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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.

PURPOSE: Left 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. METHODS: 66 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. RESULTS: LGE 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). CONCLUSION: The 5-SD gray-scale threshold technique in percentage revealed the best correlation with the visual assessment and an optimal reproducibility in MVP patient.

Background: The 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. Methods: Based on a patient with MVP and mitral annulus disjunction, we modeled the effect of excessive PM traction in a detailed medical imagederived 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. Results: In 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). Conclusion: Stretch 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 FROM AUTHOR]

Genetic factors play a significant role in the pathogenesis of mitral valve diseases, including mitral valve prolapse (MVP) and mitral valve regurgitation. Genes like Fibrillin-1 (FBN1), Filamin A (FLNA), matrix metalloproteinase 2 (MMP2), and SRY-box transcription factor 9 (SOX9) are known to influence mitral valve pathology but knowledge of the exact mechanism is far from clear. Data regarding serum parameters, transesophageal echocardiography, and genetic and histopathologic parameters were investigated in 54 patients who underwent cardiovascular surgery for mitral valve regurgitation. The possible association between Fibrillin-1, Filamin A, MMP2, and SOX9 gene expressions was checked in relationship with the parameters of systemic inflammatory response. The mRNA expression levels (RQ—relative quantification) were categorized into three distinct groups: low (RQ < 1), medium/normal (RQ = 1–2), and high (RQ > 2). Severe fibrosis of the mitral valve was reflected by high expression of FBN1 and low expression of MMP2 (p < 0.05). The myxoid degeneration level was associated with the mRNA expression level for FBN1 and a low lymphocyte-monocyte ratio was associated with an increased mRNA expression of FBN1 (p < 0.05). A high number of monocytes was associated with high values of FBN1 whereas the increase in the number of lymphocytes was associated with high levels of MMP2. In addition, we observed that the risk of severe hyalinization was enhanced by a low mRNA expression of FLNA and/or SOX9. In conclusion, a lower FLNA mRNA expression can reflect the aging process that is highlighted in mitral valve pathology as a higher risk for hyalinization, especially in males, that might be prevented by upregulation of the SOX9 gene. FBN1 and MMP2 influence the inflammation-related fibrotic degeneration of the mitral valve. Understanding the genetic base of mitral valve pathology can provide insights into disease mechanisms, risk stratification, and potential therapeutic targets. [ABSTRACT FROM AUTHOR]

Aims This study aims to explore the presence of left ventricular (LV) and left atrial (LA) morphological and functional abnormalities in patients with Barlow's disease (BD) without significant mitral regurgitation (MR) and to investigate whether these abnormalities may predict MR progression. Methods and results Consecutive patients with BD were retrospectively identified from two tertiary centres; those with MR graded from trivial to mild-to-moderate were selected and matched with healthy controls in a 1:1 ratio. Conventional and speckle-tracking echocardiographic data were collected. The development of moderate-to-severe or greater MR was evaluated on follow-up echocardiograms. Patients with BD (n = 231) showed increased LV dimensions and indexed LV mass (LVMi) in comparison with controls (P < 0.001); LV remodelling worsened with higher MR severity and was accompanied by an increased prevalence of eccentric LV hypertrophy (eLVH). Moreover, BD patients had larger LA volumes and more impaired LA reservoir strain vs. controls (P < 0.001), while LV strain was similar between the two groups. Multivariable linear regression analyses in the overall population identified BD and MR grade as independent predictors of remodelling markers (LV dimensions, LVMi, and LA volume) and BD as independent correlate of LA strain. MR progression was observed in 51 BD subjects (out of 170 patients with available follow-up). On Cox regression analysis, age, eLVH, mild-to-moderate MR, and mitral annular disjunction (MAD) emerged as independent predictors of MR progression. Conclusion BD patients without significant MR show early LV and LA remodelling, together with reduced LA strain. MR progression was associated with eccentric LV remodelling, MAD, and MR severity. [ABSTRACT FROM AUTHOR]

Valvular heart disease is a common cause of morbidity and mortality worldwide and has no effective medical therapy. Severe disease is managed with valve replacement procedures, which entail high health care-related costs and postprocedural morbidity and mortality. Robust ongoing research programs have elucidated many important molecular pathways contributing to primary valvular heart disease. However, there remain several key challenges inherent in translating research on valvular heart disease to viable molecular targets that can progress through the clinical trials pathway and effectively prevent or modify the course of these common conditions. In this scientific statement, we review the basic cellular structures of the human heart valves and discuss how these structures change in primary valvular heart disease. We focus on the most common primary valvular heart diseases, including calcific aortic stenosis, bicuspid aortic valves, mitral valve prolapse, and rheumatic heart disease, and outline the fundamental molecular discoveries contributing to each. We further outline potential therapeutic molecular targets for primary valvular heart disease and discuss key knowledge gaps that might serve as future research priorities. [ABSTRACT FROM AUTHOR]

