Age-related changes in mitral annular disjunction: A retrospective analysis using enhanced cardiac CT.

AIMS: The current recognition of mitral annular disjunction (MAD) as an anatomical abnormality potentially associated with ventricular arrhythmias has sparked controversy regarding its prevalence and clinical implications. This study aimed to investigate the prevalence and extent of MAD in individuals with no significant structural abnormalities involving the left heart using enhanced cardiac CT, while also exploring potential factors, such as age, that may be associated with MAD. METHODS: Systolic datasets of cardiac CT from 742 subjects were retrospectively included. MAD was determined by rotating orthogonal multiplanar reconstruction images around the central axis of the mitral annulus. The maximal distance of disjunction (DMAD) and segments involved (SI, 0 to 5 basal segments at left ventricular wall) was quantified to evaluate the extent of separation. RESULTS: In total, 449 (60.5%) had MAD. Subjects with MAD were significantly older (51.3 ± 19.9 years vs. 29.6 ± 20.3 years, P < 0.001). Age was found to be an independent relevant factor for MAD (OR = 1.059; 95%CI: 1.033, 1.085; P < 0.001). Subjects with MAD were then divided into 4 subgroups (G1: ≤20 years, G2: 21-40 years, G3: 41-60 years, G4: ≥61 years). DMAD and SI of each age subgroup were 1.9 ± 0.2 mm, 2.0 ± 1.2 (G1, n = 36), 2.7 ± 0.8 mm, 2.9 ± 1.3 (G2, n = 51), 3.0 ± 0.8 mm, 3.0 ± 1.3 (G3, n = 183), and 3.0 ± 1.0 mm, 3.7 ± 1.1 (G4, n = 179). Age was an independent relevant factor associated with DMAD (R2 = 0.132; ß = 0.014; 95%CI: 0.004, 0.024; P = 0.007) and SI (OR = 1.030; 95%CI: 1.005, 1.055; P = 0.016). CONCLUSIONS: MAD is a common finding on cardiac CT. Its prevalence and extent increase with age.

Characteristic of myocardial work in mitral valve prolapse with versus without mitral annular disjunction: A propensity-matched study.

BACKGROUND: Mitral annular disjunction (MAD) tends to coexist with mitral valve prolapse (MVP) and mitral regurgitation (MR), and is also highly associated with arrhythmias. Myocardial work (MW) analysis is dedicated to estimate myocardial performance by integrating strain analysis and afterload. We aimed to use MW analysis to investigate the cardiac remodeling and dysfunction in MAD, particularly the damage of some segments, and to enhance the understanding of the correlations between MW parameters and VAs within MVP patients. METHODS: A total of 22 consecutive MVP patients with MAD (MAD+) and 44 consecutive MVP patients without MAD (MAD-) (50 ± 11yeas; 18% females) were screened by propensity score matching (PSM), and were divided into subgroups based on MR severity (MR+: Grade 2+; MR-: ≤1), GWI median (GWI ≤ 2079.5 mmHg%; GWI>2079.5 mmHg%), as well as the presence of VAs (VAs+; VAs-). MW parameters consist of global work efficiency (GWE), global work index (GWI), global constructive work (GCW) and global wasted work (GWW). RESULTS: The MAD+ patients had larger LVEDD and LAVI, as well as lower GWE, GWI, and GCW (all P<0.05) compared to the MAD- patients, regardless of similar GLS and regurgitant volume(both P>0.05). When categorized by MR severity, GWI (P = 0.049) and GCW (P = 0.040) were diminished in the MR-MAD+ group. The regional analysis showed MAD+ patients had decreased MW index in the basal (posterior and inferior) and mid (posterior and inferior) segments. Multivariate linear regression showed MAD phenotype, but not MR severity, was independently associated with diminished GWE, GWI, and GCW (all P<0.05). When divided by GWI median, MAD phenotype [OR (95%CI): 5.189 (1.193-22.572), P = 0.028] was an independent predictor of decreased GCW. The receiver-operating characteristic curve identified bileaflet prolapse [AUC (95%CI): 0.664 (0.502-0.825), P = 0.045], and GWI for basal inferior [(AUC (95%CI): 0.679 (0.538-0.819), P = 0.020] as the predictors of the VAs. CONCLUSION: MAD phenotype has the ability to compromise cardiac structure and function, irrespective of volume overload, as evidenced by dilated LV and impaired MW index in basal and mid segments. Excessively decreased regional MW index can identify patients with the high risk of VAs. MW analysis can be a valuable imaging marker for detecting myocardial impairment induced by MAD.

