Background Diagnostic criteria and risk stratification in patients with arrhythmic mitral valve prolapse (AMVP) are insufficient. The current AMVP diagnosis defined by EHRA includes burden of premature ventricular complexes (PVCs) but not the origin of PVCs. Objective We aimed to assess left ventricular PVCs (LV-PVCs) as a risk factor for subsequent ventricular arrhythmia (VA) assessed by implantable cardiac monitor (ICM). We retrospectively applied the 2022 EHRA AMVP definition on our pre-2022 patient cohort. Methods We prospectively implanted ICM in patients with MVP and/or mitral annular disjunction during 2015-2020 and followed them for 3 years. We assessed occurrence of severe VA (ventricular fibrillation, sustained ventricular tachycardia [VT], or non-sustained VT [NSVT] causing syncope) and VA (severe VA and NSVT). PVCs origins were classified on 12-lead ECG. Results We included 60 patients (mean age 49±15years) with ICM. LV-PVCs were present in 30 (50%) patients. Hundred VAs were detected, 96 NSVTs and 4 severe VA. LV-PVCs were associated with VA incidence rate ratio of 10.77 (95%CI 3.23-36.06, p<0.001), adjusted for baseline NSVTs and PVC burden. Severe VA occurred only in patients with LV-PVCs (log-rank p=0.04). The 2022 EHRA AMVP definition detected patients with subsequent VA with AUC 0.63, however, missed one (2%) patient with severe VA, and missed 12 (25%) patients with subsequent NSVT due to PVCs <5% at baseline. Conclusion Presence of LV-PVCs was strongly associated with subsequent VA. The threshold of ≥5% PVCs for AMVP diagnosis appeared too high to detect MVP patients at risk of subsequent VA in our cohort.

Background:Patients with mitral valve prolapse (MVP) are at risk of ventricular arrhythmias (VAs), ranging from premature ventricular complexes (PVCs) to life-threatening VAs. The association between PVC burden and heart rate in patients with MVP is not known. We aimed to identify the association between PVC burden and heart rate in patients with MVP. Methods:In this this cross-sectional ambispective case control study we included MVP patients with available Holter monitorings. We defined PVC profiles as fast-heart-rate-dependent-PVC (F-HR-PVC) in case of positive correlation with heart rate, slow-heart-rate-dependent-PVC (S-HR-PVC) in case of negative correlation, and independent-heart-rate-PVC (I-HR-PVC) when no correlation was found. For comparison, we included a control group of age- and sex-matched patients with idiopathic PVCs. Results:We included 70 patients with MVP (48 years [interquartile range 35-58], 79% female) and 70 age- and sex-matched patients with idiopathic PVCs. A total of 153 Holter monitorings from patients with MVP were analysed and compared to 70 Holter monitorings from patients with idiopathic PVCs. In the MVP group, we found F-HR-PVC in 44 (63%) patients, I-HR-PVC in 24 (34%) and S-HR-PVC in 2 (3%). MVP patients had more frequently F-HR-PVC and less frequently S-HR-PVC than the control group (p < 0.05 for both). MVP patients with F-HR-PVC had higher rate of NSVTs (incidence rate ratio 2.9 [95% confidence interval 1.1-7.8], p = 0.03) compared to I-HR-PVC. Conclusion:Fast-heart-rate-dependent-PVC was the most common PVC profile in MVP patients, and slow-heart-rate-dependent-PVC was rare. These findings suggest a catecholamine-sensitive mechanism acting as trigger for ventricular arrhythmias in MVP patients.

