Search

Background: Mitral annular disjunction (MAD), posterior displacement of the mitral valve leaflet hinge point, predisposes to arrhythmias or sudden cardiac death. We evaluated the burden of MAD, mitral valve prolapse (MVP), and mitral regurgitation (MR) by heritable thoracic aortic disease gene in a cross-sectional analysis of 2014-2023 data in the Montalcino Aortic Consortium registry., Methods and Results: MAD was determined by direct measurement of echocardiographic images. MR and MVP were defined according to current clinical guidelines. Associations were evaluated using χ 2 or Fisher exact tests. MR and MVP were enriched in Montalcino Aortic Consortium participants (672) with pathogenic variants (PV) in transforming growth factor-β pathway genes. The combination of MR and MVP was associated with mitral surgery and arrhythmias. In the subgroup with available images, MAD was enriched in SMAD3 PV compared with other transforming growth factor-β PV (prevalence ratio 1.8 [1.1-2.8], P <0.02). Severe disjunction (>10 mm) was only observed in the transforming growth factor-β subgroup and was further enriched in participants with SMAD3 PV (prevalence ratio 3.1 [1.1-8.6]). MVP (prevalence ratio 5.2 [3.0-9.0]) and MR (PR 2.7 [1.8-3.9]) were increased in participants with MAD, but MAD was not independently associated with adverse cardiac or aortic events., Conclusions: Pathological mitral valve phenotypes are more prevalent in individuals with PV in transforming growth factor-β pathway genes, particularly SMAD3 . MR and MVP but not MAD are associated with adverse aortic and cardiac events. Because congenital mitral disease may be the primary presenting feature of SMAD3 PV, genetic testing for heritable thoracic aortic disease should be considered for such individuals, especially if they also have a family history of heritable thoracic aortic disease.

Background: Mitral annular disjunction (MAD) is associated with ventricular arrhythmia in mitral valve prolapse (MVP). The proportional risk from MAD and other predictors of ventricular arrhythmia in MVP has not been well characterized., Objective: This study aimed to identify predictors of complex or frequent ventricular ectopy (cfVE) in MVP and to quantify risk of cfVE and mortality in MVP with MAD., Methods: We studied 632 adult patients with MVP on transthoracic echocardiography at the University of North Carolina Medical Center from 2016 to 2019 (median age, 64 [interquartile range, 52-74] years; 52.7% female; 16.3% African American). Resting and ambulatory electrocardiograms were used to identify cfVE., Results: MAD was present in 94 (14.9%) patients. Independent associations of MAD were bileaflet prolapse (odds ratio [95% CI], 4.25 [2.47-7.33]; P < .0001), myxomatous valve (2.17 [1.27-3.71]; P = .005), absence of hypertension (2.00 [1.21-3.32]; P = .007), electrocardiogram inferior or lateral lead T-wave inversion (2.07 [1.23-3.48]; P = .006), and female sex (1.99 [1.21-3.25]; P = .006). cfVE was frequent with MAD (39 [41.5%] vs 93 [17.3%] without; P < .0001). Independent cfVE predictors were MAD (hazard ratio [95% CI], 2.23 [1.47-3.36]; P = .0001), bileaflet prolapse (1.86 [1.25-2.76]; P = .002), heart failure (1.79 [1.16-2.77]; P = .009), lower left ventricular ejection fraction (0.14 [0.03-0.61]; P = .009), coronary artery disease (1.60 [1.05-2.43]; P = .03), and inferior or lateral lead T-wave inversion (1.51 [1.03-2.22]; P = .03). After a median of 40 (33-48) months, there was increased mortality with MAD (P = .04)., Conclusion: MAD in MVP is associated with bileaflet or myxomatous MVP, absence of hypertension, T-wave inversion, and female sex. There is increased cfVE and mortality with MAD, highlighting the need for closer follow-up of these patients., Competing Interests: Disclosures The authors have no conflicts of interest to disclose., (Copyright © 2024 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA syndrome) is a rare congenital coronary abnormality carrying a poor prognosis. We present a clinical case series of ALCAPA-related anterior mitral valve leaflet prolapse and discuss its mechanism as a key to suspicion of ALCAPA syndrome., Competing Interests: Funding Support and Author Disclosures The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

Introduction: Malignant ventricular arrhythmia (VA) and sudden cardiac death (SCD) have been reported in patients with mitral valve prolapse (MVP); however, effective risk stratification methods are still lacking. Myocardial fibrosis is thought to play an important role in the development of VA; however, observational studies have produced contradictory findings regarding the relationship between VA and late gadolinium enhancement (LGE) in MVP patients. The aim of this meta-analysis and systematic review of observational studies was to investigate the association between left ventricular LGE and VA in patients with MVP., Methods: We searched the PubMed, Embase, and Web of Science databases from 1993 to 2023 to identify case-control, cross-sectional, and cohort studies that compared the incidence of VA in patients with MVP who had left ventricular LGE and those without left ventricular LGE., Results: A total of 1464 subjects with MVP from 12 observational studies met the eligibility criteria. Among them, VA episodes were reported in 221 individuals (15.1%). Meta-analysis demonstrated that the presence of left ventricular LGE was significantly associated with an increased risk of VA (pooled risk ratio 2.96, 95% CI: 2.26-3.88, p for heterogeneity = 0.07, I 2  = 40%). However, a meta-regression analysis of the prevalence of mitral regurgitation (MR) showed that the severity of MR did not significantly affect the association between the occurrence of LGE and VA (p = 0.079)., Conclusion: The detection of LGE could be helpful for stratifying the risk of VA in patients with MVP., (© 2024 The Author(s). Clinical Cardiology published by Wiley Periodicals, LLC.)

