ArticleLiterature Review

Characterization of ventricular arrhythmias and sudden cardiac death in subjects with mitral valve prolapse and mitral annular disjunction

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Abstract

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 nor clinically useful risk stratification schemes to determine which group of patients are at higher risk or may benefit from interventions like 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.

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... 5,28 The evaluation of 12-lead ECGs with PVCs and nonsustained VTs appeared as polymorphic complexes in 60% of our patients with inferolateral MAD, in line with previous reports showing that polymorphic ectopy can be found in patients with MAD. 21,29 The finding supports the hypothesis that an abnormal mechanical motion resulting in conduction abnormalities could be the substrate for arrhythmias in MAD. 29 The increased pro-arrhythmic profile when additional mitral valve abnormalities are present further corroborates this hypothesis. ...
... Previous studies did show an increased risk for arrhythmias with larger MAD length. 21,29 More insights into 'normal' or 'benign' MAD length could lead to a better understanding of the pathogenic mechanisms underlying MAD. ...
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Aims Previously, we demonstrated that inferolateral mitral annular disjunction (MAD) is more prevalent in patients with idiopathic ventricular fibrillation (IVF) than in healthy controls. In the present study, we advanced the insights into the prevalence and ventricular arrhythmogenicity by inferolateral MAD in an even larger IVF cohort. Methods and results This retrospective multi-centre study included 185 IVF patients [median age 39 (27, 52) years, 40% female]. Cardiac magnetic resonance images were analyzed for mitral valve and annular abnormalities and late gadolinium enhancement. Clinical characteristics were compared between patients with and without MAD. MAD in any of the 4 locations was present in 112 (61%) IVF patients and inferolateral MAD was identified in 24 (13%) IVF patients. Mitral valve prolapse (MVP) was found in 13 (7%) IVF patients. MVP was more prevalent in patients with inferolateral MAD compared with patients without inferolateral MAD (42 vs. 2%, P < 0.001). Pro-arrhythmic characteristics in terms of a high burden of premature ventricular complexes (PVCs) and non-sustained ventricular tachycardia (VT) were more prevalent in patients with inferolateral MAD compared to patients without inferolateral MAD (67 vs. 23%, P < 0.001 and 63 vs. 41%, P = 0.046, respectively). Appropriate implantable cardioverter defibrillator therapy during follow-up was comparable for IVF patients with or without inferolateral MAD (13 vs. 18%, P = 0.579). Conclusion A high prevalence of inferolateral MAD and MVP is a consistent finding in this large IVF cohort. The presence of inferolateral MAD is associated with a higher PVC burden and non-sustained VTs. Further research is needed to explain this potential interplay.
... Finally, transient increases in sympathetic tone may represent the critical factor precipitating SCD in AMVP, especially during the practice of sports [38,55]. Previous work has demonstrated an association between MVP, reduced vagal tone, and increased sympathetic activity [38]; furthermore, papillary muscle stretch may lead to spontaneous depolarization of myocardial nerve endings via mechanoelectrical feedback [56], leading to changes in the central nervous system activity and further release of catecholamines, which may in turn increase calcium release from the myocyte sarcoplasmic reticulum, modulate membrane ion channels, and favor afterdepolarizations, especially in the presence of genetic polymorphisms of the β1 adrenergic receptor leading to hypersensitivity to catecholamines [57]. ...
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Although mitral valve prolapse (MVP) is the most prevalent valvular abnormality in Western countries and generally carries a good prognosis, a small subset of patients is exposed to a significant risk of malignant ventricular arrhythmias (VAs) and sudden cardiac death (SCD), the so-called arrhythmic MVP (AMVP) syndrome. Recent work has emphasized phenotypical risk features of severe AMVP and clarified its pathophysiology. However, the appropriate assessment and risk stratification of patients with suspected AMVP remains a clinical conundrum, with the possibility of both overestimating and underestimating the risk of malignant VAs, with the inappropriate use of advanced imaging and invasive electrophysiology study on one hand, and the catastrophic occurrence of SCD on the other. Furthermore, the sports eligibility assessment of athletes with AMVP remains ill defined, especially in the grey zone of intermediate arrhythmic risk. The definition, epidemiology, pathophysiology, risk stratification, and treatment of AMVP are covered in the present review. Considering recent guidelines and expert consensus statements, we propose a comprehensive pathway to facilitate appropriate counseling concerning the practice of competitive/leisure-time sports, envisioning shared decision making and the multidisciplinary “sports heart team” evaluation of borderline cases. Our final aim is to encourage an active lifestyle without compromising patients’ safety.
... Many authors have wondered whether these are necessarily the same disease. 23,32 In their study, 33 ...
Article
Mitral valve prolapse (MVP) affects about 2-3% of the general population, mostly women, and is the most common cause of primary chronic mitral regurgitation (MR) in western countries. The natural history is heterogeneous and widely determined by the severity of MR. Although most patients remain asymptomatic with a near-normal life expectancy, approximately 5 to 10 % progress to severe MR. As largely recognized, left ventricular (LV) dysfunction due to chronic volume overload per se identifies a subgroup at risk of cardiac death. However, there is rising evidence of a link between MVP and life threating ventricular arrhythmias (VAs)/sudden cardiac death (SCD) in a small subset of middle-aged patients without significant MR, heart failure and remodeled hearts. The present review focuses on the underlying mechanism of electric instability and unexpected cardiac death in this subset of young patients, from the myocardial scarring of the LV infero-lateral wall due to mechanical stretch exerted by the prolapsing leaflets and mitral annular disjunction, to the inflammation's impact on fibrosis pathways along with a constitutional hyperadrenergic state. The heterogeneity of clinical course reveals a necessity of risk stratification, preferably through non-invasive multimodality imaging, that will help to identify and prevent adverse scenarios in young MVP patients.
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Purpose of the Review To summarize currently available data on the topic of mitral valve prolapse (MVP) and its correlation to the occurrence of atrial and ventricular arrhythmias. To assess the prognostic value of several diagnostic methods such as transthoracic echocardiography, transesophageal echocardiography, cardiac magnetic resonance, cardiac computed tomography, electrocardiography, and electrophysiology concerning arrhythmic episodes. To explore intra and extracellular biochemistry of the cardiovascular system and its biomarkers as diagnostic tools to predict rhythm disturbances in the MVP population. Recent Findings MVP is a common and mainly benign valvular disorder. It affects 2–3% of the general population. MVP is a heterogeneous and highly variable phenomenon with three structural phenotypes: myxomatous degeneration, fibroelastic deficiency, and forme fruste. Exercise intolerance, supraventricular tachycardia, and chest discomfort are the symptoms that are often paired with psychosomatic components. Though MVP is thought to be benign, the association between isolated MVP without mitral regurgitation (MR) or left ventricle dysfunction, with ventricular arrhythmia (VA) and sudden cardiac death (SCD) has been observed. The incidence of SCD in the MVP population is around 0.6% per year, which is 6 times higher than the occurrence of SCD in the general population. Summary Often asymptomatic MVP population poses a challenge to screen for VA and prevent SCD. Therefore, it is crucial to carefully assess the risk of VA and SCD in patients with MVP with the use of various tools such as diagnostic imaging and biochemical and genetic screening.
