Cardiac Arrest in a Patient With Arrhythmic Mitral Valve Prolapse Syndrome: Multiple Possible Etiologies.
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ABSTRACT:
SUBMITTER: Chivulescu M
PROVIDER: S-EPMC8603045 | biostudies-literature | 2021 Nov
REPOSITORIES: biostudies-literature
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Cardiac Arrest in a Patient With Arrhythmic Mitral Valve Prolapse Syndrome: Multiple Possible Etiologies.
Chivulescu Monica M Aabel Eivind E Dejgaard Lars L Steen Torkel T Dunlop Oona O Haugaa Kristina H KH
JACC. Case reports 20211117 16
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. (<b>Level of Difficulty: Intermediate.</b>). ...[more]
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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:BackgroundMitral valve prolapse (MVP) is relatively common in the general population with recently reported prevalence of 1% and familial clustering (Framingham Heart Study). However, its association with ventricular arrhythmias and sudden cardiac arrest (SCA) remains controversial.ObjectivesThe purpose of this study was to characterize the frequency and clinical profile of patients with MVP who suffer SCA in the community.MethodsPatients with SCA cases were prospectively identified in the population-based Oregon Sudden Unexpected Death Study (population ~1 million). The presence of MVP was identified from echocardiograms recorded prior but unrelated to the SCA event. The detailed clinical profile of patients with SCA and MVP was compared with that of SCA patients without MVP to identify potential differences.ResultsA total of 729 SCA patients were evaluated over a 12-year period (mean age 69.5 ± 14.8 years; 64.6% men). MVP was observed in 17 (2.3%) prior to the SCA event (95% confidence interval 1.2%-3.4%). Mitral regurgitation was present in 14 SCA patients with MVP (82.3%) and was moderate or severe in 10 (58.8%). Compared with SCA patients without MVP, SCA patients with MVP were younger (mean age 60.9 ± 16.4 years vs 69.7 ± 14.7 years; P = .02), with fewer risk factors (diabetes 5.9% vs 46.4%; P = .001; hypertension 41.2% vs 78.9%; P = .001) or known coronary disease (29.4% vs 65.6%; P < .001).ConclusionMVP was observed in a small proportion (2.3%) of SCA patients in the general population, suggesting a low risk overall. Since SCA patients with MVP were characterized by younger age and relatively low cardiovascular comorbidity, a focus on imaging for valve structure/insufficiency as well as genetics could aid future risk stratification approaches.
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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.
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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.
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Project description: Not available
| S-EPMC9998028 | 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:The aetiology of sudden cardiac arrest can often be identified to underlying cardiac pathology. Mitral valve prolapse is a relatively common valvular pathology with symptoms manifesting with increasing severity of mitral regurgitation (MR). It is unusual for severe MR to be present without symptoms, and there is growing evidence that this subset of patients may be at increased risk of sudden cardiac arrest or death. The difficulty lies in identifying those patients at risk and applying measures that are appropriate to halting progression to cardiac arrest. This article examines the association of mitral valve prolapse with cardiac arrests, the underlying pathophysiological process and the strategies for identifying those at risk.
| S-EPMC5402658 | biostudies-other
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:Mitral valve prolapse (MVP) is the most common nonischemic mitral regurgitation etiology and mitral abnormality requiring surgery in the Western world. There is an increasing awareness that pathological findings in MVP are not confined to the valve tissue; rather, it is a complex disease, involving the mitral valve apparatus, cardiac hemodynamics, and cardiac structure. Imaging has played a fundamental role in the understanding of the diagnosis, prevalence, and consequences of MVP. The diagnosis of MVP by imaging is based upon demonstrating valve leaflets ascending into the left atrium through the saddle-shaped annulus. Transthoracic and transesophageal echocardiography are the primary modalities in the diagnosis and assessment of MVP patients and must include careful assessment of the leaflets, annulus, chords, and papillary muscles. High-spatial-resolution imaging modalities such as cardiac magnetic resonance images and cardiac computed tomography play a secondary role in this regard and can demonstrate the anatomical relation between the mitral valve annulus and leaflet excursion for appropriate diagnosis. Ongoing development of new methods of cardiac imaging can help us to accurately understand the mechanism, diagnose the disease, develop an appropriate treatment plan, and estimate the risk for sudden death. Recently, several new observations with respect to prolapse have been derived from cardiac imaging including three-dimensional echocardiography and tissue-Doppler imaging. The aim of this article is to present these new imaging-derived insights for the diagnosis, risk assessment, treatment, and follow-up of patients with MVP.
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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