Mitral annular disjunction (MAD) is a structural abnormality of the mitral valve whereby there is a distinct separation of the mitral valve annulus-left atrial wall continuum and the basal portion of the posterolateral ventricular myocardium, a region which would normally be attached. It is usually associated with myxomatous mitral valve disease. MAD has been reported in various studies since the last four decades as constituting around 42-90% of patients with myxomatous mitral valve disease and mitral valve prolapse. It is often associated with arrhythmias ranging from benign ventricular ectopics to malignant ventricular fibrillations causing sudden cardiac deaths in the young. This condition seems to be more common in women. Last few decades have witnessed many case reports and prospective studies about this entity enhancing our understanding about its pathophysiology. We report a case of a 34 year old male with mitral valve prolapse and MAD resulting in severe mitral regurgitation who underwent mitral valve replacement. [ABSTRACT FROM AUTHOR]

Mitral valve prolapse is a common valve disorder that usually has a benign prognosis unless there is significant regurgitation or LV impairment. However, a subset of patients are at an increased risk of ventricular arrhythmias and sudden cardiac death, which has led to the recognition of "arrhythmic mitral valve prolapse" as a clinical entity. Emerging risk factors include mitral annular disjunction and myocardial fibrosis. While echocardiography remains the primary method of evaluation, cardiac magnetic resonance has become crucial in managing this condition. Cine magnetic resonance sequences provide accurate characterization of prolapse and annular disjunction, assessment of ventricular volumes and function, identification of early dysfunction and remodeling, and quantitative assessment of mitral regurgitation when integrated with flow imaging. However, the unique strength of magnetic resonance lies in its ability to identify tissue changes. T1 mapping sequences identify diffuse fibrosis, in turn related to early ventricular dysfunction and remodeling. Late gadolinium enhancement sequences detect replacement fibrosis, an independent risk factor for ventricular arrhythmias and sudden cardiac death. There are consensus documents and reviews on the use of cardiac magnetic resonance specifically in arrhythmic mitral valve prolapse. However, in this article, we propose an algorithm for the broader use of cardiac magnetic resonance in managing this condition in various scenarios. Future advancements may involve implementing techniques for tissue characterization and flow analysis, such as 4D flow imaging, to identify patients with ventricular dysfunction and remodeling, increased arrhythmic risk, and more accurate grading of mitral regurgitation, ultimately benefiting patient selection for surgical therapy. [ABSTRACT FROM AUTHOR]

Purpose: During the last decade, a number of echocardiographic studies have employed speckle tracking echocardiography (STE) for assessing myocardial deformation properties in individuals with mitral valve prolapse (MVP), reporting not univocal results. Accordingly, we performed a systematic review and meta-analysis to summarize the main findings of these studies and to examine the overall influence of MVP on left ventricular (LV) global longitudinal strain (GLS). Methods: All echocardiographic studies assessing conventional echoDoppler parameters and myocardial strain indices in MVP individuals vs. controls without MVP, selected from PubMed and EMBASE databases, were included. The risk of bias was assessed by using the National Institutes of Health (NIH) Quality Assessment of Case-Control Studies. Continuous data (LV-GLS) were pooled as a standardized mean difference (SMD) comparing MVP group with healthy controls. The overall SMD of LV-GLS was calculated using the random-effect model. Results: The full-texts of 15 studies with 1088 individuals with MVP and 591 healthy controls were analyzed. Average LV-GLS magnitude was significantly, even though modestly, reduced in MVP individuals in comparison to controls (19.4 ± 3.4% vs. 21.1 ± 2.8%, P < 0.001). The overall effect of MVP on LV-GLS was small-to-medium (SMD − 0.54, 95%CI -0.76,-0.32, P < 0.001). Substantial heterogeneity was detected for the included studies, with an overall I2 statistic value of 75.9% (P < 0.001). Egger's test for a regression intercept gave a P-value of 0.58, indicating no publication bias. On meta-regression analysis, none of the moderators (the age, the percentage of females among MVP individuals, body mass index, heart rate and systolic blood pressure of MVP individuals, the degree of mitral regurgitation, the type of ultrasound machine employed for strain echocardiographic imaging and finally the beta blocker treatment) was significantly associated with effect modification (all P < 0.05). Regional strain analysis, performed by two-third of the studies, highlighted a more enhanced reduction in myocardial strain parameters at level of the LV basal infero-lateral segments in all directions (longitudinal, circumferential and radial), with apical sparing. Conclusions: The longitudinal strain impairment detected in MVP individuals is more regional than global, with peculiar involvement of the LV basal infero-lateral segments and relative apical sparing pattern. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Cardiovascular disease is a leading global cause of mortality. Most stethoscopes still necessitate the use of tubing, which entails direct physical contact between the healthcare provider and patient. The stethoscope can serve as a means of transmission if it is utilized on individuals who have been diagnosed with airborne and droplet-borne infectious illnesses. A prototype was created to capture heart sounds using a Phonocardiography (PCG) device over website-based Bluetooth connectivity. This approach offers the benefits of being cost-effective, facilitating computer-aided diagnostics, and being wearable. In addition, the primary significance of this study resides in the identification of heart sound irregularities caused by cardio dynamic abnormalities of the heart valves, known as murmurs. The heart sound categorization process utilizes a machine learning model that involves extracting 25 Mel frequency cepstral coefficients (MFCC) as features. The model employs a hybrid approach combining convolutional neural network and long short-term memory (CNN-LSTM) techniques. The research findings indicate that the suggested model achieves an average accuracy rate of 95.9% over five distinct categories, i.e., normal, atrial stenosis, mitral regurgitation, mitral stenosis, and mitral valves prolapse. Further study can be conducted on hardware development by incorporating an infrared sensor at the fingertip of the stethoscope. [ABSTRACT FROM AUTHOR]