Article
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 endpoint 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 endpoint 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.
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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.
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Bileaflet mitral valve prolapse (Bi-MVP) is associated with increased risk for cardiac arrest. We describe a patient who presented after a cardiac arrest with Bi-MVP and variants in Lamin A/C (LMNA) and the sodium channel alpha-subunit 5a (SCN5A). Genetic variants may be the culprit for arrhythmogenesis in Bi-MVP patients. (Level of Difficulty: Intermediate.)
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Mitral annular disjunction (MAD) is routinely diagnosed by cardiac imaging, mostly by echocardiography, and shown to be a risk factor for ventricular arrhythmias. While MAD is associated with mitral valve (MV) prolapse (MVP), it is unknown which patients with MAD are at higher risk and which additional imaging features may help identify them. The value of cardiac computed tomography (CCT) for the diagnosis of MAD is unknown. Accordingly, we aimed to: (1) develop a standardized CCT approach to identify MAD in patients with MVP and severe mitral regurgitation (MR); (2) determine its prevalence and identify features that are associated with MAD in this population. We retrospectively studied 90 patients (age 63 ± 12 years) with MVP and severe MR, who had pre-operative CCT (256-slice scanner) of sufficient quality for analysis. The presence and degree of MAD was assessed by rotating the view plane around the MV center to visualize disjunction along the annulus. Additionally, detailed measurements of MV apparatus and left heart chambers were performed. Univariate logistic regression analysis was performed to determine which parameters were associated with MAD. MAD was identified in 18 patients (20%), and it was typically located adjacent to a prolapsed or flail mitral leaflet scallop. Of these patients, 75% had maximum MAD distance > 4.8 mm and 90% > 3.8 mm. Female gender was most strongly associated with MAD (p = 0.04). Additionally, smaller end-diastolic mitral annulus area (p = 0.045) and longer posterior leaflet (p = 0.03) were associated with greater MAD. No association was seen between MAD and left ventricular size and function, left atrial size, and papillary muscle geometry. CCT can be used to readily detect MAD, by taking advantage of the 3D nature of this modality. A significant portion of MVP patients referred for mitral valve repair have MAD. The presence of MAD is associated with female gender, smaller annulus size and greater posterior leaflet length.
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A 51-year-old man with a long-standing history of bileaflet mitral valve prolapse accompanied by mitral annular disjunction and mild mitral regurgitation presented for evaluation of increasingly frequent palpitations. Ambulatory Holter monitoring, cardiac magnetic resonance imaging, serial transthoracic echocardiography, and diagnostic electrophysiology studies were consistent with a diagnosis of arrhythmogenic bileaflet mitral valve prolapse syndrome. Because of the presence of a similar phenotype in the proband's mother, brother, and maternal aunt, research-based whole exome sequencing was pursued and a novel truncating variant (p.Trp34*-FLNC) in the cardiomyopathy-causative FLNC-encoded filamin C unearthed that cosegregated with disease. Unexpectedly, these observations provide the first evidence that a heritable proarrhythmic genetic substrate (ie, FLNC haploinsufficiency–mediated weakening of cell-cell adhesion) may underlie, at least in part, some cases of arrhythmogenic mitral valve prolapse syndrome.
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Objective Bileaflet mitral valve prolapse (MVP) with either focal or diffuse myocardial fibrosis has been linked to ventricular arrhythmia and/or sudden cardiac arrest. Left ventricular (LV) mechanical dispersion by speckle-tracking echocardiography (STE) is a measure of heterogeneity of ventricular contraction previously associated with myocardial fibrosis. The aim of this study is to determine whether mechanical dispersion can identify MVP at higher arrhythmic risk. Methods We identified 32 consecutive arrhythmic MVPs (A-MVP) with a history of complex ventricular ectopy on Holter/event monitor (n=23) or defibrillator placement (n=9) along with 27 MVPs without arrhythmic complications (NA-MVP) and 39 controls. STE was performed to calculate global longitudinal strain (GLS) as the average peak longitudinal strain from an 18-segment LV model and mechanical dispersion as the SD of the time to peak strain of each segment. Results MVPs had significantly higher mechanical dispersion compared with controls (52 vs 42 ms, p=0.005) despite similar LV ejection fraction (62% vs 63%, p=0.42) and GLS (−19.7 vs −21, p=0.045). A-MVP and NA-MVP had similar demographics, LV ejection fraction and GLS (all p>0.05). A-MVP had more bileaflet prolapse (69% vs 44%, p=0.031) with a similar degree of mitral regurgitation (mostly trace or mild in both groups) (p>0.05). A-MVP exhibited greater mechanical dispersion when compared with NA-MVP (59 vs 43 ms, p=0.0002). Mechanical dispersion was the only significant predictor of arrhythmic risk on multivariate analysis (OR 1.1, 95% CI 1.02 to 1.11, p=0.006). Conclusions STE-derived mechanical dispersion may help identify MVP patients at higher arrhythmic risk.
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Background: Mitral annulus disjunction (MAD) is an abnormal atrial displacement of the mitral valve leaflet hinge point. MAD has been associated with mitral valve prolapse (MVP) and sudden cardiac death. Objectives: The purpose of this study was to describe the clinical presentation, MAD morphology, association with MVP, and ventricular arrhythmias in patients with MAD. Methods: The authors clinically examined patients with MAD. By echocardiography, the authors assessed the presence of MVP and measured MAD distance in parasternal long axis. Using cardiac magnetic resonance (CMR), the authors assessed circumferential MAD in the annular plane, longitudinal MAD distance, and myocardial fibrosis. Aborted cardiac arrest and sustained ventricular tachycardia were defined as severe arrhythmic events. Results: The authors included 116 patients with MAD (age 49 ± 15 years; 60% female). Palpitations were the most common symptom (71%). Severe arrhythmic events occurred in 14 (12%) patients. Longitudinal MAD distance measured by CMR was 3.0 mm (interquartile range [IQR]: 0 to 7.0 mm) and circumferential MAD was 150° (IQR: 90° to 210°). Patients with severe arrhythmic events were younger (age 37 ± 13 years vs. 51 ± 14 years; p = 0.001), had lower ejection fraction (51 ± 5% vs. 57 ± 7%; p = 0.002) and had more frequently papillary muscle fibrosis (4 [36%] vs. 6 [9%]; p = 0.03). MVP was evident in 90 (78%) patients and was not associated with ventricular arrhythmia. Conclusions: Ventricular arrhythmias were frequent in patients with MAD. A total of 26 (22%) patients with MAD did not have MVP, and MVP was not associated with arrhythmic events, indicating MAD itself as an arrhythmogenic entity. MAD was detected around a large part of the mitral annulus circumference and was interspersed with normal tissue.