Purpose of Review: Disparities in mitral valve (MV) repair outcomes exist between men and women. This review highlights sex-specific differences in MV disease aetiology, diagnosis, as well as timing and type of intervention., Recent Findings: Females present with more complicated disease: anterior or bileaflet prolapse, leaflet dysplasia/thickening, mitral annular calcification, and mixed mitral lesions. The absence of indexed echocardiographic mitral regurgitation (MR) severity parameters contributes to delayed intervention in women, resulting in more severe symptom burden at time of surgery. The sequelae of chronic MR also necessitate concomitant procedures (e.g. tricuspid repair, arrhythmia surgery) at the time of mitral surgery. Complex MV pathology, greater patient acuity, and more complicated procedures collectively pose challenges to successful MV repair and postoperative recovery. As a consequence, women receive disproportionately more MV replacement than men. In-hospital mortality after MV repair is also greater in women than men. Long-term outcomes of MV repair are comparable after risk-adjustment for preoperative status; however, women experience a greater incidence of postoperative heart failure., Summary: To address the inequity in MV repair outcomes between sexes, indexed diagnostic measurements, diligent surveillance of asymptomatic MR, increased recruitment of women in large clinical trials, and mandatory reporting of sex-based subgroup analyses are recommended., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Introduction: Mitral valve prolapse (MVP) is a common clinical condition in the general population. A subgroup of patients with MVP may experience ventricular arrhythmias and sudden cardiac death ("arrhythmic mitral valve prolapse" [AMVP]) but how to stratify arrhythmic risk is still unclear. Our meta-analysis aims to identify predictive factors for arrhythmic risk in patients with MVP., Methods: We systematically searched Medline, Cochrane, Journals@Ovid, Scopus electronic databases for studies published up to December 28, 2022 and comparing AMVP and nonarrhythmic mitral valve prolapse (NAMVP) for what concerns history, electrocardiographic, echocardiographic and cardiac magnetic resonance features. The effect size was estimated using a random-effect model as odds ratio (OR) and mean difference (MD)., Results: A total of 10 studies enrolling 1715 patients were included. Late gadolinium enhancement (LGE) (OR: 16.67; p = .005), T-wave inversion (TWI) (OR: 2.63; p < .0001), bileaflet MVP (OR: 1.92; p < .0001) and mitral anulus disjunction (MAD) (OR: 2.60; p < .0001) were more represented among patients with AMVP than in NAMVP. Patients with AMVP were shown to have longer anterior mitral leaflet (AML) (MD: 2.63 mm; p < .0001), posterior mitral leaflet (MD: 2.96 mm; p < .0001), thicker AML (MD: 0.49 mm; p < .0001), longer MAD length (MD: 1.24 mm; p < .0001) and higher amount of LGE (MD: 1.41%; p < .0001) than NAMVP. AMVP showed increased mechanical dispersion (MD: 8.04 ms; 95% confidence interval: 5.13-10.96; p < .0001) compared with NAMVP., Conclusions: Our meta-analysis proved that LGE, TWI, bileaflet MVP, and MAD are predictive factors for arrhythmic risk in MVP patients., (© 2023 The Authors. Journal of Cardiovascular Electrophysiology published by Wiley Periodicals LLC.)

Aim      To evaluate the effect of mitral valve (MV) repair and replacement on the incidence of ventricular arrhythmias (VA) and to identify risk factors for the persistence of VA in patients with MV prolapse and severe mitral regurgitation (MR) during a mid-term follow-up.Material and methods  A single-site observational, prospective study successively enrolled 30 patients (mean age, 55.2±9.9 years, 60% men) who underwent MV repair or replacement for severe MR due to MV prolapse or chordal avulsion. Transthoracic echocardiography and Holter monitoring were performed in all patients before and annually after surgery. A pathomorphological study of MV fragments excised during surgery was performed.Results During the five-year follow-up period (144 person-years), one case of sudden cardiac death outside a health care facility was recorded. MR severity progressed in three patients after MV repair. The total number of all VAs decreased during the follow-up period, with a significant decrease in the number of paroxysms of unstable ventricular tachycardia during the first two years after surgery. The presence of VA in the postoperative period was correlated with the severity of postoperative left ventricular (LV) remodeling: end-diastolic volume (EDV) (rs=0.69; p=0.005), LV ejection fraction (EF) (rs = -0.55; p=0.004) and severity of MV myxomatous alterations according to histological study data (rτ=0.58; p=0.045). The beta-blocker treatment did not influence the VA frequency and severity (rs= -0.18; p=0.69). According to a univariate regression analysis only EDV (p = 0.001), LVEF &lt;50% (p = 0.003), and myxomatous MV degeneration (p = 0.02) were risk factors for persistent ventricular tachycardia in the postoperative period.Conclusion      Surgical intervention on MV in patients with MV prolapse and severe MR decreased the number of cases of malignant VAs and was correlated with the postoperative changes in LV volume and function, as well as the severity of MV myxomatous alterations.