Background: This case highlights several complications of a late and rare presentation of culture-negative Streptococcus pyogenes endocarditis of a previously repaired mitral valve with an annuloplasty ring including recurrent cardioembolic strokes, which was initially missed on transthoracic echocardiography. Case presentation: A 66-year-old Caucasian female with prior mitral valve prolapse status post mitral valve annuloplasty and left atrial appendage occlusion, followed by two strokes, presented with supraventricular tachycardia that resolved spontaneously. During an inpatient admission, she developed symptoms of another stroke, and imaging studies were suggestive of recurrent cardioembolic phenomenon. Additional workup revealed two small intra-atrial masses adherent to the mitral annuloplasty ring missed on prior evaluation for recurrent stroke. She underwent surgical repair in the setting of a chronic culture-negative infectious endocarditis with Streptococcus pyogenes and recovered well with no further cardioembolic phenomenon. Conclusion: This case serves to highlight the importance of having a higher index of suspicion in any cardiac prosthesis patient for endocarditis when presenting with symptoms such as recurrent stroke, arrhythmias, and abnormal cardiac lab work. It also demonstrates the need for appropriate imaging with transthoracic echocardiography followed by transesophageal echocardiography and reviews surgical indications to diagnose and treat culture-negative endocarditis. [ABSTRACT FROM AUTHOR]

Background and Aims Presentation, outcome, and management of females with degenerative mitral regurgitation (DMR) are undefined. We analysed sex-specific baseline clinical and echocardiographic characteristics at referral for DMR due to flail leaflets and subsequent management and outcomes. Methods In the Mitral Regurgitation International Database (MIDA) international registry, females were compared with males regarding presentation at referral, management, and outcome (survival/heart failure), under medical treatment, post-operatively, and encompassing all follow-up. Results At referral, females (n = 650) vs. males (n = 1660) were older with more severe symptoms and higher MIDA score. Smaller cavity diameters belied higher cardiac dimension indexed to body surface area. Under conservative management, excess mortality vs. expected was observed in males [standardized mortality ratio (SMR) 1.45 (1.27–1.65), P <.001] but was higher in females [SMR 2.00 (1.67–2.38), P <.001]. Female sex was independently associated with mortality [adjusted hazard ratio (HR) 1.29 (1.04–1.61), P =.02], cardiovascular mortality [adjusted HR 1.58 (1.14–2.18), P =.007], and heart failure [adjusted HR 1.36 (1.02–1.81), P =.04] under medical management. Females vs. males were less offered surgical correction (72% vs. 80%, P <.001); however, surgical outcome, adjusted for more severe presentation in females, was similar (P ≥.09). Ultimately, overall outcome throughout follow-up was worse in females who displayed persistent excess mortality vs. expected [SMR 1.31 (1.16–1.47), P <.001], whereas males enjoyed normal life expectancy restoration [SMR 0.92 (0.85–0.99), P =.036]. Conclusions Females with severe DMR were referred to tertiary centers at a more advanced stage, incurred higher mortality and morbidity under conservative management, and were offered surgery less and later after referral. Ultimately, these sex-related differences yielded persistent excess mortality despite surgery in females with DMR, while males enjoyed restoration of life expectancy, warranting imperative re-evaluation of sex-specific DMR management. [ABSTRACT FROM AUTHOR]