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Background Recent studies reported left ventricular (LV) fibrosis in patients with primary mitral regurgitation (MR) thought to be principally due to mitral valve prolapse (MVP). Objectives This study sought to evaluate the prevalence, characteristics, and prognostic implications of LV fibrosis in a large cohort of primary MR patients with and without MVP using cardiovascular magnetic resonance (CMR). Methods Patients referred for contrast CMR assessment of chronic primary MR were enrolled and underwent comprehensive assessment of cardiac remodeling, severity of MR, and LV replacement fibrosis. Primary MR patients were stratified into: an MVP group if there was >2 mm mitral leaflet displacement on cine-CMR, or a non-MVP group. Patients were followed for arrhythmic events (sudden cardiac death, aborted sudden cardiac arrest, and sustained or inducible ventricular arrhythmia). Results A total of 356 primary MR patients (177 MVP and 179 non-MVP) were enrolled. LV fibrosis was more prevalent in the MVP group than the non-MVP group (36.7% vs. 6.7%; p < 0.001). The presence of MVP had the strongest association (odds ratio: 6.82; p < 0.001) with LV fibrosis even after adjustment for clinical variables, measures of cardiac remodeling, and MR severity. During follow-up (median 1,354 days), MVP patients with LV fibrosis had the highest event rate for arrhythmic events. Conclusions In primary MR patients, LV fibrosis is more prevalent in MVP than non-MVP, suggesting a unique pathophysiology beyond volume overload in MVP. LV fibrosis in primary MR may represent a risk marker of arrhythmic events.
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Background— Arrhythmic mitral valve prolapse (MVP) is characterized by myxomatous leaflets and left ventricular (LV) fibrosis of papillary muscles and inferobasal wall. We searched for morphofunctional abnormalities of the mitral valve that could explain a regional mechanical myocardial stretch. Methods and Results— Thirty-six (27 female patients; median age: 44 years) arrhythmic MVP patients with LV late gadolinium enhancement on cardiac magnetic resonance and no or trivial mitral regurgitation, and 16 (6 female patients; median age: 40 years) MVP patients without LV late gadolinium enhancement were investigated by morphofunctional cardiac magnetic resonance. Mitral annulus disjunction (median: 4.8 versus 1.8 mm; P<0.001), end-systolic mitral annular diameters (median: 41.2 versus 31.5; P=0.004) and end-diastolic mitral annular diameters (median: 35.5 versus 31.5; P=0.042), prevalence of posterior systolic curling (34 [94%] versus 3 [19%]; P<0.001), and basal to mid LV wall thickness ratio >1.5 (22 [61%] versus 4 [25%]; P=0.016) were higher in MVP patients with late gadolinium enhancement than in those without. A linear correlation was found between mitral annulus disjunction and curling (R=0.85). A higher prevalence of auscultatory midsystolic click (26 [72%] versus 6 [38%]; P=0.018) was also noted. Histology of the mitral annulus showed a longer mitral annulus disjunction in 50 sudden death patients with MVP and LV fibrosis than in 20 patients without MVP (median: 3 versus 1.5 mm; P<0.001). Conclusions— Mitral annulus disjunction is a constant feature of arrhythmic MVP with LV fibrosis. The excessive mobility of the leaflets caused by posterior systolic curling accounts for a mechanical stretch of the inferobasal wall and papillary muscles, eventually leading to myocardial hypertrophy and scarring. These mitral annulus abnormalities, together with auscultatory midsystolic click, may identify MVP patients who would need arrhythmic risk stratification.
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Background: Few data exist on the relation of the 3-dimensional morphology of mitral valve and degree of mitral regurgitation (MR) in mitral valve prolapse. Methods and results: Real-time 3-dimensional transesophageal echocardiography of the mitral valve was acquired in 112 subjects, including 36 patients with mitral valve prolapse and significant MR (≥3+; MR+ group), 32 patients with mitral valve prolapse but no or mild MR (≤2+; MR- group), 12 patients with significant MR resulting from nonprolapse pathologies (nonprolapse group), and 32 control subjects. The 3-dimensional geometry of mitral valve apparatus was measured with dedicated quantification software. Compared with the normal and MR- groups, the MR+ group had more dilated mitral annulus (P<0.0001), a reduced annular height to commissural width ratio (AHCWR) (P<0.0001) indicating flattening of annular saddle shape, redundant leaflet surfaces (P<0.0001), greater leaflet billow volume (P<0.0001) and billow height (P<0.0001), longer lengths from papillary muscles to coaptation (P<0.0001), and more frequent chordal rupture (P<0.0001). Prevalence of chordal rupture increased progressively with annulus flattening (7% versus 24% versus 42% for AHCWR >20%, 15%-20%, and <15%, respectively; P=0.004). Leaflet billow volume increased exponentially with decreasing AHCWR in patients without chordal rupture (r(2)=0.66, P<0.0001). MR severity correlated strongly with leaflet billow volume (r(2)=0.74, P<0.0001) and inversely with AHCWR (r(2)=0.44, P<0.0001). In contrast, annulus dilatation but not flattening occurred in nonprolapse MR patients. An AHCWR <15% (odds ratio=7.1; P=0.0004) was strongly associated with significant MR in mitral valve prolapse. Conclusion: Flattening of the annular saddle shape is associated with progressive leaflet billowing and increased frequencies of chordal rupture and may be important in the pathogenesis of MR in mitral valve prolapse.
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Mitral annular disjunction (MAD) consists of an altered spatial relation between the left atrial wall, the attachment of the mitral leaflets, and the top of the left ventricular (LV) free wall, manifested as a wide separation between the atrial wall-mitral valve junction and the top of the LV free wall. Originally described in association with myxomatous mitral valve disease, this abnormality was recently revisited by a surgical group that pointed its relevance for mitral valve reparability. The aims of this study were to investigate the echocardiographic prevalence of mitral annular disjunction in patients with myxomatous mitral valve disease, and to characterize the clinical profile and echocardiographic features of these patients. We evaluated 38 patients with myxomatous mitral valve disease (mean age 57 ± 15 years; 18 females) and used standard transthoracic echocardiography for measuring the MAD. Mitral annular function, assessed by end-diastolic and end-systolic annular diameters, was compared between patients with and without MAD. We compared the incidence of arrhythmias in a subset of 21 patients studied with 24-hour Holter monitoring. MAD was present in 21 (55%) patients (mean length: 7.4 ± 8.7 mm), and was more common in women (61% vs 38% in men; p = 0.047). MAD patients more frequently presented chest pain (43% vs 12% in the absence of MAD; p = 0.07). Mitral annular function was significantly impaired in patients with MAD in whom the mitral annular diameter was paradoxically larger in systole than in diastole: the diastolic-to-systolic mitral annular diameter difference was -4,6 ± 4,7 mm in these patients vs 3,4 ± 1,1 mm in those without MAD (p < 0.001). The severity of MAD significantly correlated with the occurrence of non-sustained ventricular tachycardia (NSVT) on Holter monitoring: MAD›8.5 mm was a strong predictor for (NSVT), (area under ROC curve = 0.74 (95% CI, 0.5-0.9); sensitivity 67%, specificity 83%). There were no differences between groups regarding functional class, severity of mitral regurgitation, LV volumes, and LV systolic function. MAD is a common finding in myxomatous mitral valve disease patients, easily recognizable by transthoracic echocardiography. It is more prevalent in women and often associated with chest pain. MAD significantly disturbs mitral annular function and when severe predicts the occurrence of NSVT.