Background: Recently, an expert consensus statement proposed indications where implantation of a primary prevention implantable cardioverter-defibrillator (ICD) may be reasonable in patients with mitral valve prolapse (MVP). The objective was to evaluate the proposed risk stratification by the expert consensus statement., Methods: Consecutive patients with MVP without alternative arrhythmic substrates with cardiac magnetic resonance imaging (CMR) were included in a single-center retrospective registry. Arrhythmic MVP (AMVP) was defined as a total premature ventricular complex burden ≥5%, non-sustained ventricular tachycardia (VT), VT, or ventricular fibrillation. The end point was a composite of SCD, VT, inducible VT, and appropriate ICD shocks., Results: In total, 169 patients (52.1% male, median age 51.4 years) were included and 99 (58.6%) were classified as AMVP. Multivariate logistic regression identified the presence of late gadolinium enhancement (OR 2.82, 95%CI 1.45-5.50) and mitral annular disjunction (OR 1.98, 95%CI 1.02-3.86) as only predictors of AMVP. According to the EHRA risk stratification, 5 patients with AMVP (5.1%) had a secondary prevention ICD indication, while in 69 patients (69.7%) the implantation of an ICD may be reasonable. During a median follow-up of 8.0 years (IQR 5.0-15.6), the incidence rate for the composite arrhythmic end point was 0.3%/year (95%CI 0.1-0.8)., Conclusion: More than half of MVP patients referred for CMR met the AMVP diagnostic criteria. Despite low long-term event rates, in 70% of patients with AMVP the implantation of an ICD may be reasonable. Risk stratification of SCD in MVP remains an important knowledge gap and requires urgent investigation., Competing Interests: Disclosures The authors have no disclosures related to this work., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Background: There is growing evidence that mitral valve prolapse (MVP) is associated with otherwise unexplained cardiac arrest (UCA). However, reports are hindered by the absence of a systematic ascertainment of alternative diagnoses., Objectives: This study reports the prevalence and characteristics of MVP in a large cohort of patients with UCA., Methods: Patients were enrolled following an UCA, defined as cardiac arrest with no coronary artery disease, preserved left ventricular ejection fraction, and no apparent explanation on electrocardiogram. A comprehensive evaluation was performed, and patients were diagnosed with idiopathic ventricular fibrillation (IVF) if no cause was found. Echocardiography reports were reviewed for MVP. Patients with MVP were divided into 2 groups: those with IVF (AMVP) and those with an alternative diagnosis (nonarrhythmic MVP). Patient characteristics were then compared. The long-term outcomes of AMVP were reported., Results: Among 571 with an initially UCA, 34 patients had MVP (6%). The prevalence of definite MVP was significantly higher in patients with IVF than those with an alternative diagnosis (24 of 366 [6.6%] vs 5 of 205 [2.4%]; P = 0.03). Bileaflet prolapse was significantly associated with AMVP (18 of 23 [78%] vs 1 of 8 [12.5%]; P = 0.001; OR: 25.2). The proportion of patients with AMVP who received appropriate implantable cardioverter-defibrillator therapies over a median follow-up of 42 months was 21.1% (4 of 19)., Conclusions: MVP is associated with otherwise UCA (IVF), with a prevalence of 6.6%. Bileaflet prolapse appears to be a feature of AMVP, although future studies need to ascertain its independent association. A significant proportion of patients with AMVP received appropriate implantable cardioverter-defibrillator therapies during follow-up., Competing Interests: Funding Support and Author Disclosures This study was supported by the Heart and Stroke Foundation of Canada (G-13-0002775 and G-14-0005732) and the Canadian Institute of Health Research (Dr Krahn, Principal Investigator, Hearts in Rhythm Organization RN380020–406814). Dr Krahn has received support from the Sauder Family and Heart and Stroke Foundation Chair in Cardiology (Vancouver, British Columbia), the Paul Brunes Chair in Heart Rhythm Disorders (Vancouver, British Columbia), and the Paul Albrechtsen Foundation (Winnipeg, Manitoba). The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2023 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Aims: Patients with mitral valve prolapse (MVP) have high risk of life-threatening ventricular arrhythmias (VAs). Data on the impact of exercise on arrhythmic risk in these patients are lacking. We explored whether lifetime exercise dose was associated with severe VA and with established risk factors in patients with MVP. Furthermore, we explored the circumstances at the VA event., Methods and Results: In this retrospective cohort study, we included patients with MVP and assessed lifetime exercise dose as metabolic equivalents of task (MET) hours/week. Severe VA was defined as sustained ventricular tachycardia or fibrillation, aborted cardiac arrest, and appropriate shock by a primary preventive implantable cardioverter defibrillator. We included 136 MVP patients (48 years [interquartile range (IQR) 35-59], 61% female), and 17 (13%) had previous severe VA. The lifetime exercise dose did not differ in patients with and without severe VA (17 MET h/week [IQR 9-27] vs. 14 MET h/week [IQR 6-31], P = 0.34). Lifetime exercise dose > 9.6 MET h/week was a borderline significant marker for severe VA (OR 3.38, 95% CI 0.92-12.40, P = 0.07), while not when adjusted for age (OR 2.63, 95% CI 0.66-10.56, P = 0.17). Ventricular arrhythmia events occurred most frequently during wakeful rest (53%), followed by exercise (29%) and sleep (12%)., Conclusion: We found no clear association between moderate lifetime exercise dose and severe VA in patients with MVP. We cannot exclude an upper threshold for safe levels of exercise. Further studies are needed to explore exercise and risk of severe VA., Competing Interests: Conflict of interest: None declared., (© The Author(s) 2023. Published by Oxford University Press on behalf of the European Society of Cardiology.)

Arrhythmic mitral valve prolapse (MVP) has gained great interest recently because of the increasing recognition of its potential role in unexplained cardiac arrest. Although evidence has accumulated to show the association of arrhythmic MVP (AMVP) with sudden cardiac death (SCD), risk stratification and management remain unclear. Physicians are faced with the challenges of screening for AMVP among MVP patients and the dilemma of when and how to intervene to prevent SCD in these patients. In addition, there is little guidance to help approach MVP patients who present with an otherwise unexplained cardiac arrest to know whether MVP was the primary cause of cardiac arrest or just an innocent bystander. Herein we review the epidemiology and definition of AMVP, the risk and mechanisms of SCD, and summarize the clinical evidence behind risk markers of SCD and therapeutic interventions that could potentially prevent it. We also propose an algorithm that provides guidance as to how to screen for AMVP and what therapeutic interventions to use. Last, we propose a diagnostic algorithm for approaching patients with otherwise unexplained cardiac arrest who are shown to have MVP., (Copyright © 2023 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

Acute severe mitral regurgitation (MR) is rare, but often leads to cardiogenic shock, pulmonary edema, or both. Most common causes of acute severe MR are chordae tendineae (CT) rupture, papillary muscle (PM) rupture, and infective endocarditis (IE). Mild to moderate MR is often seen in patients with acute myocardial infarction (AMI). CT rupture in patients with floppy mitral valve/mitral valve prolapse is the most common etiology of acute severe MR today. In IE, native or prosthetic valve damage can occur (leaflet perforation, ring detachment, other), as well as CT or PM rupture. Since the introduction of percutaneous revascularization in AMI, the incidence of PM rupture has substantially declined. In acute severe MR, the hemodynamic effects of the large regurgitant volume into the left atrium (LA) during left ventricular (LV) systole, and in turn back into the LV during diastole, are profound as the LV and LA have not had time to adapt to this additional volume. A rapid, but comprehensive evaluation of the patient with acute severe MR is essential in order to define the underline cause and apply appropriate management. Echocardiography with Doppler provides vital information related to the underlying pathology. Coronary arteriography should be performed in patients with an AMI to define coronary anatomy and need for revascularization. In acute severe MR, medical therapy should be used to stabilize the patient before intervention (surgery, transcatheter); mechanical support is often required. Diagnostic and therapeutic steps should be individualized, and a multi-disciplinary team approach should be utilized., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

