Patient-reported outcome measures on mental health and psychosocial factors in patients with Brugada syndrome.

AIMS: Brugada syndrome (BrS) is a hereditary arrhythmic disease, associated with sudden cardiac death. To date, little is known about the psychosocial correlates and impacts associated with this disease. The aim of this study was to assess a set of patient-reported psychosocial outcomes, to better profile these patients, and to propose a tailored psychosocial care. METHODS AND RESULTS: Patients were recruited at the European reference Centre for BrS at Universitair Ziekenhuis Brussel, Belgium. Recruitment was undertaken in two phases: phase 1 (retrospective), patients with confirmed BrS, and phase 2 (prospective), patients referred for ajmaline testing who had an either positive or negative diagnosis. BrS patients were compared to controls from the general population. Two hundred and nine questionnaires were analysed (144 retrospective and 65 prospective). Collected patient-reported outcomes were on mental health (12 item General Health Questionnaire; GHQ-12), social support (Oslo Social Support Scale), health-related quality of life, presence of Type-D personality (Type-D Scale; DS14), coping styles (Brief-COPE), and personality dimensions (Ten Item Personality Inventory). Results showed higher mental distress (GHQ-12) in BrS patients (2.53 ± 3.03) than in the general population (P < 0.001) and higher prevalence (32.7%) of Type D personality (P < 0.001) in patients with confirmed Brugada syndrome (BrS +). A strong correlation was found in the BrS + group (0.611, P < 0.001) between DS14 negative affectivity subscale and mental distress (GHQ-12). CONCLUSION: Mental distress and type D personality are significantly more common in BrS patients compared to the general population. This clearly illustrates the necessity to include mental health screening and care as standard for BrS.

Electrical markers and arrhythmic risk associated with myocardial fibrosis in mitral valve prolapse.

AIMS: We aimed to characterize the substrate of T-wave inversion (TWI) using cardiac magnetic resonance (CMR) and the association between diffuse fibrosis and ventricular arrhythmias (VA) in patients with mitral valve prolapse (MVP). METHODS AND RESULTS: TWI was defined as negative T-wave ≥0.1 mV in ≥2 adjacent ECG leads. Diffuse myocardial fibrosis was assessed by T1 relaxation time and extracellular volume (ECV) fraction by T1-mapping CMR. We included 162 patients with MVP (58% females, age 50 ± 16 years), of which 16 (10%) patients had severe VA (aborted cardiac arrest or sustained ventricular tachycardia). TWI was found in 34 (21%) patients. Risk of severe VA increased with increasing number of ECG leads displaying TWI [OR 1.91, 95% CI (1.04-3.52), P = 0.04]. The number of ECG leads displaying TWI increased with increasing lateral ECV (26 ± 3% for TWI 0-1leads, 28 ± 4% for TWI 2leads, 29 ± 5% for TWI ≥3leads, P = 0.04). Patients with VA (sustained and non-sustained ventricular tachycardia) had increased lateral T1 (P = 0.004), also in the absence of late gadolinium enhancement (LGE) (P = 0.008). CONCLUSIONS: Greater number of ECG leads with TWI reflected a higher arrhythmic risk and higher degree of lateral diffuse fibrosis by CMR. Lateral diffuse fibrosis was associated with VA, also in the absence of LGE. These results suggest that TWI may reflect diffuse myocardial fibrosis associated with VA in patients with MVP. T1-mapping CMR may help risk stratification for VA.

Left atrium remodeling predicts late recurrence of paroxysmal atrial fibrillation after second generation cryoballoon ablation.

