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EHRA expert consensus statement on arrhythmic mitral valve prolapse and mitral annular disjunction complex in collaboration with the ESC Council on valvular heart disease and the European Association of Cardiovascular Imaging endorsed cby the Heart Rhythm Society, by the Asia Pacific Heart Rhythm Society, and by the Latin American Heart Rhythm Society
Abstract
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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.
Background
Mitral annular disjunction (MAD) carries an increased risk of complex ventricular arrhythmias, which can lead to sudden cardiac death. Many of these patients undergo implantable cardioverter defibrillator (ICD) implantation, but their ICD outcomes are not known.
Objective
The aim of this study was to assess the outcomes of ICD implantation and the predictors of appropriate ICD therapies in patients with MAD.
Methods
The study included patients with MAD who underwent ICD implantation. Clinical, electrocardiographic, cardiac imaging, and device therapy data were collected.
Results
Forty‐nine patients with MAD and ICD were included. Median age was 49 (21) years, and 29 (59%) were female. 13 (27%) patients underwent ICD implantation for primary prevention and 36 (73%) patients for secondary prevention. Over a median follow‐up of 27.3 (35.3) months, 23 (47%) patients received ICD therapies. 18 (37%) patients had appropriate ICD therapies, and 5 (10%) patients had inappropriate ICD shocks. Median time to first appropriate therapy was 22.3 (63.3) months. In patients with a secondary prevention ICD indication, the rate of appropriate ICD therapies was 44%, while in patients with a primary prevention ICD indication, it was 15%. Among patients with appropriate ICD therapies, the first therapies were delivered for monomorphic ventricular tachycardia (VT) in 7 (39%) patients and polymorphic VT or ventricular fibrillation (VF) in 11 (61%) patients. Patients with appropriate ICD therapies were more likely to have a history of SCA ( p = 0.003) and/or low left ventricular ejection fraction (LVEF) ( p = 0.022) before ICD implantation as compared to patients without appropriate ICD therapies.
Conclusions
In our cohort of patients with MAD and ICD, appropriate ICD therapies were common. Most appropriate ICD therapies were delivered for polymorphic VT or VF. Larger studies are needed to elucidate the mechanisms of VAs and refine risk stratification in MAD.
Sudden cardiac death in a young physically active individual or athlete is a rare but tragic event. Pre-participation screening and follow-up investigations are utilised to reduce the risk and occurrence of these events. Echocardiography plays a key role in the cardiac diagnostic pathway and aims to identify underlying inherited or congenital structural cardiac conditions. In 2013 the British Society of Echocardiography and Cardiac Risk in the Young produced a joint guidance document to support echocardiographers in this setting. The document was subsequently updated in 2018, and it is now timely to provide a further update to the guideline drawing on the advances in our knowledge alongside the developments in ultrasound technology within this nuanced area of sports cardiology.
Graphical Abstract
Background: Over the last two decades, a number of imaging studies have evaluated the characteristics and clinical implications of mitral annular disjunction (MAD) among patients with mitral valve prolapse (MVP). The present systematic review has been primarily designed to summarize the main findings of these studies and to examine the overall impact of MAD in MVP patients. Methods: All imaging studies assessing the prevalence, pathophysiological role and determinants of MAD in MVP individuals, selected from the PubMed and EMBASE databases, were included. There was no limitation in terms of time period. The risk of bias was assessed by using the National Institutes of Health (NIH) Quality Assessment Tool for Observational Cohort and Cross-Sectional Studies. Results: The full texts of 23 studies on 7718 MVP individuals were analyzed. The overall pooled prevalence of MAD in MVP individuals was 40% (range 5.4–90%). When considering the different imaging modalities for assessing MAD, the average MAD prevalence was 20% for cardiac computed tomography studies, 31.3% for transthoracic echocardiography (TTE) studies, 44.7% for transesophageal echocardiography studies and 47% for cardiac magnetic resonance studies. MAD presence was more commonly associated with female sex, young age, narrow antero-posterior thoracic diameter, symptoms of palpitations and syncope, T-wave inversion in inferolateral leads and frequent and/or complex ventricular arrhythmias (VAs) on electrocardiogram, myxomatous leaflets, bileaflet prolapse, larger mitral valve annulus and non-severe mitral regurgitation on TTE. A total of 12 studies (52.2%) provided follow-up data. Over a median follow-up time of 3.9 yrs (range 1–10.3 yrs), MVP individuals with MAD showed increased risk of clinical arrhythmic events, no difference in survival rate and good surgical outcomes. Conclusions: MAD was present in more than one-third of MVP patients, with a wide range of variability depending on the specific imaging method used for assessing MAD presence and on a nonunivocal MAD definition, with a possible overestimation due to Pseudo-MAD rather than True-MAD measurement. A multimodality imaging approach comprehensive of noninvasive chest shape assessment might improve MAD detection among MVP individuals. It appears that careful serial monitoring for VAs should be mandatory for MAD patients.
BACKGROUND
Patients with arrhythmogenic mitral valve prolapse syndrome are at increased risk for life-threatening ventricular arrhythmias, but studies have been limited by small sample sizes. We sought to assemble an international arrhythmogenic mitral valve prolapse syndrome registry to delineate the clinical, imaging, and treatment characteristics of patients with arrhythmogenic mitral valve prolapse syndrome who survived sudden cardiac arrest (SCA) or had sustained ventricular tachycardia (VT) or ventricular fibrillation.
METHODS
In this descriptive registry, we characterized patients with arrhythmogenic mitral valve prolapse syndrome who survived SCA, sustained VT, or ventricular fibrillation. Deidentified data were abstracted locally and combined centrally.
RESULTS
We included 148 patients who had SCA or VT/ventricular fibrillation. Patients had a mean age of 43.7±15.4 years; 68% were women, 73% had bileaflet prolapse, 65% had mitral annular disjunction, 67% had nonsustained VT, and 59% had inferolateral T-wave inversions. Syncope (n=54, 48%) and anterolateral T-wave inversion (n=26, 22%) were relatively common. Catheter ablation was performed in 50 (35%) patients for premature ventricular complexes and in 18 (17.7%) patients for VT. Sites of origin for arrhythmias were commonly in the papillary muscles, fascicles, mitral annulus, and inferior/inferolateral left ventricle.
CONCLUSIONS
In this international descriptive registry of patients with arrhythmogenic mitral valve prolapse syndrome and SCA, patients were young, women, and had bileaflet mitral valve prolapse, mitral annular disjunction, inferolateral T-wave inversions, and nonsustained VT. A history of syncope and anterolateral T-wave inversions was relatively common in patients who survived SCA or sustained VT/ventricular fibrillation.
Mitral valve prolapse (MVP) is a common condition affecting approximately 3% of the population, typically with a benign clinical course. However, a small subset of patients (5–10%) may develop severe mitral regurgitation or arrhythmias, which can lead to sudden cardiac death (SCD). Among the morphological features of MVP, mitral annular disjunction (MAD) has emerged as a potential marker of malignant MVP, with some studies suggesting an association with ventricular arrhythmias and SCD. MAD refers to a structural abnormality where there is a separation between the posterior mitral annulus and the ventricular myocardium, particularly during systole. Initially described in the 1980s, MAD has been primarily studied through echocardiography, although its dynamic nature during the cardiac cycle has complicated its diagnosis. The clinical significance of MAD has been debated, as its presence is not exclusive to pathological MVP, being observed in structurally normal mitral valves as well. Recent research, using advanced imaging techniques such as three-dimensional echocardiography, cardiac magnetic resonance and computed tomography, has provided a more refined understanding of MAD. These studies suggest that MAD can be found in normal hearts, particularly in the posterior mitral annulus, and is often considered a benign anatomical variant. However, the occurrence of MAD in patients with MVP, especially those with leaflet redundancy, has been linked to an increased risk of arrhythmias and SCD. The exact role of MAD in arrhythmogenesis remains unclear, but it is hypothesized that MAD may contribute to electrical instability by altering the mechanical properties of the mitral valve, potentially promoting fibrosis in the surrounding myocardium. Despite these associations, the direct causal role of MAD in SCD requires further investigation, and it may ultimately prove to be an innocent bystander rather than the primary cause of fatal arrhythmias.
Graphical Abstract
Schematic illustration of the current definition of MAD and the new hypothesis
Keratoconus is a progressive eye disease that results in thinning of the cornea, leading to visual impairment. Mitral valve prolapse (MVP) is a common disorder affecting around 2–4% of the general population. Previous studies have found an overrepresentation of MVP in individuals with keratoconus, with a prevalence of 38–65%, suggesting a shared underlying mechanism. In this case-control study, patients with keratoconus were enrolled from a quality and research registry. They were examined by a 2D echocardiography to identify if they had MVP, billowing or normal mitral leaflets. Controls were matched from the population-based Trøndelag Health Study. Patients and controls underwent a detailed echocardiographic examination to detect abnormal mitral valves. We included 101 patients (age 33 [IQR 29–40], 75% male) with keratoconus and 101 matched individuals. MVP was found in 2 (2%), while billowing was found in 5 (5%) of keratoconus patients. No significant association was found between keratoconus and the prevalence of MVP or billowing compared to the control group. Moreover, no associations were found between severity of keratoconus with presence of MVP nor with billowing of the mitral valves. We could not confirm the previously reported association between keratoconus and MVP, suggesting that routine screening for MVP in keratoconus patients may not be warranted. However, we cannot rule out the possibility of an association in other gender, age and ethnic groups different than ours.
BACKGROUND
A subset of patients with mitral valve prolapse (MVP), a highly heritable condition, experience sudden cardiac arrest (SCA) or sudden cardiac death (SCD). However, the inheritance of phenotypic imaging features of arrhythmic MVP remains unknown.
METHODS
We recruited 23 MVP probands, including 9 with SCA/SCD and 14 with frequent/complex ventricular ectopy. Participants underwent interviews, 2-dimensional and speckle-tracking echocardiography, and 48-hour Holter/event monitoring. Each individual was categorized as having a normal mitral valve, MVP, or borderline MVP. We assessed mitral annular disjunction, curling, global longitudinal strain, segmental peak longitudinal strain, electrical/mechanical dispersion, and the postsystolic shortening index in family members and unrelated healthy controls.
RESULTS
We enrolled 23 pedigrees (14 extended pedigrees, 4 trios, and 5 duos) with a total of 121 participants (mean age 45 years, 50% women). Multigenerational SCA/SCD occurred in 2 of 14 extended pedigrees (14%) with an SCA/SCD proband. Mitral annular disjunction was present in 2 generations among 3 families (13%) and absent in 3 of 9 (33%) SCA/SCD cases. Compared with nonarrhythmic cases, arrhythmic MVP cases had more bileaflet involvement, mitral annular disjunction, curling, and abnormal valvular-myocardial mechanics, as expressed by a higher mid-inferior/inferolateral postsystolic shortening index ( P <0.05). Among arrhythmic MVP cases, those with SCA had the highest mechanical dispersion ( P =0.04). Family members with normal valves had lower global longitudinal strain and greater mechanical dispersion compared with nonpedigree controls (both P <0.05).