We herein presented a 17-year-old female with history of mild mitral valve prolapse who was admitted for methicillin-sensitive Staphylococcus aureus endocarditis and diagnosed with mitral annular disjunction and perforated posterior mitral valve leaflet on two-dimensional and three-dimensional echocardiography (P 1 -P 2 ). A perforation in the posterior leaflet was confirmed and repaired during surgical intervention. This is a rare presentation of leaflet perforation in the area of mitral annular disjunction.

There is an increasing awareness on the association between mitral valve prolapse (MVP) and sudden cardiac death. Mitral annular disjunction (MAD) is a phenotypic risk feature that can help in risk stratification. We present a case of a 58-year-old woman who experienced an out-of-hospital cardiac arrest caused by ventricular fibrillation interrupted by a direct current shock. No coronary lesions were documented. Echocardiogram showed myxomatous MVP. Nonsustained ventricular tachycardia have been registered during hospital stay. Interestingly, cardiac magnetic resonance revealed MAD and a late gadolinium enhancement area in inferior wall. Finally, a defibrillator has been implanted. For arrhythmic risk stratification of MVP with MAD, multimodality imaging is the diagnostic tool to find out the disease behind many cardiac arrests of unknown cause.

Background: Myxomatous mitral valve degeneration (MMVD) is the most common degenerative heart disease in dogs and is associated with irreversible changes in the valve tissue. Although traditional cardiac biomarkers are efficient for diagnosing MMVD, there are limitations, therefore, it is important to find novel biomarkers. Cartilage intermediate layer protein 1 (CILP1), an extracellular matrix-derived protein, acts as a transforming growth factor-β antagonist and is involved in myocardial fibrosis. This study aimed to evaluate serum CILP1 levels in canines with MMVD. Dogs with MMVD were staged according to the American College of Veterinary Internal Medicine consensus guidelines. Data analysis was performed using the Mann-Whitney U test, Spearman's correlation, and receiver operating characteristic (ROC) curves., Results: CILP1 levels were elevated in dogs with MMVD (n = 27) compared to healthy controls (n = 8). Furthermore, results showed that CILP1 levels were significantly higher in stage C group dogs compared to healthy controls. The ROC curve of CILP1 and NT-proBNP were good predictors of MMVD, although no similarity was observed between the two. Left ventricular end-diastolic diameter normalized to the body weight (LVIDdn) and left atrial to aorta dimension (LA/Ao) showed a strong association with CILP1 levels; however, no correlation was observed between CILP1 levels and vertebral heart size (VHS) and vertebral left atrial score (VLAS). The optimal cut-off value was selected from the ROC curve and dogs were classified according to the cut-off value (1.068 ng/mL, sensitivity 51.9%, specificity 100%). Results showed a significant association of CILP1 with cardiac remodeling indicators, such as VHS, VLAS, LA/Ao, and LVIDdn., Conclusions: CILP1 can be an indicator of cardiac remodeling in canines with MMVD and therefore, can be used as an MMVD biomarker., (© 2023. The Author(s).)

Background: Pectus anomalies constitute 95% of chest anomalies. Pectus carinatum (PC) and excavatum (PE) are often asymptomatic in childhood. However, symptoms and signs such as chest pain, dyspnea, and mitral valve prolapse (MVP) can be seen in pectus anomalies. Demographic characteristics and accompanying cardiac signs in children with pectus deformity were investigated., Methods: In this study, the clinical findings for children with pectus deformity, and the incidence of MVP and other concomitant heart diseases detected in echocardiographic examinations were evaluated., Results: Eighty-two children with PE, 27 with PC, and 107 healthy children were included in this study. In the echocardiographic examination of PE, PC patients, and healthy children, MVP was detected with frequencies of 25%, 33%, and 2% respectively., Conclusions: The study showed that pectus anomalies were associated with an increased incidence of MVP. All patients with pectus deformity should therefore undergo a screening echocardiogram in adolescence to assess for the presence of MVP., (© 2023 Japan Pediatric Society.)

Sudden cardiac death is reported as the leading cause of mortality in developed nations. Arrhythmic mitral valve disease, encompassing mitral valve prolapse and/or mitral annular disjunction, is thought to be responsible in a sizable portion of these deaths. Despite this evidence, there are no reliable methods or clinically useful risk stratification schemes to determine which group of patients are at higher risk or may benefit from interventions such as catheter ablation or prophylactic implantation of a defibrillator. The reasons for this lack of guidance include our incomplete understanding of the mechanisms of ventricular arrhythmias and the fact that mitral valve prolapse and disjunction are frequently diagnosed, yet carry an overall low risk of sudden cardiac death. This heterogeneity makes the development of a reliable prediction model based on the presence of common risk factors very difficult. In this review, we summarize the relevant literature regarding the epidemiology, diagnosis, pathophysiology, and management of mitral valve prolapse and mitral annular disjunction and elucidate their role in sudden cardiac death., (Copyright © 2022 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Objectives: Barlow's mitral valve disease with late systolic mitral regurgitation provides diagnostic and therapeutic challenges. The mechanisms of the regurgitation are still unclear. We hypothesized that the onset and the severity of late systolic regurgitation are determined by annulus dynamics and the mechanical stresses imposed by the left ventricle., Methods: Ten patients with Barlow's mitral valve disease and mitral annulus disjunction (MAD) were compared with 10 healthy controls. Resting blood pressure was measured, and transthoracic three-dimensional echocardiography was analyzed using a holographic display that allows tracking and measurements of mitral annulus surface area (ASA) throughout the cardiac cycle. A novel annulus elastance index (dASA/dP) was calculated between aortic valve opening and onset of mitral regurgitation. Severity of MAD was quantified as the disjunction index (mm × degree). Leaflet coaptation area was calculated using a finite element model., Results: Peak systolic ASAs in controls and patients were 9.3 ± 0.6 and 21.1 ± 3.1 cm 2 , respectively (P < .001). In patients, the ASA increased rapidly during left ventricular ejection, and onset of mitral regurgitation coincided closely with peak upslope of annulus area change (dASA/dt). The finite element model showed a close association between rapid annulus displacement and coaptation area deficit in Barlow's mitral valve disease. Systolic annulus elastance index (0.058 ± 0.036 cm 2 /mm Hg) correlated strongly with disjunction index (r = 0.91, P < .0001). Moreover, regurgitation volume showed a positive correlation with systolic blood pressure (r = 0.80, P < .01)., Conclusion: The present pilot study supports the hypothesis that annulus dilatation may accentuate mitral valve regurgitation in patients with Barlow's mitral valve disease. A novel annulus elastance index may predict the severity of mitral valve regurgitation in selected patients., (Copyright © 2022 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.)