Background The diagnostic role of signal‐averaged ECG (SAECG) in arrhythmogenic right ventricular cardiomyopathy (ARVC) has lately been questioned. We assessed the value of SAECG‐derived late ventricular potentials (LP) in ARVC diagnosis and its association with disease manifestations. Methods and Results Patients with definite ARVC diagnosis or genotype‐positive family members who underwent SAECG were included in register‐based observational study (n=357, mean age 41 years, 47% female, 43% probands). LP and terminal activation duration (TAD) were defined by Task Force Criteria 2010. We assessed the association of TAD and LP with structural RV abnormalities and ventricular tachycardia (VT), defined as sustained VT, appropriate implantable cardioverter‐defibrillator shock, aborted cardiac arrest, or sudden cardiac death, at diagnosis. LP were documented in 210 patients (59%) and abnormal TAD in 66 patients (18%). Each of the SAECG parameters was significantly associated with definite ARVC diagnosis in receiver‐operator characteristics curve analysis with area under the curve between 0.67 and 0.74. Exclusion of SAECG from diagnostic workup led to reclassification of 37 patients (16%) from definite to borderline ARVC (13 probands, 9 of whom had prevalent VT). Ninety patients (25%) had history of VT. LP, but not TAD, were associated with VT (adjusted odds ratio [ORadj], 2.42 [95%CI, 1.07–5.48]). LP had lower specificity (72% versus 97%) but higher sensitivity (71% versus 25%) for association with RV structural abnormalities than TAD. Conclusions In the Nordic ARVC cohort SAECG‐derived LP are associated with VT and structural RV abnormalities and were critical for ascertainment of ARVC diagnosis in 16% of patients with narrow QRS complexes, including 8% of all probands.

Keratoconus is a progressive eye disease that results in thinning of the cornea, leading to visual impairment. Mitral valve prolapse (MVP) is a common disorder affecting around 2–4% of the general population. Previous studies have found an overrepresentation of MVP in individuals with keratoconus, with a prevalence of 38–65%, suggesting a shared underlying mechanism. In this case-control study, patients with keratoconus were enrolled from a quality and research registry. They were examined by a 2D echocardiography to identify if they had MVP, billowing or normal mitral leaflets. Controls were matched from the population-based Trøndelag Health Study. Patients and controls underwent a detailed echocardiographic examination to detect abnormal mitral valves. We included 101 patients (age 33 [IQR 29–40], 75% male) with keratoconus and 101 matched individuals. MVP was found in 2 (2%), while billowing was found in 5 (5%) of keratoconus patients. No significant association was found between keratoconus and the prevalence of MVP or billowing compared to the control group. Moreover, no associations were found between severity of keratoconus with presence of MVP nor with billowing of the mitral valves. We could not confirm the previously reported association between keratoconus and MVP, suggesting that routine screening for MVP in keratoconus patients may not be warranted. However, we cannot rule out the possibility of an association in other gender, age and ethnic groups different than ours.

Background Arrhythmic mitral valve prolapse (AMVP) is a cause of sustained ventricular tachyarrhythmias (VAs) and sudden cardiac death (SCD), but the arrhythmias remain only partially understood. Objectives In this worldwide collaboration, this study aimed to characterize the VAs occurring in AMVP patients, explore factors associated with various types of sustained VA, and describe common triggering mechanisms. Methods In this multicenter retrospective cohort study, we collected patients with AMVP and documented VA. Clinical and imaging data, and detailed data of the arrhythmic events were collected. In addition, electrocardiograms or intracardiac tracings capturing the arrhythmic events were analyzed by a core laboratory. Results We included 225 patients from 35 centers (age 44 ± 17 years, 57% female). Late gadolinium enhancement (LGE) was found in 61%. We collected 278 arrhythmic events, of which ventricular fibrillation (VF) was the most frequent (65%), followed by sustained monomorphic ventricular tachycardia (SMVT; 26%). Triggers were most commonly exercise or stress (37%), but 31% had no discernable trigger. SMVT was associated with increasing age (P = 0.03), family history of SCD (P = 0.03), history of syncope (P = 0.05), and myocardial LGE (P = 0.003). Of the 278 events, 140 (50%) had available tracings, where 25% of events were triggered by a short-coupled premature ventricular contraction. Pause-dependent initiation was most frequent (49%), and more likely to lead to VF than to SMVT (P = 0.01). Conclusions The dominant VA in AMVP was VF, although SMVT was also common and associated with older age, family history of SCD, syncope, and myocardial LGE. The most common initiation was pause dependent leading to VF.

