Arrhythmic Mitral Valve Prolapse Research Articles

Mitral annular disjunction and mitral valve prolapse extrapolating adult data to an adolescent cohort?

The aim of this study is to provide an update on mitral valve prolapse (MVP) and mitral annular disjunction (MAD) and who may be at risk for ventricular arrhythmias and sudden cardiac death. MVP is generally considered a benign condition. However, a small subset of patients may be at risk for life-threatening ventricular arrhythmias. Among the risk factors identified in adults include patients with bileaflet mitral valves, myxomatous changes, myocardial fibrosis, and the presence of MAD. Advances in multimodal imaging have allowed for improved identification of fibrosis, anatomical valve derangements, and the amount of MAD. Recent guidelines have suggested that patients with MVP with or without MAD may be at risk for life-threatening arrhythmias if they have had prior ventricular arrhythmias, ventricular dysfunction, or unexplained syncope. Yet, extrapolation of adult data to a pediatric cohort with similar MVP and MAD at this juncture is challenging. There is, however, early evidence that some pediatric patients with significant myocardial fibrosis or abnormal tissue Doppler may be at risk for ventricular tachycardia. Mitral valve prolapse and mitral annular disjunction at times coexist and at other times can be seen as isolated entities. While the incidence of arrhythmic MVP is quite rare, there is increasing evidence that certain select adults with MVP may be at risk for ventricular tachycardia and sudden cardiac death. Future multicenter studies are needed to better understand the natural history of arrhythmic mitral valve disease and how early disease manifestation in children may impact findings now being reported in young adults.

Arrhythmic Mitral Valve Prolapse and Sports Activity: Pathophysiology, Risk Stratification, and Sports Eligibility Assessment.

Although mitral valve prolapse (MVP) is the most prevalent valvular abnormality in Western countries and generally carries a good prognosis, a small subset of patients is exposed to a significant risk of malignant ventricular arrhythmias (VAs) and sudden cardiac death (SCD), the so-called arrhythmic MVP (AMVP) syndrome. Recent work has emphasized phenotypical risk features of severe AMVP and clarified its pathophysiology. However, the appropriate assessment and risk stratification of patients with suspected AMVP remains a clinical conundrum, with the possibility of both overestimating and underestimating the risk of malignant VAs, with the inappropriate use of advanced imaging and invasive electrophysiology study on one hand, and the catastrophic occurrence of SCD on the other. Furthermore, the sports eligibility assessment of athletes with AMVP remains ill defined, especially in the grey zone of intermediate arrhythmic risk. The definition, epidemiology, pathophysiology, risk stratification, and treatment of AMVP are covered in the present review. Considering recent guidelines and expert consensus statements, we propose a comprehensive pathway to facilitate appropriate counseling concerning the practice of competitive/leisure-time sports, envisioning shared decision making and the multidisciplinary "sports heart team" evaluation of borderline cases. Our final aim is to encourage an active lifestyle without compromising patients' safety.

Kiosk 2R-FC-02 - Unveiling the Distinctive Phenotypes of Arrhythmic Mitral Valve Prolapse by High-density Myocardial Strain Imaging and Late-gadolinium-enhancement Microstructure Analysis

Kiosk 2R-FC-02 - Unveiling the Distinctive Phenotypes of Arrhythmic Mitral Valve Prolapse by High-density Myocardial Strain Imaging and Late-gadolinium-enhancement Microstructure Analysis

Left ventricular fibrosis in arrhythmic mitral valve prolapse: quantification and comparison of semi-automated techniques assessed by cardiac magnetic resonance

PurposeLeft ventricular (LV) fibrosis has a key role in arrhythmogenesis in patients with mitral valve prolapse (MVP). Cardiac magnetic resonance identifies LV fibrosis by using late gadolinium enhancement (LGE) technique. LGE assessment and quantification in patients with MVP lacks of standardization protocols.Methods66 MVP patients with normal systolic function and without significant regurgitation were enrolled. Semi-automated gray-scale thresholding techniques using full width at half maximum (FWHM) and 2, 3 and 5 standard deviation (SD) above the remote myocardium were used and compared with the visual assessment, considered as the gold standard.ResultsLGE was identified in 41 MVP patients (62%) and quantified. The mean quantity of LGE visually assessed was 2.40 ± 1.07% or 1.40 ± 0.82 g. With FWHM, LGE resulted 3.56 ± 1.23% or 1.99 ± 1.13 g. Using thresholding, the mean LGE quantity was 9.2 ± 3.1% or 4.82 ± 2.28 g for 2-SD, 5.72 ± 1.75% or 3.06 ± 1.47 g for 3-SD and 2.36 ± 0.99% or 1.29 ± 0.79 g for 5-SD. The 5-SD measurement in percentage demonstrated a good correlation with LGE quantification visually assessed (2.40 ± 1.07 vs. 2.363 ± 0.9909, p = 0.543). When compared with the gold standard, the 5-SD threshold quantification, both in percentage and in grams, revealed the least intra-observer (respectively, ICC: 0.976 and 0.966) and inter-observer variability (respectively ICC: 0.948 and 0.935).ConclusionThe 5-SD gray-scale threshold technique in percentage revealed the best correlation with the visual assessment and an optimal reproducibility in MVP patient.