CONCLUSIONS
In the context of familial MVP, SCA/SCD is rarely observed in multiple generations and is not consistently linked to mitral annular disjunction. Instead, SCA may result from combination of abnormal valvular-myocardial mechanics and a substrate of increased mechanical/electrical dispersion. Family members with normal mitral valves also exhibit mildly abnormal global strain parameters, suggesting an underlying myopathy independent of MVP expression. Future studies are needed to determine whether SCA/SCD in MVP requires the concomitant presence of abnormal mechanics and a primary genetic myopathy.
T wave inversion (TWI) on the electrocardiogram (ECG) is a relatively common finding in athletes. It poses a diagnostic challenge, as it can indicate either a benign physiological pattern or an early sign of serious cardiac pathology. This expert opinion statement provides a comprehensive review of the current understanding of TWI in athletes, emphasizing the importance of its localization, associated clinical features, and demographic factors in guiding its interpretation and management. We explore the potential causes of TWI, including physiological adaptations such as the juvenile pattern and training-induced repolarization variants, as well as pathological conditions like cardiomyopathies, ion channel diseases, and other cardiac abnormalities. Additionally, we discuss the implications of TWI in different ECG leads-anterior, inferior, and lateral-and the diagnostic work-up needed to exclude underlying disease. The importance of follow-up in athletes with TWI is highlighted, particularly for young athletes, to monitor the potential development of cardiomyopathy. Finally, we address considerations for sports eligibility in athletes with TWI, stressing the need for a balanced approach that ensures athlete safety without imposing unnecessary restrictions and investigations.
With a prevalence of 2–3% in the general population, mitral valve prolapse (MVP) is the most common valvular heart disease. The clinical course is benign in the majority of patients, although severe mitral regurgitation, heart failure, and sudden cardiac death affect a non-negligible subset of patients. Imaging of MVP was confined to echocardiography until a few years ago when it became apparent that cardiovascular magnetic resonance (CMR) could offer comparative advantages for detecting and quantifying mitral valve abnormalities alongside tissue myocardial characterization. The present review highlights the growing body of evidence supporting the role of CMR in patients with MVP. Based on the recent literature, CMR appears not as a simple alternative to echocardiography in patients with poor acoustic windows, but as a complementary imaging modality instrumental for better quantifying mitral valve abnormalities, mitral regurgitation severity, ventricular remodeling, and myocardial tissue changes. In this respect, pivotal CMR studies highlight that mitral annular disjunction and myocardial fibrosis by late gadolinium enhancement are associated with a heightened risk of life-threatening ventricular arrhythmias (arrhythmic MVP). We also delineate how these and other markers (e.g., the severity of mitral regurgitation) could enable a personalized risk assessment in patients with MVP and implement clinical decision-making. Here, we provide a comprehensive review of the current literature, with an emphasis on the arrhythmic MVP phenotype. The review also provides some practical suggestions on how to carry out a dedicated CMR protocol in MVP and composes a thorough report to inform clinicians on key aspects of this valvular heart disease.
Mitral annular disjunction (MAD) was a frequent incidental finding at cardiac MRI examinations performed for various clinical indications; longer MAD length and coexisting bileaflet mitral valve prolapse were associated with a higher prevalence of arrhythmia.
Purpose of Review
The purpose of this manuscript is to provide a comprehensive review for the clinician regarding the epidemiology, pathophysiology, diagnosis, and management of patients with mitral valve prolapse and disjunction. We will start the case with an illustrative case which consolidates these points.
Recent Findings
After providing a historical background, we will also update the reader on newer studies in approaching catheter ablation of ventricular arrhythmias in these patients and the impact of surgical repair on outcomes. We then provide our own framework and risk stratification schema to observe, treat, and manage this heterogeneous patient cohort.
Summary
The association of mitral valve dysplasia and ventricular arrhythmias and sudden cardiac death has long been recognized. The most important high-risk features include the presence of thickened mitral valve leaflets with bileaflet prolapse, fast, non-sustained ventricular tachycardia, late gadolinium enhancement in cardiac MRI, and the presence of mitral annular disjunction. Although marked progress has been made in recognizing this entity and managing these patients, more insights into arrhythmia mechanisms and prospective data evaluating the impact of treatments are needed.
Fundamento: Embora seja uma condição comumente benigna, o prolapso de valva mitral (PVM) pode estar associado a risco aumentado de arritmias ventriculares (AV), condição conhecida como prolapso de valva mitral arrítmico (PVMA). Objetivos: Apresentar as diversas manifestações do PVMA por meio de casos clínicos que ilustrem os sintomas, os achados no eletrocardiograma (ECG), no Holter de 24h, no ecocardiograma transtorácico (ETT) e na ressonância magnética cardíaca (RMC) desses pacientes, além de discutir as condutas tomadas diante de desfechos clínicos distintos. Métodos: Estudo retrospectivo, descritivo e observacional que analisou 5 pacientes com PVMA atendidos entre os anos de 2019 e 2024, sendo investigados, nessa amostra, marcadores de risco elevado para desfechos clínicos graves, sobretudo morte súbita cardíaca (MSC). Resultados: Dos 5 pacientes avaliados, a apresentação clínica mais comum foi de palpitações (100% dos casos), seguida por síncopes (40%). Três pacientes (60%) apresentaram arritmia severa ou muito severa no Holter de 24 horas, enquanto 4 (80%) apresentaram disjunção do anel mitral (DAM). Realce tardio positivo foi observado em 2 (40%) pacientes. Em 2 casos (40%), foi indicado implante de cardiodesfibrilador implantável (CDI). Em 1 dos casos, foi optado pela troca valvar mitral, porém sem resolução das arritmias e, por conseguinte, indicada ablação por radiofrequência. Embora descrito na literatura, nenhum caso de MSC foi observado na amostra. Conclusão: O PVMA pode apresentar distintas manifestações clínicas, inclusive com desfechos graves. Identificar os marcadores de risco é essencial para o diagnóstico e tratamento precoces dessa condição, objetivando-se reduzir a mortalidade relacionada à MSC nesses pacientes.
This paper describes the role of cardiovascular magnetic resonance (CMR) imaging in assessing patients with mitral valve disease. Mitral regurgitation (MR) is one of the most prevalent valvular heart diseases. It often progresses without significant symptoms, leading to left ventricular overload, dysfunction, frequent decompensated heart failure episodes, and excess mortality. Cardiovascular magnetic resonance assessment is recommended for MR when routine ultrasound imaging information is insufficient or discordant. A well-planned CMR can provide an in-depth assessment of the mitral valve apparatus, leaflet morphology, and papillary muscles. In addition, it can precisely inform the impact of MR on left atrial and ventricular remodelling. The review aims to highlight established and emerging techniques for morphological assessment, flow assessment (including regurgitation and stenosis), myocardial assessment, and haemodynamic assessment of mitral valve disease by CMR. It also proposes a simplified clinical flow chart for CMR assessment of the mitral valve.
Degenerative mitral valve disease is common. Up to a quarter of patients with degenerative mitral valve disease may be asymptomatic despite having severe valve regurgitation. Current guideline indications for intervention in asymptomatic patient are centred on left ventricular dimensions and ejection fraction and may include consideration in atrial fibrillation, pulmonary hypertension and those with left atrial dilatation. However, despite intervention according to these recommendations, patients remain at risk of post-operative heart failure and mortality. Newer risk markers have been developed including left ventricular and atrial strain, myocardial fibrosis demonstrated using late gadolinium enhancement, mitral annular disjunction and ventricular arrhythmia burden. Translating newer markers into clinical practice will require integrating and identifying high-risk phenotypes that benefit from early intervention using machine learning techniques and artificial intelligence. Valve repair is the recommended intervention. However, repair rate and durability are dependent on both operator and centre volumes as well as valve characteristics. Recent advancements, including robotic surgery, may enhance repair rates; however, larger datasets are necessary to confirm these improvements. Efforts should focus on establishing high-volume regional centres of excellence for mitral valve repair.
The problem of managing of patients with mitral valve prolapse and ventricular arrhythmias — arrhythmogenic mitral valve prolapse — is quite relevant in routine clinical practice, which led to the creation in 2022 of an expert consensuses on the management of such patients. Based on the criteria, it is possible to identify a group of people at high risk of sudden cardiac death and implement measures to prevent death. How to manage patients at moderate risk of sudden cardiac death remains unclear. A clinical case of successful treatment of ventricular arrhythmias in a patient with arrhythmogenic mitral valve prolapse is presented.
We present the case of a 52-year-old man suffering from malignant mitral valve prolapse syndrome. He underwent a right-sided thoracotomy for mitral valve repair but required implantable cardioverter-defibrillator (ICD) implantation 4 years later. He chose the option of a substernal ICD, which was implanted successfully without any complications and good electrical parameters.
Purpose
Mitral valve prolapse (MVP) has been associated with left heart remodeling. This study explored cardiac remodeling in patients with MVP without significant regurgitation, focusing on the right heart.
Methods
This single‐center study enrolled consecutive patients referred to trans‐thoracic‐echocardiography (TTE) with MVP, excluding those with significant regurgitation or known cardiovascular or pulmonary diseases. A group of healthy controls was included.
Results
Forty‐nine patients with MVP and 54 controls were finally selected (mean age of 62, 52–71; 52% males), and echocardiographic parameters were compared among groups. Twenty‐nine (41%) patients with MVP showed tricuspid valve prolapse (TVP). Patients with MVP, irrespective of TVP, showed greater tricuspid annulus (systolic annulus: 31±6 vs. 32±5 vs. 27±3 mm for MVP ⁺ /TVP ⁻ , MVP ⁺ /TVP ⁺ , and controls, respectively; all p < 0.01) and greater minimum right atrial volume indexed (13, 12–15 mL/m ² vs. 15, 12–20 mL/m ² vs. 11, 10–14 mL/m ² ; all p < 0.05). Right ventricular dimensions and systolic indexes did not differ among groups, except TAPSE, which was significantly greater in MVP ⁺ /TVP ⁺ patients compared to controls (25±4 vs. 22±3 mm, p = 0.004). A significant correlation ( ρ = 0.43; p < 0.001) and an independent association at multivariate analysis ( ß = 0.28; 95% CI 0.09–0.47; p = 0.004) were observed between end‐systolic tricuspid diameter and TAPSE.
Conclusion
In patients with MVP with less‐than‐moderate mitral or tricuspid regurgitation, dilation of the right atrium and tricuspid annulus was found. The latter finding was associated with increased values of TAPSE, which should then be used with caution, while other indexes may be preferred when assessing the systolic function of these patients.
Background
Interstitial 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.
Methods
We 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).
Results
We 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).
Conclusion
In 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.
Aims
To address the limitations of traditional diagnostic methods for mitral valve prolapse (MVP), specifically fibroelastic deficiency (FED) and Barlow’s disease (BD), by introducing an automated diagnostic approach utilizing multi-view echocardiographic sequences and deep learning.
Methods and results
An echocardiographic data set, collected from Zhongshan Hospital, Fudan University, containing apical 2 chambers (A2C), apical 3 chambers (A3C), and apical 4 chambers (A4C) views, was employed to train the deep learning models. We separately trained view-specific and view-agnostic deep neural network models, which were denoted as MVP-VS and MVP view-agonistic (VA), for MVP diagnosis. Diagnostic accuracy, precision, sensitivity, F1-score, and specificity were evaluated for both BD and FED phenotypes. MVP-VS demonstrated an overall diagnostic accuracy of 0.94 for MVP. In the context of BD diagnosis, precision, sensitivity, F1-score, and specificity were 0.83, 1.00, 0.90, and 0.92, respectively. For FED diagnosis, the metrics were 1.00, 0.83, 0.91, and 1.00. MVP-VA exhibited an overall accuracy of 0.95, with BD-specific metrics of 0.85, 1.00, 0.92, and 0.94 and FED-specific metrics of 1.00, 0.83, 0.91, and 1.00. In particular, the MVP-VA model using mixed views for training demonstrated efficient diagnostic performance, eliminating the need for repeated development of MVP-VS models and improving the efficiency of the clinical pipeline by using arbitrary views in the deep learning model.