Objective: Mitral valve prolapse (MVP) is the most common cause of mitral regurgitation in developed countries. The role of inflammation in the pathogenesis of MVP is still not clear. In this study, we aimed to investigate how inflammatory markers such as monocyte/high-density lipoprotein ratio (MHR), lymphocyte/monocyte ratio (LMR), neutrophil/lymphocyte ratio (NLR), and platelet/neutrophil ratio (PLR) are affected in MVP patients., Methods: In this retrospective study, we included 461 patients with MVP and 459 normal echocardiographic patients, matched with gender and age. Inflammatory markers and all variables were compared between the two groups., Results: There were no statistically significant differences in age, sex, or body mass index between the two groups. Neutrophil counts (4,960 [3,900-6,780]. 4,200 [3,800-5,600], p < 0.001), NLR (2.488 [1.72-4.51], 1.857 [1.49-2.38], p < 0.001), MHR (14.9 [11.9-18.6], 12.2 [9.4-17.3], p = 0.003), PLR (122.4 [85-171], 104.4 [85-130], p < 0.001), and CRP (0.71 ± 0.50, 0.67 ± 0.33 p < 0.001) were significantly higher, and LMR (3.75 [2.75-5.09], 4.06 [3.12-4.83] p = 0.016) was significantly lower in the MVP group than the control group, respectively. In logistic regression analysis, NLR (odds ratio [OR]: 1.058 [1.047-1.072]; p < 0.001), LMR (OR: 1.560 [1.211-2.522]; p = 0.027), and PLR (OR: 1.015 [1.012-1.019]; p = 0.003) were found to be independent predictors for MVP presence., Conclusions: These parameters can be used as a simple, low-cost, practical tool to detect inflammation in MVP., (Copyright: © 2022 Permanyer.)

Mitral valve prolapse (MVP) is the most common primary valvular abnormality, associated with various degrees of incompetent function and sequelae, including heart failure and sudden cardiac death. Recent improvements in echocardiographic techniques and new insights into mitral valve anatomy and physiology have rendered the diagnosis of this condition more accurate and reliable. Here we review the genetic etiology, clinical significance, diagnosis, and treatment options for MVP patients.

Mitral valve prolapse (MVP) belongs to cardiac disorders characterized by impaired closure of mitral leaflets. We studied adolescent group of patients with MVP suffering from symptomatology that cannot be explained by mitral regurgitation alone. Several studies suggested that symptoms can be explained by autonomic, in particular sympathetic-linked dysfunction. Thus, we assessed non-invasive sympathetic indices of blood pressure and heart rate variability and electrodermal activity (EDA). Fifty-three adolescents with MVP (age: 15.1+/-0.4 years) and 43 healthy age- and gender-matched adolescents (age: 14.9+/-0.4 years) were examined. Blood pressure, heart rate and EDA were continuously recorded during 6-min rest. Evaluated parameters were: low frequency band of systolic blood pressure variability, systolic, diastolic and mean blood pressure, mean RR interval, cardiac sympathetic indices: symbolic dynamics (0V%), left ventricular ejection time (LVET), pre-ejection period (PEP), and EDA. Our findings revealed significantly higher systolic, diastolic, and mean blood pressure values, shortened mean RR interval, increased 0V%, and shortened LVET in MVP patients vs. controls (p=0.028, p<0.001, p=0.002, p<0.001, p=0.050, p<0.001; respectively). Our study revealed enhanced cardiovascular sympathetic regulation in adolescent MVP patients. We suggest that evaluation of non-invasive sympathetic parameters could represent potential biomarkers for early diagnosis of cardiovascular complications associated with MVP already at adolescent age.

Objectives: Barlow´s disease represents a wide spectrum of mitral valve pathologies associated with regurgitation (MR), excess leaflet tissue, and prolapse. Repair strategies range from complex repairs with annuloplasty plus neochords through resection to annuloplasty-only. The latter requires symmetric prolapse patterns and central regurgitant jets. We aimed to assess repair success and durability, survival, and intraoperative outcomes with symmetric and asymmetric Barlow's disease., Methods: Between 09/10 and 03/20, 103 patients (of 1939 with mitral valve surgery) presented with Barlow´s disease. All received surgery through mini-thoracotomy with annuloplasty plus neochords (n = 71) or annuloplasty-only (n = 31). One valve was replaced for endocarditis (repair rate: 99%)., Results: Annuloplasty-only patients were older (64 ± 16 vs. 55 ± 11 years, p = 0.008) and presented with higher risk (EuroSCORE II: 4.2 ± 4.9 vs. 1.6 ± 1.7, p = 0.007). Annuloplasty-only patients had shorter cross-clamp times (53 ± 18 min vs. 76 ± 23 min, p < 0.001) and received more tricuspid annuloplasty (15.5% vs. 48.4%, p < 0.001). Operating times were similar (170 ± 41 min vs. 164 ± 35, p = 0.455). In three patients, annuloplasty-only caused intraoperative systolic anterior motion (SAM), which was fully resolved by neochords to the posterior leaflet. There were no conversions to sternotomy or deaths at 30-days. Three patients required reoperation for recurrent MR (at 25 days, 2.8 and 7.8 years). At the latest follow-up, there was no MR in 81.4%, mild in 14.7%, and moderate in 2.9%. Three patients died due to non-cardiac reasons. Surviving patients report the absence of relevant symptoms., Conclusions: Minimally-invasive Barlow's repair is safe with good durability. Annuloplasty-only may be a simple solution for complex but symmetric pathologies. However, it may carry an increased risk of intraoperative SAM., (© 2021. The Author(s).)