Predictors of and outcomes in arrhythmic mitral valve prolapse

Abstract Background Recently, the European Heart Rhythm Association (EHRA) defined the phenotype of the arrhythmic mitral valve prolapse (AMVP) complex and proposed management approaches. Assessing the incidence of SCD in this small subset of patients is challenging due to the low event-rate and longitudinal real-world data remain scarce. Purpose To determine the predictors of AMVP and study the clinical outcome of patients stratified by ICD indication as proposed by the EHRA consensus statement. Methods All consecutive patients with MVP who underwent cardiac magnetic resonance imaging (CMR) were enrolled in a single-center retrospective registry. AMVP was defined as the presence of frequent or complex ventricular arrhythmias (>5% total PVC burden, non-sustained ventricular tachycardia (VT), VT or ventricular fibrillation) in a patient with MVP without alternative arrhythmic substrates. The medical records of these patients included a comprehensive clinical, rhythmic and echocardiographic characterization allowing to collect the key date of transition to AMVP. A forward conditional multivariable logistic regression was performed to identify clinical risk factors for AMVP reporting the odds ratio (OR) and corresponding 95% confidence interval (95% CI). Incidence rates were calculated for the composite endpoint of SCD, sustained VT, ICD shocks, and all-cause mortality. Results A total of 169 patients (52.1% male, median age 51.4 years) were included, of which 99 (58.6%) were classified as AMVP. Clinical characteristics are presented in Image 1. Patients with non-arrhythmic MVP and AMVP had comparable left ventricular function, mitral valve regurgitation grade and extent of MVP. Multivariable logistic regression identified the presence of late gadolinium enhancement (LGE, OR 2.82, 95%CI 1.45–5.50, p=0.002) and mitral annular disjunction (MAD, OR 1.98, 95%CI 1.02–3.86, p=0.045) on CMR as only predictors of AMVP. Cardiac CMR results are presented in Image 2. The median overall follow-up since first cardiac contact was 8.0 years (IQR 5.0–15.6), while this was 5.0 years (IQR 2.1–7.4 years) after transition to AMVP. The incidence for the composite endpoint in the overall population was 0.4%/year (95%CI 0.2 – 0.9). The 5-year cumulative event rate was 1.8%. According to the EHRA risk stratification the implantation of an ICD would have been reasonable in 69 patients (69.7%). In total, 5 patients were implanted with an ICD for secondary prevention, but all those patients did not classify as AMVP prior to the life threathening arrhythmic event. Conclusion CMR is an important tool in the challenging risk stratification of AMVP given that the presence of MAD and LGE enhancement are predictors of AMVP. There is a lack of longitudinal data to determine when patients transition from MVP to AMVP and how this modifies their outcome.Clinical characteristics and ECG resultsOverview of CMR measurements

Mitral annular disjunction; how accurate are we? A cardiovascular MRI study defining risk

AimsMitral Annular Disjunction (MAD) refers to embryologic fibrous separation between mitral annular ring and basal left ventricular myocardium. Since its original description, the role of MAD in arrhythmic mitral valve prolapse (MVP) has been the subject of active research. In this study we sought to assess prognostic and imaging characteristics of MVP patients with and without underlying MAD. Methods and resultsPatients with posterior or bi-leaflet MVP were retrospectively identified via a review of all patients referred to our cardiac magnetic resonance (CMR) imaging laboratory from January 2015 to May 2022. MVP patients were further stratified by underlying MAD status. A total of 100 MVP patients undergoing CMR imaging (52 MVP patients with posterior MAD) were retrospectively identified with female comprising 55 % of the cohort. MVP patients with MAD were more likely to have an abnormal basal inferolateral/ papillary muscles LGE (51 % vs 21 %, p < 0.01). Posterior MAD longitudinal disjunction gap in ‘mm’ was a predictor of ventricular tachycardia (VT) [1.29, p = 0.01)]. Using ROC curve analysis, a disjunction gap of ≥ 4 mm was predictive of VT (AUC-0.71, p < 0.01), and incorporation of LGE in ROC model further improved AUC to 0.78 confirmed via Akaike information criterion (p < 0.01). ConclusionAbnormal LGE involving basal inferolateral myocardium and papillary muscles may provide etiologic substrate for arrythmia in MVP patients.