Mitral Valve Prolapse With Mitral Annular Disjunction Causing Ventricular Tachycardia During a Stress Test: A Case Report.

Mitral valve prolapse (MVP) is a relatively common valvular disorder characterized by displacement of one or both mitral valve leaflets into the left atrium (LA) during systole. Mitral annular disjunction (MAD) is an associated abnormality where a portion of the mitral valve annulus attaches superiorly in the left atrial wall. Although MVP is often considered benign, it can rarely lead to serious complications such as ventricular arrhythmias, especially when MAD is present. Herein, we present a case of a 63-year-old male with MVP and MAD who experienced sustained ventricular tachycardia (VT) during cardiac stress testing. This case underscores the importance of recognizing MVP with MAD as a potential substrate for ventricular arrhythmias, notably under heightened physiological or induced periods of stress.

Arrhythmogenic Mitral Valve Prolapse: Can We Risk Stratify and Prevent Sudden Cardiac Death?

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.

Case report: arrhythmic mitral valve prolapse syndrome-are risk factors underdiagnosed?

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.

Severe tricuspid regurgitation with biannular disjunction requiring surgical treatment: a case report.

Background: Tricuspid annular disjunction (TAD) is an annular disjunction of the right-sided heart. Although TAD is often concomitant with mitral annular disjunction (MAD), it often presents as mitral regurgitation (MR), rather than tricuspid regurgitation (TR). While the clinical significance of MAD has been well-established, there is still little data on TAD. This is a rare case of severe TR due to TAD that appears to be isolated from MAD. Case summary: A 63-year-old female complaining of pre-syncope and dyspnoea on exertion was referred to our department. Initial transthoracic echocardiography showed MR and TR due to tricuspid valve prolapse (TVP). On transoesophageal echocardiography, the TVP consisted of an excessively redundant anterior leaflet, where the annular disjunction and severe regurgitation were formed. She recently underwent mitral and tricuspid valve plasties for symptomatic primary severe TR. Discussion: This case report emphasizes the clinical significance of TAD as a potential cause of severe TR, even without significant MR. Tricuspid annular disjunction progresses more gradually compared with MAD. This case suggests that remodelling of the right atrium, particularly in chronic atrial fibrillation, may contribute to the development of TR. Despite diagnostic challenges due to the flexible and dynamic nature of the tricuspid annulus, this is the first report of TAD-induced severe TR necessitating surgical intervention. Accurately diagnosing TAD remains challenging with current imaging modalities, emphasizing the need for improved diagnostic tools to optimize treatment strategies.

Mitral valve prolapse: arrhythmic risk during pregnancy and postpartum.

BACKGROUND AND AIMS: Arrhythmic mitral valve prolapse (AMVP) is linked to life-threatening ventricular arrhythmias (VAs), and young women are considered at high risk. Cases of AMVP in women with malignant VA during pregnancy have emerged, but the arrhythmic risk during pregnancy is unknown. The authors aimed to describe features of women with high-risk AMVP who developed malignant VA during the perinatal period and to assess if pregnancy and the postpartum period were associated with a higher risk of malignant VA. METHODS: This retrospective international multi-centre case series included high-risk women with AMVP who experienced malignant VA and at least one pregnancy. Malignant VA included ventricular fibrillation, sustained ventricular tachycardia, or appropriate shock from an implantable cardioverter defibrillator. The authors compared the incidence of malignant VA in non-pregnant periods and perinatal period; the latter defined as occurring during pregnancy and within 6 months after delivery. RESULTS: The authors included 18 women with AMVP from 11 centres. During 7.5 (interquartile range 5.8-16.6) years of follow-up, 37 malignant VAs occurred, of which 18 were pregnancy related occurring in 13 (72%) unique patients. Pregnancy and 6 months after delivery showed increased incidence rate of malignant VA compared to the non-pregnancy period (univariate incidence rate ratio 2.66, 95% confidence interval 1.23-5.76). CONCLUSIONS: The perinatal period could impose increased risk of malignant VA in women with high-risk AMVP. The data may provide general guidance for pre-conception counselling and for nuanced shared decision-making between patients and clinicians.

Serial Cardiac Magnetic Resonance Imaging in Patients with Mitral Valve Prolapse-A Single-Center Retrospective Registry.