BACKGROUND: Atrial fibrillation (AF) is the most common arrhythmia worldwide. Nowadays, AF ablation is a valuable treatment option. It has been shown that the left atrium (LA) diameter is a predictor of AF recurrence after cryoballoon ablation (CBA). Since it does not reflect the true LA size, we compared the role of different LA anatomical parameters using echocardiography for the prediction of AF recurrence after CBA. METHODS: We retrospectively included 209 patients (mean age 56.1 ± 13.6 years, male 62%) with paroxysmal AF undergoing CBA. A transthoracic echocardiography was performed in all patients. RESULTS: At a mean follow-up of 16.9 ± 6.3 months, AF recurred in 25.4% of the patients. LA anterior - posterior diameter (LAD), LA minimum volume (LAmin) and early AF recurrence were independent predictors of recurrence. Based on receiver operating characteristics, cut - off values for LAD and, LAmin were 41 mm, 23.69 mL, respectively. The negative predictive values for recurrence were 73% and 87.3% respectively. In patients with AF recurrence, a significant proportion (30.2%) showed LA longitudinal remodeling (LA superior - inferior diameter) even though classically measured LAD was normal. CONCLUSIONS: Longitudinal LA remodeling plays an additional role for predicting AF recurrence after CBA, in patients without LAD dilation. Moreover, LAmin had a high negative predictive value and was an independent predictor of AF recurrence. Therefore, a more complete LA anatomical assessment allows a better prediction of AF recurrences after CBA.

Are disease-specific patient-reported outcomes measures (PROMs) used in cardiogenetics? A systematic review.

Patient-reported outcome measures (PROMs) are used to facilitate patient-centered care (PCC). While studies in patients with cardiac conditions have revealed poorer health-related quality of life (HRQoL) and elevated emotional stress, studies in inherited cardiac conditions (ICC) seem rare. A systematic review evaluated which (specific domains of) PROMs are used in patients with ICC. From three databases (PubMed, PsychINFO, and Web of Science) quantitative studies investigating PROMs in patients with ICC were included. A Cochrane-based assessment tool was used to evaluate quality and potential risk of bias per subdomain. Data from 17 eligible articles were extracted. Among the included studies, risk of bias was predominantly high (35%) or unclear (30%). Most (n = 14) studies used a generic health status measure (SF-36, SF-12); 3 studies used a disease-specific PROM (KCCQ- cardiomyopathy and MLFHQ-heart failure). In addition to HRQoL measures, several studies used affective psychological measures (i.e., HADS, CAQ-18, IES-R, and IPQ). The mental health component of the PROMs showed lower scores overall in patients with ICC compared to population norms. Nine studies using HADS and GAD-7/PHQ-9 showed a prevalence of clinically significant anxiety (17-47%) and depression levels (8.3-28%) that were higher than the population norm (8.3% and 6.3%, respectively). HRQoL in patients with ICC is primarily assessed with generic PROMs. Results further confirmed high psychological morbidity in this population. Generic PROMS measures evaluate overall health status, but lack sensitivity to ICC-specific factors like heredity-related concerns. We propose developing a PROM specific for ICC to optimize PCC.

Feasibility, Reproducibility and Validation of Right Ventricular Volume and Function Assessment Using Three-Dimensional Echocardiography.

Three-dimensional echocardiography (3DE) is advised for right ventricular (RV) assessment. Data regarding the optimal acquisition settings and optimization are still scarce. We aimed to evaluate the feasibility, reproducibility and validation of 3DE for RV volume and function assessment, using cardiac magnetic resonance (CMR) as gold standard. Thirty healthy volunteers and 36 consecutive patients were prospectively included. CMR was performed in the latter. Standard apical four-chamber view (A4CV), focused A4CV and modified A4CV were used for 3DE RV acquisition. Feasibility (and the effect of changes in settings) was evaluated. Intra and interobserver analyses were performed by three observers (expert vs. novice). RV parameters by echocardiography were compared to CMR. Feasibility of acquisition was 16.7% for A4CV, 80.0% for focused A4CV and 16.7% for modified A4CV. Changes in settings had no significant influence on feasibility and further analysis. Intraobserver variability was good in both expert and novice, interobserver variability was good between experienced observers. Compared to CMR, 3DE volumes were significantly lower with fair to moderate correlation (EDV: 91.1 ± 24.4 mL vs. 144.3 ± 43.0 mL (p < 0.001), r = 0.653 and ESV: 48.1 ± 16.4 mL vs. 60.4 ± 21.2 mL (p < 0.001), r = 0.530, by multi-beat 3DE and CMR respectively). These findings suggest that standardization is needed in order to implement this technique in clinical practice, thus further studies are required.

Additional value of left atrium remodeling assessed by three-dimensional echocardiography for the prediction of atrial fibrillation recurrence after cryoballoon ablation.

