ECG, an essential tool to guide arrhythmic mitral valve prolapse diagnosis.
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ABSTRACT:
SUBMITTER: Garcia AR
PROVIDER: S-EPMC9998028 | biostudies-literature | 2023 Mar
REPOSITORIES: biostudies-literature
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ECG, an essential tool to guide arrhythmic mitral valve prolapse diagnosis.
Garcia Alvaro Riesgo AR Iñarra Ander Larrea AL de Miguel Sara Martinez SM
European heart journal. Case reports 20230201 3
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Project description:BackgroundArrhythmic mitral valve prolapse (AMVP) is a relatively new entity in the field of arrhythmia and sudden cardiac death.Case summaryA 19-year-old female patient with a history of aborted sudden cardiac death, initially diagnosed as idiopathic ventricular fibrillation in 2012 and received implantable cardioverter defibrillator. Gradual decline in left ventricular ejection fraction and receiving multiple appropriate implantable cardioverter defibrillator shocks was odd for us. After review of her past medical documents and detailed cardiac imaging diagnosis of AMVP was made.DiscussionIndividuals with AMVP are prone to left ventricular mechanical dispersion, leading to an increased susceptibility to malignant ventricular arrhythmias independent of the severity of MR.Take-home messageThis case emphasizes the importance of reviewing old documents and consultation with multiple subspecialists. Follow-up may alter the primary suspected diagnosis.
| S-EPMC11830245 | biostudies-literature
Project description:Patients with heritable aortic disease (HAD) have an increased risk of ventricular arrhythmias and sudden cardiac death. Although mitral valve prolapse is common in HAD, mitral annulus disjunction (MAD) has only recently been described in these patients. This under-recognized condition may be a contributing factor to otherwise unexplained ventricular arrhythmias and sudden cardiac death in patients with HAD. This case series describes 3 patients in an adult HAD clinic who have concomitant mitral valve prolapse, MAD, and malignant arrhythmias. These cases may represent a unique disease entity or overlap syndrome, and they introduce MAD as a potential arrhythmogenic risk marker in HAD.
| S-EPMC8640620 | biostudies-literature
Project description:Arrhythmic mitral valve prolapse syndrome (ARMV) is a recognized but underdiagnosed disease pattern. Risk factors for ARMV are established but not very well known, and the association of the structural abnormality with ventricular arrhythmias is incompletely understood. Here, we present the case of a young man who presented at our hospital for radiofrequency catheter ablation and mitral valve surgery after two episodes of survived sudden cardiac arrest. We discuss the diagnostic and therapeutic strategies that were used. We shine light on the risk factors for ARMV and why early identification is crucial. We address the topic of primary prevention and its limitations. Finally, we discuss different treatment modalities for patients with ARMV. More awareness for ARMV is crucial. A consensus statement on clinical management exists, but scientific gaps in prospective data for primary prevention need to be filled and there is a need for a better understanding of the pathogenesis of ARMV.
| S-EPMC11328526 | biostudies-literature
Project description:BackgroundInterstitial fibrosis as quantified by cardiovascular magnetic resonance (CMR) has been demonstrated in arrhythmic mitral valve prolapse (AMVP), a condition with known female predominance. Prior studies of interstitial fibrosis in AMVP have only included cases with significant mitral regurgitation (MR) or mitral annular disjunction (MAD), limiting our understanding of alternative arrhythmic mechanisms in mitral valve prolapse (MVP). We sought to evaluate the association between interstitial fibrosis and AMVP, regardless of MAD and without severe MR, while also investigating the contribution of sex to this association.MethodsWe performed research-based contrast CMR in consecutive individuals with MVP between 2019 and 2022. Extracellular volume fraction (ECV%), a surrogate marker for interstitial fibrosis, was quantified using T1 mapping in the basal and mid-left ventricular slices. Replacement fibrosis was assessed using late gadolinium enhancement (LGE). AMVP was defined as MVP with frequent premature ventricular contractions and/or non-sustained/sustained ventricular tachycardia (VT) or fibrillation (VF).ResultsWe identified 65 MVP cases without severe MR (30 [46%] women, 22 [34%] no/trace, 30 [44%] mild, and 13 [21%] moderate MR) and with adequate ECV% measurement. Among these, 38% were classified as AMVP, including two cases of aborted VF arrest, both in premenopausal females. Global ECV% was significantly higher in AMVP vs non-AMVP (31% [27-33] vs 27% [23-30], p = 0.002). In the AMVP group, higher segmental ECV% was not limited to the inferolateral/inferior walls, typically subject to myocardial traction by the prolapsing leaflets/MAD but was more diffuse and involved atypical segments such as the anterior/anterolateral walls (p < 0.05). The association between AMVP and global ECV% was driven by female sex (32% [30-34] vs 27% [25-30], p = 0.002 in females; 28% [23-32] vs 26% [23-30], p = 0.41 in males). ECV% remained independently associated with an increased risk of arrhythmic events, including VT/VF (p < 0.01), even after adjustment for cardiovascular risk factors, MAD, and LGE (p < 0.01).ConclusionIn MVP without significant MR, interstitial fibrosis by CMR is associated with an increased risk of arrhythmic events, suggesting a primary myopathic process. The selective association between interstitial fibrosis and AMVP in females may explain why severe arrhythmic complications are more prevalent among women.