BACKGROUND:Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inheritable heart disease, whereas exercise-induced arrhythmogenic cardiomyopathy (EiAC) is a proposed acquired similar phenotype in athletes. The differences in disease progression between these entities are not well understood. OBJECTIVES:This study aims to assess structural, functional, and arrhythmic disease progression in EiAC compared with ARVC. METHODS:This longitudinal cohort study included EiAC patients who were competitive endurance athletes (>24 MET-hours/week for >6 consecutive years) referred due to ventricular arrhythmias (VA), without inherited or genetic factors or other evident causes, and genotype-positive ARVC patients with a definite diagnosis and their genotype-positive family members for comparison. Disease progression was assessed by repeated echocardiographic examinations and incident VA during long-term follow-up. RESULTS:The authors included 125 ARVC patients (61 women, aged 38 ± 17 years) and 41 EiAC patients (6 women, aged 45 ± 13 years) and followed them for 96 months (Q1-Q3: 73-132 months) and 82 months (Q1-Q3: 50-118 months), respectively. The authors analyzed 730 echocardiographic examinations (538 ARVC, 192 EiAC). Right ventricular (RV) structure and function remained stable in EiAC patients, whereas those in ARVC patients deteriorated during follow-up. The 5-year and 10-year cumulative incidences of VA were similar between EiAC and ARVC patients. CONCLUSIONS:RV structure and function deteriorated in ARVC patients but remained stable in EiAC patients during follow-up. The incidence of VA was high in both populations. These results indicate that EiAC patients should be followed closely over time regardless of structural and functional progression.

BACKGROUND: Patients with arrhythmogenic mitral valve prolapse syndrome are at increased risk for life-threatening ventricular arrhythmias, but studies have been limited by small sample sizes. We sought to assemble an international arrhythmogenic mitral valve prolapse syndrome registry to delineate the clinical, imaging, and treatment characteristics of patients with arrhythmogenic mitral valve prolapse syndrome who survived sudden cardiac arrest (SCA) or had sustained ventricular tachycardia (VT) or ventricular fibrillation. METHODS: In this descriptive registry, we characterized patients with arrhythmogenic mitral valve prolapse syndrome who survived SCA, sustained VT, or ventricular fibrillation. Deidentified data were abstracted locally and combined centrally. RESULTS: We included 148 patients who had SCA or VT/ventricular fibrillation. Patients had a mean age of 43.7±15.4 years; 68% were women, 73% had bileaflet prolapse, 65% had mitral annular disjunction, 67% had nonsustained VT, and 59% had inferolateral T-wave inversions. Syncope (n=54, 48%) and anterolateral T-wave inversion (n=26, 22%) were relatively common. Catheter ablation was performed in 50 (35%) patients for premature ventricular complexes and in 18 (17.7%) patients for VT. Sites of origin for arrhythmias were commonly in the papillary muscles, fascicles, mitral annulus, and inferior/inferolateral left ventricle. CONCLUSIONS: In this international descriptive registry of patients with arrhythmogenic mitral valve prolapse syndrome and SCA, patients were young, women, and had bileaflet mitral valve prolapse, mitral annular disjunction, inferolateral T-wave inversions, and nonsustained VT. A history of syncope and anterolateral T-wave inversions was relatively common in patients who survived SCA or sustained VT/ventricular fibrillation.

Background:Several reports exist of an acquired exercise-induced arrhythmogenic cardiomyopathy. Little is known about myocardial disease progression and arrhythmia prediction in this population. Objective:The study sought to explore the evolution of myocardial function and structure and its relation to incident life-threatening ventricular arrhythmias (VA), to identify markers of impending events. Methods:We included athletes (individuals with exercise doses >24 metabolic equivalent of task hours per week, >6 consecutive years, participating in organized and competitive sports) who had VA, absence of family history and known genetic variants associated with cardiac disease, and no other identified etiology, in a tertiary referral single-center, longitudinal cohort study of patients with exercise-induced arrhythmogenic cardiomyopathy (EiAC). Evolution of myocardial function and structure was assessed by repeated echocardiographic examinations during long-term follow-up. Life-threatening VA were assessed at baseline and during long-term follow-up. Results:Forty-one EiAC patients (15% women, age 45 ± 13 years) were followed for 80 (interquartile range 48-115) months. There were no changes in myocardial function or structure in the overall population during follow-up. We observed high incidence rate and high recurrence rate of life-threatening VA in EiAC patients. Subtle deterioration of right ventricular function was strongly associated with subsequent first-time VA (odds ratio 1.12, 95% confidence interval 1.01-1.25, P = .031, per 1% deterioration of right ventricular free wall longitudinal strain). Conclusion:There were no clear changes in myocardial function or structure during follow-up in the overall population, but there was a high incidence rate and high recurrence rate of life-threatening VA. Subtle right ventricular deterioration by free wall longitudinal strain was a strong predictor of impending first-time life-threatening VA during follow-up.