Cryoballoon ablation (CBA) is a safe and efficient therapeutic option for atrial fibrillation (AF). However, AF recurrence occurs in 25% of the patients, leading to repeated ablations and complications. Previous reports have shown that left atrium (LA) assessed by M-Mode and two-dimensional echocardiography (2DE) predicts AF recurrence. Nevertheless, these methods imply geometrical assumptions of the LA remodeling, which is a three-dimensional process. We hypothesized that LA remodeling by three-dimensional echocardiography (3DE) has an additional value for AF recurrence prediction post-CBA. 172 consecutive patients (62.2 ± 12.2 years, 61% male) were prospectively recruited. Echocardiography was performed before CBA. Blanking period was defined as the first three months post-ablation. The primary endpoint was AF recurrence after the blanking period. 50 (29%) patients had AF recurrence. 3DE LA maximum volume index (LAVI) had the highest incremental predictive value for AF recurrence (HR 5.50, 95% CI 1.34 -22.45, p < 0.001). In patients with non-dilated LA diameter index and LAVI by 2DE, LAVI by 3DE was able to discriminate AF recurrence with a sensitivity of 90% and a specificity of 66%, for an optimal cut-off value of 30.4 ml/m2. LA remodeling by 3DE predicted AF recurrence, even in patients with non-dilated LA by M-Mode and 2DE, suggesting that 3DE might reflect better and earlier the asymmetric and variable nature of LA remodeling and it should be considered for systematic use to evaluate AF recurrence risk post-CBA.

Mitral annulus disjunction is associated with adverse outcome in Marfan and Loeys-Dietz syndromes.

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.

Troponin T in COVID-19 hospitalized patients: Kinetics matter.

BACKGROUND: Coronavirus disease 2019 (COVID-19) emerged as a worldwide health crisis, overwhelming healthcare systems. Elevated cardiac troponin T (cTn T) at admission was associated with increased in-hospital mortality. However, data addressing the role of cTn T in major adverse cardiovascular events (MACE) in COVID-19 are scarce. Therefore, we assessed the role of baseline cTn T and cTn T kinetics for MACE and in-hospital mortality prediction in COVID-19. METHODS: Three hundred and ten patients were included prospectively. One hundred and eight patients were excluded due to incomplete records. Patients were divided into three groups according to cTn T kinetics: ascending, descending, and constant. The cTn T slope was defined as the ratio of the cTn T change over time. The primary and secondary endpoints were MACE and in-hospital mortality. RESULTS: Two hundred and two patients were included in the analysis (mean age 64.4 ± 16.7 years, 119 [58.9%] males). Mean duration of hospitalization was 14.0 ± 12.3 days. Sixty (29.7%) patients had MACE, and 40 (19.8%) patients died. Baseline cTn T predicted both endpoints (p = 0.047, hazard ratio [HR] 1.805, 95% confidence interval [CI] 1.009-3.231; p = 0.009, HR 2.322, 95% CI 1.234-4.369). Increased cTn T slope predicted mortality (p = 0.041, HR 1.006, 95% CI 1.000-1.011). Constant cTn T was associated with lower MACE and mortality (p = 0.000, HR 3.080, 95% CI 1.914-4.954, p = 0.000, HR 2.851, 95% CI 1.828-4.447). CONCLUSIONS: The present study emphasizes the additional role of cTn T testing in COVID-19 patients for risk stratification and improved diagnostic pathway and management.

Incremental value of left atrial strain to predict atrial fibrillation recurrence after cryoballoon ablation.

OBJECTIVE: Atrial fibrillation (AF) recurrence occurs in approximately 25% of the patients undergoing cryoballoon ablation (CBA), leading to repeated ablations and complications. Left atrial (LA) dilation has been proposed as a predictor of AF recurrence. However, LA strain is a surrogate marker of LA mechanical dysfunction, which might appear before the enlargement of the LA. The purpose of this study was to evaluate the additional predictive value of LA function assessed using strain echocardiography for AF recurrence after CBA. METHODS: 172 consecutive patients (62.2 ± 12.2 years, 61% male) were prospectively analyzed. Echocardiography was performed before CBA. Blanking period was defined as the first three months post-ablation. The primary endpoint was AF recurrence after the blanking period. RESULTS: 50 (29%) patients had AF recurrence. In the overall study population, peak atrial longitudinal strain (PALS) ≤ 17% had the highest incremental predictive value for AF recurrence (HR = 9.45, 95%CI: 3.17-28.13, p < 0.001). In patients with non-dilated LA, PALS≤17% remained an independent predictor of AF recurrence (HR = 5.39, 95%CI: 1.66-17.52, p = 0.005). CONCLUSIONS: This study showed that LA function assessed by PALS provided an additional predictive value for AF recurrence after CBA, over LA enlargement. In patients with non-dilated LA, PALS also predicted AF recurrence. These findings emphasize the added value of LA strain, suggesting that it should be implemented in the systematic evaluation of AF patients before CBA.