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Mitral-valve prolapse has been described as a common disease with frequent complications. To determine the prevalence of mitral-valve prolapse in the general population, as diagnosed with the use of current two-dimensional echocardiographic criteria, we examined the echocardiograms of 1845 women and 1646 men (mean [+/-SD] age, 54.7+/-10.0 years) who participated in the fifth examination of the offspring cohort of the Framingham Heart Study. Classic mitral-valve prolapse was defined as superior displacement of the mitral leaflets of more than 2 mm during systole and as a maximal leaflet thickness of at least 5 mm during diastasis, and nonclassic prolapse was defined as displacement of more than 2 mm, with a maximal thickness of less than 5 mm. A total of 84 subjects (2.4 percent) had mitral-valve prolapse: 47 (1.3 percent) had classic prolapse, and 37 (1.1 percent) had nonclassic prolapse. Their age and sex distributions were similar to those of the subjects without prolapse. None of the subjects with prolapse had a history of heart failure, one (1.2 percent) had atrial fibrillation, one (1.2 percent) had cerebrovascular disease, and three (3.6 percent) had syncope, as compared with unadjusted prevalences of these findings in the subjects without prolapse of 0.7, 1.7, 1.5, and 3.0 percent, respectively. The frequencies of chest pain, dyspnea, and electrocardiographic abnormalities were similar among subjects with prolapse and those without prolapse. The subjects with prolapse were leaner (P<0.001) and had a greater degree of mitral regurgitation than those without prolapse, but on average the regurgitation was classified as trace or mild. In a community based sample of the population, the prevalence of mitral-valve prolapse was lower than previously reported. The prevalence of adverse sequelae commonly associated with mitral-valve prolapse in studies of patients referred for that diagnosis was also low.
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Open in new tabDownload slide Risk stratification scheme. Risk stratification aiming at assessing the risk of VAs and SCD in patients with MVP, involving two phases based on the clinical and imaging context and the uncovered arrhythmia. In the absence of ventricular tachycardia, phenotypic risk features will trigger the intensity of screening for arrhythmia. Green boxes indicate green heart consensus statements and yellow boxes indicate yellow heart consensus statements. High risk - sustained VT, polymorphic NSVT, fast (>180 bpm) NSVT, VT/NSVT resulting in syncope. ICD = implantable cardioverter defibrillator; LA = left atrium; LGE = late gadolinium enhancement; LV-EF = left ventricular ejection fraction; MAD = mitral annular disjunction; MV = mitral valve; PVCs = premature ventricular contractions; TWI = T-wave inversion; VT = ventricular tachycardia. #Additional ECG monitoring method may be used such as loop recorders.
Article
Background: Mitral annular disjunction (MAD) dynamic consequences on the mitral apparatus and the left ventricle (LV) remain unclear and are crucial in the context of mitral surgery. Thus, we aimed to assess mitral valvular/annular/ventricular dynamics in mitral valve prolapse (MVP) stratified by MAD presence. Methods: In 61 patients (62±11 years; 25% women) with MVP and severe mitral-regurgitation undergoing mitral-surgery between 2009 and 2016, valvular/annular dimensions/dynamics by 2D transthoracic and 3D-transesophageal-echocardiography, and LV dimensions/dynamics were analyzed stratified by MAD presence, pre and post-surgery. Results: MAD (8±3mm) was diagnosed in 27 (44%) patients (with effective-regurgitant-orifice area of 0.55±0.20cm² and similar to no-MAD), more frequently in bileaflet-prolapse (52 vs. 18% in no-MAD, P=0.004), involving consistently P2 (P=0.005). MAD displayed larger diastolic annular area (1646±410 vs. 1380±348mm²), circumference (150±19 vs. 137±16mm) and intercommissural diameter (48±7 vs. 43±6mm) vs. no-MAD (all P≤0.008). Dynamically, mid- and late-systolic excess inter-commissural diameter, annular area and circumference enlargement were associated with MAD (all P≤0.01). MAD was also associated with dynamically annular slippage, larger prolapse volume and height (P≤0.007), and larger leaflet area (2053±620 vs. 1692±488mm², P=0.01). While MAD vs no-MAD showed similar ejection fraction (65±5 vs 62±8% respectively, P=0.1), systolic basal posterior thickness was increased in MAD (19±2 vs. 15±2mm, P <0.001) with higher systolic thickening of basal posterior wall (74±27 vs. 50±28%) and higher ratio basal wall-thickness/diameter (both P≤0.01). However after mitral repair, MAD disappeared, and LV diameters/wall-thickness/wall-thickening showed no difference in MAD vs. no-MAD (all P≥0.1). Conclusions: MAD in MVP involves a predominant phenotype of bileaflet-MVP, and causes profound annular dynamics alterations with considerable expansion and excess annular enlargement in systole potentially affecting leaflets’ coaptation. MAD myocardial/annular slippage simulates vigorous LV function without true benefit post-surgical annular suture. Thus, while MAD does not hinder the feasibility and quality of valve repair, it requires careful suture of ring to ventricular myocardium lest it may persist postoperatively.
Article
Objectives The aim of this study was to assess in patients with mitral valve prolapse (MVP) mitral annular disjunction (MAD) prevalence, phenotypic characteristics, and long-term outcomes (clinical arrhythmic events and excess mortality). Background Clinical knowledge regarding MAD of MVP remains limited and controversial, and its potential link with untoward outcomes is unsubstantiated. Methods A cohort of 595 (278 women, mean age 61 ± 16 years) consecutive patients with isolated MVP, with comprehensive clinical, rhythmic, Doppler echocardiographic, and consistent MAD assessment, were examined. MAD prevalence, associated MVP phenotypes, and outcomes (survival, clinical arrhythmic events) starting at diagnostic echocardiography were analyzed. To balance important baseline differences, propensity scoring matching was conducted among patients with and those without MAD. Results The presence of MAD was common (n = 186 [31%]) in patients with MVP, generally in younger patients, and was not random but was independently associated with severe myxomatous disease involving bileaflet MVP and marked leaflet redundancy (both P ≤ 0.0002). The presence of MAD was also independently associated with a larger left ventricle (P = 0.005). Age-matched cohort survival after MVP diagnosis was not worse with MAD (10-year survival 93% ± 2% for patients without MAD and 97% ± 1% for those with MAD; P = 0.40), even adjusted comprehensively for MVP characteristics (P = 0.80) and accounting for time-dependent mitral surgery (P = 0.60). During follow-up, 170 patients had clinical arrhythmic events (ventricular tachycardia, n = 159; arrhythmia ablation, n = 14; cardioverter-defibrillator implantation, n = 14; sudden cardiac death, n = 3). MAD was independently associated with higher risk for arrhythmic events (adjusted HR: 2.60; 95% CI: 1.87-3.62; P < 0.0001). The link between MAD and arrhythmic events persisted with time-dependent mitral surgery (adjusted HR: 2.54; 95% CI: 1.84-3.50; P < 0.0001), was strong under medical management (adjusted HR: 3.21; 95% CI: 2.03-5.06; P < 0.0001) but was weaker after mitral surgery (adjusted HR: 2.07; 95% CI: 1.24-3.43; P = 0.005). Conclusions This large cohort with MVP comprehensively characterized shows that MAD is frequent at MVP diagnosis and is strongly linked to advanced myxomatous degeneration. The presence of MAD was independently associated with long-term excess incidence of clinical arrhythmic events. However, within the first 10 years post-diagnosis, MAD was not linked to excess mortality, and although reassurance should be provided from the survival point of view, careful monitoring for arrhythmias is in order for MAD.