Background: Mitral valve prolapse (MVP) and mitral annular disjunction (MAD) are common valvular abnormalities that have been associated with ventricular arrhythmias (VA). Cardiac magnetic resonance imaging (CMR) has a key role in risk stratification of VA, including assessment of late gadolinium enhancement (LGE). Methods: Single-center retrospective analysis of patients with MVP or MAD who had >1 CMR and >1 24 h Holter registration available. Data are presented in detail, including evolution of VA and presence of LGE over time. Results: A total of twelve patients had repeated CMR and Holter registrations available, of which in four (33%) patients, it was conducted before and after minimal invasive mitral valve repair (MVR). After a median of 4.7 years, four out of eight (50%) patients without surgical intervention had new areas of LGE. New LGE was observed in the papillary muscles and the mid to basal inferolateral wall. In four patients, presenting with syncope or high-risk non-sustained ventricular tachycardia (VT), programmed ventricular stimulation was performed and in two (50%), sustained monomorphic VT was easily inducible. In two patients who underwent MVR, new LGE was observed in the basal inferolateral wall of which one presented with an increased burden of VA. Conclusions: In patients with MVP and MAD, repeat CMR may show new LGE in a small subset of patients, even shortly after MVR. A subgroup of patients who presented with an increase in VA burden showed new LGE upon repeat CMR. VA in patients with MVP and MAD are part of a heterogeneous spectrum that requires further investigation to establish risk stratification strategies.

Mitral annular disjunction and its progression during childhood in Marfan syndrome.

AIMS: Data on mitral annular disjunction (MAD) in children with Marfan syndrome (MFS) are sparse. To investigate the diagnostic yield of MAD by echocardiography and cardiac magnetic resonance imaging (CMR), its prevalence and progression during childhood. METHODS AND RESULTS: We included patients <21 years old with MFS, defined by 2010 Ghent criteria and a pathogenic FBN1 variant or ectopia lentis. Two readers measured systolic separation between the mitral valve (MV) posterior hinge point and left ventricular (LV) myocardium on initial and subsequent imaging. MAD was defined as MV-LV separation ≥2 mm, MV prolapse (MVP) as atrial displacement ≥2 mm. Kappa coefficients evaluated echocardiogram-CMR agreement. Bland-Altman and intraclass correlation coefficients (ICCs) assessed inter-rater and inter-modality reliability. Univariable mixed-effects linear regression was used to evaluate longitudinal changes of MAD. MAD was detected in 60% (110/185) eligible patients. MVP was present in 48% (53/110) of MAD and MAD in 90% (53/59) of MVP. MAD detection by CMR and echocardiography had 96% overall agreement (Kappa = 0.89, P < 0.001) and a 0.32 mm estimate bias (95% CI 0.00, 0.65). ICC by echocardiography, CMR, and between modalities were 0.97 (95% CI 0.93, 0.98), 0.92 (95% CI 0.79, 0.97), and 0.91 (95% CI 0.85, 0.94), respectively. MAD was associated with aortic root dilation (P < 0.001). MAD was found in children of all ages, increased +0.18 mm/year (95% CI +0.14, +0.22) during a median duration of 5.5 years (IQR 3.1, 7.5 years). MAD indexed by height yielded a constant value +0.0002 mm/m/year (95% CI -0.0002, +0.0005 mm/m/year). CONCLUSION: MAD was common in pediatric MFS and was associated with aortic root dilation. MAD detection by echocardiography and CMR was highly reliable, suggesting that routine assessment in MFS is feasible. MAD was present in neonates and progressed over time but remained constant when indexing by height. Further studies are needed to evaluate MAD as a biomarker for clinical outcomes in pediatric MFS.

Evaluation of left ventricular function in patients with mitral annular disjunction using speckle tracking echocardiography.