Prognostic Value of Coronary Artery Calcium Score in Hospitalized COVID-19 Patients.

Background: The association of known cardiovascular risk factors with poor prognosis of coronavirus disease 2019 (COVID-19) has been recently emphasized. Coronary artery calcium (CAC) score is considered a risk modifier in the primary prevention of cardiovascular disease. We hypothesized that the absence of CAC might have an additional predictive value for an improved cardiovascular outcome of hospitalized COVID-19 patients. Materials and methods: We prospectively included 310 consecutive hospitalized patients with COVID-19. Thirty patients with history of coronary artery disease were excluded. Chest computed tomography (CT) was performed in all patients. Demographics, medical history, clinical characteristics, laboratory findings, imaging data, in-hospital treatment, and outcomes were retrospectively analyzed. A composite endpoint of major adverse cardiovascular events (MACE) was defined. Results: Two hundred eighty patients (63.2 ± 16.7 years old, 57.5% male) were included in the analysis. 46.7% patients had a CAC score of 0. MACE rate was 21.8% (61 patients). The absence of CAC was inversely associated with MACE (OR 0.209, 95% CI 0.052-0.833, p = 0.027), with a negative predictive value of 84.5%. Conclusion: The absence of CAC had a high negative predictive value for MACE in patients hospitalized with COVID-19, even in the presence of cardiac risk factors. A semi-qualitative assessment of CAC is a simple, reproducible, and non-invasive measure that may be useful to identify COVID-19 patients at a low risk for developing cardiovascular complications.

Feasibility and Reproducibility of Left Atrium Measurements Using Different Three-Dimensional Echocardiographic Modalities.

Left atrium (LA) volume is a biomarker of cardiovascular outcomes. Three-dimensional echocardiography (3DE) provides an accurate LA evaluation, but data regarding the optimal 3DE method is scarce. We assessed the feasibility and reproducibility of LA measurements using different 3DE methods. One hundred and ninety-four patients were prospectively analyzed. Conventional 3DE and two semi-automatic 3DE algorithms (Tomtec™ and Dynamic Heart Model (DHM)) were used in 110 patients. Intra- and interobserver reproducibility and intervendor comparison were performed in additional patients' subsets. Forty patients underwent cardiac magnetic resonance (CMR). Feasibility was 100% for Tomtec, 98.2% for DHM, and 72.8% for conventional 3DE. Tomtec volumes were higher than 3DE and DHM (p < 0.001). Reproducibility was better for DHM (intraobserver LA maximum volume (LAmax) ICC 0.99 (95% CI 1.0-0.99), LA minimum volume (LAmin) 0.98 (95% CI 0.95-0.99), LApreA 0.96 (95% CI 0.91-0.98); interobserver LAmax ICC 0.98 (95% CI 0.96-0.99), LAmin 0.99 (95% CI 0.99-1.00), and LApreA 0.97 (95% CI 0.94-0.99)). Intervendor comparison showed differences between left ventricle (LV) software adapted for LA (p < 0.001). Tomtec underestimated the least LA volumes compared to CMR. These findings emphasize that dedicated software should be used for LA assessment, for consistent clinical longitudinal follow-up and research.

Worse Prognosis in Brugada Syndrome Patients With Arrhythmogenic Cardiomyopathy Features.