Abstract 16105: Sex-Based Differences in Potential Risk Factors for Sudden Death in Patients With Mitral Valve Prolapse

Background: The majority of individuals experiencing sudden arrhythmic death attributable to mitral valve prolapse (MVP) are women. However, factors leading to this increased risk in women are unknown. Aims: To describe sex differences in factors associated with MVP. Methods: We studied 637 patients aged 18-90 with MVP diagnosed by echocardiography at our center between 2016 and 2019. Clinical, echocardiographic and ECG parameters were compared between men and women using Fisher’s exact and Wilcoxon rank-sum tests, including features associated with arrhythmic MVP (bileaflet prolapse, mitral annular disjunction [MAD], ECG T-wave inversion (TWI), and complex or frequent ventricular ectopy (cfVE) including nonsustained ventricular tachycardia [NSVT]). All statistical tests are univariate, reported as OR [95% CI], and p-values are two-sided. Results: Among patients with MVP, 335 (52.6%) were women, 103 (16.2%) were African American and median (IQR) age was 67 (55-76.5) years. Women were more likely than men to have MAD (60/335 [17.9%] vs 35/302 [11.6%] respectively, 1.66 [1.06-2.61], p=0.026) and less likely to have moderate or severe mitral regurgitation (85/335 [25.4%] vs 99/302 [32.8%] respectively, 0.70 [0.49-0.98], p=0.044). There was no difference between women and men in presence of other features associated with arrhythmic MVP. Men were more likely than women to have cardiometabolic risk factors for sudden death including hypertension, diabetes, dyslipidemia, coronary artery disease, and heart failure. Conclusions: In patients with mitral valve prolapse, potential risk factors for sudden death vary by sex. Women are more likely than men to have mitral annular disjunction, while men are more likely to have cardiometabolic risk factors. Further studies are needed to determine whether mitral annular disjunction-associated ventricular arrhythmias can explain the increased incidence of sudden arrhythmic death in women with mitral valve prolapse.

The Prevalence and Characteristics of Arrhythmic Mitral Valve Prolapse in Patients With Unexplained Cardiac Arrest

BackgroundThere is growing evidence that mitral valve prolapse (MVP) is associated with otherwise unexplained cardiac arrest (UCA). However, reports are hindered by the absence of a systematic ascertainment of alternative diagnoses. ObjectivesThis study reports the prevalence and characteristics of MVP in a large cohort of patients with UCA. MethodsPatients were enrolled following an UCA, defined as cardiac arrest with no coronary artery disease, preserved left ventricular ejection fraction, and no apparent explanation on electrocardiogram. A comprehensive evaluation was performed, and patients were diagnosed with idiopathic ventricular fibrillation (IVF) if no cause was found. Echocardiography reports were reviewed for MVP. Patients with MVP were divided into 2 groups: those with IVF (AMVP) and those with an alternative diagnosis (nonarrhythmic MVP). Patient characteristics were then compared. The long-term outcomes of AMVP were reported. ResultsAmong 571 with an initially UCA, 34 patients had MVP (6%). The prevalence of definite MVP was significantly higher in patients with IVF than those with an alternative diagnosis (24 of 366 [6.6%] vs 5 of 205 [2.4%]; P = 0.03). Bileaflet prolapse was significantly associated with AMVP (18 of 23 [78%] vs 1 of 8 [12.5%]; P = 0.001; OR: 25.2). The proportion of patients with AMVP who received appropriate implantable cardioverter-defibrillator therapies over a median follow-up of 42 months was 21.1% (4 of 19). ConclusionsMVP is associated with otherwise UCA (IVF), with a prevalence of 6.6%. Bileaflet prolapse appears to be a feature of AMVP, although future studies need to ascertain its independent association. A significant proportion of patients with AMVP received appropriate implantable cardioverter-defibrillator therapies during follow-up.