A young woman presented with palpitations. Holter monitoring revealed frequent premature ventricular complexes; echocardiography showed high-degree mitral regurgitation due to mitral valve prolapse. While mitral valve replacement or repair were being discussed, a different therapy was applied after further diagnostic work-up of the patient which in retrospect appears even more efficient., (© 2021. Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

Mitral valve prolapse (MVP) is characterized by excessive leaflet tissue leading to a wide spectrum of mitral regurgitation (MR) ranging from trivial to severe. The prolapse volume (PV) below the prolapsing leaflets in end-systole was suspected to impact both chamber remodeling and MR grading in MVP. Based on 157 consecutive patients (45 women; mean age 62±15) referred for CMR assessment of MR, either from MVP (n = 91; 58%) or fibroelastic disease (FED) (n = 66; 42%), we sought to study (i) the interaction between PV and cardiac chamber geometry (ii) to study the impact of PV on MR quantification in MVP. Despite similar left ventricular (LV) size, PV was larger in MVP (11±9ml) than in FED (2±2ml). PV progressively increased with the severity of MR in MVP but not in FED. Despite a low regurgitant volume (32±18ml), some MVP patients with less than moderate MR exhibit significant cardiac chambers remodeling compared to 52 age and sex-matched controls. PV correlated significantly (r = 0.52) with the LV dilatation in severe MR but also in less than moderate MR. In MVP, PV>14ml was associated with a significant underestimation (Bias=-26±32ml) of regurgitant volume by PISA compared to CMR. In conclusion, in MVP, PV may play a role in left cardiac chambers remodeling, even in patients without severe MR, and in discordant grading of MR between echocardiography and CMR., (Copyright © 2021 Elsevier Inc. All rights reserved.)

We present the case of a 28-year-old man with a history of unexplained syncope, frequent ventricular arrhythmias, familial LMNA-related dilated cardiomyopathy (DCM), and mitral annular disjunction (MAD). We provide the first association of a novel truncating LMNA variant serving as a potential vulnerable substrate for arrhythmogenic MAD syndrome. This could suggest a possible synergistic role between concealed genetic variants (resulting in fibrosis as a "substrate" for arrhythmogenesis) and the presence of mitral annular disjunction (the "trigger" with mechanical stretch initiating ventricular arrhythmias), which may provide a link between mitral valve prolapse and sudden cardiac death., (Copyright © 2021 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

Importance: Mitral annular disjunction (MAD) has received particular interest in patients with mitral valve prolapse, ventricular tachycardia, and sudden cardiac death. The clinical significance of MAD for patients with Marfan syndrome (MFS) remains largely unexplored., Objective: To define the prevalence of MAD and examine its association with cardiovascular outcomes and arrhythmia among patients with MFS., Design, Setting, and Participants: This retrospective, single-center cohort study included 142 patients with a diagnosis of MFS based on the revised Ghent criteria and a confirmed (likely) pathogenic variant in the FBN1 gene who underwent regular follow-up between January 1, 2004, and December 31, 2019., Main Outcomes and Measures: The presence of MAD was assessed by echocardiography, and the extent of MAD was categorized in tertiles. Patients also underwent resting electrocardiography and 24-hour Holter monitoring. Outcomes included aortic events (aortic dissection or prophylactic aortic surgery), arrhythmic events (defined as sustained ventricular tachycardia or sudden cardiac death), and mitral valve surgery., Results: A total of 142 patients (72 female patients [51%]; median age at first examination, 25 years [range, 2-64 years]) were evaluated. Forty-eight patients (34%) had MAD. Patients with MAD had larger aortic root z scores than patients without MAD (4.1 [interquartile range, 2.8-5.7] vs 3.0 [interquartile range, 1.8-4.0]; P < .001) and more often had mitral valve prolapse (34 of 48 [71%] vs 14 of 94 [15%]; P < .001), ventricular ectopy (14 of 33 [42%] vs 15 of 70 [21%]; P = .03), and nonsustained ventricular tachycardia (13 of 33 [39%] vs 12 of 70 [17%]; P = .01). During follow-up, aortic events occurred at similar rates among patients with vs without MAD (15 of 43 [35%] vs 21 of 84 [25%]; P = .24), but patients in the upper MAD tertile (>10 mm) showed a higher occurrence of aortic events compared with patients with MAD of 10 mm or smaller (9 of 15 [60%] vs 6 of 28 [21%]; P = .01). Patients with arrhythmic events (n = 5) and patients requiring mitral valve surgery (n = 7) were observed exclusively in the group displaying MAD., Conclusions and Relevance: This study suggests that MAD among patients with MFS is associated with the occurrence of arrhythmic events, a higher need for mitral valve intervention, and, among patients with extensive MAD, more aortic events. Cardiac imaging for patients with MFS should consider the assessment of MAD as a potential marker for adverse outcomes.