Conclusion
This study pioneers the integration of artificial intelligence into MVP diagnosis and demonstrates the effectiveness of deep neural networks in overcoming the challenges of traditional diagnostic methods. The efficiency and accuracy of the proposed automated approach suggest its potential for clinical applications in the diagnosis of valvular heart disease.
Background
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.
Case summary
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.
Discussion
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.
Mitral valve prolapse is a well-known condition that is generally benign, but it can be associated with cardiac arrhythmias, particularly malignant ventricular arrhythmias and sudden cardiac death. This association and its outcome have been described in medical literature, but the low incidence leads to a lack of evidence regarding its management and stratification.The case of a young woman is presented, whose initial manifestation was syncope, with subsequent development of severe mitral insufficiency, followed by severe mitral regurgitation and the discovery of frequent ventricular extrasystoles. She underwent surgical repair with mitral valve plasty and cryoablation of the anterior papillary muscle. The clinical outcome was favorable, as evidenced by an improvement in symptoms and control of arrhythmia burden.
Mitral valve prolapse is a common valve disorder that usually has a benign prognosis unless there is significant regurgitation or LV impairment. However, a subset of patients are at an increased risk of ventricular arrhythmias and sudden cardiac death, which has led to the recognition of “arrhythmic mitral valve prolapse” as a clinical entity. Emerging risk factors include mitral annular disjunction and myocardial fibrosis. While echocardiography remains the primary method of evaluation, cardiac magnetic resonance has become crucial in managing this condition. Cine magnetic resonance sequences provide accurate characterization of prolapse and annular disjunction, assessment of ventricular volumes and function, identification of early dysfunction and remodeling, and quantitative assessment of mitral regurgitation when integrated with flow imaging. However, the unique strength of magnetic resonance lies in its ability to identify tissue changes. T1 mapping sequences identify diffuse fibrosis, in turn related to early ventricular dysfunction and remodeling. Late gadolinium enhancement sequences detect replacement fibrosis, an independent risk factor for ventricular arrhythmias and sudden cardiac death. There are consensus documents and reviews on the use of cardiac magnetic resonance specifically in arrhythmic mitral valve prolapse. However, in this article, we propose an algorithm for the broader use of cardiac magnetic resonance in managing this condition in various scenarios. Future advancements may involve implementing techniques for tissue characterization and flow analysis, such as 4D flow imaging, to identify patients with ventricular dysfunction and remodeling, increased arrhythmic risk, and more accurate grading of mitral regurgitation, ultimately benefiting patient selection for surgical therapy.
Ventricular arrhythmias associated with mitral valve prolapse (MVP) and the capacity to cause sudden cardiac death (SCD), referred to as ‘malignant MVP’, are an increasingly recognised, albeit rare, phenomenon. SCD can occur without significant mitral regurgitation, implying an interaction between mechanical derangements affecting the mitral valve apparatus and left ventricle. Risk stratification of these arrhythmias is an important clinical and public health issue to provide precise and targeted management. Evaluation requires patient and family history, physical examination and electrophysiological and imaging-based modalities. We provide a review of arrhythmogenic MVP, exploring its epidemiology, demographics, clinical presentation, mechanisms linking MVP to SCD, markers of disease severity, testing modalities and management, and discuss the importance of risk stratification. Even with recently improved understanding, it remains challenging how best to weight the prognostic importance of clinical, imaging and electrophysiological data to determine a clear high-risk arrhythmogenic profile in which an ICD should be used for the primary prevention of SCD.
Mitral annular disjunction (MAD) is defined as a separation between the mitral annulus and the left ventricular myocardium and is most often seen in association with mitral valve prolapse (MVP). MAD has been linked to ventricular arrhythmias in adults, independent of MVP. However, it has rarely been reported in children. We, hereby, report two cases of MAD associated with a large atrial septal defect (ASD). Thus far, there are no consensus guidelines for the management of MAD. The additional association of large ASD further complicates the decision-making in these patients. To the best of our knowledge, this is the first report of the association of MAD with ASD. We further discuss the challenges in the management of this condition.
Mitral valve prolapse (MVP) affects 2–3% of the general population, and despite its benign prognosis overall, it is associated with sudden death in a small subset of patients. The term “arrhythmic MVP syndrome” (AMVPS) refers to the presence of frequent or complex ventricular arrhythmias, commonly reported in female patients with a stereotypical phenotype including bileaflet myxomatous disease, ECG repolarisation abnormalities in inferior leads, mitral annular disjunction, and significant fibrosis in the inferolateral LV and papillary muscles. Modern imaging technologies have led to the identification of new risk factors that have been implemented in recent risk stratification guidelines; however, screening for patients with MVP who are at risk of sudden cardiac death (SCD) remains challenging. In addition, there is a limited amount of data on the outcomes of different treatment approaches in AMVP and no specific indication for targeted or disease-modifying therapies within current guidelines. Potential arrhythmic substrates in patients with AMVP syndrome have been the subject of interest in previous studies, with areas consisting of fibrosis at the papillary muscle level and the Purkinje system. Premature ventricular contractions (PVCs) originating from these areas have been shown to play an important role as triggers for ventricular fibrillation and SCD in patients with AMVP. Catheter ablation has emerged as a potential treatment modality in patients with MVP and ventricular arrhythmias (VAs), targeting arrhythmic substrates and triggering PVC foci. The aim of this review is to explore the role of catheter ablation in treating patients with AMVP.
Purpose
During the last decade, a number of echocardiographic studies have employed speckle tracking echocardiography (STE) for assessing myocardial deformation properties in individuals with mitral valve prolapse (MVP), reporting not univocal results. Accordingly, we performed a systematic review and meta-analysis to summarize the main findings of these studies and to examine the overall influence of MVP on left ventricular (LV) global longitudinal strain (GLS).
Methods
All echocardiographic studies assessing conventional echoDoppler parameters and myocardial strain indices in MVP individuals vs. controls without MVP, selected from PubMed and EMBASE databases, were included. The risk of bias was assessed by using the National Institutes of Health (NIH) Quality Assessment of Case-Control Studies. Continuous data (LV-GLS) were pooled as a standardized mean difference (SMD) comparing MVP group with healthy controls. The overall SMD of LV-GLS was calculated using the random-effect model.
Results
The full-texts of 15 studies with 1088 individuals with MVP and 591 healthy controls were analyzed. Average LV-GLS magnitude was significantly, even though modestly, reduced in MVP individuals in comparison to controls (19.4 ± 3.4% vs. 21.1 ± 2.8%, P < 0.001). The overall effect of MVP on LV-GLS was small-to-medium (SMD − 0.54, 95%CI -0.76,-0.32, P < 0.001). Substantial heterogeneity was detected for the included studies, with an overall I² statistic value of 75.9% (P < 0.001). Egger’s test for a regression intercept gave a P-value of 0.58, indicating no publication bias. On meta-regression analysis, none of the moderators (the age, the percentage of females among MVP individuals, body mass index, heart rate and systolic blood pressure of MVP individuals, the degree of mitral regurgitation, the type of ultrasound machine employed for strain echocardiographic imaging and finally the beta blocker treatment) was significantly associated with effect modification (all P < 0.05). Regional strain analysis, performed by two-third of the studies, highlighted a more enhanced reduction in myocardial strain parameters at level of the LV basal infero-lateral segments in all directions (longitudinal, circumferential and radial), with apical sparing.
Conclusions
The longitudinal strain impairment detected in MVP individuals is more regional than global, with peculiar involvement of the LV basal infero-lateral segments and relative apical sparing pattern.
Background
The presence of mitral annulus disjunction (MAD) has been considered a high-risk feature for sudden cardiac death based on selected study populations. We aimed to assess the prevalence of MAD in consecutive patients undergoing clinically indicated cardiovascular magnetic resonance (CMR), its association with ventricular arrhythmias, mitral valve prolapse (MVP), and other CMR features.
Methods
This single-center retrospective study included consecutive patients referred to CMR at our institution between June 2021 and November 2021. MAD was defined as a ≥1 mm displacement between the left atrial wall-mitral valve leaflet junction and the left ventricular wall during end-systole. MAD extent was defined as the maximum longitudinal displacement. Associates of MAD were evaluated at univariable and multivariable regression analysis. The study endpoint, a composite of (aborted) sudden cardiac death, unexplained syncope, and sustained ventricular tachycardia, was evaluated at a 12-month follow-up.
Results
Four hundred and forty-one patients 55 ± 18 years, 267/441 (61%) males) were included, and 29/441 (7%) had MVP. The prevalence of MAD ≥1 mm, 4 mm, and 6 mm was 214/441 (49%), 63/441 (14%), and 15/441 (3%), respectively. Patients with MVP showed a higher prevalence of MAD greater than 1 mm (26/29 (90%) vs 118/412 (46%)); p < 0.001), 4 mm (14/29 (48%) vs 49/412 (12%)); p < 0.001), and 6 mm (3/29 (10%) vs 12/412 (3%)); p = 0.03), and a greater MAD extent (4.2 mm, 3.0–5.7 mm vs 2.8 mm, 1.9–4.0 mm; p < 0.001) compared to patients without MVP. MVP was the only morpho-functional abnormality associated with MAD at multivariable analysis (p < 0.001). A high burden of ventricular ectopic beats at baseline Holter-electrocardiogram was associated with MAD ≥4 mm and MAD extent (p < 0.05). The presence of MAD ≥1 mm (0.9% vs 1.8%; p = 0.46), MAD ≥4 mm (1.6% vs 1.3%; p = 0.87), or MVP (3.5% vs 1.2%; p = 0.32) were not associated with the study endpoint, whereas patients with MAD ≥6 mm showed a trend toward a higher likelihood of the study endpoint (6.7% vs 1.2%; p = 0.07).
Conclusion
MAD of limited severity was common in consecutive patients undergoing CMR. Patients with MVP showed higher prevalence and greater extent of MAD. Extended MAD was rarer and showed association with ventricular arrhythmias at baseline. The mid-term prognosis of MAD seems benign; however, prospective studies are warranted to search for potential “malignant MAD extents” to improve patients’ risk stratification.
Introduction
Malignant ventricular arrhythmia (VA) and sudden cardiac death (SCD) have been reported in patients with mitral valve prolapse (MVP); however, effective risk stratification methods are still lacking. Myocardial fibrosis is thought to play an important role in the development of VA; however, observational studies have produced contradictory findings regarding the relationship between VA and late gadolinium enhancement (LGE) in MVP patients. The aim of this meta‐analysis and systematic review of observational studies was to investigate the association between left ventricular LGE and VA in patients with MVP.
Methods
We searched the PubMed, Embase, and Web of Science databases from 1993 to 2023 to identify case–control, cross‐sectional, and cohort studies that compared the incidence of VA in patients with MVP who had left ventricular LGE and those without left ventricular LGE.