Mitral valve prolapse (MVP) is the most common valve disease in the western world and recently emerged as a possible substrate for sudden cardiac death (SCD). It is estimated an annual risk of sudden cardiac death of 0.2 to 1.9% mostly caused by complex ventricular arrhythmias (VA). Several mechanisms have been recognized as potentially responsible for arrhythmia onset in MVP, resulting from the combination of morpho-functional abnormality of the mitral valve, structural substrates (regional myocardial hypertrophy, fibrosis, Purkinje fibers activity, inflammation), and mechanical stretch. Echocardiography plays a central role in MVP diagnosis and assessment of severity of regurgitation. Several abnormalities detectable by echocardiography can be prognostic for the occurrence of VA, from morphological alteration including leaflet redundancy and thickness, mitral annular dilatation, and mitral annulus disjunction (MAD), to motion abnormalities detectable with "Pickelhaube" sign. Additionally, speckle-tracking echocardiography may identify MVP patients at higher risk for VA by detection of increased mechanical dispersion. On the other hand, cardiac magnetic resonance (CMR) has the capability to provide a comprehensive risk stratification combining the identification of morphological and motion alteration with the detection of myocardial replacement and interstitial fibrosis, making CMR an ideal method for arrhythmia risk stratification in patients with MVP. Finally, recent studies have suggested a potential role in risk stratification of new techniques such as hybrid PET-MR and late contrast enhancement CT. The purpose of this review is to provide an overview of the mitral valve prolapse syndrome with a focus on the role of imaging in arrhythmic risk stratification. Clinical relevance statement: Mitral valve prolapse is the most frequent valve condition potentially associated with arrhythmias. Imaging has a central role in the identification of anatomical, functional, mechanical, and structural alterations potentially associated with a higher risk of developing complex ventricular arrhythmia and sudden cardiac death. Key Points: • Mitral valve prolapse is a common valve disease potentially associated with complex ventricular arrhythmia and sudden cardiac death. • The mechanism of arrhythmogenesis in mitral valve prolapse is complex and multifactorial, due to the interplay among multiple conditions including valve morphological alteration, mechanical stretch, myocardial structure remodeling with fibrosis, and inflammation. • Cardiac imaging, especially echocardiography and cardiac magnetic resonance, is crucial in the identification of several features associated with the potential risk of serious cardiac events. In particular, cardiac magnetic resonance has the advantage of being able to detect myocardial fibrosis which is currently the strongest prognosticator. [ABSTRACT FROM AUTHOR]

Background Some patients affected by mitral valve (MV) prolapse (MVP) are at higher risk of ventricular arrhythmias (VAs), but the underlying pathogenesis, as well as the effects of surgery on VA, remain not fully understood. Mitral valve repair, however, represents a privileged point of view to deepen the understanding of arrhythmogenesis in this context. Hence, we report an interesting case of MV re-repair. Case summary A 52-year-old man was referred to our institution for severe mitral regurgitation (MR) due to P2 prolapse in the context of myxomatous MV degeneration. Pre-operative imaging showed systolic mitral annular disjunction, left ventricular (LV) wall curling, Pickelhaube's sign, and a prolapsing tricuspid valve (TV) with only mild regurgitation. Twenty-four-hour electrocardiogram (ECG) Holter revealed a significant burden of premature ventricular contractions (PVCs), most of them originating from anterior papillary muscle (APM), posterior papillary muscle (PPM), and mitral annulus (MA). Quadrangular resection of P2 and mitral annuloplasty were performed. One year later, relapse of severe MR due to a residual P2M1 prolapse occurred. Twenty-four-hour ECG Holter showed no PVCs from PPM and MA, while those from APM persisted. A central edge-to-edge repair was effectively used to fix the residual prolapse. After 1 year from REDO surgery, a third ECG Holter confirmed the absence of any remaining LV PVCs, but still few ectopic beats originating from TV were recorded. Discussion Here, we report a case of VA resolution after specific, anatomical triggers addressing surgical gestures. Our experience confirms that MV surgery may have a role in MVP patients' arrhythmias correction. [ABSTRACT FROM AUTHOR]