BackgroundThe electrophysiological mechanism connecting mitral valve prolapse (MVP), premature ventricular complexes and life-threatening ventricular arrhythmia is unknown. A common hypothesis is that stretch activated channels (SACs) play a significant role. SACs can trigger depolarizations or shorten repolarization times in response to myocardial stretch. Through these mechanisms, pathological traction of the papillary muscle (PM), as has been observed in patients with MVP, may induce irregular electrical activity and result in reentrant arrhythmia.MethodsBased on a patient with MVP and mitral annulus disjunction, we modeled the effect of excessive PM traction in a detailed medical image-derived ventricular model by activating SACs in the PM insertion region. By systematically varying the onset of SAC activation following sinus pacing, we identified vulnerability windows for reentry with 1 ms resolution. We explored how reentry was affected by the SAC reversal potential (ESAC) and the size of the region with simulated stretch (SAC region). Finally, the effect of global or focal fibrosis, modeled as reduction in tissue conductivity or mesh splitting (fibrotic microstructure), was investigated.ResultsIn models with healthy tissue or fibrosis modeled solely as CV slowing, we observed two vulnerable periods of reentry: For ESAC of −10 and −30 mV, SAC activated during the T-wave could cause depolarization of the SAC region which lead to reentry. For ESAC of −40 and −70 mV, SAC activated during the QRS complex could result in early repolarization of the SAC region and subsequent reentry. In models with fibrotic microstructure in the SAC region, we observed micro-reentries and a larger variability in which times of SAC activation triggered reentry. In these models, 86% of reentries were triggered during the QRS complex or T-wave. We only observed reentry for sufficiently large SAC regions (>= 8 mm radius in models with healthy tissue).ConclusionStretch of the PM insertion region following sinus activation may initiate ventricular reentry in patients with MVP, with or without fibrosis. Depending on the SAC reversal potential and timing of stretch, reentry may be triggered by ectopy due to SAC-induced depolarizations or by early repolarization within the SAC region.

Abstract Background Insertable cardiac monitors (ICM) have the capability to continuously monitor premature ventricular contraction (PVC) burden over time [1] as well as detect ventricular tachycardia and fibrillation (VT/VF). Objective We investigated the association of PVC burden with incidence of VT/VF in a large real-world cohort of patients implanted with ICMs. Methods Patients implanted with an ICM for various reasons for monitoring and PVC detection turned on were included from the ICM manufacturer's de-identified data warehouse. Patients were included if they had at least 90 days of PVC burden follow-up. Tachycardia episodes that were detected by the ICM were first classified as VT/VF, SVT, or oversensing using an artificial intelligence (AI) model that was pre-trained using over 60,000 manually adjudicated ICM detected tachycardia episodes. If the AI model output probability for VT/VF was greater than 0.2, then those episodes were manually adjudicated for incidence of non-induced spontaneous VT/VF. The PVC burden trends recorded by the device were divided into 4 mutually exclusive patient groups: (1) 0% PVC burden on all days, (2) 1-4% PVC burden on ≥1 day, (3) 5-9% PVC burden on ≥1 day, and (4) >10% PVC on ≥1 day. The incidence rate of spontaneous VT/VF was compared between the PVC burden patient groups using a Generalized Estimating Equations model with negative binomial distribution. Time to first spontaneous VT/VF occurrence after the first day of occurrence of PVC burden for the respective PVC burden groups were estimated using Kaplan-Meier analysis and the groups were compared using the Cox proportional hazards model. Results A total of 5,521 patients were included in the analysis. Patients had an average age of 69±15 years and 49.8% being males. There was a total of 33,393 tachycardia episodes from 2,641 patients that were detected by the ICM. After AI model probability-based adjudications, 691 spontaneous VT/VF episodes were identified from 277 patients. Patients with ≥1 day of PVC burden of 1-4%, 5-9%, and ≥10% were associated with 2.9, 7.3, and 7.8 times increased incidence rate of VT/VF episodes during follow-up period relative to patients with 0% PVC burden on all days of follow-up (Figure A). Kaplan Meier curves for incidence of first spontaneous VT/VF episode for the four groups following occurrence of the first day of qualifying PVC burden for the respective groups are shown in figure B. Patients with a day of PVC burden ≥10% were 3.5 times more likely to develop VT/VF in the future compared to patients with 0% PVC burden on all days. Conclusion Days with high PVC burden, as detected by an ICM with continuous PVC detection capability, were associated with increased risk of VT/VF events in a group of real-world patients implanted with ICMs. PVC burden measured by ICMs may be a risk stratification tool for further investigation to prevent sudden cardiac arrest.