Article
Introduction We aimed to assess mitral valvular/annular/ventricular dynamics in mitral valve prolapse (MVP) stratified by mitral annular disjunction (MAD) presence, in the context of mitral surgery. Method In 61 patients (62 ± 11 years; 25% women) with MVP and severe mitral-regurgitation undergoing mitral-surgery between 2009-2016, 2D transthoracic and 4D-transesophageal echocardiographic characteristics and left ventricular (LV) dimensions/dynamics were analyzed, stratified by to MAD presence, pre and post-surgery. Results MAD (8 ± 3 mm) was diagnosed in 27 (44%) patients [ERO of 0.50(0.36–65)cm²], more frequently in bileaflet MVP (52 vs.19%, P = 0.007), involving consistently P2 (P = 0.03). MAD displayed larger diastolic annular area (1646 ± 410 vs. 1380 ± 348 mm², P = 0.008), circumference (150 ± 19 vs. 137 ± 16 mm, P = 0.005) and intercommissural diameter (48 ± 7 vs. 43 ± 6 mm, P = 0.03) vs. no-MAD. Dynamically, early-systolic annular contraction and saddle shape accentuation were unaffected but mid- and late-systolic excess inter-commissural diameter, annular area and circumference enlargement were associated with MAD (all P < 0.0001). MAD was also associated with dynamically larger prolapse volume and height (P ≤ 0.007), with larger leaflet area (2053 ± 620 vs. 1692 ± 488 mm², P = 0.01). LV diastolic dimensions were unaffected by MAD (all P > 0.07). However, despite similar ejection fraction (65 ± 5 vs. 62 ± 8%, P = 0.1), systolic basal posterior thickness was increased in MAD (19 ± 2 vs. 15 ± 2 mm, P < 0.001). After mitral repair, MAD disappeared, and LV diameters/wall-thickness showed no difference in MAD vs. no-MAD (all P > 0.50) (Fig. 1). Conclusion MAD is frequent in MVP, mostly associated with bileaflet/deep MVP and compounds profound annular alterations. MAD myocardial/annular slippage masquerades stronger LV function without benefit post-surgery. Hence, MAD valvular/ventricular alterations warrant careful detection and interpretation.
Article
Background Mitral valve prolapse (MVP) is often considered benign but recent suggestion of an arrhythmic MVP (AMVP) form remains incompletely defined and uncertain. Objectives This study determined ventricular arrhythmia prevalence, severity, phenotypical context, and independent impact on outcome in patients with MVP. Methods A cohort of 595 (age 65 ± 16 years; 278 women) consecutive patients with MVP and comprehensive clinical, arrhythmia (24-h Holter monitoring) and Doppler-echocardiographic characterization, was identified. Long-term outcomes were analyzed. Results Ventricular arrhythmia was frequent (43% with at least ventricular ectopy ≥5%), most often moderate (ventricular tachycardia [VT]; 120 to 179 beats/min) in 27%, and rarely severe (VT ≥180 beats/min) in 9%. Presence of ventricular arrhythmia was associated with male sex, bileaflet prolapse, marked leaflet redundancy, mitral annulus disjunction (MAD), a larger left atrium and left ventricular end-systolic diameter, and T-wave inversion/ST-segment depression (all p ≤ 0.001). Severe ventricular arrhythmia was independently associated with presence of MAD, leaflet redundancy, and T-wave inversion/ST-segment depression (all p < 0.0001) but not with mitral regurgitation severity or ejection fraction. Overall mortality after arrhythmia diagnosis (8 years; 13 ± 2%) was strongly associated with arrhythmia severity (8 years; 10 ± 2% for no/trivial, 15 ± 3% for mild and/or moderate, and 24 ± 7% for severe arrhythmia; p = 0.02). Excess mortality was substantial for severe arrhythmia (univariate hazard ratio [HR]: 2.70; 95% confidence interval [CI]: 1.27 to 5.77; p = 0.01 vs. no/trivial arrhythmia), even after it was comprehensively adjusted, including for MVP characteristics (adjusted HR: 2.94; 95% CI: 1.36 to 6.36; p = 0.006) and by time-dependent analysis (adjusted HR: 3.25; 95% CI: 1.56 to 6.78; p = 0.002). Severe arrhythmia was also associated with higher rates of mortality, defibrillator implantation, VT ablation (adjusted HR: 4.68; 95% CI: 2.45 to 8.92; p < 0.0001), particularly under medical management (adjusted HR: 5.80; 95% CI: 2.75 to 12.23; p < 0.0001), and weakly post-mitral surgery (adjusted HR: 3.69; 95% CI: 0.93 to 14.74; p = 0.06). Conclusions In this large cohort of patients with MVP, ventricular arrhythmia by Holter monitoring was frequent but rarely severe. AMVP was independently associated with phenotype dominated by MAD, marked leaflet redundancy, and repolarization abnormalities. Long-term severe arrhythmia was independently associated with notable excess mortality and reduced event-free survival, particularly under medical management. Therefore, AMVP is a clinical entity strongly associated with outcome and warrants careful risk assessment and well-designed clinical trials.
Article
Importance Malignant arrhythmic mitral valve prolapse (MVP) phenotype poses a substantial risk of sudden cardiac death (SCD), and an estimated 26 000 individuals in the United States are at risk of SCD per year. Thus, identifying risk-stratification strategies for SCD is imperative. Observations Patients with MVP have a heterogenous clinical spectrum, ranging from a benign course to a devastating complication such as SCD. Some of the high-risk markers of MVP, which are identified electrocardiographically, include inverted or biphasic T waves, QT dispersion, QT prolongation, and premature ventricular contractions originating from the left ventricular outflow tract and papillary muscles. Morphofunctional characteristics of SCD are leaflet thickness of 5 mm or greater, mitral annulus disjunction, paradoxical systolic increase of the mitral annulus diameter, increased tissue Doppler velocity of the mitral annulus, and higher mechanical dispersion on echocardiography and fibrosis identified by late gadolinium enhancement on cardiac magnetic resonance imaging. Conclusions and Relevance Findings from this review suggest that SCD can occur earlier in the course of MVP from complex arrhythmias that are triggered by the repeated tugging and traction of the chordopapillary muscle unit and basal mid-myocardium, even before macrofibrosis can be identified in these regions by late gadolinium enhancement on cardiac magnetic resonance imaging. Some of the newer markers identified by speckle-tracking Doppler, such as mechanical dispersion, myocardial work index, and postsystolic shortening, need further validation in a larger population.