Introduction: Malignant ventricular arrhythmia (VA) and sudden cardiac death (SCD) have been reported in patients with mitral valve prolapse (MVP); however, effective risk stratification methods are still lacking. Myocardial fibrosis is thought to play an important role in the development of VA; however, observational studies have produced contradictory findings regarding the relationship between VA and late gadolinium enhancement (LGE) in MVP patients. The aim of this meta‐analysis and systematic review of observational studies was to investigate the association between left ventricular LGE and VA in patients with MVP. Methods: We searched the PubMed, Embase, and Web of Science databases from 1993 to 2023 to identify case–control, cross‐sectional, and cohort studies that compared the incidence of VA in patients with MVP who had left ventricular LGE and those without left ventricular LGE. Results: A total of 1464 subjects with MVP from 12 observational studies met the eligibility criteria. Among them, VA episodes were reported in 221 individuals (15.1%). Meta‐analysis demonstrated that the presence of left ventricular LGE was significantly associated with an increased risk of VA (pooled risk ratio 2.96, 95% CI: 2.26−3.88, p for heterogeneity = 0.07, I2 = 40%). However, a meta‐regression analysis of the prevalence of mitral regurgitation (MR) showed that the severity of MR did not significantly affect the association between the occurrence of LGE and VA (p = 0.079). Conclusion: The detection of LGE could be helpful for stratifying the risk of VA in patients with MVP. [ABSTRACT FROM AUTHOR]

(1) Background and Objectives: Mitral regurgitation is a common valve disease requiring surgical repair. Even with satisfactory results, repair techniques may underlie subjectivity and variability and require long learning curves. A novel approach, the "Roman Arch" technique, may ease the technical burden. This study assessed an automated suturing device's feasibility and time efficiency for a proposed simplified technique. (2) Materials and Methods: Using the MiStitch™ and MiKnot™ devices (LSI Solutions, Inc., Victor, NY, USA), the suture pattern was performed in a cadaver model. Three surgeons with different expertise levels conducted the procedures. Repair and suture placement times were recorded and analyzed. (3) Results: The modified "Roman Arch" repair was completed on all ten human heart specimens with an average total repair time of 3:01 ± 00:59 min and a trend toward reduced times as experience increased. The study confirmed the technical feasibility with 90% of the attempts rated as rather satisfactory or very satisfactory. (4) Conclusions: The MiStitch™ system effectively facilitated the modified "Roman Arch" repair in an ex vivo setting, suggesting its potential to reduce the technical complexity of mitral valve repairs. Further studies are needed to confirm its efficacy and safety in clinical practice. [ABSTRACT FROM AUTHOR]

Mitral annular disjunction (MAD) is defined as a separation between the mitral annulus and the left ventricular myocardium and is most often seen in association with mitral valve prolapse (MVP). MAD has been linked to ventricular arrhythmias in adults, independent of MVP. However, it has rarely been reported in children. We, hereby, report two cases of MAD associated with a large atrial septal defect (ASD). Thus far, there are no consensus guidelines for the management of MAD. The additional association of large ASD further complicates the decision-making in these patients. To the best of our knowledge, this is the first report of the association of MAD with ASD. We further discuss the challenges in the management of this condition.

Objectives: The aim of this study is to evaluate echocardiographically (and determine pathology if there is any) the diameters and Z-scores of aortic annulus, sinus of valsalva and ascending aorta in patients with bicuspid aortic valve, aortic and mitral valve prolapse and healthy children. Materials and Methods: This retrospective cross-sectional study includes three hundred and fifty patients with bicuspid aortic valve, aortic and mitral valve prolapse and healthy children. One hundred and ninety of them (54.3%) are non-syndromic, non-operated, hemodynamically stable patients. One hundred and sixty (45.7%) are heathy control group. Three hundred and fifty of 0-18 (average 10.47) years old patients are boys (58.6%) and 150 are girls (42.9%). Aortic annulus, sinus of valsalva and ascending aorta were measured on the parasternal long axis view. The indexed values were obtained by dividing all measured parameters by body surface area. The Z-scores and percentile values are calculated for each group. Results: Average indexed aortic annulus value is 1.59 cm/m2; sinus of Valsalva value is 2.27 cm/m2 and ascending aortic value is 2.07 cm/m2 in our study. Average aortic annulus Z-score is 0.4; sinus of valsalva Z-score is 0.08 and ascending aorta Z-score is 0.15. Conclusion: In the present study, children aged between 0.33-17.8 years, mean aortic diameters were determined in three levels. According to the nomogram we used in our study, our dilation rate was 33.6% in the patient group, 21.4% in the entire patient group, and 6.9% in the control group.

