5A:p.His558Arg polymorphism. Both variants are shared between the mother and her daughter with a history of resuscitated SCD and syncopes, respectively. The second daughter with prodromal MVP as well as her healthy father carried only the SCN5A:p.His558Arg polymorphism. Our study is highly suggestive of the contribution of SCN5A mutations as a potential genetic cause of the electric instability leading to ventricular arrhythmias in familial MVP cases with syncope and/or SCD history.

SCN5A Variants as Genetic Arrhythmias Triggers for Familial Bileaflet Mitral Valve Prolapse.

Mitral valve prolapse (MVP) is a common valvular heart defect with variable outcomes. Several studies reported MVP as an underestimated cause of life-threatening arrhythmias and sudden cardiac death (SCD), mostly in young adult women. Herein, we report a clinical and genetic investigation of a family with bileaflet MVP and a history of syncopes and resuscitated sudden cardiac death. Using family based whole exome sequencing, we identified two missense variants in the SCN5A gene. A rare variant SCN5A:p.Ala572Asp and the well-known functional SCN5A:p.His558Arg polymorphism. Both variants are shared between the mother and her daughter with a history of resuscitated SCD and syncopes, respectively. The second daughter with prodromal MVP as well as her healthy father and sister carried only the SCN5A:p.His558Arg polymorphism. Our study is highly suggestive of the contribution of SCN5A mutations as the potential genetic cause of the electric instability leading to ventricular arrhythmias in familial MVP cases with syncope and/or SCD history.

Case of Mitral Valve Prolapse – Associated Sudden Cardiac Death in Pregnancy

BACKGROUND Mitral valve prolapse (MVP) is a frequent echocardiographic finding that can be accompanied by symptoms ranging from a benign course to occasionally catastrophic complications, such as heart failure, and rarely, sudden cardiac death. Female sex, younger age, physiological or psychological stress, electrical instability, and changes in the structure of the mitral apparatus all seem to be risk factors for fatal ventricular arrhythmias in patients with MVP. We report a case of MVP-related cardiac arrest in a pregnant woman, which is rarely reported. CASE REPORT A 34-year-old woman who had collapsed at home from cardiac arrest was transported to the hospital. She had no history of cardiac diseases and was 8 weeks pregnant. Premature ventricular complexes and sinus tachycardia were observed on the 12-lead electrocardiogram as she arrived at the Emergency Department. The second cardiac arrest she experienced while in the hospital was observed to be from torsades de pointes. Further investigations revealed severe mitral valve regurgitation due to posterior leaflet prolapse and regional hypokinesis of the inferior wall and interventricular septum. CONCLUSIONS Ventricular arrhythmia is a frequent finding of mitral valve regurgitation. However, it rarely results in serious consequences. Malignant arrhythmic mitral valve regurgitation can result in sudden cardiac death; therefore, physicians need to be aware of patients with MVP who exhibit characteristics of a potential high-risk profile in order to avoid tragic outcomes.

Cryoablation of Papillary Muscles at Surgery for Malignant Ventricular Arrhythmias Due to Mitral Valve Prolapse.

Mitral valve prolapse (MVP) is relatively common condition and while generally benign a small subset of patient suffers from malignant ventricular arrhythmias (MVA) and sudden cardiac death (SCD). We report three cases of mitral valve prolapse, mitral regurgitation and malignant ventricular arrhythmias refractory to medical therapy, who had surgical cryoablation at the time of surgery on the mitral valve. During a follow-up period ranging from 3 to 11 years all three patients have remained free of ventricular arrhythmias and cryoablation lesions targeting the base of the papillary muscles have not caused any detrimental effect on the valve function. Surgical cryoablation of papillary muscles as described in this article should be considered in MVP who suffer from MVA, aborted SCD or frequent ventricular ectopics likely to cause LV dysfunction.

Genome-Wide Association Meta-Analysis Supports Genes Involved in Valve and Cardiac Development to Associate With Mitral Valve Prolapse.