| S-EPMC11652916 | biostudies-literature
Project description:ImportanceMyocardial replacement fibrosis has been reported to occur in one-third of patients with mitral valve prolapse (MVP) and significant mitral regurgitation (MR). However, it remains unknown whether there are detectable changes in myocardial metabolism suggestive of inflammation or ischemia that accompany the development of fibrosis.ObjectivesTo characterize the burden and distribution of fluorine 18-labeled (18F) fluorodeoxyglucose (FDG) uptake and late gadolinium enhancement (LGE) in patients with degenerative MVP and ventricular ectopy.Design, setting, and participantsProspective observational study of 20 patients with MVP and significant primary degenerative MR who were referred for mitral valve repair and underwent hybrid positron emission tomography/magnetic resonance imaging (PET/MRI). Ventricular arrhythmias were categorized as either complex (n = 12) or minor (n = 8). Coregistered hybrid 18F FDG-PET and MRI LGE images were assessed and categorized. Recruitment occurred in the new patient clinic of a mitral valve repair reference center. This study was conducted from January 11, 2018, to June 26, 2019.ExposuresSimultaneous cardiac 18F FDG-PET and MRI with LGE imaging on a hybrid PET/MRI system and ambulatory rhythm monitoring.Main outcomes and measuresPatients were categorized by the presence and pattern of FDG uptake and LGE, the severity of ventricular arrhythmias, and the indication for mitral valve surgery.ResultsIn the cohort of 20 patients, the median age was 59.5 years (interquartile range, 52.5-63.2 years). Focal, or focal-on-diffuse uptake, of 18F-FDG (PET positive) was detected in 17 of 20 patients (85%). The FDG uptake coexisted with areas of LGE (PET/MRI positive) in 14 patients (70%). Of the 5 asymptomatic patients with normal ventricular indices and absence of any surgical indications, all were PET/MRI positive.Conclusions and relevanceIn this pilot study, we demonstrate a novel association between degenerative MVP and FDG uptake, a surrogate for myocardial inflammation and/or ischemia. Such evidence of myocardial injury, even in asymptomatic patients, suggests an ongoing subclinical disease process. These findings warrant further investigation into whether imaging for myocardial inflammation, ischemia, and scar has a role in arrhythmic risk stratification and whether it provides incremental prognostic value in patients with chronic severe mitral regurgitation undergoing active surveillance.
| S-EPMC7254450 | biostudies-literature
Project description:Arrhythmic mitral valve prolapse syndrome is associated with a high risk of death. A 60-year-old man with arrhythmic mitral valve prolapse syndrome was monitored with an implantable loop recorder. Nine months later dyspnea developed, followed by cardiac arrest. Echocardiography showed mitral valve chordal rupture. He underwent successful surgical mitral valve repair. (Level of Difficulty: Intermediate.).
| S-EPMC8603045 | biostudies-literature
Project description:Aim: Test the hypothesis that 5HT receptors (5HTRs) signaling contributes to MVP pathophysiology. Methods and results: MV RNA was used for microarray analysis of MVP patients versus control, highlighting genes that indicate the involvement of 5HTR pathways and extracellular matrix remodeling in MVP. These canine MVP leaflets (N=5/group) showed 5HTR2B upregulation. Conclusion: In humans, MVP is associated with an upregulation in 5HTR2B expression and increased 5HT receptor signaling in the leaflets. 5HTR signaling is involved not only in previously reported 5HTrelated valvulopathies, but it is also involved in the pathological remodeling of MVP.