Arrhythmic Mitral Valve Prolapse: A Comprehensive Review.

Mitral valve prolapse (MVP) is a prevalent cardiac disorder that impacts approximately 2% to 3% of the overall population. While most patients experience a benign clinical course, there is evidence suggesting that a subgroup of MVP patients face an increased risk of sudden cardiac death (SCD). Although a conclusive causal link between MVP and SCD remains to be firmly established, various factors have been associated with arrhythmic mitral valve prolapse (AMVP). This study aims to provide a comprehensive review encompassing the historical background, epidemiology, pathology, clinical manifestations, electrocardiogram (ECG) findings, and treatment of AMVP patients. A key focus is on utilizing multimodal imaging techniques to accurately diagnose AMVP and to highlight the role of mitral annular disjunction (MAD) in AMVP.

Effectiveness of the risk stratification proposed by the 2022 European Heart Rhythm Association Expert Consensus statement on arrhythmic mitral valve prolapse

Recently, an expert consensus statement proposed indications where implantation of a primary prevention implantable cardioverter-defibrillator (ICD) may be reasonable in patients with mitral valve prolapse (MVP). The objective was to evaluate the proposed risk stratification by the expert consensus statement. Consecutive patients with MVP without alternative arrhythmic substrates with cardiac magnetic resonance imaging (CMR) were included in a single-center retrospective registry. Arrhythmic MVP (AMVP) was defined as a total premature ventricular complex burden ≥5%, non-sustained ventricular tachycardia (VT), VT, or ventricular fibrillation. The end point was a composite of SCD, VT, inducible VT, and appropriate ICD shocks. In total, 169 patients (52.1% male, median age 51.4 years) were included and 99 (58.6%) were classified as AMVP. Multivariate logistic regression identified the presence of late gadolinium enhancement (OR 2.82, 95%CI 1.45-5.50) and mitral annular disjunction (OR 1.98, 95%CI 1.02-3.86) as only predictors of AMVP. According to the EHRA risk stratification, 5 patients with AMVP (5.1%) had a secondary prevention ICD indication, while in 69 patients (69.7%) the implantation of an ICD may be reasonable. During a median follow-up of 8.0 years (IQR 5.0-15.6), the incidence rate for the composite arrhythmic end point was 0.3%/year (95%CI 0.1-0.8). More than half of MVP patients referred for CMR met the AMVP diagnostic criteria. Despite low long-term event rates, in 70% of patients with AMVP the implantation of an ICD may be reasonable. Risk stratification of SCD in MVP remains an important knowledge gap and requires urgent investigation.

High-risk phenotypes of arrhythmic mitral valve prolapse: a systematic review and meta-analysis

Background Mitral valve prolapse (MVP) is associated with aggravated risk of ventricular tachycardia (VT), ventricular fibrillation (VF) and sudden cardiac death (SCD). There is a lack of specific guideline recommendation regarding risk stratification and management, despite multiple proposed high-risk phenotypes. We performed systematic review and meta-analysis to evaluate high-risk phenotypes for malignant arrhythmias in patients with MVP. Methods We comprehensively searched the databases of MEDLINE, SCOPUS, and EMBASE from inception to April 2023. Included studies were cohort and case-control comparing between MVP patients with and without VT, VF, cardiac arrest, ICD placement, or SCD. Data from each study were combined using the random-effects. Pooled odds ratios (OR) and 95% confidence intervals (CI) were calculated. Results Nine studies from 1985 to 2023 were included involving 2,279 patients with MVP. We found that T-wave inversion (OR 2.52; 95% CI: 1.90–3.33; p < 0.001), bileaflet involvement (OR 2.28; 95% CI: 1.69–3.09; p < 0.001), late gadolinium enhancement (OR 17.05; 95% CI: 3.41–85.22; p < 0.001), mitral annular disjunction (OR 3.71; 95% CI: 1.63–8.41; p < 0.002), and history of syncope (OR 6.96; 95% CI: 1.05–46.01; p = 0.044), but not female (OR 0.96; 95% CI: 0.46–2.01; p = 0.911), redundant leaflets (OR 4.30; 95% CI: 0.81–22.84; p = 0.087), or moderate-to-severe mitral regurgitation (OR 1.24; 95% CI: 0.65–2.37; p = 0.505), were associated with those events. Conclusion Bileaflet prolapse, T-wave inversion, mitral annular disjunction, late gadolinium enhancement, and history of syncope are high-risk phenotypes among population with MVP. Further research is needed to validate the risk stratification model and justify the role of primary prophylaxis against malignant arrhythmias.