Mitral valve prolapse (MVP) is a common cardiac valve disease, which affects 1 in 40 in the general population. Previous genome-wide association study has identified 6 risk loci for MVP. But these loci explained only partially the genetic risk for MVP. We aim to identify additional risk loci for MVP by adding data set from the UK Biobank. We also incorporated 434 MVP cases and 4527 controls from the UK Biobank for discovery analyses. Genetic association was conducted using SNPTEST and meta-analyses using METAL. We used Functional Mapping and Annotation of Genome-Wide Association Studies for post-genome-wide association study annotations and Multi-marker Analysis of GenoMic Annotation for gene-based and gene-set analyses. We found Trans-Omics for Precision Medicine imputation to perform better in terms of accuracy in the lower ranges of minor allele frequency below 0.1. Our updated meta-analysis included UK Biobank study for ≈8 million common single-nucleotide polymorphisms (minor allele frequency >0.01) and replicated the association on Chr2 as the top association signal near TNS1. We identified an additional risk locus on Chr1 (SYT2) and 2 suggestive risk loci on chr8 (MSRA) and chr19 (FBXO46), all driven by common variants. Gene-based association using Multi-marker Analysis of GenoMic Annotation revealed 6 risk genes for MVP with pronounced expression levels in cardiovascular tissues, especially the heart and globally part of enriched GO terms related to cardiac development. We report an updated meta-analysis genome-wide association study for MVP using dense imputation coverage and an improved case-control sample. We describe several loci and genes with MVP spanning biological mechanisms highly relevant to MVP, especially during valve and heart development.

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?

Cardiovascular abnormalities in patients with oral cleft: a clinical-electrocardiographic-echocardiographic study

OBJECTIVES:The present study aims to describe the clinical, electrocardiographic, and echocardiographic cardiological findings in a group of patients with oral clefts.METHODS:This is a prospective cross-sectional study on 70 children (age range from 13 days to 19 years) with oral clefts who attended the multidisciplinary program of a university hospital from March 2013 to September 2014. The patients were evaluated by a pediatric cardiologist and underwent detailed anamnesis, physical examination, electrocardiogram, and echocardiogram.RESULTS:Sixty percent of the patients were male; 55.7% presented with cleft lip and palate, and 40.0% presented with health complaints. Comorbidities were found in 44.3%. Relevant pregnancy, neonatal, family and personal antecedents were present in 55.7%, 27.1%, 67.2%, and 24.3% of the patients, respectively. Regarding the antecedents, 15.2% of the patients presented with a cardiac murmur, 49.0% with a familial risk of developing plurimetabolic syndrome, and 6% with family antecedents of rheumatic fever. Electrocardiographic evaluation showed one case of atrioventricular block. Echocardiograms were abnormal in 35.7% of the exams, including 5 cases of mitral valve prolapse — one of which was diagnosed with rheumatic heart disease.CONCLUSION:The finding of a family risk of developing plurimetabolic syndrome and a diagnosis of rheumatic heart disease indicates that patients with oral clefts may be more prone to developing acquired heart disease. Thus, our findings highlight the importance of anamnesis and methodological triangulation (clinical-electrocardiographic-echocardiographic) in the investigation of patients with oral clefts and emphasize that cardiological follow-up to evaluate acquired and/or rhythm heart diseases is necessary. This strategy permits comorbidity prevention and individualized planned treatment.

Mitral Valve Prolapse Classification from an Echocardiography Sequence using Coherent Point Drift Method based on Fractal Dimension

Background: Mitral Valve Prolapse (MVP) is now recognized as the most common cardiovalvular abnormality (CVA) in the USA, but there has been much less agreement about other aspects of its epidemiology, diagnosis and clinical features. CVA analysis still relies mainly on the visual assessment of echocardiographic imaging and manual measurements by experienced cardiologists. So, automating or semi-automating analysis of echocardiographic images for MVP detection is, therefore, highly desirable, but also challenging due to low image quality and high amount of speckle noise. Objective: The main objective of this paper was to test the hypothesis that fractal dimension (FD) values are significantly higher in severe MVP and moderate MVP cases than those of healthy subjects in motion pattern of their mitral valves. Materials and Methods: In this paper, we present a noninvasive algorithm that does not require any segmentation. This approach has three steps: first, we extracted corners as landmarks from echocardiographic sequences based on SUSAN corner detector. Then, landmarks were tracked using Coherent Point Drift (CPD) non-rigid registration. After that, the motion patterns of the landmarks were analyzed to extract features (FD) in order to classify the mitral valve behavior of 46 patients and 25 normals as normal, moderate or severe MVP. Results: The results show that moderate MVP detection has a relatively good sensitivity (82%) and specificity (82%). Conclusion: The motion pattern of landmarks in MVP patients, obtained by CPD method, differed from that of normals in such a way that the difference can be quantified and analyzed by FD.