Results
A total of 1464 subjects with MVP from 12 observational studies met the eligibility criteria. Among them, VA episodes were reported in 221 individuals (15.1%). Meta‐analysis demonstrated that the presence of left ventricular LGE was significantly associated with an increased risk of VA (pooled risk ratio 2.96, 95% CI: 2.26−3.88, p for heterogeneity = 0.07, I² = 40%). However, a meta‐regression analysis of the prevalence of mitral regurgitation (MR) showed that the severity of MR did not significantly affect the association between the occurrence of LGE and VA (p = 0.079).
Conclusion
The detection of LGE could be helpful for stratifying the risk of VA in patients with MVP.
Background
Some patients affected by mitral valve prolapse (MVP) are at higher risk of ventricular arrhythmias (VA), but the underlying pathogenesis, as well as the effects of surgery on VA, remain not fully understood. Mitral valve (MV) repair, however, represents a privileged point of view to deepen the understanding of arrhythmogenesis in this context. Hence, we report an interesting case of MV re- repair.
Case Summary
A 52-year-old man was referred to our Institution for severe mitral regurgitation (MR) due to P2 prolapse in the context of myxomatous MV degeneration. Pre-operative imaging showed systolic mitral annular disjunction, left ventricular (LV) wall curling, Pickelhaube sign and a prolapsing tricuspid valve (TV) with only mild regurgitation. 24-hour ECG Holter revealed a significant burden of premature ventricular contractions (PVCs), most of them originating from anterior papillary muscle (APM), posterior papillary muscle (PPM) and mitral annulus (MA). Quadrangular resection of P2 and mitral annuloplasty were performed. One year later, relapse of severe MR due to a residual P2M1 prolapse occurred. 24-hour ECG Holter showed no PVCs from PPM and MA, while those from APM persisted. A central edge-to-edge repair was effectively used to fix the residual prolapse. After one year from REDO surgery, a third ECG Holter confirmed the absence of any remaining LV PVCs, but still few ectopic beats originating from TV were recorded.
Discussion
Here we report a case of VA resolution after specific, anatomical triggers-addressing surgical gestures. Our experience confirms that MV surgery may have a role in MVP patients’ arrhythmias correction.
Purpose To use unsupervised machine learning to identify phenotypic clusters with increased risk of arrhythmic mitral valve prolapse (MVP). Materials and Methods This retrospective study included patients with MVP without hemodynamically significant mitral regurgitation or left ventricular (LV) dysfunction undergoing late gadolinium enhancement (LGE) cardiac MRI between October 2007 and June 2020 in 15 European tertiary centers. The study end point was a composite of sustained ventricular tachycardia, (aborted) sudden cardiac death, or unexplained syncope. Unsupervised data-driven hierarchical k-mean algorithm was utilized to identify phenotypic clusters. The association between clusters and the study end point was assessed by Cox proportional hazards model. Results A total of 474 patients (mean age, 47 years ± 16 [SD]; 244 female, 230 male) with two phenotypic clusters were identified. Patients in cluster 2 (199 of 474, 42%) had more severe mitral valve degeneration (ie, bileaflet MVP and leaflet displacement), left and right heart chamber remodeling, and myocardial fibrosis as assessed with LGE cardiac MRI than those in cluster 1. Demographic and clinical features (ie, symptoms, arrhythmias at Holter monitoring) had negligible contribution in differentiating the two clusters. Compared with cluster 1, the risk of developing the study end point over a median follow-up of 39 months was significantly higher in cluster 2 patients (hazard ratio: 3.79 [95% CI: 1.19, 12.12], P = .02) after adjustment for LGE extent. Conclusion Among patients with MVP without significant mitral regurgitation or LV dysfunction, unsupervised machine learning enabled the identification of two phenotypic clusters with distinct arrhythmic outcomes based primarily on cardiac MRI features. These results encourage the use of in-depth imaging-based phenotyping for implementing arrhythmic risk prediction in MVP. Keywords: MR Imaging, Cardiac, Cardiac MRI, Mitral Valve Prolapse, Cluster Analysis, Ventricular Arrhythmia, Sudden Cardiac Death, Unsupervised Machine Learning Supplemental material is available for this article. © RSNA, 2024.
Background
Pedometer-based walking programs hold promise as a health promotion strategy for stroke prevention in community-dwelling older adults, particularly when targeted at physical activity-related modifiable risk factors. The question arises: What is the effectiveness of pedometer-based walking program interventions in improving modifiable stroke risk factors among community-dwelling older adults?
Method
Eight databases were searched up to December 2nd, 2023, following the Preferred Reporting Items for Systematic Review and Meta-Analysis protocol. Inclusion criteria focused on randomized controlled trials (RCTS) involving community-dwelling older adults and reported in English. Two independent reviewers utilized Physiotherapy Evidence Database (PEDro) tool to extract data, assess eligibility, evaluate study quality, and identify potential bias. Standardized mean difference (SMD) was employed as summary statistics for primary —physical activity level —and secondary outcomes related to cardiovascular function (blood pressure) and metabolic syndrome, including obesity (measured by body mass index and waist circumference), fasting blood sugar, glycated hemoglobin, high-density lipoprotein cholesterol (HDL-C), and triglycerides. A random-effects model was used to generate summary estimates of effects.
Results
The review analyzed eight studies involving 1546 participants aged 60-85 years, with 1348 successfully completing the studies. Across these studies, pedometer-based walking programs were implemented 2-3 times per week, with sessions lasting 40-60 minutes, over a duration of 4-26 weeks. The risk of bias varied from high to moderate. Our narrative synthesis revealed positive trends in HDL-C levels, fasting blood sugar, and glycated hemoglobin, suggesting improved glycemic control and long-term blood sugar management. However, the impact on triglycerides was only marginal. Primary meta-analysis demonstrated significantly improved physical activity behavior (SMD=0.44,95%CI:0.26, 0.61,p=<0.00001;I²=0%;4 studies; 532 participants) and systolic blood pressure (SMD=-0.34,95%CI:-0.59,-0.09;p=<0.008;I²=65%,2 studies;249 participants), unlike diastolic blood pressure (SMD=0.13,95%CI:-0.13,-0.38,p=0.33; I²=91%; 2 studies; 237 participants). Interventions based on social cognitive, self-efficacy, and self-efficiency theory(ies), and social cognitive theory applied in an ecological framework, were linked to successful physical activity behavior outcomes.
Conclusion
Pedometer-based walking programs, utilizing interpersonal health behavior theory/ecological framework, enhance physical activity behavior and have antihypertensive effects in community-dwelling older adults. While they do not significantly affect diastolic blood pressure, these programs potentially serve as a primary stroke prevention strategy aligning with global health goals.
Trial registration
Registration Number: INPLASY202230118
Premature ventricular contraction (PVC), a common arrhythmia affecting 1–2% of the general population, has been considered to have a benign clinical course. However, people with PVC often develop heart failure and ventricular arrhythmias such as ventricular tachycardia. We aimed to clarify the risk of heart failure and lethal ventricular arrhythmias in people with PVC. The Korean National Health Insurance Service database was used for this study. People who underwent nationwide health check-ups in 2009 were enrolled in this study and clinical follow-up data until December 2018 were analyzed. Newly diagnosed PVC in 2009 (≥ 1 inpatient or outpatient claim) were identified and cumulative incidence of heart failure (≥ 1 inpatient claim) and ventricular arrhythmias (≥ 1 inpatient or outpatient claim) were compared. A total of 4515 people were first diagnosed with PVC in 2009 among 9,743,582 people without prior history of PVC, heart failure, or ventricular arrhythmias. People with newly diagnosed PVC in 2009 had a significantly higher incidence of heart failure compared to those without PVC [adjusted hazard ratio (HR) 1.371; 95% confidence interval (CI) 1.177–1.598; p < 0.001]. Significant interaction was observed between age and PVC with young age people at greater risk of developing heart failure for having PVC. The incidence of ventricular arrhythmia was also significantly increased in people with PVC (HR 5.588; 95% CI 4.553–6.859; p < 0.001). Age and chronic kidney disease had significant interactions with PVC. In conclusion, the incidence of heart failure and ventricular arrhythmia was significantly increased in people with PVC. Outpatient follow-up of people with PVC can be helpful to detect early signs of heart failure or advanced forms of ventricular arrhythmia.
Mitral valve prolapse (MVP) associated with severe mitral regurgitation is a debilitating disease with no pharmacological therapies available. MicroRNAs (miRNA) represent an emerging class of circulating biomarkers that have never been evaluated in MVP human plasma. Our aim was to identify a possible miRNA signature that is able to discriminate MVP patients from healthy subjects (CTRL) and to shed light on the putative altered molecular pathways in MVP. We evaluated a plasma miRNA profile using Human MicroRNA Card A followed by real-time PCR validations. In addition, to assess the discriminative power of selected miRNAs, we implemented a machine learning analysis. MiRNA profiling and validations revealed that miR-140-3p, 150-5p, 210-3p, 451a, and 487a-3p were significantly upregulated in MVP, while miR-223-3p, 323a-3p, 340-5p, and 361-5p were significantly downregulated in MVP compared to CTRL (p ≤ 0.01). Functional analysis identified several biological processes possible linked to MVP. In addition, machine learning analysis correctly classified MVP patients from CTRL with high accuracy (0.93) and an area under the receiving operator characteristic curve (AUC) of 0.97. To the best of our knowledge, this is the first study performed on human plasma, showing a strong association between miRNAs and MVP. Thus, a circulating molecular signature could be used as a first-line, fast, and cheap screening tool for MVP identification.
Aims
We aimed to assess the prevalence of mitral annulus disjunction (MAD) and to explore the association with aortic disease and mitral valve surgery in patients with Marfan syndrome (MFS) and Loeys–Dietz syndrome (LDS).
Methods and results
We included consecutive MFS patients fulfilling Revised Ghent Criteria and LDS patients fulfilling Loeys–Dietz Revised Nosology. MAD was identified by echocardiography and was quantified as the longitudinal distance from the ventricular myocardium to the hinge point of the posterior mitral leaflet. Aortic events were defined as aortic dissection or prophylactic aortic surgery. We recorded the need of mitral valve surgery including mitral valve repair or replacement. We included 168 patients (103 with MFS and 65 with LDS). The prevalence of MAD was 41%. MAD was present in all age groups. Aortic events occurred in 112 (67%) patients (27 with dissections and 85 with prophylactic surgical interventions). Patients with MAD were younger at aortic event than those without MAD (log rank = 0.02) Patients with aortic events had greater MAD distance in posterolateral wall [8 (7–10) mm vs. 7 (6–8) mm, P = 0.04]. Mitral events occurred more frequently in patients with MAD (P < 0.001).
Conclusion
MAD was highly prevalent in patients with MFS and LDS. MAD was a marker of severe disease including aortic events at younger age and need of mitral valve surgery. Screening patients with MFS an LDS for MAD may provide prognostic information and may be relevant in planning surgical intervention. Detection of MAD in patients with MFS and LDS may infer closer clinical follow-up from younger age.