Background: Although frequently considered a benign condition, new evidence has shown that mitral valve prolapse (MVP) is associated with complex ventricular arrhythmias (VAs) and sudden cardiac death (SCD). Therefore, we conducted a systematic review and meta-analysis of the relevant studies to investigate the parameters that could identify MVP patients at higher risk of experiencing complex VAs., Method: We performed a systematic literature search of PubMed for potential studies between January 2010 and January 2021. Our meta-analysis included studies comparing MVP patients with complex VAs (A-MVP) and those without (NA-MVP). We used the fixed-effects model to obtain the odds ratio (OR), risk ratio (RR), or mean difference (MD) and 95% confidence interval (CI) for each analyzed parameter., Results: Six studies with 848 individuals were included in the meta-analysis. As compared to the NA-MVP patients, A-MVP patients had a higher prevalence of inverted T-wave (OR: 2.73; 95% CI: 1.85-4.02; p < .00001) and longer QTc interval on the resting ECG (MD: 14.73; 95% CI: 9.39-20.08; p < .00001), longer anterior mitral leaflet length (MD: 2.67; 95% CI: 2.02-3.31; p < .00001), bi-leaflet prolapse (OR: 1.65; 95% CI: 1.22-2.24; p = .001), and mitral annulus disjunction (MAD) on echocardiogram (RR: 1.90; 95% CI: 1.50-2.40; p < .00001), and late gadolinium enhancement (LGE) on cardiac magnetic resonance (RR: 4.38; 95% CI: 1.77-10.86; p = .001)., Conclusion: Our comprehensive meta-analysis suggests that risk factors related to A-MVP are T-wave inversion, longer QTc interval, bi-leaflet prolapse, longer anterior mitral valve leaflet, MAD, and LGE., (© 2021 Wiley Periodicals LLC.)

Mitral valve prolapse (MVP) is a commonly occurring heart condition defined by enlargement and superior displacement of the mitral valve leaflet(s) during systole. Although commonly seen as a standalone disorder, MVP has also been described in case reports and small studies of patients with various genetic syndromes. In this review, we analyzed the prevalence of MVP within syndromes where an association to MVP has previously been reported. We further discussed the shared biological pathways that cause MVP in these syndromes, as well as how MVP in turn causes a diverse array of cardiac and noncardiac complications. We found 105 studies that identified patients with mitral valve anomalies within 18 different genetic, developmental, and connective tissue diseases. We show that some disorders previously believed to have an increased prevalence of MVP, including osteogenesis imperfecta, fragile X syndrome, Down syndrome, and Pseudoxanthoma elasticum, have few to no studies that use up-to-date diagnostic criteria for the disease and therefore may be overestimating the prevalence of MVP within the syndrome. Additionally, we highlight that in contrast to early studies describing MVP as a benign entity, the clinical course experienced by patients can be heterogeneous and may cause significant cardiovascular morbidity and mortality. Currently only surgical correction of MVP is curative, but it is reserved for severe cases in which irreversible complications of MVP may already be established; therefore, a review of clinical guidelines to allow for earlier surgical intervention may be warranted to lower cardiovascular risk in patients with MVP.

Background: Cardiac fibrosis in mitral valve prolapse (MVP) is implicated in the development of sudden cardiac death (SCD); however, the pattern remains poorly characterized., Objective: The purpose of this study was to systematically quantify left and right ventricular fibrosis in individuals with isolated MVP and SCD (iMVP-SCD), whereby other potential causes of death are excluded, compared to a control cohort., Methods: Individuals with iMVP-SCD were identified from the Victorian Institute of Forensic Medicine, Australia, and matched for age, sex, and body mass index to control cases with noncardiac death. Cardiac tissue sections were analyzed to determine collagen deposition in the left ventricular free wall (anterior, lateral, and posterior portions), interventricular septum, and right ventricle. Within the iMVP-SCD cases, the endocardial-to-epicardial distribution of fibrosis within the left ventricle was specifically characterized., Results: Seventeen cases with iMVP-SCD were matched 1:1 with 17 controls, yielding 149 samples and 1788 histologic regions. The iMVP-SCD group had increased left ventricular (anterior, lateral, and posterior; all P <.001) and interventricular septum fibrosis (P <.001), but similar amounts of right ventricular fibrosis (P = .62) compared to controls. In iMVP-SCD, left ventricular fibrosis was significantly higher in the lateral and posterior walls compared to the anterior wall and interventricular septum (all P <.001). Within the lateral and posterior walls, iMVP-SCD cases had a significant endocardial-to-epicardial gradient of cardiac fibrosis (P <.01) similar to other known conditions that cause cardiac remodeling., Conclusion: Our study indicates that nonuniform left ventricular remodeling with both localized and generalized left ventricular fibrosis is important in the pathogenesis of SCD in individuals with MVP., (Copyright © 2020 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Mitral valve prolapse (MVP) affects approximately 170 million people worldwide; however, phenotypically, there is a wide variety of heterogeneity. In particular subsets, the incidence of sudden cardiac death is calculated to be 998 per 100,000 person-years, which is significantly increased when compared with the general population of MVP patients. Individuals with high-risk features have been identified as young females with bileaflet MVP and electrocardiogram findings of frequent complex ectopy, ST-T wave changes, and inferior T wave inversions. Supplemental imaging modalities in this subgroup demonstrate redundant leaflets and chordae on 2-dimensional transthoracic echocardiography along with varying severity of mitral annular disjunction. Detailed morphologic assessment by 3-dimensional echocardiography provides a quantitative assessment of annular disjunction along with left ventricular longitudinal and basal circumferential strain patterns. Late gadolinium enhancement on cardiac magnetic resonance imaging identifies diffuse and isolated left ventricle fibrosis involving the fascicles and papillary muscles, which has been visualized in isolation during autopsy. Findings of this review propose that sudden cardiac death as a result of malignant arrhythmias arises from automaticity, complex ectopy, and reentry at the level of the fascicles and papillary muscles. The repetitive mechanical stress provides a nidus for the development of both micro- and macrofibrosis easily identified by late gadolinium enhancement on cardiac magnetic resonance imaging. Escalation to electrophysiology studies and early intervention could provide new targeted lifesaving therapies., (Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.)