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. Structured Graphical Abstract In this international multi-centre retrospective case series, we contacted 27 centres from four different continents for possible collaboration, and we included 18 women with AMVP from 11 centres. Malignant ventricular arrhythmia occurred 37 times, of which 18 (48%) occurred during pregnancy and 6 months postpartum. This period showed higher incidence rate of malignant VA, suggesting increased arrhythmic risk during the perinatal period. The findings from this study provide guidance for shared decision-making in high-risk AMVP women wanting to become pregnant. AMVP, arrhythmic mitral valve prolapse; CI, confidence interval; ICD, implantable cardioverter defibrillator; VA, ventricular arrhythmia; VT, ventricular tachycardia. The image graphics were generated using the Midjourney AI application, Midjourney.com.

Abstract Background A subset of patients with mitral valve prolapse (MVP) are at risk of life-threatening ventricular arrhythmias (VAs), and a high burden of premature ventricular contractions (PVCs) is associated with increased mortality in patients with MVP. Previous studies indicate that PVC burden could either increase with increasing heart rate (HR), decrease with increasing HR, or with no relationship to HR. The association between PVC burden and HR in MVP is not known, nor if there is an association with VAs. Purpose We aimed to explore the relationship between PVC burden and HR in patients with MVP. Furthermore, we aimed to explore whether the different PVC profiles were associated with severe VA. Methods In this cross-sectional ambispective outcome study, we included patients with MVP and available Holter monitorings with >100 PVCs per 24-hours seen at our tertiary centre. The Holter monitoring with the highest PVC burden was used in case of multiple recordings. We investigated the relationship between hourly PVC burden and hourly mean HR by univariable mixed linear regression. We defined PVC profiles as (1) fast-HR-dependent-PVC (F-HR-PVC) when there was a significant positive correlation, (2) slow-HR-dependent-PVC (S-HR-PVC) when there was a significant negative correlation, and (3) independent-HR-PVC (I-HR-PVC) when no correlation between PVC and HR was found. The significance was set to 0.05. Severe VA was defined as sustained ventricular tachycardia, non-sustained ventricular tachycardia with haemodynamic instability, ventricular fibrillation, aborted cardiac arrest, or appropriate shock by a primary preventive implantable cardioverterefibrillator. Severe VA was recorded from retrospective medical history, prospective ICD interrogation or monitoring with implantable loop recorder. Results We included 75 patients with >100 PVCs at Holter monitoring (median age 48 years [IQR 35-58], 58% female). The median PVC burden was 1.9% per 24-hours (IQR 0.4-7.2). We found F-HR-PVC in 49 patients (65%), S-HR-PVC in 1 (1%), and I-HR-PVC in 25 (33%). Twelve (16%) patients had severe VA and these had higher PVC burden compared to those without severe VA (7.3% per 24-hours [IQR 4.2-10.4] vs 1.1% per 24-hours [IQR 0.3-5.3], p=0.004). The median PVC burden was 1.5% (IQR 0.3-8.0) for F-HR-PVC, 5.7% for S-HR-PVC and 1.5% (IQR 0.3-8.0) for I-HR-PVC. We found no association between different PVC profiles and the presence of severe VA (F-HR-PVC 65% vs 67%, S-HR-PVC 2% vs 0%, I-HR-PVC 33% vs 33%, p>0.05 for all groups). Conclusion Fast-HR-dependent-PVC was most common in MVP patients, and slow-HR-dependent-PVC were rare. A higher PVC burden was associated with severe VA. However, different PVC profiles did not infer higher risk of severe VA in our study. Further studies are needed to explore whether distinct PVC profiles can predict severe VA in larger patient populations.