BACKGROUND: Mitral annular disjunction (MAD) is a structural abnormality characterized by the systolic detachment of the posterior mitral annulus and the ventricular myocardium. It is usually observed coexistent with mitral valve prolapse (MVP) and associated with a mechanical dysfunction despite preserved electrical isolation function of the mitral annulus. This study aimed to evaluate left ventricular (LV) function using speckle tracking echocardiography in MVP patients with MAD. METHODS: This study was designed as a prospective, single-center study including 103 patients with MVP and 40 age- and sex-matched control subjects. Transthoracic echocardiography and cardiac magnetic resonance imaging were performed to assess LV function and MAD presence. RESULTS: MAD (+) MVP (n = 34), MAD (-) MVP (n = 69), and control (n = 40) groups were enrolled in the study. Among the MVP patients, 34 (33%) had MAD. T-negativity in the inferior leads on electrocardiography was more frequent in the MAD (+) group than in the MAD (-) patients (4.3% vs. 20.6%, p = .014). Mitral regurgitation degree, Pickelhaube sign (17.6% vs. 1.4%, p = .005), and late gadolinium enhancement frequency (35.3% vs. 10.6%, p = .002) were significantly higher in MAD (+) patients. MAD (+) patients had significantly impaired global longitudinal strain (-23.1 ±  2.1 vs. -23.5 ± 2.3, p < .001), basal longitudinal strain (BLS) (-19.6 ±  1.5 vs. -20.5 ± 1.9, p < .001), Mid-Ventricular Longitudinal Strain (-22.2 ± 1.7 vs. -23.2 ± 2.2, p < .001) and LA strain (-24.5 ± 3.9 vs. -27.2 ± 3.6, p < .001) when compared to MAD (-) MVP patients, despite similar LV ejection fraction. All these values of MVP patients were also significantly lower than the control group. The mean MAD distance was 7.8 ± 3.2 mm in MAD (+) patients. Patients with two or more symptoms were higher in the MAD (+) group than in the MAD (-) group (4.3% vs. 44.1%, p < .001). CONCLUSION: This study demonstrated a significant decrease in longitudinal strain in MVP patients with MAD, indicating myocardial dysfunction. These findings suggest that MAD may contribute to LV dysfunction and highlight the importance of early detection in younger patients. Further research is needed to explore the functional implications and long-term outcomes of MAD.

Mitral annular disjunction in out-of-hospital cardiac arrest patients-a retrospective cardiac MRI study.

BACKGROUND: Mitral annular disjunction (MAD), defined as defective attachment of the mitral annulus to the ventricular myocardium, has recently been linked to malignant arrhythmias. However, its role and prognostic significance in patients requiring cardiopulmonary resuscitation (CPR) remain unknown. This retrospective analysis aimed to describe the prevalence and significance of MAD by cardiac magnetic resonance (CMR) imaging in out-of-hospital cardiac arrest (OHCA) patients. METHODS: Eighty-six patients with OHCA and a CMR scan 5 days after CPR (interquartile range (IQR): 49 days before - 9 days after) were included. MAD was defined as disjunction-extent ≥ 1 mm in CMR long-axis cine-images. Medical records were screened for laboratory parameters, comorbidities, and a history of arrhythmia. RESULTS: In 34 patients (40%), no underlying cause for OHCA was found during hospitalization despite profound diagnostics. Unknown-cause OHCA patients showed a higher prevalence of MAD compared to definite-cause patients (56% vs. 10%, p < 0.001) and had a MAD-extent of 6.3 mm (IQR: 4.4-10.3); moreover, these patients were significantly younger (43 years vs. 61 years, p < 0.001), more often female (74% vs. 21%, p < 0.001) and had fewer comorbidities (hypertension, hypercholesterolemia, coronary artery disease, all p < 0.005). By logistic regression analysis, the presence of MAD remained significantly associated with OHCA of unknown cause (odds ratio: 8.49, 95% confidence interval: 2.37-30.41, p = 0.001) after adjustment for age, presence of hypertension, and hypercholesterolemia. CONCLUSIONS: MAD is rather common in OHCA patients without definitive aetiology undergoing CMR. The presence of MAD was independently associated to OHCA without an identifiable trigger. Further research is needed to understand the exact role of MAD in OHCA patients.

Description and prevalence of ventricular mitral annular disjunction: variation of normality or pathological variant?