23. Epidemiological aspects and clinical outcomes of mitral valve prolapse in Saudi adults over a 10 year period

Mitral valve prolapse (MVP) is a well recognized clinical entity that is associated with significant morbidity. Epidemiology, echocardiographic (echo) characteristics and clinical outcome of MVP in Saudi Arabia have not been studied. To determine the prevalence, echo features and clinical outcome of MVP among the adult Saudi patients who underwent echo evaluation over a 10-year period.Retrospective review of consecutive cases of MVP or any of its components as diagnose by echocardiogram. Study was conducted in King Abdulaziz Cardiac Center, Riyadh and included 121,419 adult echo studies done between January 2003 and December 2012. Study population consisted of 77,176 patients after removing duplicate studies. Echo parameters for all Saudi nationals ⩾14 y of age were collected from the Xcelera database. Mitral valve disease due to non-myxomatous prolapse were excluded. Among the study population ( n = 77,176) 600 patients were labled as having MVP or any of its echo features (0.7%). Mean age was 64 years and 62% were males. Majority of patients (54.4%) had mild MVP, while moderate and severe prolapse were present in 21.1% and 24.5% respectively. Severe mitral regurgitation was present in 16.5% and chordal rupture was noted in 9%. Left ventricular size was moderately dilated in 7.6% and severely dilated in 1.3%.Prevalence of MVP in Saudi nationals at a referral cardiac center is less than the reported international figure of 1-3%. In contrary to published literature MVP in Saudi population seems to be more common in males and seems to be diagnosed at a later age.

Genetic association analyses highlight biological pathways underlying mitral valve prolapse.

Non-syndromic mitral valve prolapse (MVP) is a common degenerative cardiac valvulopathy of unknown aetiology that predisposes to mitral regurgitation, heart failure and sudden death1. Previous family and pathophysiological studies suggest a complex pattern of inheritance2–5. We performed a meta-analysis of two genome-wide association studies in 1,442 cases and 2,439 controls. We identified and replicated in 1,422 cases and 6,779 controls six loci and provide functional evidence for candidate genes. We highlight LMCD1 encoding a transcription factor6, for which morpholino knockdown in zebrafish results in atrioventricular (AV) valve regurgitation. A similar zebrafish phenotype was obtained for tensin1 (TNS1), a focal adhesion protein involved in cytoskeleton organization. We also show the expression of tensin1 during valve morphogenesis and describe enlarged posterior mitral leaflets in Tns1−/− mice. This study identifies the first risk loci for MVP and suggests new mechanisms involved in mitral valve regurgitation, the most common indication for mitral valve repair7.

産科大出血に対する大量輸血後の肺うっ血を契機に診断された僧帽弁逸脱症の1例

産科大出血に対する大量輸血後の肺うっ血を契機に診断された僧帽弁逸脱症の1例

Abstract 18977: Non-Syndromic Mitral Valve Prolapse From Gene Mutations to Modifiable Mechanisms