2018-01-29 | GSE109744 | GEO
Project description:Mitral valve prolapse (MVP) is a common condition present in 1-3% of the population. There has been evidence that a subset of MVP patients is at higher risk of sudden cardiac death. The arrhythmogenic mechanism is related to fibrotic changes in the papillary muscles caused by the prolapsing valve. ECG features include ST-segment depression, T wave inversion or biphasic T waves in inferior leads, and premature ventricular contractions arising from the papillary muscles and the fascicular system. Echocardiography can identify MVP and mitral annular disjunction, a feature that has significant negative prognostic value in MVP. Cardiac MRI is indicated for identifying fibrosis. Patients with high-risk features should be referred for further evaluation. Catheter ablation and mitral valve repair might reduce the risk of malignant arrhythmia. MVP patients with high-risk features and clinically documented ventricular arrhythmia may also be considered for an ICD.
| S-EPMC9376835 | biostudies-literature
Project description:BackgroundInitial studies have suggested the familial clustering of mitral valve prolapse, but most of them were either community based among unselected individuals or applied non-specific diagnostic criteria. Therefore little is known about the familial distribution of mitral regurgitation in a referral-type population with a more severe mitral valve prolapse phenotype. The objective of this study was to evaluate the presence of familial mitral regurgitation in patients undergoing surgery for mitral valve prolapse, differentiating patients with Barlow's disease, Barlow forme fruste and fibro-elastic deficiency.MethodsA total of 385 patients (62 ± 12 years, 63% men) who underwent surgery for mitral valve prolapse were contacted to assess cardiac family history systematically. Only the documented presence of mitral regurgitation was considered to define 'familial mitral regurgitation'. In the probands, the aetiology of mitral valve prolapse was defined by surgical observations.ResultsA total of 107 (28%) probands were classified as having Barlow's disease, 85 (22%) as Barlow forme fruste and 193 (50%) patients as fibro-elastic deficiency. In total, 51 patients (13%) reported a clear family history for mitral regurgitation; these patients were significantly younger, more often diagnosed with Barlow's disease and also reported more sudden death in their family as compared with 'sporadic mitral regurgitation'. In particular, 'familial mitral regurgitation' was reported in 28 patients with Barlow's disease (26%), 15 patients (8%) with fibro-elastic deficiency and eight (9%) with Barlow forme fruste (P < 0.001).ConclusionsIn a large cohort of patients operated for mitral valve prolapse, the self-reported prevalence of familial mitral regurgitation was 26% in patients with Barlow's disease and still 8% in patients with fibro-elastic deficiency, highlighting the importance of familial anamnesis and echocardiographic screening in all mitral valve prolapse patients.
| S-EPMC7008556 | biostudies-literature
Project description:Background Mitral valve prolapse (MVP) is one of the most common forms of cardiac valve disease and affects 2% to 3% of the population. Previous imaging reports have indicated that myocardial fibrosis is common in MVP and described its association with sudden cardiac death. These data combined with evidence for postrepair ventricular dysfunction in surgical patients with MVP support a link between fibrosis and MVP. Methods and Results We performed histopathologic analysis of left ventricular (LV) biopsies from peripapillary regions, inferobasal LV wall and apex on surgical patients with MVP, as well as in a mouse model of human MVP (Dzip1S14R/+). Tension-dependent molecular pathways were subsequently assessed using both computational modeling and cyclical stretch of primary human cardiac fibroblasts in vitro. Histopathology of LV biopsies revealed regionalized fibrosis in the peripapillary myocardium that correlated with increased macrophages and myofibroblasts. The MVP mouse model exhibited similar regional increases in collagen deposition that progress over time. As observed in the patient biopsies, increased macrophages and myofibroblasts were observed in fibrotic areas within the murine heart. Computational modeling revealed tension-dependent profibrotic cellular and molecular responses consistent with fibrosis locations related to valve-induced stress. These simulations also identified mechanosensing primary cilia as involved in profibrotic pathways, which was validated in vitro and in human biopsies. Finally, in vitro stretching of primary human cardiac fibroblasts showed that stretch directly activates profibrotic pathways and increases extracellular matrix protein production. Conclusions The presence of prominent regional LV fibrosis in patients and mice with MVP supports a relationship between MVP and progressive damaging effects on LV structure before overt alterations in cardiac function. The regionalized molecular and cellular changes suggest a reactive response of the papillary and inferobasal myocardium to increased chordal tension from a prolapsing valve. These studies raise the question whether surgical intervention on patients with MVP should occur earlier than indicated by current guidelines to prevent advanced LV fibrosis and potentially reduce residual risk of LV dysfunction and sudden cardiac death.
| S-EPMC9075228 | biostudies-literature