OBJECTIVES: This study aimed to assess the presence of echocardiographic and electrocardiographic similarities in patients with Brugada syndrome (BrS) and arrhythmogenic cardiomyopathy (AC) and the prevalence and prognostic value of AC structural/electrical features in patients with BrS. BACKGROUND: BrS and AC are genetic cardiac diseases with high risk for sudden cardiac death. Although BrS and AC display different features, previous reports suggest a phenotypic overlap. METHODS: We acquired clinical data, electrocardiogram, and transthoracic echocardiography in patients with BrS and AC. We assessed the presence of AC diagnostic criteria according to the 2010 AC task force criteria for right ventricular outflow tract (RVOT), fractional area change, depolarization, and repolarization in the patients with BrS. We compared arrhythmic outcome in BrS patients with and without AC structural/electrical criteria. RESULTS: A total of 116 BrS and 141 AC patients were included. AC electrical features were present in 28 (24%) BrS patients and structural features in 97 (84%) BrS patients. BrS patients with an RVOT or depolarization AC criterion showed a trend towards worse severe arrhythmia-free survival compared to BrS patients without (p = 0.05). The criterion for RVOT dilation showed high sensitivity and improved detection of arrhythmic BrS patients when added to type 1 electrocardiogram pattern and syncope (area under the curve 0.73 [95% confidence interval: 0.59 to 0.87] vs. area under the curve 0.79 [95% confidence interval: 0.69 to 0.90]); p = 0.009). CONCLUSIONS: In this large cohort comparison, Brugada syndrome (BrS) and arrhythmogenic cardiomyopathy patients had phenotypic overlap. The presence of arrhythmogenic cardiomyopathy diagnostic criteria in BrS patients was associated with a trend towards higher arrhythmic risk. The right ventricular outflow tract dilation criterion improved detection of arrhythmic BrS patients.

Contraction alterations in Brugada syndrome; association with life-threatening ventricular arrhythmias.

BACKGROUND: Brugada syndrome (BrS) is characterized by a high risk of sudden cardiac death. The clinical value of deformation imaging in patients with BrS is unknown. We aimed to assess whether echocardiographic speckle tracking parameters differ between: 1) BrS patients and healthy controls, 2) BrS patients with and without life-threatening ventricular arrhythmias. METHODS: Left ventricle (LV) and right ventricle (RV) longitudinal strain and mechanical dispersion (MD) were derived from echocardiography at inclusion. Clinical and ECG data were retrospectively assessed. A life-threatening ventricular arrhythmia was defined as an aborted cardiac arrest or sustained ventricular tachyarrhythmia. RESULTS: We included 175 BrS patients and 82 controls. LV and RV longitudinal strain were lower (-18.1 ±â€¯2.6% vs. -18.8 ±â€¯2.0%, p = 0.01 and - 24.4 ±â€¯5.4% vs. 25.6 ±â€¯3.7%, p = 0.04), while MD was higher [38 ±â€¯11 ms vs. 33 ±â€¯8 ms, p = 0.001 and 15 (8-25) ms vs. 11 (6-19) ms, p = 0.03] in BrS patients compared to controls. BrS patients who experienced a life-threatening ventricular arrhythmia (n = 19) had higher LV MD compared to those without events (43 ±â€¯11 ms vs. 37 ±â€¯11 ms, p = 0.02). An LV MD ≥40 ms was optimally associated with life-threatening ventricular arrhythmias [odds ratio 4.62 (95%CI 1.58-13.50), p = 0.005]. CONCLUSIONS: BrS patients had lower longitudinal strain and more heterogeneous contractions than healthy controls. Furthermore, BrS patients with a history of life-threatening ventricular arrhythmia had more heterogeneous LV contractions than those without. Therefore, LV MD may be a risk marker in BrS and its evaluation in prospective studies is needed.

Speckle tracking echocardiography data in Brugada syndrome patients.

Brugada syndrome is characterized by typical electrocardiogram changes and a high risk for sudden cardiac death (Priori et al., 2013). In addition to the well known electrical substrate, morphological and functional alterations appeared to be present in a subset of the Brugada syndrome patients (Catalano et al., 2009). Echocardiographic speckle tracking enables us to detect subtle contraction alterations (Smiseth et al.,2016). We performed transthoracic echocardiography with speckle tracking analysis in 82 healthy controls and 175 Brugada syndrome patients. Main findings are presented and discussed in the article "Contraction alterations in Brugada syndrome; association with life-threatening ventricular arrhythmias" (Scheirlynck et al., 2019). This related Data article contains segmental longitudinal strain values for RV and LV, and the comparison of echocardiographic parameters between Brugada syndrome patients with spontaneous and drug-induced type 1 pattern and between patients with and without ventricular arrhythmia inducibility during electrophysiological study.