Mitral valve prolapse (MVP) is a common disease affecting nearly 1 in 40 individuals. It is the leading surgical indication for mitral valve regurgitation. Despite a clear heritable component, the genetic etiology of non-syndromic MVP has remained elusive. Individuals from a large multigenerational family segregating non-syndromic MVP underwent capture sequencing of a previously linked interval on chromosome 11. We found a novel missense mutation in DCHS1, the human homologue of the Drosophila cell polarity gene dachsous (ds) that segregates with MVP in the family. The familial mutation reduced protein stability in zebrafish and cultured cells. Morpholino knockdown of zebrafish dachsous1b yielded a regurgitant atrioventricular canal defect that could be rescued by wild-type human DCHS1 but not by mRNA with the familial mutation. Adult haploinsufficient Dchs1 mice had anatomic and functional prolapse of thickened, elongated leaflets recapitulating the human MVP phenotype. Dchs1 haploinsufficiency was associated with developmental defects in valve shape, cell migration and patterning during fetal morphogenesis, suggesting cell polarity as a previously unrecognized facet of valve morphogenesis. Studies of downstream changes identified increased MAP kinase activities and α-SMA+ cells (myofibroblasts) in the valves preceding myxomatous degeneration; these suggest potential future directions for blunting disease progression. Additional genetic studies identified a second family with a DCHS1 mutation and a significantly increased burden of novel DCHS1 mutations in sporadic MVP cases Interdisciplinary studies strongly support DCHS1 as the first identified disease-causing gene for non-syndromic MVP and suggest potential mechanisms for modifying disease natural history.

Mitral valve repair is an effective treatment for ventricular arrhythmias in mitral valve prolapse syndrome

The prognosis of ventricular arrhythmias associated with mitral valve prolapse (MVP) syndrome is unclear. We present a case of MVP that emphasizes the role of mitral valve repair in the treatment of ventricular arrhythmias resulting from this condition. The case also illustrates the need for high vigilance in those with malignant features of MVP, to prevent sudden cardiac death.

Screening of TGFBR1, TGFBR2, and FLNA in familial mitral valve prolapse

So far only mutations in the filamin A gene (FLNA) have been identified as causing familial mitral valve prolapse (MVP). Previous studies have linked dysregulation of the transforming growth factor beta (TGF-β) cytokine family to MVP. We investigated whether mutations in the TGF-β receptors genes type I (TGFBR1) and II (TGFBR2) underlie isolated familial MVP cases. Eight families with isolated familial MVP were evaluated clinically and genetically. Ventricular arrhythmias were present in five of the eight families and sudden cardiac death occurred in six patients. Tissue obtained during mitral valve surgery or autopsy was available for histological examination in six cases; all demonstrated myxomatous degeneration. A previously described FLNA missense mutation (p.G288R) was identified in one large family, but no mutations were discovered in TGFBR1 or TGFBR2. An FLNA missense mutation was identified in one family but we found no TGFBR1 or TGFBR2 mutations. Our results suggest that TGFBR1 and TGFBR2 mutations do not play a major role in isolated myxomatous valve dystrophy. Screening for FLNA mutations is recommended in familial myxomatous valvular dystrophy, particularly if X-linked inheritance is suspected.

A New Definition for an Old Entity: Improved Definition of Mitral Valve Prolapse Using Three-Dimensional Echocardiography and Color-Coded Parametric Models

Differentiating between mitral valve (MV) prolapse (MVP) and MV billowing (MVB) on two-dimensional echocardiography is challenging. The aim of this study was to test the hypothesis that color-coded models of maximal leaflet displacement from the annular plane into the atrium derived from three-dimensional transesophageal echocardiography would allow discrimination between these lesions. Three-dimensional transesophageal echocardiographic imaging of the MV was performed in 50 patients with (n=38) and without (n=12) degenerative MV disease. Definitive diagnosis of MVP versus MVB was made using inspection of dynamic three-dimensional renderings and multiple two-dimensional cut planes extracted from three-dimensional data sets. This was used as a reference standard to test an alternative approach, wherein the color-coded parametric models were inspected for integrity of the coaptation line and location of the maximally displaced portion of the leaflet. Diagnostic interpretations of these models by two independent readers were compared with the reference standard. In all cases of MVP, the color-coded models depicted loss of integrity of the coaptation line and maximal leaflet displacement extending to the coaptation line. MVB was depicted by preserved leaflet apposition with maximal displacement away from the coaptation line. Interpretation of the 50 color-coded models by novice readers took 5 to 10 min and resulted in good agreement with the reference technique (κ=0.81 and κ=0.73 for the two readers). Three-dimensional color-coded models provide a static display of MV leaflet displacement, allowing differentiation between MVP and MVB, without the need to inspect multiple planes and while taking into account the saddle shape of the mitral annulus.