Article
Importance Myocardial 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. Objectives To 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 Participants Prospective 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. Exposures Simultaneous cardiac ¹⁸F FDG-PET and MRI with LGE imaging on a hybrid PET/MRI system and ambulatory rhythm monitoring. Main Outcomes and Measures Patients were categorized by the presence and pattern of FDG uptake and LGE, the severity of ventricular arrhythmias, and the indication for mitral valve surgery. Results In 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 Relevance In 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.
Article
Background Mitral valve prolapse (MVP) is a common valve condition and has been associated with sudden cardiac death. Premature ventricular contractions (PVCs) from the papillary muscles (PMs) may play a role as triggers for ventricular fibrillation (VF) in these patients. Objectives To characterize the electrophysiological substrate and outcomes of catheter ablation in patients with MVP and PM PVCs. Methods Of 597 patients undergoing ablation of ventricular arrhythmias during the period 2012‐2015, we identified 25 patients with MVP and PVCs mapped to the PMs (54.7±15.7 years, 64% female, 72% bileaflet). The LVEF was 50.5±11.8% and PVC burden was 24.4±13.1%. A cardiac magnetic resonance imaging was performed in 9 cases and areas of late gadolinium enhancement were found in 4 of them. A detailed LV voltage map was performed in 11 patients, 3 of which exhibited bipolar voltage abnormalities. Complete PVC elimination was achieved in 19 patients (76%), a significant reduction in PVC burden was observed in 2 patients (8%) and no effect was seen in 4 patients (16%). The average follow‐up was 31.5±15.1 months. In patients in which the ablation was successful the PVC burden decreased from 20.4±10.8% to 6.3±9.5% (p=0.001). In 5/6 patients with depressed LVEF and successful ablation, the LV function improved post ablation. No significant differences were identified between patients with and without VF. Conclusions PM PVCs are a source of VF in patients with MVP and can induce PVC‐mediated cardiomyopathy that reverses after PVC suppression. Catheter ablation is highly successful, with >80% PVC elimination or burden reduction. This article is protected by copyright. All rights reserved.
Article
Background A particular phenotype of mitral valve prolapse (MVP) was identified as linked to sudden-death (SD) associating myxomatous, bi-leaflet and prolapse with mitral annular disjunction (MAD), so called ‘severe myxomatous mitral valve disease’ (SMMVP). Purpose Description of the prevalence and the echocardiographic characteristics of SMMVP in a cohort of MVP patients. Methods From a single center echocardiographic database, patients with MVP were retrospectively enrolled from January 2016 to December 2017 and echocardiographic characteristics, based for all patients on reports and additional measurement, were reported. Results Among 101 patients included (female, n = 50; 59 ± 16 years), we reported 71 patients with myxomatous MVP and 30 patients with fibro-elastic deficiency (FED). All patients with MAD (n = 22/101) had myxomatous bi-leaflet MVP (i.e. SMMVP). Compared to FED, SMMVP patients were younger (52 ± 13 vs. 68 ± 13 years, P = 0.02), without flail leaflet (0 vs.7 patients, P < 0.0001), with lower left ventricle (LV) ejection fraction (65 ± 9% vs. 76 ± 5%, P = 0.002) and greater LV end-systolic diameter (31 ± 5 mm vs. 25 ± 5 mm, P = 0.005) as systolic mitral annulus diameter (43 ± 7 vs. 33 ± 4 mm, P = 0.0009). SMMVP patients had, compared to FED and Barlow groups, a deeper MVP (6.5 ± 2.1 mm vs. 3.8 ± 1.2 mm, P = 0.0096; vs. 4.4 ± 1.4 mm, P = 0.009) with thicker distal posterior mitral leaflet (5.5 ± 1.2 mm vs. 3.4 ± 0.8 mm, P 0.004; vs. 4.3 ± 1.6 mm, P = 0.01) and lower mitral regurgitation (MR) (3/22 vs. 22/30 vs. 24/49 patients with severe MR, P < 0.0001). Among 101 MVP, 5 had SD history, all had a SMMVP. SMMVP patients with SD had larger MAD (10.3 ± 1.5 vs. 6.9 ± 1.7 mm, P = 0.007), deeper MVP (8.8 ± 2. vs. 5.1 ± 1.9 mm, P = 0.04) and larger systolic annulus diameter (48 ± 2 vs. 40 ± 6 mm, P = 0.02) than the other SMMVP. Conclusion Among MVP population, SMMVP is an independent MVP entity. This phenotype is all the more specific because it is linked to SD, associating severe deep myxomatous bi-leaflet prolapse with large MAD.
Article
Objectives Mitral valve prolapse (MVP) is commonly observed as a benign finding. However, the literature suggests that it may be associated with sudden cardiac death (SCD). We performed a meta-analysis and systematic review to determine the: (1) prevalence of MVP in the general population; (2) prevalence of MVP in all SCD and unexplained SCD; (3) incidence of SCD in MVP and (4) risk factors for SCD. Methods The English medical literature was searched for: (1) MVP community prevalence; (2) MVP prevalence in SCD cohorts; (3) incidence SCD in MVP and (4) SCD risk factors in MVP. Thirty-four studies were identified for inclusion. This study was registered with PROSPERO (CRD42018089502). Results The prevalence of MVP was 1.2% (95% CI 0.5 to 2.0) in community populations. Among SCD victims, the cause of death remained undetermined in 22.1% (95% CI 13.4 to 30.7); of these, MVP was observed in 11.7% (95% CI 5.8 to 19.1). The incidence of SCD in the MVP population was 0.14% (95% CI 0.1 to 0.3) per year. Potential risk factors for SCD include bileaflet prolapse, ventricular fibrosis complex ventricular ectopy and ST-T wave abnormalities. Conclusion The high prevalence of MVP in cohorts of unexplained SCD despite low population prevalence provides indirect evidence of an association of MVP with SCD. The absolute number of people exposed to the risk of SCD is significant, although the incidence of life-threatening arrhythmic events in the general MVP population remains low. High-risk features include bileaflet prolapse, ventricular fibrosis, ST-T wave abnormalities and frequent complex ventricular ectopy. Trial registration PROSPERO (CRD42018089502).