Non-syndromic mitral valve prolapse (MVP) is a common disease. In most cases, its benign course is noted, however, a connection between MVP and ventricular arrhythmias is reported, as well as sudden cardiac death. Clinical signs (chest pain, syncope/lipotymia, mid-systolic click), results of instrumental studies (electrocardiographic, echocardiographic, magnetic resonance imaging) allow us to identify known ("old") risk factors and new phenomena encountered in rhythm and conduction disturbances during MVP. Signs of "arrhythmic" MVP, often detected in young women, include prolapse of thickened both mitral valve leaflets, T wave inversion in the inferior basal leads on the electrocardiogram, annulus fibrosus disjunction, fibrosis of the papillary muscles and myocardium in the inferior basal wall of the left ventricle, determined by magnetic resonance imaging. resonance tomography and myocardial biopsies. In 2022, the European Heart Rhythm Association Expert Consensus presented risk stratification and treatment principles for patients with arrhythmic MVP, identifying low, intermediate and high-risk groups, and in 2024, the first meta-analysis of studies was performed to identify prognostic risk factors of arrhythmic MVP. The most significant predictors of arrhythmias included late gadolinium enhancement on magnetic resonance imaging, T-wave inversion on the electrocardiogram, prolapse of both mitral valve leaflets, and mitral annulus disjunction. When choosing treatment for patients with arrhythmic MVP, clinical characteristics are taken into account and a personalized approach is used to prevent sudden cardiac death and severe ventricular arrhythmias. As a rule, to prevent sudden cardiac death in patients with arrhythmic MVP, four treatment options are considered: medications (beta-blockers or non-dihydropyridine slow calcium channel blockers, a combination of a beta-blocker and flecainide, or amiodarone), radiofrequency ablation, surgical treatment of the mitral valve, implantable cardioverter — defibrillator (for primary or secondary prevention of sudden cardiac death).The rationale for surgical, electrophysiological, and/or therapeutic treatments depends on the specific rhythm disorder.

Abstract Objective Mitral valve failure is one of the most common valvular heart diseases worldwide. Valve replacement and repair have an impact on the quality of life of patients. Therefore, the present study was conducted to compare the quality of life in patients with mitral valve replacement and those who underwent mitral valve repair. Methods In this cross-sectional study, we considered all cardiac patients with ischemic mitral insufficiency who underwent mitral valve repair and patients with a history of valve replacement in Imam Ali Hospital of Kermanshah between 2014 and 2020. Two Minnesota and general quality of life questionnaires along with a checklist for demographic variables were used for data collection. Data analysis was performed using SPSS version 21 software. Results The mean quality of life score based on the general quality of life scale in the valve repair group was 32.33 (SD = 2.29) and in the valve replacement group 32.89(SD = 2.60), (p = 0.917). Also, mean quality of life, as measured by the Minnesota MLHFQ was 60.89(SD = 17.67) in the valve repair group and 63.42 (SD = 12.13) in the valve replacement group (p = 0.308). The results showed that the average general quality of life was different in study groups regarding education. Tukey’s post hoc test showed that the average general quality of life in illiterate people is significantly lower than in people with academic degrees (P-value = 0.001). Conclusion The quality of life of the patients in both the valve repair and replacement groups was at an average level. There was no significant difference between the general quality of life and the Minnesota scales, suggesting that both tools can be effectively used to measure patients’ quality of life. The study’s findings can be valuable for monitoring patients, screening for conditions, and enhancing communication between doctors and patients.

Background and Aim: Mitral valve prolapse is a cardiac condition affecting the valve between the heart's left chambers, with genetic factors playing a significant role. This study aimed to assess the relationship between ACE gene deletion/insertion polymorphism and mitral valve prolapse in Mazandaran Province, located in northern Iran. Methods: This case-control study included 90 patients with mitral valve prolapse and 95 healthy individuals as the control group. Genomic DNA was extracted from blood leukocytes, and genotypes and allelic frequencies were determined using the GAP-PCR method. Results: The frequencies of II, ID, and DD genotypes of the ACE gene in patients were 22.22%, 42.22%, and 35.46%, respectively, while in the control group, they were 32.63%, 45.26%, and 22.1%, respectively. A statistically significant difference was observed in the frequency of the DD genotype in patients compared to the control group (P=0.048, CI=1.00-3.70, OR=1.92). The odds ratio indicated that the D allele increased the probability of mitral valve prolapse by 1.58 times more than the I allele (OR=1.58). Furthermore, the frequency of the DD genotype was significantly higher in patients with high blood pressure (33.9%) compared to those without high blood pressure (12.7%) (P=0.02). Conclusion: The results suggest a potential association between the I/D polymorphism of the ACE gene and mitral valve prolapse. However, further studies involving larger populations are necessary to confirm these findings.