INTRODUCTION AND OBJECTIVES: The aim of this study was to investigate a new variation of the atrial wall-mitral annulus-ventricular wall junction along the mural mitral leaflet and commissures: the ventricular mitral annular disjunction (v-MAD). This new variant is characterized by spatial displacement of the mitral leaflet hinge line by more than 2mm toward the left ventricle. METHODS: We examined a cohort of autopsied human hearts (n=224, 21.9% females, 47.9±17.6 years) from patients without known cardiovascular disease to identify the presence of v-MAD. RESULTS: More than half (57.1%) of the hearts showed no signs of MAD in the mural mitral leaflet or mitral commissures. However, v-MAD was found in 23.6% of cases, located within 20.1% of mural leaflets, 2.2% in superolateral commissures, and 1.3% in inferoseptal commissures. V-MAD was not uniformly distributed along the mitral annulus circumference, with the most frequent site being the P2 scallop (19.6% of hearts). The v-MAD height was significantly greater in mural leaflets than in commissures (4.4 mm±1.2 mm vs 2.1 mm±0.1 mm; P<.001). No specific variations in mitral valve morphology or anthropometrical features of donors were associated with the presence or distribution of v-MADs. Microscopic examinations revealed the overlap of the thin layer of atrial myocardium over ventricular myocardium in areas of v-MAD. CONCLUSIONS: Our study is the first to present a detailed definition and morphometric description of v-MAD. Further studies should focus on the clinical significance of v-MAD to elucidate whether it represents a benign anatomical variant or a significant clinical anomaly.

Mitral Annular Disjunction: An Under-Recognized Entity in Pediatrics.

Mitral annular disjunction (MAD) is a rare and under-recognized entity in the pediatric population. We present 2 cases of MAD in previously healthy pediatric patients and highlight clinical scenarios where MAD should be suspected.

Mitral annular disjunction in idiopathic ventricular fibrillation patients: just a bystander or a potential cause?

AIMS: Previously, we demonstrated that inferolateral mitral annular disjunction (MAD) is more prevalent in patients with idiopathic ventricular fibrillation (IVF) than in healthy controls. In the present study, we advanced the insights into the prevalence and ventricular arrhythmogenicity by inferolateral MAD in an even larger IVF cohort. METHODS AND RESULTS: This retrospective multi-centre study included 185 IVF patients [median age 39 (27, 52) years, 40% female]. Cardiac magnetic resonance images were analyzed for mitral valve and annular abnormalities and late gadolinium enhancement. Clinical characteristics were compared between patients with and without MAD. MAD in any of the 4 locations was present in 112 (61%) IVF patients and inferolateral MAD was identified in 24 (13%) IVF patients. Mitral valve prolapse (MVP) was found in 13 (7%) IVF patients. MVP was more prevalent in patients with inferolateral MAD compared with patients without inferolateral MAD (42 vs. 2%, P < 0.001). Pro-arrhythmic characteristics in terms of a high burden of premature ventricular complexes (PVCs) and non-sustained ventricular tachycardia (VT) were more prevalent in patients with inferolateral MAD compared to patients without inferolateral MAD (67 vs. 23%, P < 0.001 and 63 vs. 41%, P = 0.046, respectively). Appropriate implantable cardioverter defibrillator therapy during follow-up was comparable for IVF patients with or without inferolateral MAD (13 vs. 18%, P = 0.579). CONCLUSION: A high prevalence of inferolateral MAD and MVP is a consistent finding in this large IVF cohort. The presence of inferolateral MAD is associated with a higher PVC burden and non-sustained VTs. Further research is needed to explain this potential interplay.

Implications of Mitral Annular Disjunction in Patients Undergoing Transcatheter Edge-to-Edge Repair for Degenerative Mitral Regurgitation.

BACKGROUND: Little is known about mitral transcatheter edge-to-edge repair (TEER) in patients with mitral annular disjunction (MAD). OBJECTIVES: The authors sought to explore TEER for degenerative mitral regurgitation (MR) according to MAD status. METHODS: We retrospectively analyzed 271 consecutive patients (median age 82 [Q1-Q3: 75-88] years, 60.9% men) undergoing an isolated, first-ever TEER for whom there were viewable preprocedural echocardiograms. Stratified by MAD status at baseline, the cohort was evaluated for all-cause mortality, heart failure hospitalizations, and mitral reinterventions-the composite of which constituted the primary outcome-as well as functional capacity and residual MR, all along the first postprocedural year. RESULTS: Individuals with (n = 62, 22.9%) vs without MAD had more extensive prolapse and larger valve dimensions. Although the former's procedures were longer, utilizing more devices per case, technical success rate and residual MR were comparable. MAD presence was associated with higher mortality risk (HR: 2.64; 95% CI: 1.82-5.52; P = 0.014), and increased MAD length-with lower odds of functional class ≤II (OR: 0.65; 95% CI: 0.47-0.88; P = 0.006). Among 47 MAD patients with retrievable 1-month data, MAD regressed in 91.5% and by an overall 50% (Q1-Q3: 22%-100%) compared with baseline (P < 0.001). A greater MAD shortening conferred attenuated risk for the primary outcome. CONCLUSIONS: In our experience, TEER for degenerative MR accompanied by MAD was feasible and safe; however, its postprocedural course was somewhat less favorable. MAD shortening following TEER was observed in most patients and proved prognostically beneficial.