Article
Background The left ventricular papillary muscles are important components of the mitral valve apparatus. Catheter ablation of ventricular arrhythmias from these sites is challenging. We aim to describe the association between LV papillary muscle ventricular arrhythmias (VAs) and mitral valve prolapse (MVP), and to determine the outcomes of ablation in these patients with a focus on those with MVP and cardiomyopathy. Methods 152 patients underwent 170 consecutive procedures for ablation of focal VAs. Mitral valve prolapse and cardiomyopathy were diagnosed by echocardiography. Outcomes following ablation were assessed in 3 groups: i) LV papillary muscle VAs vs other sites ii) LV papillary muscle VAs by the presence of MVP iii) LV papillary muscle VAs in the setting of cardiomyopathy. Results 9 of 23 (39%) patients undergoing ablation of LV papillary muscle VAs had MVP compared to none of 129 (0%) patients at other sites (p < 0.001). In the former group, acute procedural success was achieved in 60% and 80% of those with and without MVP respectively (p = 0.28). Medium term outcomes were comparable (p = 0.75). In patients with cardiomyopathy, the median LV ejection fraction improved from 40% to 54% following ablation (p = 0.007). Conclusions Although MVP is strongly associated with LV papillary muscle VAs, MVP does not adversely affect the acute or medium‐term outcomes of ablation. Systolic function can improve following ablation in patients with ectopy‐mediated cardiomyopathy due to papillary muscle VAs. This article is protected by copyright. All rights reserved
Article
Background Mitral annular disjunction is a structural abnormality of the mitral annulus fibrosus and is pathologically defined by a separation between the atrial wall–mitral valve junction and the left ventricular attachment. Mitral annular disjunction can cause hypermobility of the mitral valve apparatus and is often associated with mitral valve prolapse (MVP). The aim of this study was to investigate the frequency and characteristics of mitral annular disjunction in the patients referred to an echocardiography laboratory and to compare these with previously reported pathological data. Methods and resultsWe retrospectively studied 1439 patients (mean age 65 ± 17 years, 58% male) referred to our echocardiography laboratory from 6 January 2014 to 31 March 2014. The echocardiographic parameters were compared between the patients with and without mitral annular disjunction. There were 125 cases (8.7%) with mitral annular disjunction, of which 15 (12%) also had MVP. The number of MVP patients in the group with mitral annular disjunction was significantly larger than in the group without mitral annular disjunction (p < 0.0001). The grade of mitral regurgitation was not significantly different between the two groups. Conclusions Mitral annular disjunction was detected not only in patients with a myxomatous mitral valve but also in normal cases. The number of MVPs was significantly larger in patients with mitral annular disjunction than patients without mitral annular disjunction. Further investigation is needed to clarify the clinical significance of the mitral annular disjunction detected by routine echocardiography.
Article
Background: Although the vast majority of mitral valve prolapse (MVP) is benign, a small subset of patients, predominantly women, with bileaflet prolapse, complex ventricular ectopy (VE), and abnormal T waves comprise the recently described bileaflet MVP syndrome. We compared findings on electrophysiological study in bileaflet MVP syndrome patients with and without cardiac arrest to identify factors that may predispose to malignant ventricular arrhythmia. Methods and results: Fourteen consecutive bileaflet MVP syndrome patients (n=13 women; median [limits], age at index ablation, 33.8 [21.0-58.7] years; ejection fraction, 60% [45%-67%]; all ≤ moderate mitral regurgitation; n=6 with previous cardiac arrest and implantable cardioverter defibrillator shocks for ventricular fibrillation; and n=8 without implantable cardioverter defibrillator although with symptomatic complex VE) were included. The 2 groups had similar baseline echocardiographic and electrocardiographic characteristics. All patients had at least 1 left ventricular papillary or fascicular VE focus. Purkinje origin VE was identified as the ventricular fibrillation trigger in 6 of 6 cardiac arrest patients (4 from papillary muscle) and Purkinje origin of dominant VE was seen in 5 of 8 (3 from papillary muscle) nonarrest patients. Acute success was seen in 17 of 19 procedures, and a ventricular fibrillation storm occurred within 24 hours of ablation in a single patient. Repeat ablation for recurrent symptomatic arrhythmia was performed in 6 patients. At 478 (39-2099) days of follow-up, 2 cardiac arrest patients received appropriate shocks. Symptoms from VE were reduced in 12 of 14. Conclusions: Bileaflet MVP syndrome is characterized by fascicular and papillary muscle VE that triggers ventricular fibrillation. Ablation of clinically dominant VE foci improves symptoms and reduces appropriate implantable cardioverter defibrillator shocks.
Article
-Mitral valve prolapse (MVP) may present with ventricular arrhythmias and sudden cardiac death (SCD) even in the absence of hemodynamic impairment. The structural basis of ventricular electrical instability remains elusive. -A) The Cardiac Registry of 650 young adults (≤40 yrs) with SCD was reviewed and cases with MVP as the only cause of SCD were reexamined. Forty-three MVP cases (26 female, age range 19-40, median 32 yrs) were identified (7% of all SCD, 13% of women). Among 12 with available ECG, 10 (83%) had inverted T waves on inferior leads and all right bundle branch block ventricular arrhythmias. A bileaflet involvement was found in 70%. LV fibrosis was detected at histology at the level of papillary muscles in all and infero-basal wall in 88%. B) MVP patients with complex ventricular arrhythmias (N=30) and without (controls, N=14) underwent a study protocol including contrast-enhanced cardiac magnetic resonance (CE-CMR). Patients with complex ventricular arrhythmias (22 female, age range 28-43, median 41 yrs), either right bundle branch block-type or polymorphic, showed a bileaflet involvement in 70% of cases. LV late-enhancement was identified by CE-CMR in 93% vs. 14% of controls (p<0.001), with a regional distribution overlapping the histopathology findings. -MVP is an under-estimated cause of arrhythmic SCD, mostly in young adult women. Fibrosis of papillary muscles and infero-basal LV wall, suggesting a myocardial stretch by the prolapsing leaflet, is the structural hallmark and correlates with ventricular arrhythmias origin. CE-CMR may help to identify this concealed substrate for risk stratification.
Article
Objectives: The aim of this study was to investigate the prevalence of mitral valve prolapse (MVP) and its association with ventricular arrhythmias in a cohort with "unexplained" out-of-hospital cardiac arrest. Background: Ventricular arrhythmias are an important cause of sudden unexpected death in the young. The role of MVP in sudden unexpected death remains controversial. Methods: Of 1,200 patients evaluated between July 2000 and December 2009 in the Mayo Clinic's Long QT Syndrome/Genetic Heart Rhythm Clinic, all 24 (16 women, median age 33.5 years) with idiopathic out-of-hospital cardiac arrest (i.e., negative for ischemia, cardiomyopathy, and channelopathy) were reviewed. Results: All 24 patients had implantable cardioverter-defibrillators (ICDs). Out-of-hospital cardiac arrest was the sentinel event in 22 (92%). Bileaflet MVP was found in 10 (42%). Compared with patients with normal mitral valves, patients with bileaflet MVP: 1) were over-represented by women (9 of 10 [90%] vs. 7 of 14 [50%], p = 0.04); 2) had a higher prevalence of biphasic or inverted T waves (7 of 9 [77.8%] vs. 4 of 14 [29%], p = 0.04); and 3) on Holter interrogation had higher prevalence of ventricular bigeminy (9 of 9 [100%] vs. 1 of 10 [10%], p < 0.0001), ventricular tachycardia (7 of 9 [78%] vs. 1 of 10 [10%], p = 0.006), and premature ventricular contractions originating from the outflow tract alternating with the papillary muscle or fascicular region (7 of 9 [78%] vs. 2 of 10 [20%], p = 0.02). Over a median 1.8 years (range: 0.1 to 11.9 years) from ICD placement, 13 of 24 patients (54%) received appropriate ventricular fibrillation-terminating ICD shocks. Only bileaflet MVP was associated with ventricular fibrillation recurrences requiring ICD therapy on follow-up (logistic regression odds ratio: 7.2; 95% confidence interval: 1.1 to 48; p = 0.028). Conclusions: The authors describe a "malignant" subset of patients with MVP who experienced life-threatening ventricular arrhythmias. This phenotype is characterized by bileaflet MVP, female sex, and frequent complex ventricular ectopic activity, including premature ventricular contractions of the outflow tract alternating with papillary muscle or fascicular origin.