The phonocardiogram (PCG) can be used as an affordable way to monitor heart conditions. This study proposes the training and testing of several classifiers based on SVMs (support vector machines), k-NN (k-Nearest Neighbor), and NNs (neural networks) to perform binary ("Normal"/"Pathologic") and multiclass ("Normal", "CAD" (coronary artery disease), "MVP" (mitral valve prolapse), and "Benign" (benign murmurs)) classification of PCG signals, without heart sound segmentation algorithms. Two datasets of 482 and 826 PCG signals from the Physionet/CinC 2016 dataset are used to train the binary and multiclass classifiers, respectively. Each PCG signal is pre-processed, with spike removal, denoising, filtering, and normalization; afterward, it is divided into 5 s frames with a 1 s shift. Subsequently, a feature set is extracted from each frame to train and test the binary and multiclass classifiers. Concerning the binary classification, the trained classifiers yielded accuracies ranging from 92.4 to 98.7% on the test set, with memory occupations from 92.7 kB to 11.1 MB. Regarding the multiclass classification, the trained classifiers achieved accuracies spanning from 95.3 to 98.6% on the test set, occupying a memory portion from 233 kB to 14.1 MB. The NNs trained and tested in this work offer the best trade-off between performance and memory occupation, whereas the trained k-NN models obtained the best performance at the cost of large memory occupation (up to 14.1 MB). The classifiers' performance slightly depends on the signal quality, since a denoising step is performed during pre-processing. To this end, the signal-to-noise ratio (SNR) was acquired before and after the denoising, indicating an improvement between 15 and 30 dB. The trained and tested models occupy relatively little memory, enabling their implementation in resource-limited systems. [ABSTRACT FROM AUTHOR]

Background: Mitral annulus (MA) area is derived during transthoracic echocardiography (TTE) assuming of a circular shape using the MA diameter from the apical 4 chamber (A4c) view. Since the MA is not a circular structure, we hypothesized that an elliptical model using parasternal long-axis (PLAX) and apical 2 chamber (A2c) view measured MA diameters would have better agreement with 3-dimensional transesophageal echocardiography (3D TEE) measured MA in degenerative mitral valve disease (DMVD). Methods: Seventy-six patients with moderate-to-severe DMVD had 2D TTE and 3D TEE performed. MA area was measured retrospectively using semi-automatic modeling of 3D data (3D TEEsa) and considered as the reference method. MA diameters were measured using different 2D TTE views. MA area was calculated using assumptions of a circular or an elliptical shape. 2D TTE derived and 3D TEEsa. MA areas were compared using linear regression and Bland-Altman analysis. Results: The median MA area measured at 3D TEEsa was 1,386 (1,293–1,673) mm2. With 2D TTE, the circular model using A4c view diameter resulted in a small systematic underestimation of MA area (6%), while the elliptical model using PLAX and A2c diameters resulted in 25% systematic underestimation. The standard deviations of the distributions of inter-method differences were wide for all 2D TTE methods (265–289 mm2) when compared to 3D TEEsa, indicating imprecision. Conclusions: When compared with 3D TEEsa modeling of the MA as the reference, the assumption of a circular shape using A4c TTE view diameter was the method with the least systematic error to assess MA area in DMVD and moderate to severe regurgitation. [ABSTRACT FROM AUTHOR]

Heart diseases have a great impact on human health. Heart sound signals contain a lot of useful information about heart diseases. Therefore, various heart diseases can be judged by heart sound auscultation. In order to improve the accuracy of classification and judgment, a heart sound signal classification method based on the mixed characteristics of Mel cepstrum coefficient and second-order spectrum is proposed: first, a class of normal heart sounds and aortic stenosis, mitral regurgitation, mitral stenosis, mitral valve prolapse. The Mel cepstrum coefficients and second-order spectral features of four kinds of abnormal heart sounds with mitral valve prolapse are extracted separately and then combined into a new feature. The convolution neural network is used for learning and classification. The whole data set has a total of 1000 audio records, which are randomly divided into test sets and training sets by 2:3. From the experimental results, it can be seen that the accuracy rate in the training set is 99.6%, and the accuracy rate in the test set is 98.5%. Compared with other traditional classification and recognition methods, the accuracy is significantly improved. [ABSTRACT FROM AUTHOR]