This international multidisciplinary document intends to provide clinicians with evidence-based practical patient-centered recommendations for evaluating patients and decedents with (aborted) sudden cardiac arrest and their families. The document includes a framework for the investigation of the family allowing steps to be taken, should an inherited condition be found, to minimize further events in affected relatives. Integral to the process is counseling of the patients and families, not only because of the emotionally charged subject, but because finding (or not finding) the cause of the arrest may influence management of family members. The formation of multidisciplinary teams is essential to provide a complete service to the patients and their families, and the varied expertise of the writing committee was formulated to reflect this need. The document sections were divided up and drafted by the writing committee members according to their expertise. The recommendations represent the consensus opinion of the entire writing committee, graded by Class of Recommendation and Level of Evidence. The recommendations were opened for public comment and reviewed by the relevant scientific and clinical document committees of the Asia Pacific Heart Rhythm Society (APHRS) and the Heart Rhythm Society (HRS); the document underwent external review and endorsement by the partner and collaborating societies. While the recommendations are for optimal care, it is recognized that not all resources will be available to all clinicians. Nevertheless, this document articulates the evaluation that the clinician should aspire to provide for patients with sudden cardiac arrest, decedents with sudden unexplained death, and their families.
In bileaflet mitral valve prolapse (BMVP) systolic leaflet displacement creates a pocket of blood on the left ventricular (LV) side of the leaflets, but on the atrial side of the annulus. This blood is excluded from the LV end-systolic volume if the mitral valve annulus is used to determine the most basal extent of the LV. The purpose of this study is to describe the quantitative implications of defining the LV base on mitral regurgitant severity and LV systolic function in BMVP. In 30 consecutive patients (53% male, 58 ± 14 years) with BMVP, LV endocardial and epicardial borders were determined from SSFP images. The LV base at end-systole was defined by the “Functional” method (at the mitral valve annulus) or the “Anatomic” method (at the mitral valve leaflets). Regurgitant volume was the difference between the LV stroke volume and mean forward flow. LV myocardial strain measurements were determined from the short axis endocardial and epicardial borders. The “Functional” method resulted in higher regurgitant volumes (mean difference: 22 ml, range 0–40 ml) and higher ejection fractions (mean difference: 9%, range 0–21%). The correlation between LV end-diastolic volume and regurgitant volume was better with the “Functional” method (r = 0.79, p < 0.0001) than the “Anatomic” method (r = 0.67, p < 0.0001). The correlation between global myocardial radial strain and LV EF was better with the “Functional” method (r = 0.86, p < 0.0001) than the “Anatomic” method (r = 0.68, p < 0.0001). In BMVP, the base of the LV should be defined at the level of the mitral valve annulus so that regurgitant volume most accurately reflects the functional significance of the mitral valve disease and LVEF most accurately reflects global systolic LV function. Defining the basal extent of the LV at the mitral valve leaflets leads to substantially lower regurgitant volumes and lower ejection fractions that could have important clinical consequences.
PurposePrior studies reporting efficacy of radiofrequency catheter ablation for complex ventricular ectopy in mitral valve prolapse (MVP) are limited by selective inclusion of bileaflet MVP, papillary muscle only ablation, or short-term follow-up. We sought to evaluate the long-term incidence of hemodynamically significant ventricular tachycardia (VT) or fibrillation (VF) in patients with MVP after initial ablation.Methods
We studied consecutive patients with MVP undergoing ablation for complex ventricular ectopy between 2013 and 2017 at our institution. Of 580 patients with MVP, we included 15 (2.6%, 10 women; mean age 50 ± 14 years, 53% bileaflet) with complex ventricular ectopy treated with initial ablation.ResultsOver a median follow-up of 3406 (1875-6551) days or 9 years, 5 of 15 (33%) patients developed hemodynamically significant VT/VF after their initial ablation and underwent placement of an implantable cardioverter defibrillator (ICD). Three of 5 also underwent repeat ablations. Sustained VT was inducible prior to index ablation in all 5 who developed VT/VF, compared to none of the 10 patients who did not develop VT/VF after index ablation (p = 0.002). Complex ventricular ectopy at index ablation was multifocal in all 5 patients who underwent repeat intervention versus 4 of 10 patients (40%) who did not (p = 0.04). All 3 patients with subsequent VT/VF who underwent repeat ablation had a new clinically dominant focus of ventricular arrhythmia and 3 of the patients with ICD had appropriate VT/VF therapies.Conclusions
In the long term, a subset of MVP patients treated with ablation for ventricular arrhythmias, all with multifocal ectopy on initial EP study, develop hemodynamically significant VT/VF. Our findings suggest the progressive nature of ventricular arrhythmias in patients with MVP and multifocal ectopy.
Background
The association between mitral valve prolapse (MVP) and sudden death remains controversial. We aimed to describe histopathological changes in individuals with autopsy‐determined isolated MVP ( iMVP ) and sudden death and document cardiac arrest rhythm.
Methods and Results
The Australian National Coronial Information System database was used to identify cases of iMVP between 2000 and 2018. Histopathological changes in iMVP and sudden death were compared with 2 control cohorts matched for age, sex, height, and weight (1 group with noncardiac death and 1 group with cardiac death). Data linkage with ambulance services provided cardiac arrest rhythm for iMVP cases. From 77 221 cardiovascular deaths in the National Coronial Information System database, there were 376 cases with MVP . Individual case review yielded 71 cases of iMVP . Mean age was 49±18 years, and 51% were women. Individuals with iMVP had higher cardiac mass (447 g versus 355 g; P <0.001) compared with noncardiac death, but similar cardiac mass (447 g versus 438 g; P =0.64) compared with cardiac death. Individuals with iMVP had larger mitral valve annulus compared with noncardiac death (121 versus 108 mm; P <0.001) and cardiac death (121 versus 110 mm; P =0.002), and more left ventricular fibrosis (79% versus 38%; P <0.001) compared with noncardiac death controls. In those with iMVP and witnessed cardiac arrest, 94% had ventricular fibrillation.
Conclusions
Individuals with iMVP and sudden death have increased cardiac mass, mitral annulus size, and left ventricular fibrosis compared with a matched cohort, with cardiac arrest caused by ventricular fibrillation. The histopathological changes in iMVP may provide the substrate necessary for development of malignant ventricular arrhythmias.
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.
Ventricular arrhythmias are an important cause of morbidity and mortality and come in a variety of forms, from single premature ventricular complexes to sustained ventricular tachycardia and fibrillation. Rapid developments have taken place over the past decade in our understanding of these arrhythmias and in our ability to diagnose and treat them. The field of catheter ablation has progressed with the development of new methods and tools, and with the publication of large clinical trials. Therefore, global cardiac electrophysiology professional societies undertook to outline recommendations and best practices for these procedures in a document that will update and replace the 2009 EHRA/HRS Expert Consensus on Catheter Ablation of Ventricular Arrhythmias. An expert writing group, after reviewing and discussing the literature, including a systematic review and meta-analysis published in conjunction with this document, and drawing on their own experience, drafted and voted on recommendations and summarized current knowledge and practice in the field. Each recommendation is presented in knowledge byte format and is accompanied by supportive text and references. Further sections provide a practical synopsis of the various techniques and of the specific ventricular arrhythmia sites and substrates encountered in the electrophysiology lab. The purpose of this document is to help electrophysiologists around the world to appropriately select patients for catheter ablation, to perform procedures in a safe and efficacious manner, and to provide follow-up and adjunctive care in order to obtain the best possible outcomes for patients with ventricular arrhythmias.
Background:
Mitral annular disjunction (MAD) is a structural abnormality where there is a separation between the mitral valve annulus and the left atrial wall which is not well understood.
Methods:
We conducted a systematic review to evaluate the prevalence of MAD, factors associated with MAD and clinical outcomes among patients with MAD.
Results:
A total of 19 studies were included in this review, and the number of noncase report studies had between 23 and 1439 patients. The pooled rate of MAD in studies of myxomatous mitral valve patients was 66/130 (50.8%, 3 studies), and among patients with mitral valve prolapse was 95/291 (32.6%, 3 studies). One study suggests that 78% of patients with MAD had mitral valve prolapse, and another suggested it was strongly associated with myxomatous mitral valve disease (HR 5.04 95% CI 1.66-15.31). In terms of clinical significance, it has been reported that MAD with disjunction > 8.5 mm was associated with nonsustained ventricular tachycardia (OR 10 95% CI 1.28-78.1). There is also evidence that gadolinium enhancement in papillary muscle (OR 4.09 95% CI 1.28-13.05) and longitudinal MAD distance in posterolateral wall (OR 1.16 95% CI 1.02-1.33) was predictive of ventricular arrhythmia and late gadolinium enhancement in anterolateral papillary muscle was strongly associated with serious arrhythmic event (OR 7.35 95% CI 1.15-47.02).
Conclusions:
Mitral annular disjunction appears to be common in myxomatous mitral valve disease and mitral valve prolapse which can be detected on cardiac imaging and may be important because of its association with ventricular arrhythmias and sudden cardiac death.
Valvular regurgitation represents an important cause of cardiovascular morbidity and mortality. Imaging is pivotal in the evaluation of native valve regurgitation and echocardiography is the primary imaging modality for this purpose. The imaging assessment of valvular regurgitation should integrate quantification of the regurgitation, assessment of the valve anatomy and function, and the consequences of valvular disease on cardiac chambers. In clinical practice, the management of patients with valvular regurgitation largely relies on the results of imaging. It is crucial to provide standards that aim at establishing a baseline list of measurements to be performed when assessing native valve regurgitation. The present document aims to present clinical guidance for the multi-modality imaging assessment of native valvular regurgitation.
Background:
Mitral annular disjunction (MAD) is a structural abnormality involving a distinct separation of the left atrium/mitral valve annulus and myocardium continuum. The literature around MAD has increased over recent years, thus we sought to review the current data on the definition, prevalence, and clinical outcomes of MAD.
Methods:
A search of MEDLINE and EMBASE was conducted to identify studies which evaluated MAD in any patient cohort. The study results were synthesized narratively.
Results:
A total of 12 studies were included with 3925 patients (average age 62 years, 63% male). The pooled prevalence of MAD in patients with mitral valve prolapse and/or Barlow's disease was 30.1%. In a general population, MAD prevalence was 8.7%. The definition of MAD was not consistent across all studies. In terms of clinical outcomes, only one study reported MAD to be associated with ventricular arrhythmias.
Conclusions:
MAD is an increasingly recognized finding amongst patients undergoing cardiac imaging. This review highlights the need for agreed definitions for clinically significant MAD and how identified MAD should be managed. At present, there is insufficient evidence that MAD is associated adverse clinical outcomes.
Background
The prognosis of exercise-induced premature ventricular contractions (PVCs) in asymptomatic individuals is unclear.
Objectives
This study sought to investigate whether high-grade PVCs during stress testing predict mortality in asymptomatic individuals.
Methods
A cohort of 5,486 asymptomatic individuals who took part in the Lipid Research Clinics prospective cohort had baseline interview, physical examination, blood tests, and underwent Bruce protocol treadmill testing. Adjusted Cox survival models evaluated the association of exercise-induced high-grade PVCs (defined as either frequent (>10 per minute), multifocal, R-on-T type, or ≥2 PVCs in a row) with all-cause and cardiovascular mortality.