Article
The mitral annulus plays an important role in leaflet coaptation, in unloading mitral valve closing forces, and in promoting left atrial and left ventricular filling and emptying. Perturbations of annular mechanics figure prominently in a number of disorders including functional and ischemic mitral regurgitation, mitral valve prolapse, atrial fibrillation, mitral annular calcification, and annular submitral aneurysm. This review discusses the role of annular dysfunction in the pathogenesis of these disorders.
Article
A prospective long-term follow-up study was made of 300 patients with idiopathic mitral valve prolapse, diagnosed by clinical, cineangiographic and echocardiographic criteria. There were 136 male and 164 female patients, ranging in age from 10 to 87 years (mean 42.2). The study included all patients with primary mitral valve prolapse, irrespective of clinical condition at the onset, and excluded only those patients with "secondary" mitral valve prolapse attributable to an accompanying established disorder. The average follow-up period was 6.1 years (range 6 months to 20 years). Two patients died of a noncardiac cause. The clinical condition of 153 patients remained stable. In 27 patients a supraventricular tachycardia occurred that was readily controlled with medication and caused no serious clinical complications. In 20 patients signs of mitral regurgitation appeared, but the patients remained clinically asymptomatic. Serious complications developed in 100 patients. Sudden death, most likely due to ventricular fibrillation, occurred in three patients; documented ventricular fibrillation was seen in two. Ventricular tachycardia developed in 56 patients, but in all instances the rhythm disorder was managed effectively and durably with medication. Infective endocarditis occurred in 18 patients, 4 of whom died during treatment and 6 of whom needed mitral valve replacement. The remaining eight patients suffer from severe mitral regurgitation that will require surgery in the near future. Twenty-eight patients underwent mitral valve operation because of progressive regurgitation. Cerebrovascular accidents occurred in 11 patients, but lifelong treatment with coumarin derivatives or antiplatelet aggregation agents was not considered necessary.(ABSTRACT TRUNCATED AT 250 WORDS)
Article
Floppy mitral valve is usually attributed to connective-tissue degeneration. However, we have observed several instances in which both a floppy mitral valve and an abnormal mitral annulus fibrosus were present at autopsy. To study this association, we examined 900 hearts (after postmortem arteriography and fixation in distention) from autopsies of adults at The Johns Hopkins Hospital. Twenty-five (3 percent) of the hearts had a morphologically typical floppy mitral valve; in 23 of them (92 percent), the mitral annulus fibrosus showed disjunction--i.e., a separation between the atrial wall-mitral valve junction and the left ventricular attachment. In 42 other hearts (5 percent), which were from significantly younger patients (mean age [+/- SE], 60 +/- 2 years vs. 68 +/- 3; P less than 0.05), there was mitral annulus disjunction but no floppy mitral valve. Two hearts had a floppy mitral valve but no disjunction of the annulus; both of them had old infarcts of the papillary muscle. Our results show that floppy mitral valve is significantly associated with disjunction of the mitral annulus fibrosus (P less than 0.001). We suggest that floppy mitral valve develops from hypermobility of the valve apparatus, and that it is usually secondary to disjunction of the mitral annulus fibrosus, an anatomic variation in the morphology of the annulus.
Article
We determined the long-term prognosis for patients with mitral-valve prolapse documented by echocardiography by following 237 minimally symptomatic or asymptomatic patients for a mean of 6.2 years (range, 1 to 10.4). The actuarial eight-year probability of survival was 88 per cent, which is not significantly different from that for a matched control population. An initial left ventricular diastolic dimension exceeding 60 mm was the best echocardiographic predictor of the subsequent need for mitral-valve replacement (17 patients). Of the 97 patients with redundant mitral-valve leaflets identified echocardiographically, 10 (10.3 per cent) had sudden death, infective endocarditis, or a cerebral embolic event; in contrast, of the 140 patients with nonredundant valves, only 1 (0.7 per cent) had such complications (P less than 0.001). Most patients with echocardiographic evidence of mitral-valve prolapse have a benign course, but subsets at high risk for the development of progressive mitral regurgitation, sudden death, cerebral embolic events, or infective endocarditis can be identified by echocardiography.
Article
To describe the epidemiology of echocardiographic mitral valve prolapse (MVP) and its anthropometric, physiologic, and psychobehavioral correlates with a cross-sectional analysis at 4 urban clinical centers. A biethnic, community-based sample of 4136 young (aged 23 to 35 years) adult participants in the Coronary Artery Risk Development in Young Adults (CARDIA) study who had echocardiograms during their third examination between 1990 and 1991. Echocardiographic mitral valve prolapse, Doppler mitral regurgitation, blood pressure, anthropometry, and 4 psychobehavioral scales. Definite echocardiographic MVP prevalence was 0.6% overall and was similar across the 4 ethnicity/sex groups. Most participants (21 of 26, 80%) with definite echocardiographic MVP were unaware of their condition. Relative to persons with normal echocardiograms, those with echocardiographic MVP were taller (174.6 cm vs 171.0 cm, P <.01), leaner (26.7 mm vs 37.4 mm sum of triceps and subscapular skinfolds, P <.01), had lower body mass index (22.0 kg/m(2) vs 26.2 kg/m(2), P <.01), and more often has Doppler mitral regurgitation (34.8% vs 11. 8%, P <.01). Women with echocardiographic MVP had higher ethnicity-adjusted hostility scores (19.9 vs 16.1, P <.05) than women with no MVP. Among 111 (2.7%) of 4136 participants reporting prior physician diagnosis of MVP, only 5 (0.45%) of 111 had definite echocardiographic MVP. These data document a low prevalence of definite echocardiographic MVP and suggest a constellation of anthropometric, physiologic, and psychobehavioral characteristics in young adults with echocardiographic MVP. Most definite echocardiographic MVP diagnoses were discordant with self-reported MVP status, and false-positive diagnoses of echocardiographic MVP were made more often in women and whites.
Article
Mitral annular disjunction is a structural abnormality of the mitral annulus fibrosus described by pathologists in association with mitral leaflet prolapse and defined as a separation between the atrial wall-mitral valve (MV) junction and the left ventricular attachment allowing for hypermobility of the MV apparatus. The transesophageal echocardiographic characteristics of this abnormality have not been previously described. In patients undergoing MV repair for myxomatous MV degeneration and evaluated using a standardized transesophageal echocardiographic protocol, annular disjunction (mean value 10 +/- 3 mm) was seen at the base of the posterior leaflet in 98% of patients with advanced, and in 9% of patients with mild/moderate MV degeneration. There was a significant correlation between the magnitude of disjunction and the number of segments with prolapse/flail (r = 0.397, P = .001). We found annular disjunction to be a common component of MV apparatus in advanced MV degeneration. Its recognition on transesophageal echocardiography is important to facilitate optimal MV repair. The modification of the repair technique allows surgical correction of the annular disjunction, which seems to optimize long-term results in these challenging cases.
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