Mitral Valve Prolapse and Sudden Cardiac Death in Athletes at High Risk.

Mitral valve prolapse (MVP) is the most frequent valvulopathy in the general population, with usually a favourable prognosis. Although it can be associated with some complications, ventricular arrhythmias (VA) and sudden cardiac death (SCD) are the most worrying. The estimated risk of SCD in MVP is between 0.2% to 1.9% per year, including MVP patients with and without severe mitral regurgitation (MR). The association between SCD and MVP is expressed by a phenotype called "malignant MVP" characterized by transthoracic echocardiography (TTE) findings such as bileaflet myxomatous prolapse and mitral annulus disjunction (MAD), ECG findings such as repolarization abnormalities, complex ventricular arrhythmias (c-VAs) and LV fibrosis of papillary muscles (PMs) and inferobasal wall visualized by late gadolinium enhancement cardiac magnetic resonance (LGE-CMR). Therefore, attention is raised for patients with "arrhythmic MVP" characterized from an ECG point of view by frequent premature ventricular contractions (PVCs) arising from one or both PMs as well as by T-wave inversion in the inferolateral leads. In athletes, SCD is the most frequent medical cause of death and in young subjects (< 35 years) usually is due to electrical mechanism affecting who has a silent cardiovascular disease and are not considered per se a cause of increased mortality. In MVP, SCD was reported to happen during sports activity or immediately after and valve prolapse was the only pathological aspect detected. The aim of the present paper is to explore the association between SCD and MVP in athletes, focusing attention on ECG, TTE in particular, and CMR findings that could help to identify subjects at high risk for complex arrhythmias and eventually SCD. In addition, it is also examined if sports activity might predispose patients with MVP to develop major arrhythmias.

P387 “THE IMPORTANCE OF NEW ADVANCED IMAGING TECHNIQUES IN SINE CAUSA VENTRICULAR ARRHYTHMIAS”

Abstract Case Presentation A 61–years–old woman was referred to our center after her 33 years old daughter suffered a sudden cardiac death. Cardiovascular risk factors: dyslipidemia and family history of ischemic cardiomyopathy. Diagnostic and Therapeutic Work Up Patient’s ECG was normal. Holter ECG showed frequent ventricular extrasystoles (2000/die) isolated and not premature, with LBB morphology, associated with not sustained ventricular tachycardias with LLB and low axis morphology (HR mean 140–170 beats/min). Exercise Stress Test did not show a clear correlation between physical activity and arrhythmias. Beta blocker therapy was started and was only partially effective in reducing ventricular arrhythmias, and a loop recorder was implanted. In 2019 a cardiac MRI with adenosine was performed, showing a mild globular shaped left ventricle with a non–specific linear distribution of LGE in the middle septum. Genetic analysis for channelopathies and cardiomyopathies resulted negative. In the last advanced echocardiogram (2022) with 3D reconstruction and strain analyses, characteristics suspected for arrhythmic mitral valve prolapse (MAD and curling of inferolateral wall) were identified. Accordingly, it has been decided to perform a second cardiac MRI to better characterize the underlying cardiac condition and to define the appropriate risk stratification algorithm. Conclusions Arrhythmic mitral valve prolapse is defined by ventricular arrhythmias in the absence of other known causes and it is present in up to 13% of sudden cardiac deaths in female patients. According to the recent consensus document by EHRA, sudden cardiac death risk stratification is complex and based firstly on the evaluation of arrhythmic burden, and secondly on symptoms and additional risk factors (i.e. MAD, left ventricle disfunction, flap redundancy, LGE in specific areas and so on). This case shows the importance of advanced imaging techniques and multimodal approach in ventricular arrhythmias evaluation.

Arrhythmic mitral valve prolapse in 2023: Evidence-based update.