Results
Mean baseline age was 45.4 ± 10.8 years; 42% were women. During a mean follow-up of 20.2 ± 3.9 years, 840 deaths occurred, including 311 cardiovascular deaths. High-grade PVCs occurred during exercise in 1.8% of individuals, during recovery in 2.4%, and during both in 0.8%. After adjusting for age, sex, diabetes, hypertension, lipids, smoking, body mass index, and family history of premature coronary disease, high-grade PVCs during recovery were associated with cardiovascular mortality (hazard ratio [HR]: 1.82; 95% CI: 1.19-2.79; P = 0.006), which remained significant after further adjusting for exercise duration, heart rate recovery, achieving target heart rate, and ST-segment depression (HR: 1.68; 95% CI: 1.09-2.60; P = 0.020). Results were similar by clinical subgroups. High-grade PVCs occurring during the exercise phase were not associated with increased risk. Recovery PVCs did not improve 20-year cardiovascular mortality risk discrimination beyond clinical variables.
Conclusions
High-grade PVCs occurring during recovery were associated with long-term risk of cardiovascular mortality in asymptomatic individuals, whereas PVCs occurring only during exercise were not associated with increased risk.
Background
: Multiple spikes within the QRS complex, known as fragmented QRS (fQRS), are associated with the occurrences of ventricular arrhythmic events (VAEs) in patients with Brugada syndrome and hypertrophic cardiomyopathy. However, the association between fQRS and occurrence of VAEs in patients with cardiac sarcoidosis (CS) has not been elucidated.
Methods
: We evaluated the associations between fQRS and cardiac events including VAEs [non-sustained ventricular tachycardia (NSVT), sustained ventricular tachycardia (VT), and ventricular fibrillation (VF)], hospitalization for heart failure, and all-cause death in 68 patients with CS (30 patients with fQRS vs. 38 patients without fQRS) over a 5-year period.
Results
: Cardiac events occurred in 22 patients with fQRS and 18 patients without fQRS (73% vs. 47%, p=0.009). Of the cardiac events that occurred in CS patients, VAEs occurred more frequently in patients with fQRS than in patients without fQRS (VAEs: 70% vs. 45%, p=0.017; NSVT: 70% vs. 45%, p=0.010; VT: 43% vs. 18%, p=0.011, and VF: 6.7% vs. 2.6%, p=0.34), whereas there was no significant difference in hospitalization for heart failure or all-cause death between patients with and those without fQRS (hospitalization for heart failure: 6.7% vs. 5.3%, p=0.75; all-cause death: 6.7% vs. 5.3%, p=0.64). Multivariate analysis showed that fQRS in the baseline electrocardiogram was independently associated with VAEs (hazard ratio: 2.21, 95% confidence interval: 1.15–4.25, p=0.017).
Conclusion
: fQRS is a predictor of VAEs in patients with CS.
Despite what was considered adequate pharmacological treatment, the conditon of six patients with severe mitral valve prolapse but with trivial or no mitral regurgitation deteriorated. These patients had marked weakness, chest pain, dyspnea, and arrhythmias. Because these patients found their condition to be intolerable, the prolapsed mitral valve was repaired. Electrocardiography, treadmill stress testing, and left ventriculography performed following operation showed complete repair of the valve and significant improvement over the preoperative findings in all six patients. Repair of the floppy mitral valve did not eradicate all abnormalities; however, it did significantly improve the chest pain, weakness, dyspnea, and arrhythmias in all six patients. Five patients no longer require any medication. The prolapsed mitral valve contributed significantly to the symptoms and arrhythmias, but it could not have been the sole cause for these patients’ signs and symptoms. With complete correction of the prolapse in all six patients, few of the signs and symptoms of the disease persisted. Repair of severe mitral valve prolapse without mitral regurgitation is recommended only for those patients who continue to be severely symptomatic from chest pain, dyspnea, or ventricular arrhythmias after an extensive trial of adequate medical therapy.
BACKGROUND
Myxomatous mitral valve prolapse (MVP) and mitral-annular disjunction (Barlow disease) are at-risk for ventricular arrhythmias (VA). Fibrosis involving the papillary muscles and/or the infero-basal left ventricular (LV) wall was reported at autopsy in sudden cardiac death (SCD) patients with MVP.
OBJECTIVES
We investigated the electrophysiological substrate subtending VA in MVP patients with Barlow disease phenotype.
METHODS
Twenty-three patients with VA were enrolled, including five with syncope and 4 with a history of SCD. Unipolar (Uni<8.3mV) and bipolar (Bi<1.5 mV) low-voltage areas were analysed with electro-anatomical mapping (EAM), and VA inducibility was evaluated with programmed ventricular stimulation (PES). Electrophysiological parameters were correlated with VA patterns, ECG inferior negative T wave (nTW), and late gadolinium enhancement (LGE) assessed by cardiac magnetic resonance.
RESULTS
Premature ventricular complex (PVC) burden was 12061.9±12994.6/24 hours with a papillary-muscle type (PM-PVC) in 18 patients (68%). Twelve-lead ECG showed nTW in 12 patients (43.5%). A large Uni<8.3mV area (62.4+/- 45.5 cm2) was detected in the basal infero-lateral LV region in 12 (73%) patients, and in the papillary muscles (2.2+/-2.9 cm2) in 5 (30%) of 15 patients undergoing EAM. A concomitant Bi<1.5 mV area (5.0±1.0 cm2) was identified in 2 patients. A history of SCD, and the presence of nTW, and LGE were associated with a greater Uni<8.3mV extension: (32.8+/-3.1 cm2 vs. 9.2+/-8.7 cm2), nTW (20.1+/-11.0vs.4.1+/-3.8cm2), and LGE (19.2 ± 11.7 cm2 vs. 1.0 ± 2.0 cm2, p=0.013), respectively. All patients with PM-PVC had a Uni<8.3mV area. Sustained VA (ventricular tachycardia 2, VF 2) were induced by PES only in 4 patients (one with resuscitated SCD).
CONCLUSIONS
Low unipolar low voltage areas can be identified with EAM in the basal infero-lateral LV region and in the papillary muscles as a potential electrophysiological substrate for VA and SCD in patients with MVP and Barlow disease phenotype.
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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.
Importance:
Mitral annular disjunction (MAD) has received particular interest in patients with mitral valve prolapse, ventricular tachycardia, and sudden cardiac death. The clinical significance of MAD for patients with Marfan syndrome (MFS) remains largely unexplored.
Objective:
To define the prevalence of MAD and examine its association with cardiovascular outcomes and arrhythmia among patients with MFS.
Design, setting, and participants:
This retrospective, single-center cohort study included 142 patients with a diagnosis of MFS based on the revised Ghent criteria and a confirmed (likely) pathogenic variant in the FBN1 gene who underwent regular follow-up between January 1, 2004, and December 31, 2019.
Main outcomes and measures:
The presence of MAD was assessed by echocardiography, and the extent of MAD was categorized in tertiles. Patients also underwent resting electrocardiography and 24-hour Holter monitoring. Outcomes included aortic events (aortic dissection or prophylactic aortic surgery), arrhythmic events (defined as sustained ventricular tachycardia or sudden cardiac death), and mitral valve surgery.
Results:
A total of 142 patients (72 female patients [51%]; median age at first examination, 25 years [range, 2-64 years]) were evaluated. Forty-eight patients (34%) had MAD. Patients with MAD had larger aortic root z scores than patients without MAD (4.1 [interquartile range, 2.8-5.7] vs 3.0 [interquartile range, 1.8-4.0]; P < .001) and more often had mitral valve prolapse (34 of 48 [71%] vs 14 of 94 [15%]; P < .001), ventricular ectopy (14 of 33 [42%] vs 15 of 70 [21%]; P = .03), and nonsustained ventricular tachycardia (13 of 33 [39%] vs 12 of 70 [17%]; P = .01). During follow-up, aortic events occurred at similar rates among patients with vs without MAD (15 of 43 [35%] vs 21 of 84 [25%]; P = .24), but patients in the upper MAD tertile (>10 mm) showed a higher occurrence of aortic events compared with patients with MAD of 10 mm or smaller (9 of 15 [60%] vs 6 of 28 [21%]; P = .01). Patients with arrhythmic events (n = 5) and patients requiring mitral valve surgery (n = 7) were observed exclusively in the group displaying MAD.
Conclusions and relevance:
This study suggests that MAD among patients with MFS is associated with the occurrence of arrhythmic events, a higher need for mitral valve intervention, and, among patients with extensive MAD, more aortic events. Cardiac imaging for patients with MFS should consider the assessment of MAD as a potential marker for adverse outcomes.
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.
Aims :
The term idiopathic ventricular fibrillation (IVF) describes survivors of unexplained cardiac arrest (UCA) without a specific diagnosis after clinical and genetic testing. Previous reports have described a subset of IVF individuals with ventricular arrhythmia initiated by short-coupled trigger premature ventricular contractions (PVCs) for which the term short-coupled ventricular fibrillation (SCVF) has been proposed. The aim of this article is to establish the phenotype and frequency of SCVF in a large cohort of UCA survivors.
Methods and results :
We performed a multicentre study including consecutive UCA survivors from the CASPER registry. Short-coupled ventricular fibrillation was defined as otherwise unexplained ventricular fibrillation initiated by a trigger PVC with a coupling interval of <350 ms. Among 364 UCA survivors, 24/364 (6.6%) met diagnostic criteria for SCVF. The diagnosis of SCVF was obtained in 19/24 (79%) individuals by documented ventricular fibrillation during follow-up. Ventricular arrhythmia was initiated by a mean PVC coupling interval of 274 ± 32 ms. Electrical storm occurred in 21% of SCVF probands but not in any UCA proband (P < 0.001). The median time to recurrent ventricular arrhythmia in SCVF was 31 months. Recurrent ventricular fibrillation resulted in quinidine administration in 12/24 SCVF (50%) with excellent arrhythmia control.
Conclusion :
Short-coupled ventricular fibrillation is a distinct primary arrhythmia syndrome accounting for at least 6.6% of UCA. As documentation of ventricular fibrillation onset is necessary for the diagnosis, most cases are diagnosed at the time of recurrent arrhythmia, thus the true prevalence of SCVF remains still unknown. Quinidine is effective in SCVF and should be considered as first-line treatment for patients with recurrent episodes.
Mitral annular disjunction is a structural abnormality of the mitral annulus fibrosus, which has been described by pathologists to be associated with mitral leaflet prolapse. Mitral annular disjunction is a common finding in patients with myxomatous mitral valve diseases. The prevalence of mitral annular disjunction should be checked routinely during presurgical imaging. Otherwise, mitral annular disjunction itself might be an arrhythmogenic entity, irrespective of the presence of mitral valve prolapse (MVP). Therefore, we should check echocardiography keeping in mind mitral annular disjunction. Further prospective studies are needed to address whether a causative mechanistic link exists between mitral annular disjunction and arrhythmic MVP.
Aims
Mitral annular disjunction is fibrous separation between the attachment of the posterior mitral leaflet and the basal left ventricular myocardium initially described in dissected hearts. Currently, it is commonly evaluated by echocardiography, and potential relationships with mitral valve prolapse and ventricular arrhythmia have been suggested. However, controversy remains as its prevalence and extent have not been fully elucidated in normal living subjects.