ABSTRACTBackgroundPatients with the arrhythmogenic mitral valve prolapse syndrome (AMVPS) are at increased risk for life-threatening ventricular arrhythmias (VAs), but studies have been limited by small sample sizes. We sought to assemble an international AMVPS registry to delineate clinical, imaging, treatment characteristics, and risk factors for sudden cardiac arrest (SCA).MethodsWe retrospectively identified two groups of subjects with AMVPS: 1) the MVP-SCA group with SCA, sustained ventricular tachycardia (VT), and ventricular fibrillation (VF); and 2) the MVP-PVC group with significant premature ventricular complexes (PVCs) only. Deidentified data was abstracted locally and combined centrally.ResultsWe included 217 subjects with AMVPS: 148 (68%) had SCA or VT/VF (MVP-SCA group) and 69 (32%) had PVCs only (MVP-PVC group). Phenotypically, both groups were similar [mean age 44.2±16.7 years, 66% female, 76% with bileaflet prolapse, 55% with mitral annular disjunction (MAD)]. Syncope was more common in the MVP-SCA group than the MVP-PVC group (47% vs 22%, p=0.001) as were anterolateral T-wave inversions (TWIs, 22% vs 7%, p=0.011). Prior mitral valve surgery was less common in the MVP-SCA group (6% vs 20%, p=0.002). These differences remained significant after multivariable adjustment. An electrophysiology (EP) study was negative in 15/45 (33%) of the MVP-SCA subjects.ConclusionsIn this international registry, AMVPS subjects were young, female, and had bileaflet prolapse with MAD. A history of syncope and anterolateral TWIs were associated with SCA. Prior mitral valve surgery was less common in SCA subjects. A negative EP study had limited negative predictive value in high-risk patients.

Aims Arrhythmic mitral valve syndrome is linked to life-threatening ventricular arrhythmias. The incidence, morphology and methods for risk stratification are not well known. This prospective study aimed to describe the incidence and the morphology of ventricular arrhythmia and propose risk stratification in patients with arrhythmic mitral valve syndrome. Methods Arrhythmic mitral valve syndrome patients were monitored for ventricular tachyarrhythmias by implantable loop recorders (ILR) and secondary preventive implantable cardioverter-defibrillators (ICD). Severe ventricular arrhythmias included ventricular fibrillation, appropriate or aborted ICD therapy, sustained ventricular tachycardia and non-sustained ventricular tachycardia with symptoms of hemodynamic instability. Results During 3.1 years of follow-up, severe ventricular arrhythmia was recorded in seven (12%) of 60 patients implanted with ILR [first event incidence rate 4% per person-year, 95% confidence interval (CI) 2-9] and in four (20%) of 20 patients with ICD (re-event incidence rate 8% per person-year, 95% CI 3-21). In the ILR group, severe ventricular arrhythmia was associated with frequent premature ventricular complexes, more non-sustained ventricular tachycardias, greater left ventricular diameter and greater posterolateral mitral annular disjunction distance (all P < 0.02). Conclusions The yearly incidence of ventricular arrhythmia was high in arrhythmic mitral valve syndrome patients without previous severe arrhythmias using continuous heart rhythm monitoring. The incidence was even higher in patients with secondary preventive ICD. Frequent premature ventricular complexes, non-sustained ventricular tachycardias, greater left ventricular diameter and greater posterolateral mitral annular disjunction distance were predictors of first severe arrhythmic event.