Mitral annular disjunction in surgical mitral valve prolapse: prevalence, characteristics and outcomes.

BACKGROUND: There is a paucity of literature regarding outcomes of patients with mitral valve prolapse (MVP) and mitral annular disjunction (MAD) after mitral surgery, with many unanswered questions including the post-surgical persistence of MAD, effect of MAD on mitral valve reparability, and incidence of arrhythmia after mitral valve surgery. We aimed to examine the prevalence, imaging characteristics and clinical associations of mitral annular disjunction among patients undergoing mitral valve surgery for mitral valve prolapse, as well as outcomes after surgery including the persistence of MAD, arrhythmic events and excess mortality. RESULTS: A retrospective analysis of 111 consecutive patients who underwent mitral valve surgery for MVP was performed. A total of 32 patients (28.8%) had MAD. Patients with MAD were younger (64 vs 67 yrs, p = 0.04), with lower rates of hypertension (21.9% vs 50.6%, p = 0.01) and hyperlipidaemia (25% vs 50.6%; p = 0.01) and were more likely to be female (43.8% vs 21.4%, p = 0.04) with myxomatous leaflets > 5mm (90.6% vs 15.2%, p = < 0.01) and bileaflet prolapse (31.3% vs 10.1%, p = 0.02). Mitral valve repair was performed in 29/32 patients (90.6%) in the MAD positive group, and no patients had the persistence of MAD post-surgery. Post-operative ventricular arrhythmia was higher in the MAD positive group (28.13% vs 11.69%, p = 0.04) with no difference in mortality, 30-day hospital re-admission, or post-operative mitral regurgitation between patients with and without MAD over 3.91 years of follow up. CONCLUSION: In this study of consecutive patients with MVP undergoing surgery, MAD was a common finding (almost 1 in 3). MAD does not compromise mitral valve surgical reparability, and both repair and replacement are effective at correcting disjunction. Our data suggest that concurrent MAD in MVP patients undergoing surgery has no significant effect on post surgical outcomes. Further research as to whether this patient cohort requires post-surgical arrhythmia monitoring is warranted.

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

Aims: 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 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 results: 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. 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). Conclusion: Abnormal LGE involving basal inferolateral myocardium and papillary muscles may provide etiologic substrate for arrythmia in MVP patients.

Lifetime exercise dose and ventricular arrhythmias in patients with mitral valve prolapse.

AIMS: Patients with mitral valve prolapse (MVP) have high risk of life-threatening ventricular arrhythmias (VAs). Data on the impact of exercise on arrhythmic risk in these patients are lacking. We explored whether lifetime exercise dose was associated with severe VA and with established risk factors in patients with MVP. Furthermore, we explored the circumstances at the VA event. METHODS AND RESULTS: In this retrospective cohort study, we included patients with MVP and assessed lifetime exercise dose as metabolic equivalents of task (MET) hours/week. Severe VA was defined as sustained ventricular tachycardia or fibrillation, aborted cardiac arrest, and appropriate shock by a primary preventive implantable cardioverter defibrillator. We included 136 MVP patients (48 years [interquartile range (IQR) 35-59], 61% female), and 17 (13%) had previous severe VA. The lifetime exercise dose did not differ in patients with and without severe VA (17 MET h/week [IQR 9-27] vs. 14 MET h/week [IQR 6-31], P = 0.34). Lifetime exercise dose > 9.6 MET h/week was a borderline significant marker for severe VA (OR 3.38, 95% CI 0.92-12.40, P = 0.07), while not when adjusted for age (OR 2.63, 95% CI 0.66-10.56, P = 0.17). Ventricular arrhythmia events occurred most frequently during wakeful rest (53%), followed by exercise (29%) and sleep (12%). CONCLUSION: We found no clear association between moderate lifetime exercise dose and severe VA in patients with MVP. We cannot exclude an upper threshold for safe levels of exercise. Further studies are needed to explore exercise and risk of severe VA.