Methods and results
Systolic datasets of cardiac computed tomography obtained from 98 patients (mean age, 69.1 ± 12.6 years; 81% men) with structurally normal hearts were assessed retrospectively. Circumferential extent of both mitral leaflets and disjunction was determined by rotating orthogonal multiplanar reconstruction images around the central axis of the mitral valvar orifice. Distribution angle within the circumference of the mitral valvar attachment and maximal height of disjunction were quantified. In total, 96.0% of patients demonstrated disjunction. Average distribution angles of the anterior and posterior mitral leaflets were 91.3 ± 9.4° and 269.8 ± 9.7°, respectively. Average distribution angle of the disjunction was 105.1 ± 49.2°, corresponding to 39.0 ± 18.2% of the entire posterior mitral valvar attachment. Median value of the maximal height of disjunction was 3.0 (1.5–7.0) mm. Distribution prevalence map of the disjunction revealed characteristic double peaks, with frequent sites of the disjunction located at the anterior to antero-lateral and inferior to infero-septal regions.
Conclusion
Mitral annular disjunction is a rather common finding in the normal adult heart with bimodal distribution predominantly observed involving the P1 and P3 scallops of the posterior mitral leaflet.
Background: Mitral valve prolapse (MVP) is a frequent disease that can be complicated by mitral regurgitation (MR), heart failure, arterial embolism, rhythm disorders and death. Left ventricular (LV) replacement myocardial fibrosis, a marker of maladaptive remodeling, has been described in patients with MVP, but the implications of this finding remain scarcely explored. We aimed at assessing the prevalence, pathophysiological and prognostic significance of LV replacement myocardial fibrosis through late gadolinium enhancement (LGE) by cardiac magnetic resonance (CMR) in patients with MVP.
Methods: Four hundred patients (53±15 years, 55% male) with MVP (trace to severe MR by echocardiography) from 2 centers, who underwent a comprehensive echocardiography and LGE CMR, were included. Correlates of replacement myocardial fibrosis (LGE+), influence of MR degree, and ventricular arrhythmia were assessed. The primary outcome was a composite of cardiovascular events (cardiac death, heart failure, new-onset atrial fibrillation, arterial embolism, and life-threatening ventricular arrhythmia).
Results: Replacement myocardial fibrosis (LGE+) was observed in 110 patients (28%; 91 myocardial wall including 71 basal inferolateral wall, 29 papillary muscle). LGE+ prevalence was 13% in trace-mild MR, 28% in moderate and 37% in severe MR, and was associated with specific features of mitral valve apparatus, more dilated LV and more frequent ventricular arrhythmias (45 vs 26%, P<0.0001). In trace-mild MR, despite the absence of significant volume overload, abnormal LV dilatation was observed in 16% of patients and ventricular arrhythmia in 25%. Correlates of LGE+ in multivariable analysis were LV mass (OR 1.01, 95% CI [1.002-1.017], P=0.009) and moderate-severe MR (OR: 2.28, 95% CI [1.21-4.31], P=0.011). LGE+ was associated with worse 4-year cardiovascular event-free survival (49.6±11.7 in LGE+ vs 73.3±6.5% in LGE-, P<0.0001). In a stepwise multivariable Cox model, MR volume and LGE+ (HR: 2.6 [1.4-4.9], P=0.002) were associated with poor outcome.
Conclusions: LV replacement myocardial fibrosis is frequent in patients with MVP, is associated with mitral valve apparatus alteration, more dilated LV, MR grade, ventricular arrhythmia, and is independently associated with cardiovascular events. These findings suggest a MVP-related myocardial disease. Finally, CMR provides additional information to echocardiography in MVP.
Objectives
The goal of this study was to investigate the characteristics of mitral valve prolapse (MVP) in a post-mortem study of consecutive sudden cardiac deaths (SCDs) in subjects up to 90 years of age.
Background
Up to 2.3% of subjects with MVPs experience SCD, but by convention SCD is rarely confirmed by autopsy. In a post-mortem study of persons <40 years of age, 7% of SCDs were caused by MVP; bileaflet involvement, mitral annular disjunction (MAD), and replacement fibrosis were common.
Methods
In the San Francisco POST SCD (Postmortem Systematic Investigation of Sudden Cardiac Death) study, autopsies have been performed on >1000 consecutive World Health Organization–defined (presumed) cases of SCD in subjects aged 18 to 90 years since 2011; a total of 603 were adjudicated. Autopsy-defined sudden arrhythmic death (SAD) required absence of nonarrhythmic cause; MVP diagnosis required leaflet billowing. One hundred antemortem echocardiograms were revised to identify additional MVPs missed on autopsy.
Results
Among the 603 presumed SCDs, 339 (56%) were autopsy-defined SADs, with MVP identified in 7 (1%). Six additional MVPs were identified by review of echocardiograms, for a prevalence of at least 2% among 603 presumed SCDs and 4% among 339 SADs (vs. 264 non-SADs; p = 0.02). All 6 additional MVPs had monoleaflet rather than bileaflet involvement and mild mitral regurgitation, ruling out hemodynamic cause. Less than one-half had MAD with replacement fibrosis, but all had multisite interstitial fibrosis.
Conclusions
In a countywide post-mortem study of all adult cases of SCD, MVP prevalence was at least 4% of SADs, but one-half were missed on autopsy. Monoleaflet MVP was often underdiagnosed post-mortem. Compared with young cases of SCD, lethal MVP in older cases of SCD did not consistently have bileaflet anatomy, replacement fibrosis, or MAD.
BACKGROUND
The American Heart Association, in conjunction with the National Institutes of Health, annually reports the most up-to-date statistics related to heart disease, stroke, and cardiovascular risk factors, including core health behaviors (smoking, physical activity, diet, and weight) and health factors (cholesterol, blood pressure, and glucose control) that contribute to cardiovascular health. The Statistical Update presents the latest data on a range of major clinical heart and circulatory disease conditions (including stroke, congenital heart disease, rhythm disorders, subclinical atherosclerosis, coronary heart disease, heart failure, valvular disease, venous disease, and peripheral artery disease) and the associated outcomes (including quality of care, procedures, and economic costs).
METHODS
The American Heart Association, through its Statistics Committee, continuously monitors and evaluates sources of data on heart disease and stroke in the United States to provide the most current information available in the annual Statistical Update. The 2021 Statistical Update is the product of a full year’s worth of effort by dedicated volunteer clinicians and scientists, committed government professionals, and American Heart Association staff members. This year’s edition includes data on the monitoring and benefits of cardiovascular health in the population, an enhanced focus on social determinants of health, adverse pregnancy outcomes, vascular contributions to brain health, the global burden of cardiovascular disease, and further evidence-based approaches to changing behaviors related to cardiovascular disease.
RESULTS
Each of the 27 chapters in the Statistical Update focuses on a different topic related to heart disease and stroke statistics.
CONCLUSIONS
The Statistical Update represents a critical resource for the lay public, policy makers, media professionals, clinicians, health care administrators, researchers, health advocates, and others seeking the best available data on these factors and conditions.
Objectives:
Barlow's disease (BD) is characterized by thick, redundant mitral valve (MV) leaflets, which can lead to prolapse and significant mitral regurgitation (MR). MV annular abnormalities are also commonly observed and increasingly recognized as possible primary pathology, with leaflet thickening being secondary to increased stress on the MV apparatus. To provide more insights into this hypothesis, the evolution of MV abnormalities over time in patients with BD was assessed.
Methods:
A total of 64 patients (54 ± 12 years, 72% male) with BD who underwent MV surgery and had multiple transthoracic echocardiograms (TTE) before surgery were included. In total, 186 TTE were analysed (median time interval 4.2, interquartile range 2.2-6.5 years) including specific MV characteristics.
Results:
At baseline, MV leaflet length, thickness, billowing height and annular diameter were larger in patients with BD compared to 59 healthy subjects. Systolic outward motion (curling) of the annulus was observed in 77% and severe mitral annular disjunction (≥5 mm) in 38% of patients with BD. Forty (63%) patients had MR grade I-II and 24 (37%) MR grade III-IV; at baseline, the 2 groups only differed in left atrial volume and in thickness and billowing height of the posterior leaflet, showing comparable MV annular abnormalities and dilatation despite different grades of MR. Over time, MV annulus diameter, leaflet length and billowing height increased significantly along with MR grade.
Conclusions:
In patients with BD, MV annulus abnormalities are present at an early stage and precede the development of significant MR, suggesting their substantial role in the pathophysiology of this disease and as an important target for surgical treatment.
Mitral valve prolapse affects up to 3% of the population, with an annual risk of death of up to 2% a year. Presentation is often in the third to fourth decade of life. This report details an adolescent with mitral valve prolapse with non-specific symptoms but investigative findings of ventricular ectopy at higher heart rates. These patients warrant close surveillance to monitor for progressive arrhythmias and may progress to need an ICD.
Background
Mitral annular disjunction is a structural abnormality of the mitral annulus fibrosus which is often associated with mitral leaflet prolapse. However, few reports have described mitral annular disjunction in mitral valve prolapse (MVP). This study aimed to investigate the characteristics of mitral annular disjunction in patients with severe mitral regurgitation (MR) caused by MVP.
Methods
We reviewed 185 consecutive patients with severe MR caused by fibroelastic deficiency (FED) and Barlow's syndrome from March 2009 to December 2010. The upper limit of the disjunction was defined at the level of the posterior scallop's insertion into the left atrial wall, whereas the lower limit was defined at the level of the left atrium's connection to the ventricular myocardium. The distance between the two levels was called mitral annular disjunction. Prolapse sites in FED patients were categorized into anterior leaflet, posterior leaflet, and commissure groups. Patients with a disjunction distance of ≥2 mm were diagnosed with mitral annular disjunction.
Results
Annular disjunction was found in 45 patients (24%). Among them, the most common site of prolapse was the posterior leaflet (n = 35, 77.8%). During a median follow‐up of 20.3 years, arrhythmic events and sudden death occurred in seven patients (3.8%).
Conclusions
Mitral annular disjunction was detected in 24% of patients with severe MR and in 90% of the patients with Barlow's syndrome. There were significant differences at its sites of prolapse in FED patients. The presence and site of prolapse with mitral annular disjunction should be actively determined in FED patients.
Asymptomatic arrhythmias are frequently encountered in clinical practice. Although studies specifically dedicated to these asymptomatic arrhythmias are lacking, many arrhythmias still require proper diagnostic and prognostic evaluation and treatment to avoid severe consequences, such as stroke or systemic emboli, heart failure, or sudden cardiac death. The present document reviews the evidence, where available, and attempts to reach a consensus, where evidence is insufficient or conflicting.
Objectives:
T1 mapping (T1-map) and cardiac magnetic resonance feature tracking (CMR-FT) techniques have been introduced for the early detection of interstitial myocardial fibrosis and deformation abnormalities. We sought to demonstrate that T1-map and CMR-FT may identify the presence of subclinical myocardial structural changes in patients with mitral valve prolapse (MVP).
Methods:
Consecutive MVP patients with moderate-to-severe mitral regurgitation and comparative matched healthy subjects were prospectively enrolled and underwent CMR-FT analysis to calculate 2D global and segmental circumferential (CS) and radial strain (RS) and T1-map to determine global and segmental native T1 (nT1) values.