Degree of correlation between cardiac auscultatory and echocardiographic findings among young athletes

Purpose: The goal of our study was to find an association between auscultatory and echocardiographic findings and to determine diagnostic value of each of these methods. Methods: 186 soccer players (13 to 16 years of age) underwent pre-participation screening, including physical examination and echocardiography with color doppler. Results: The study revealed that 17.1% of all investigated persons had neither auscultatory nor echocardiographic abnormalities. Simultaneous auscultatory and echocardiographic changes were observed in 43.8% of all investigated athletes (including systolic murmurs, accentuation of second heart sound, mitral regurgitation, mitral valve prolapse, tricuspid regurgitation and etc). Only auscultatory changes, not visible on echocardiographic examination, were observed in 30.1% of athletes. 8.9% of athletes had only echocardiographic changes, including two cases of mitral valve prolapse and regurgitation, requiring further assessment and observation. Conclusion: Obtained results demonstrate considerably high frequency of auscultatory and echocardiographic changes among young athletes. Furthermore, rate of discordance between the findings obtained by two different methods is almost as high (39.0%), as rate of concordance between them (43.8%). Therefore, we suggest that it is desirable to conduct echocardiographic examination along with physical examination of athletes, in order to exclude possibility of overlooking relatively severe pathologies on one hand, and to avoid false positive diagnosis of valvular disease, on the other hand.

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

Purpose: Ventricular arrhythmias are known to be common in symptomatic patients with Mitral Valve Prolapse (MVP). The aim of our study was to investigate the prevalence, echocardiographic and biochemical predictors, and possible mechanisms of ventricular arrhythmias in young asymptomatic patients with MVP without significant mitral regurgitation. Methods: We studied 78 asymptomatic young subjects (mean age 19.7±1.6, 72% male) in sinus rhythm with MVP in comparison with 80 sex- and age-matched healthy subjects. Transthoracic echocardiography (Vivid 7 Dimension, GE) and 24-hour Holter monitoring were performed. MVP was diagnosed by billowing of 1 or both mitral leaflets >2 mm above the mitral annulus in the long-axis parasternal view. Longitudinal strain were determined from three apical views, using spackle tracking (EchoPAC'08, GE) with grey-scale frame rate 50-55/sec. Concentration of Monocyte Chemoattractant Protein-1 (MCP-1) in serum were determined by enzyme-linked immunosorbent assay. Results: The frequency of isolated Ventricular Premature Contractions (VPCs) was similar in both groups (32.4% and 33.3%; χ2=1.2, p=0.90). Ventricular tachycardias were not found in either MVP nor in control group. However, the ventricular couplets were recorded only in patients with MVP (in 8.1% of MVP group; χ2=5.2, p=0.01). The presence of couplets negatively correlated with Left Ventricular (LV) ejection fraction (r=–0.66; p=0.0001), with a global LV longitudinal strain (r=–0.78; p<0.0001), but positively correlated with the concentration of profibrotic chemokine MCP-1 (r=0.56; p=0.008). In patients with couplets more often were revealed local wall motion abnormalities (z=–2.0, p=0.04). Based on logistic regression analysis for MVP cases, in the case of ventricular couplets, the longitudinal strain (p=0.001) and MCP-1 serum level (p=0.01) were the independent predictors of couplets. Conclusion: In young asymptomatic patients with MVP more often than in the healthy population recorded ventricular couplets, the origin of which may be associated with impaired LV contractility, probably due to fibrosis of the myocardium.

English

Mitral valve prolapse (MVP) is a common valvular cardiac abnormality in the general population and is very rarely associated with Wolf Parkinson White (WPW) syndrome. These patients are prone for life threatening arrhythmias. Anaesthetics tend to change the electrophysiology of the atrio-ventricular conduction system and hence they tend to affect the behavior of the patient under anaesthesia. We present a case of mitral valve prolapse with WPW syndrome who underwent successful general anaesthesia for open nephrolithotomy.