Patients with mitral valve prolapse (MVP) may develop ventricular arrhythmias, ranging from premature ventricular contractions through more complex non-sustained ventricular tachycardia to sustained life-threatening ventricular arrhythmias. The prevalence of MVP in autopsy series of young adults who died suddenly has been estimated to be between 4% and 7%. Thus, "arrhythmic MVP" has been reported as an underappreciated cause of sudden cardiac death, leading to a renewed interest in the study of this association. The term "arrhythmic MVP" refers to a small subset of patients who have, in the absence of any other arrhythmic substrate, MVP, with or without mitral annular disjunction, and frequent or complex ventricular arrhythmias. Our understanding of their coexistence in terms of contemporary management and prognosis is still incomplete. While literature regarding the arrhythmic MVP may be contrasting despite recent consensus document, the present review summarizes the relevant evidence concerning the diagnostic approach, prognostic implications, and targeted therapies for MVP-related ventricular arrhythmias. We also summarize recent data supporting left ventricular remodeling, which complicates the coexistence of MVP with ventricular arrhythmias. As the evidence for a putative link between MVP-associated ventricular arrhythmias and sudden cardiac death is scarce and based on scant and retrospective data, risk prediction remains a challenge. Thus, we aimed at listing potential risk factors from available seminal reports for further use in a more reliable prediction model that requires additional prospective data. Finally, we summarize evidence and guidelines on targeted therapies of ventricular arrhythmias in the setting of MVP, including implantable cardioverter defibrillators and catheter ablation. Our review highlights current knowledge gaps and provides an action plan for structured research on the pathophysiological genesis, diagnosis, prognostic impact, and optimal management of patients with arrhythmic MVP.

Left Ventricular Fibrosis in Arrhythmic Mitral Valve Prolapse: Quantification and Comparison of Semi-Automated Techniques Assessed by Cardiac Magnetic Resonance

Left Ventricular Fibrosis in Arrhythmic Mitral Valve Prolapse: Quantification and Comparison of Semi-Automated Techniques Assessed by Cardiac Magnetic Resonance

347 ARRHYTHMIC MITRAL VALVE PROLAPSE AND MITRAL ANNULUS DISJUNCTION: WHEN PRIMARY PREVENTION IMPLANTATION OF ICD IS POSSIBLE?

Abstract Mitral valve prolapse (MVP) is the most common valvular heart disease. Although MVP is generally considered benign, it can be associated with important complications, including sudden cardiac death (SCD). MVP is often related to mitral annular disjunction (MAD), a structural abnormality involving a distinct separation of the left atrium/mitral valve annulus and myocardium continuum. It is associated with an increased risk of arrhythmias and is an integral component of the arrhythmic MVP (AMVP) complex. Here we present a case report of a patient with MAD, Barlow's syndrome with bileaflet mitral prolapse and a history of recurrent non sustained ventricular tachycardia (NSVT) who underwent subcutaneous cardioverter defibrillator (ICD) implant in primary prevention. A 49 year old woman, with a history of moderate mitral regurgitation and posterior leaflet prolapse, was sent to our hospital by her cardiologist for the evidence on holter ecg of several tracts of non sustained ventricular arrhythmias. Transesophageal echocardiogram revealed the presence of moderate mitral regurgitation, bileaflet prolapse (mainly of the redundant posterior leaflet), dilation of the left atrium and positive Pickelhaube sign, suggestive of MAD. After few days was performed cardiac magnetic resonance imaging with evidence of mitral annulus disjunction associated with bileaflet prolapse and moderate mitral regurgitation. Delayed gadolinium enhanced (DGE) signal was found in the posterior basal wall. During hospitalization, she presented daily episodes of high frequency (&amp;gt;180 bpm) NSVT with right bundle block (RBBB) morphology and several premature ventricular complex. It was therefore decided to discharge the patient with Life Vest and five months later, after asking second opinion to an Italian high specialization center, we decided to place a subcutaneous ICD in primary prevention for the high arrhythmic risk of the patient. Arrhythmic mitral valve prolapse (AMVP) is an estimated cause of sudden cardiac death. It is known that patients with MVP and MAD have a high arrhythmic risk but there aren't trials that demonstrate the benefits of primary ICD implantation. The recent “EHRA expert consensus statement on arrhythmic mitral valve prolapse and mitral annular disjunction “ affirms that can be reasonable, primary ICD implant, in patients with AMVP, 1 high risk features (VT (hemodynamically tolerated), NSVT, unexplained syncope) and 2 or more phenotypic risk features (—T-wave inversion in the inferior leads, repetitive documented polymorphic PVCs, MAD phenotype, redundant MV leaflet, enlarged left atrium or ejection fraction≤50%, LGE). For this reason, our patient underwent subcoutanues ICD implant. Therefore, we believe that further studies, to develop guidelines for risk stratification of patients with evident MVP, would be beneficial in reducing the incidence of potentially fatal events in these patients.