Increased levels of sST2 in patients with mitral annulus disjunction and ventricular arrhythmias.

Objective: Displacement of the mitral valve, mitral annulus disjunction (MAD), is described as a possible aetiology of sudden cardiac death. Stress-induced fibrosis in the mitral valve apparatus has been suggested as the underlying mechanism. We aimed to explore the association between stretch-related and fibrosis-related biomarkers and ventricular arrhythmias in MAD. We hypothesised that soluble suppression of tumourigenicity-2 (sST2) and transforming growth factor-ß1 (TGFß1) are markers of ventricular arrhythmias in patients with MAD. Methods: We included patients with ≥1 mm MAD on cardiac MRI. We assessed left ventricular ejection fraction (LVEF) and fibrosis by late gadolinium enhancement (LGE). The occurrence of ventricular arrhythmia, defined as aborted cardiac arrest, sustained or non-sustained ventricular tachycardia, was retrospectively assessed. We assessed circulating sST2 and TGFß1 levels. Results: We included 72 patients with MAD, of which 22 (31%) had ventricular arrhythmias. Patients with ventricular arrhythmias had lower LVEF (60 % (±6) vs 63% (±6), p = 0.04), more frequently papillary muscle fibrosis (14 (64%) vs 10 (20%), p < 0.001) and higher sST2 levels (31.6 ± 10.1 ng/mL vs 25.3 ± 9.2 ng/mL, p = 0.01) compared with those without, while TGFß1 levels did not differ (p = 0.29). Combining sST2 level, LVEF and papillary muscle fibrosis optimally detected individuals with arrhythmia (area under the curve 0.82, 95% CI 0.73 to 0.92) and improved the risk model (p < 0.05) compared with single parameters. Conclusion: Circulating sST2 levels were higher in patients with MAD and ventricular arrhythmias compared with arrhythmia-free patients. Combining sST2, LVEF and LGE assessment improved risk stratification in patients with MAD.

The Mitral Annulus Disjunction Arrhythmic Syndrome.

BACKGROUND: Mitral annulus disjunction (MAD) is an abnormal atrial displacement of the mitral valve leaflet hinge point. MAD has been associated with mitral valve prolapse (MVP) and sudden cardiac death. OBJECTIVES: The purpose of this study was to describe the clinical presentation, MAD morphology, association with MVP, and ventricular arrhythmias in patients with MAD. METHODS: The authors clinically examined patients with MAD. By echocardiography, the authors assessed the presence of MVP and measured MAD distance in parasternal long axis. Using cardiac magnetic resonance (CMR), the authors assessed circumferential MAD in the annular plane, longitudinal MAD distance, and myocardial fibrosis. Aborted cardiac arrest and sustained ventricular tachycardia were defined as severe arrhythmic events. RESULTS: The authors included 116 patients with MAD (age 49 ± 15 years; 60% female). Palpitations were the most common symptom (71%). Severe arrhythmic events occurred in 14 (12%) patients. Longitudinal MAD distance measured by CMR was 3.0 mm (interquartile range [IQR]: 0 to 7.0 mm) and circumferential MAD was 150° (IQR: 90° to 210°). Patients with severe arrhythmic events were younger (age 37 ± 13 years vs. 51 ± 14 years; p = 0.001), had lower ejection fraction (51 ± 5% vs. 57 ± 7%; p = 0.002) and had more frequently papillary muscle fibrosis (4 [36%] vs. 6 [9%]; p = 0.03). MVP was evident in 90 (78%) patients and was not associated with ventricular arrhythmia. CONCLUSIONS: Ventricular arrhythmias were frequent in patients with MAD. A total of 26 (22%) patients with MAD did not have MVP, and MVP was not associated with arrhythmic events, indicating MAD itself as an arrhythmogenic entity. MAD was detected around a large part of the mitral annulus circumference and was interspersed with normal tissue.