Introduction: To describe the signalment, physical examination, and echocardiographic findings of a series of English Springer Spaniels (ESSs) diagnosed with congenital mitral valve dysplasia (MD)., Animals: Eight client-owned ESSs with congenital MD referred for murmur investigation and/or suspected congestive heart failure (CHF)., Materials and Methods: Retrospective case series. Medical records and echocardiograms were reviewed to collect relevant data. Echocardiograms were assessed for the following abnormalities consistent with MD: thickened valve leaflets or leaflet tips, a 'hockey stick' appearance to the valve leaflets, abnormal length of one leaflet with respect to the other, and tethering of one or both leaflets to the papillary muscles., Results: All eight dogs showed the typical echocardiographic lesions associated with MD: thickened leaflet tips (5/8), 'hockey stick' appearance (5/8), elongated anterior leaflet (4/8), tethering of one or both leaflets (7/8). Seven of the eight dogs presented in CHF. Six of the eight dogs had left ventricular dilation in both systole and diastole. Two of the eight dogs had reduced systolic function as assessed by ejection fraction/fractional shortening; however end-systolic volume index was increased in 6/8 dogs. Two dogs subsequently developed atrial fibrillation., Conclusions: Congenital MD should be considered in ESSs with a left-sided apical systolic murmur, particularly in younger dogs. The valve changes seen are similar to those reported in other breeds with MD (thickened leaflet tips, hockey stick appearance to open leaflet tips, abnormal leaflet tethering, abnormally shaped leaflets) and may result in marked remodeling and CHF., Competing Interests: Conflict of Interest Statement The authors do not have any conflicts of interest to disclose., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Background: Mitral valve prolapse is the most frequent valvular defect associated with a wide range of electro-hemodynamic abnormalities, leading to heart failure, arrhythmias and sudden cardiac death. Mitral valve prolapse, first described from Barlow in the 1960s, is defined as displacement of mitral leaflet tissue into the left atrium past the mitral annular plane during systole. The correlation between mitral valve prolapse and sudden cardiac death has been investigated and clarified by various studies in recent years. However, identifying patients at risk and applying measures to prevent those from sudden cardiac death is challenging., Case Presentation: We report on a 61-year-old female patient who had undergone an aborted sudden cardiac death. An arrythmogenic mitral valve prolapse was diagnosed. In addition, electrocardiographically and morphologically risk markers for sudden cardiac death were found in this case. We performed an ICD implantation as secondary prophylaxis and intended to reconstruct the mitral valve., Conclusion: This article examines the association of mitral valve prolapse with sudden cardiac death, the underlying pathophysiological mechanisms and the strategies leading to identify the risk group.

Objective: Left atrial (LA) and left ventricular (LV) remodelling are the adaptive changes that occur in primary mitral regurgitation (MR) and are related to its clinical outcomes. Despite the pathophysiological differences in MR in rheumatic heart disease (RHD) and mitral valve prolapse (MVP), whether the pattern of LV and LA remodelling is different between the two conditions remains unknown. Hence, we compared the LA and LV strain pattern in MR due to RHD, the predominant etiology in developing countries topatients with MVP and age and sex-matched controls., Methods: A total of 50 patients of severe MR which included 30 MVP MR and 20 RHD MR were assessed by strain imaging by speckle tracking echocardiography (STE) and were compared with age and sex-matched controls. 2D STE was used for LA and 3D STE was used for LV strain analysis. LA and LV strain parameters were compared between MVP MR and RHD MR groups., Results: 30 patients with MVP and 20 with RHD were studied. 60% (n = 30) were symptomatic. Mean GLS was -17.2 ± 4.4% compared to -20 ± 3.2% among controls and mean LA strain was 17.35 ± 10.3% compared to 51.34 ± 11.5% among controls which were significantly lower (both p < 0.01). No significant difference in LA strain and GLS was found between MVP and RHD subgroups (LA strain 20.45 ± 11.9% and 14.63 ± 8.85%; p = 0.08; GLS - 18.25 ± 4.3% and-16.2 ± 4.6%; p = 0.12). PALS in the RHD group was lower compared to MVP(p = 0.08) which showed a trend towards significance. LV strain parameters showed no significant difference among the MVP and RHD groups., Conclusion: LA and LV strain parameters showed no significant difference in MR due to either RHD or MVP. There was a trend towards lower LA strain in RHD which needs validation with large multicentric studies. The current strain parameters from MVP with the prognostic value may be applied to MR of RHD etiology, pending confirmation of our results by other groups., Competing Interests: Declaration of competing interest The authors report no relationships that could be construed as a conflict of interest., (Copyright © 2020 Cardiological Society of India. Published by Elsevier B.V. All rights reserved.)

Mitral valve prolapse affects up to 3% of the population, with an annual risk of death of up to 2% a year. Presentation is often in the third to fourth decade of life. This report details an adolescent with mitral valve prolapse with non-specific symptoms but investigative findings of ventricular ectopy at higher heart rates. These patients warrant close surveillance to monitor for progressive arrhythmias and may progress to need an ICD., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: We aimed to assess the clinical and echocardiographic characteristics of patients who underwent surgery for degenerative mitral valve prolapse (MVP) in our center, and its relation to outcomes., Methods: We enrolled 117 consecutive patients from North-East China with an echocardiographic diagnosis of MVP related mitral regurgitation (MR) between April 2018 and November 2019. A complexity scoring system was used for valve anatomy, and patients were re-evaluated at 3-6 months after surgery., Results: Most patients (57.3%) were 40-59 years old. Ejection fraction was <60% in one third, and pulmonary hypertension was present in 64.3% of operated patients. Etiology was myxomatous in 58.9%, with flail as main lesion. Leaflet involvement was posterior in 59.8% patients, anterior in 32.5%, bileaflet in 6%, and commissural in 25.6%. Lesion score was intermediate in >50% of patients, and myxomatous lesions scored higher compared to fibroelastic deficiency (FED). Degree of MR left atrial volume and estimated wedge pressure were significantly higher in intermediate and complex lesions. Repair was performed in 93/101 patients (95.8% success rate). No in-hospital major adverse events, nor deaths at follow-up were reported. Residual MR was ≤ mild in 86.7% of patients at follow-up and was associated with FED etiology and complex lesions., Conclusions: Compared to Western countries, in our sample of Chinese population degenerative severe MR occurred in younger patients. The MVP lesion characteristics are similar, can be accurately detected by non-invasive preoperative evaluation, allowing predictable results. Advanced tailored repair techniques allow excellent immediate and short-term results regardless of the underlying complexity.