363 TRICUSPID ANNULUS DISJUNCTION: THE MISSING PIECE IN THE ARRHYTHMIC MITRAL VALVE PROLAPSE?

Abstract Background Arrhythmic mitral valve prolapse (MVP) is characterized by left ventricular (LV) fibrosis, due to an excessive myocardial stretch on the LV basal-mid inferolateral wall. Mitral annulus disjunction (MAD) and curling are common findings in arrhythmic MVP patients, thus representing the excessive traction of mitral apparatus on myocardium. Cardiac magnetic resonance (CMR) demonstrated to clearly identify these typical morpho-functional alterations, as well as LV fibrosis, in MVP patients. The involvement of right-sided annulus disease recently emerged as an interesting missing piece in the natural history of MVP patients. However, its role in a population of arrhythmic MVP patients in absence of significant mitral regurgitation (MR) has not been evaluated. Purpose The primary aim of the study was to evaluate the prevalence and the role of tricuspid annulus disjunction (TAD) in the arrhythmic MVP patients. Secondly, we evaluated the role of TAD in the prevalence and severity of the common morpho-functional alterations of MVP, MAD and curling. Methods Consecutive MVP patients with normal systolic function and without significant MR, referred for palpitations, were enrolled. Morpho-biventricular evaluations, including MAD and curling assessment, as well as LV fibrosis were evaluated using CMR. Results 66 patients met the inclusion criteria (29% male, median age 44 years). TAD was identified in 22 MVP patients (27% male, median age 52 years). No differences for ventricular arrhythmias (p: 0.459) between MVP patients without (n: 44) and with TAD (n: 22) were identified. MVP patients with TAD were older (52 vs 42 years, p: 0.009) and presented reduced biventricular end-diastolic volumes, despite normal, in comparison with those without TAD (left ventricle: 80.5 vs 93.5 ml/m2, p: 0.002; right ventricle: 72.5 vs 85.5 ml/m2, p: 0.018). MAD and curling were more common in patients without TAD (respectively, 25 vs 19, p:0.014 and 24 vs 20, p: 0.002) than in those with TAD. Conversely, MAD and curling are more severe in patients with TAD (respectively, 5.9 vs 4.7 mm and 4 vs 3 mm, both p: 0.034) than in those without TAD. No differences for LGE distribution were identified in the two groups (p: 0.062). Conclusion In arrhythmic MVP patients, presence of TAD was associated with a great severity of MAD and curling. So, despite uncommon, the contextual presence of TAD and MAD in arrhythmic MVP patients suggests a more severe biannular disease, characterized by an increased biventricular myocardial stretch. Conversely to MAD, TAD wasn't associated with ventricular arrhythmias. Thus, its role in ventricular arrhythmogenesis in MVP patients appeared limited.

Abstract 12600: Mitral Annular Disjunction: How Accurate Are We? A Cardiovascular MRI Study Defining Risk

Background: Mitral Annular Disjunction (MAD) refers to embryologic fibrous separation between mitral annular ring and basal left ventricular myocardium. Since its original description, the role of MAD in arrhythmic mitral valve prolapse (MVP) has been subject of active research. In this study we sought to assess prognostic and imaging characteristics of MVP patients with and without underlying MAD. Methods: Patients with posterior or bi-leaflet MVP were retrospectively identified via a review of all patients referred to our cardiac magnetic resonance (CMR) imaging laboratory from January 2015 to May 2022. MVP patients were further stratified by underlying MAD status. CMR characteristics including late gadolinium enhancement (LGE) distribution, clinical characteristics, and data on mitral valve intervention were determined. Results: A total of 100 MVP patients undergoing CMR imaging (52 MVP patients with posterior MAD) were retrospectively identified with female comprising 55% of the cohort. MVP patients with MAD were more likely to have an abnormal basal inferolateral/ papillary muscles LGE (51% vs 21%, p&lt;0.01). Posterior MAD longitudinal disjunction gap in ‘mm’ was a predictor of ventricular tachycardia (VT) [1.23, p=0.03)]. Similarly, posterior MAD as a binary variable was predictor of VT (11, p&lt;0.01), ventricular arrhythmia (composite of VT, and non-sustained VT) (2.4, p=0.03), and major adverse cardiac events (MACE) (composite of VT, syncope, and mitral valve intervention) (2.5, p=0.03). Using ROC curve analysis, a disjunction gap of &gt; 4 mm was predictive of VT (AUC-0.71, p&lt;0.01), and incorporation of LGE in ROC model further improved AUC to 0.78 confirmed via Akaike information criterion (p&lt;0.05), see Figure. Conclusion: Abnormal LGE involving basal inferolateral myocardium and papillary muscles may provide substrate for arrythmia in MVP patients. Further, for the first time, a MAD threshold of &gt; 4 mm is demonstrated to stratify the VT risk.