Results:
Seventy-three MVP patients (mean age, 57 ± 13 years old; male, 76%; regurgitant volume, 57 ± 21 mL) and 42 matched control subjects (mean age, 56 ± 18 years; male, 74%) were included. MVP patients showed a lower global CS (- 16.3 ± 3.4% vs. - 17.8 ± 1.9%, p = 0.020) and longer global nT1 (1124.9 ± 97.7 ms vs. 1007.4 ± 26.1 ms, p < 0.001) as compared to controls. Moreover, MVP patients showed lower RS and CS in basal (21.6 ± 12.3% vs. 27.6 ± 8.9%, p = 0.008, and - 13.0 ± 6.7% vs. - 14.9 ± 4.1%, p = 0.013) and mid-inferolateral (20.6 ± 10.7% vs. 28.4 ± 8.7%, p < 0.001, and - 12.8 ± 6.3% vs. - 16.5 ± 4.0%, p < 0.001) walls as compared to other myocardial segments. Similarly, MVP patients showed longer nT1 values in basal (1080 ± 68 ms vs. 1043 ± 43 ms, p < 0.001) and mid-inferolateral (1080 ± 77 ms vs. 1034 ± 37 ms, p < 0.001) walls as compared to other myocardial segments. Of note, nT1 values were significantly correlated with CS (r, 0.36; p < 0.001) and RS (r, 0.37; p < 0.001) but not with regurgitant volume.
Conclusions:
T1-map and CMR-FT identify subclinical left ventricle tissue changes in patients with MVP. Further studies are required to correlate these subclinical tissue changes with the outcome.
Key points:
• T1 mapping (T1-map) and cardiac magnetic resonance feature tracking (CMR-FT) techniques have been introduced for the early detection of interstitial myocardial fibrosis and deformation abnormalities. • In MVP patients, we demonstrated a longer global nT1 with associated reduced global circumferential (CS) and radial strain (RS) as compared to control subjects. • Among MVP patients, the mid-basal left ventricle inferolateral wall showed longer nT1 with reduced CS and RS as compared to other myocardial segments. Further studies are required to correlate these subclinical tissue changes with the outcome.
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.
Objective
Mitral annular disjunction (MAD) is an abnormality linked to mitral valve prolapse (MVP), possibly associated with malignant ventricular arrhythmias. We assessed the agreement among different imaging techniques for MAD identification and measurement.
Methods
131 patients with MVP and significant mitral regurgitation undergoing transthoracic echocardiography (TTE) and cardiac magnetic resonance (CMR) were retrospectively enrolled. Transoesophageal echocardiography (TOE) was available in 106 patients. MAD was evaluated in standard long-axis views (four-chamber, two-chamber, three-chamber) by each technique.
Results
Considering any-length MAD, MAD prevalence was 17.3%, 25.5%, 42.0% by TTE, TOE and CMR, respectively (p<0.05). The agreement on MAD identification was moderate between TTE and CMR (κ=0.54, 95% CI 0.49 to 0.59) and good between TOE and CMR (κ=0.79, 95% CI 0.74 to 0.84). Assuming CMR as reference and according to different cut-off values for MAD (≥2 mm, ≥4 mm, ≥6 mm), specificity (95% CI) of TTE and TOE was 99.6 (99.0 to 100.0)% and 98.7 (97.4 to 100.0)%; 99.3 (98.4 to 100.0)% and 97.6 (95.8 to 99.4)%; 97.8 (96.2 to 99.3)% and 93.2 (90.3 to 96.1)%, respectively; sensitivity (95% CI) was 43.1 (37.8 to 48.4)% and 74.5 (69.4 to 79.5)%; 54.0 (48.7 to 59.3)% and 88.9 (85.2 to 92.5)%; 88.0 (84.5 to 91.5)% and 100.0 (100.0 to 100.0)%, respectively. MAD length was 8.0 (7.0-10.0), 7.0 (5.0-8.0], 5.0 (4.0-7.0) mm, respectively by TTE, TOE and CMR. Agreement on MAD measurement was moderate between TTE and CMR (ρ=0.73) and strong between TOE and CMR (ρ=0.86).
Conclusions
An integrated imaging approach could be necessary for a comprehensive assessment of patients with MVP and symptoms suggestive for arrhythmias. If echocardiography is fundamental for the anatomic and haemodynamic characterisation of the MV disease, CMR may better identify small length MAD as well as myocardial fibrosis.
Background
Premature ventricular contractions (VPC) have hour-to-hour and day-to-day variation. High VPC burden correlates with cardiomyopathy.
Objective
To determine the optimal duration for ambulatory ECG monitoring for accurate assessment of VPC burden.
Methods
Our group performed a retrospective analysis on patch monitors used for any indication with overall VPC burden ≥5.0% between February 1, 2016 and February 1, 2020. We generated cumulative daily VPC averages for each day of wear and performed linear regression analysis between each cumulative daily average and overall burden. Patients were divided into groups based on low or high VPC frequency, and the analysis was repeated. Split-sample validation was used to internally validate the overall prediction model.
Results
116 patches representing 107 patients (mean age: 64.5; F: 48%) were analyzed. Mean overall VPC burden was 13.4 ± 7.5% (range: 5.0-42.0). Day 1 R² was 60%, p<0.001 and continued to increase to R² 88%, p<0.001 at day 14. Median percent and absolute error decreased from 22.70% (IQR: 9.73-34.39) and 2.58% (IQR: 1.24-4.59) at day 1 to 5.62% (IQR: 2.82-8.39) and 0.55% (IQR: 0.28-1.05) at day 14. Patients with higher overall VPC frequencies achieved a more rapid rise in R² relative to those with lower frequencies. Split-sample validation supported the internal validity of our linear regression prediction model.
Conclusion
Mobile telemetry for a period of ∼7 days accurately reflects overall VPC burden. Measurement of VPC burden for only 24-48 hours may not accurately reflect total burden. Monitoring for 2 weeks or longer adds little additional VPC information.
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.
Floppy mitral valve/mitral valve prolapse (FMV/MVP) is a common valvular abnormality affecting 2% to 3% of the general population. It occurs in a heterogeneous group of patients with varying and age dependent expressions. FMV/MVP can be familial or sporadic, isolated (called non-syndromic) or as a part of a well-defined syndrome of heritable connective tissue disorders or other diseases. A wide range of phenotypic expression exists ranging from asymptomatic to non-specific symptoms related to neuroendocrine or autonomic nervous system functional abnormalities, varying degrees of mitral regurgitation that may require interventional therapy, heart failure, infective endocarditis, cardiac arrhythmias and/or sudden cardiac death. FMV/MVP is predominantly considered a heritable disorder with clinical manifestations not present at birth, but appearing later in life. Though a variant gene may initiate the development of FMV/MVP, precise phenotypic expression may be related to multiple other molecular, genetic and epigenetic factors that modify the final expression of the disease. A better understanding of these mechanisms will help to better define the natural history of the disease, inhibit disease progression and even prevent the phenotypic expression of FMV/MVP.
Background:
Although mitral valve prolapse (MVP) is a benign disease, several studies have indicated its association with ventricular arrhythmias (VAs). Some histopathological studies have pointed to left ventricular fibrosis as the underlying cause of arrhythmia in MVP patients. Fragmented QRS (fQRS) on electrocardiography (ECG) has been shown to be a marker of myocardial fibrosis. This study aimed to investigate the association between fQRS and complex VAs in patients with MVP.
Methods:
A total of 230 consecutive patients who were diagnosed with MVP were included in the study. The control group consisted of 302 healthy individuals matched according to age and sex. fQRS was defined as additional R' wave or notching/splitting of S wave in two contiguous ECG leads. All patients underwent 24-hour Holter monitoring and VAs were classified using Lown's scoring system. Lown class ≥ 3 VAs were considered as complex VAs.
Results:
As compared to the healthy individuals, prevalence of fQRS (40% vs 9.6%, p<0.001) and complex VAs (18.7% vs 0%, p<0.001) were significantly higher in patients with MVP. Furthermore, complex VAs (35.9% vs 7.2%, p=0.001) were significantly higher in MVP patients with fQRS. In multiple logistic regression analysis, the presence of bileaflet prolapse (OR: 2.567, 95%CI: 1.434 to 4.367; p=0.002) and presence of fQRS (OR: 3.021, 95%CI: 1.556 to 6.232; p<0.001) were independent predictors for complex VAs.
Conclusions:
The presence of fQRS may be associated with complex VAs in patients with MVP. Therefore, fQRS may be used in risk stratification of complex VAs in patients with MVP.
Mitral annulus disjunction (MAD) is characterized by a separation between the atrial wall mitral junction and the left ventricular (LV) free wall. Little is known regarding cardiac magnetic resonance (CMR) performance to detect MAD and its prevalence in mitral valve prolapse (MVP). Based on 89 MVP patients (63 women; mean age 6413) referred for CMR assessment of MR, either from Myxomatous Mitral Valve Disease (MMVP) (n=40; 45%) or fibroelastic disease (FED) (n=49; 55%), we sought to assess MAD prevalence and its consequences on LV morphology. Patients were classified in 2 groups according to MAD presence (MAD+) or absence (MAD-). MAD (measuring 8±4mm) was diagnosed in 35% (31/89) of MVP patients, more frequently in MMVP than FED (60% vs 14%). MMVP, bileaflet prolapse and non-sustain ventricular tachycardia (NSVT) were associated to MAD presence but severity of MR was not and MAD was longer in patients with NSVT (55 mm vs 24 mm). Diagnostic accuracy of TTE for the detection of MAD was fair (65% sensitivity, 96% specificity) with CMR as reference. MAD+ showed significantly enlarged basal and mid LV diameters and enlarged mitral-annulus diameter. Among patients with late gadolinium enhancement, presence of LV fibrosis at level of papillary muscle was more frequent in MAD+. After adjustment on age and MR severity, MMVP and enlarged end-systolic mitral annulus diameter were independently associated with MAD+. In conclusion, MAD was present in about 1/3 of MVP patients, mostly in MMVP and independent of MR severity. Enlarged mitral-annulus and basal LV diameters, NSVT and papillary muscle fibrosis were associated with MAD presence.
Despite a 2% to 3% prevalence of echocardiographically defined mitral valve prolapse (MVP) in the general population, the actual burden, risk stratification, and treatment of the so-called arrhythmic MVP are unknown. The clinical profile is characterized by a patient, usually female, with mostly bileaflet myxomatous disease, mid-systolic click, repolarization abnormalities in the inferior leads, and complex ventricular arrhythmias with polymorphic/right bundle branch block morphology, without significant regurgitation. Among the various pathophysiologic mechanisms of electrical instability, left ventricular fibrosis in the papillary muscles and inferobasal wall, mitral annulus disjunction, and systolic curling have been recently described by pathological and cardiac magnetic resonance studies in sudden death victims and patients with arrhythmic MVP. In addition, premature ventricular beats arising from the Purkinje tissue as ventricular fibrillation triggers have been documented by electrophysiologic studies in MVP patients with aborted sudden death.
The genesis of malignant ventricular arrhythmias in MVP probably recognizes the combination of the substrate (regional myocardial hypertrophy and fibrosis, Purkinje fibers) and the trigger (mechanical stretch) eliciting premature ventricular beats because of a primary morphofunctional abnormality of the mitral valve annulus.
The main clinical challenge is how to identify patients with arrhythmic MVP (which imaging technique and in which patient) and how to treat them to prevent sudden death. Thus, there is a necessity for prospective multicenter studies focusing on the prognostic role of cardiac magnetic resonance and electrophysiologic studies and on the therapeutic efficacy of targeted catheter ablation and mitral valve surgery in reducing the risk of life-threatening arrhythmias, as well as the role of implantable cardioverter defibrillators for primary prevention.