Abstract 15502: Exercise as a Risk Factor for Arrhythmic Mitral Valve Prolapse

Background: Mitral valve prolapse (MVP) is a common valvular abnormality found in approximately 2.4% of the population. Whereas most cases are benign, an increasingly recognized sequela of MVP is sudden cardiac death (SCD), but risk factors for SCD are not well-defined. Exercise has not been explored as a potential risk factor for SCD in MVP. Hypothesis: We tested the hypothesis that patients with MVP and SCD would be more likely to exercise or be competitive athletes than MVP controls with the capacity to exercise. Methods - The study population was generated from the Duke Epic EHR and determined by ICD-10 codes for MVP and SCD. A comparison group of patients with MVP with exercise data was used. Patients were included if they had guideline-based diagnosis of MVP on echocardiogram or cardiac magnetic resonance imaging. Exercise &lt;1 hour per week was defined as being sedentary and time &gt;1 hour per week or status as a competitive athletes were defined as habitual exercisers. Results: Of the 27 patients with MVP and SCD 18 had documentation of exercise time per week. There were 42 in the control group. Patients with SCD were younger [(54.8 +/-15.1) vs. (63.9 +/- 14.1) p= 0.03], and more likely to have bileaflet prolapse [13/18 (72%) vs. 15/42 (36%), p=0.01]. Patients with SCD were more likely to be habitual exercisers vs. sedentary [16/18 (89%) vs. 17/42 (40.5%), p = .025]. 20 patients with MVP and SCD had documentation of activity level at the time of event. 10 were exercising (50%), 5 (25%) working or doing housework, 4 (20%) resting and 1 (5%) sleeping. Conclusion: Exercise time &gt;1 hour per week is associated with sudden cardiac death in Mitral Valve Prolapse. Exercise may be a trigger of SCD; however, more data is needed to better characterize exercise as a risk factor for SCD in this population.

Abstract 11350: Arrhythmogenic Mitral Valve Prolaspe and Mitral Annular Disjunction

Background: Mitral valve prolapse (MVP) affects about 2-3% of the general population. A small proportion of persons may have arrhythmic symptoms, leading to ventricular arrhythmias and sudden cardiac death (SCD). Mitral annular disjunction is a recently described entity that may be present with MVP or independently. Case: A 55-year-old male patient presented to the hospital with cardiac arrest. He has a history of percutaneous coronary intervention with stenting to left anterior descending (LAD) and right coronary artery (RCA) in 2015. He had ventricular fibrillation (VF) and subsequently arrested and underwent resuscitation. Electrocardiogram (EKG) showed sinus rhythm with prolonged QTc 487msec with no ischemia; echocardiography showed mitral annular disjunction, severe left ventricular global hypokinesis with ejection fraction of 10%. Left heart catheterization revealed nonobstructive moderate coronary artery disease. Right heart catheterization showed a severely reduced cardiac index of 2.1, and he was placed on mechanical support (Impella) for cardiogenic shock. On day 5, repeat echocardiography revealed left ventricular ejection fraction of 60%. Subsequent cardiac magnetic resonance (CMR) showed no evidence of infiltrative diseases but revealed an inferolateral wall scar from previous myocardial infarction and MVP with MAD. An implantable cardiac defibrillator was placed for secondary prevention of SCD. Discussion: The potential etiology of our patient's ventricular fibrillation cardiac arrest may include a scar from previous myocardial infarction or arrhythmogenic MVP with MAD. Myocardial fibrosis arising from MAD may be an origin of ectopic activity and arrhythmia. EKG, echocardiographic, and cardiac magnetic resonance imaging (CMR) are essential in diagnosing arrhythmic MVP and MAD. Young patients with premature ventricular contractions without clear etiology should be evaluated for MAD, and incidental findings of MAD should warrant further investigations, given its arrhythmogenic potential. Conclusion: MVP with MAD is associated with life-threatening arrhythmias. Further studies are required to explore treatment options to reduce the incidence of malignant VAs and SCDs in patients with MVP.