In vitro modeling of the left heart relies on accurately replicating the physiological conditions of the native heart. The targeted physiological conditions include the complex fluid dynamics coming along with the opening and closing of the aortic and mitral valves. As the mitral valve possess a highly sophisticated apparatus, thence, accurately modeling it remained a missing piece in the perfect heart duplicator puzzle. In this study, we explore using a hydrogel-based mitral valve that offers a full representation of the mitral valve apparatus. The valve is tested using a custom-made mock circulatory loop to replicate the left heart. The flow analysis includes performing particle image velocimetry measurements in both left atrium and ventricle. The results showed the ability of the new mitral valve to replicate the real interventricular and atrial flow patterns during the whole cardiac cycle. Moreover, the investigated valve has a ventricular vortex formation time of 5.2, while the peak e- and a-wave ventricular velocities was 0.9 m/s and 0.4 m/s respectively. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Aims Although systolic expansion of the annulus has been recognized in Barlow's disease, the mechanisms of the unique pathological movement of the annulus and its relation to the leaflet augmentation have not yet been clarified. We aimed to investigate the detailed mechanisms of the characteristic mitral apparatus dynamics in Barlow's disease by frame-by-frame sequential geometric analysis using real-time 3D transoesophageal echocardiography. Methods and results Fifty-three patients with Barlow's disease and severe mitral regurgitation without torn chordae, as well as 10 controls, were included. We evaluated geometric changes in the mitral complex using 3D transoesophageal echocardiography at five points during systole. To identify early systolic billowing of leaflets, the annulo-leaflet angle was measured. We also performed a more detailed analysis in four consecutive frames just before and after leaflet free-edge prolapse above the annulus plane. The median annulo-leaflet angle of both leaflets in early systole was >0° (above annulus plane) in patients with Barlow's disease, and billowing of the leaflet body was observed from early systole. The prolapse volume of both leaflets increased markedly from early to mid-systole [1.60 (0.85–2.80) to 4.00 (2.10–6.45) mL; analysis of variance (ANOVA), P < 0.001; post hoc , P < 0.05]. With frame-by-frame analysis, dynamic augmentation of the annulus and leaflets developed between frames just before and just after leaflet free-edge prolapse (ANOVA, P < 0.01; post hoc , P < 0.05). Conclusion In Barlow's disease, early systolic billowing of the mitral leaflet induces systolic annulus expansion followed by leaflet augmentation and leaflet free-edge prolapse. [ABSTRACT FROM AUTHOR]

Objective: Mitral valve failure is one of the most common valvular heart diseases worldwide. Valve replacement and repair have an impact on the quality of life of patients. Therefore, the present study was conducted to compare the quality of life in patients with mitral valve replacement and those who underwent mitral valve repair. Methods: In this cross-sectional study, we considered all cardiac patients with ischemic mitral insufficiency who underwent mitral valve repair and patients with a history of valve replacement in Imam Ali Hospital of Kermanshah between 2014 and 2020. Two Minnesota and general quality of life questionnaires along with a checklist for demographic variables were used for data collection. Data analysis was performed using SPSS version 21 software. Results: The mean quality of life score based on the general quality of life scale in the valve repair group was 32.33 (SD = 2.29) and in the valve replacement group 32.89(SD = 2.60), (p = 0.917). Also, mean quality of life, as measured by the Minnesota MLHFQ was 60.89(SD = 17.67) in the valve repair group and 63.42 (SD = 12.13) in the valve replacement group (p = 0.308). The results showed that the average general quality of life was different in study groups regarding education. Tukey's post hoc test showed that the average general quality of life in illiterate people is significantly lower than in people with academic degrees (P-value = 0.001). Conclusion: The quality of life of the patients in both the valve repair and replacement groups was at an average level. There was no significant difference between the general quality of life and the Minnesota scales, suggesting that both tools can be effectively used to measure patients' quality of life. The study's findings can be valuable for monitoring patients, screening for conditions, and enhancing communication between doctors and patients. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Adaptation of the heart is often a blessing for the patient, but sometimes a diagnostic challenge for the responsible physician. The clinical difficulty may be enhanced when employing diagnostic tools that are hard to interpret. Ratio‐based metrics are notorious in this respect, and particularly risky in the follow‐up evaluation of heart transplant patients. However, measures expressed as physical units contribute to a comprehensive clinical evaluation and guide proper patient management. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Aims: Mitral valve prolapse (MVP) is an accepted cause of sudden cardiac death (SCD) in most autopsy series. Diagnosis at autopsy relies upon subjective assessment with no established objective pathological criteria. This study set out to establish objective measurements to help pathologists dealing with SCD. Methods: We diagnosed 120 (1.5%) cases of MVP in 8108 cases of SCD. We measured the mitral annulus, anterior and posterior leaflets, rough zone and mitral annular disjunction (MAD) in 27 MVP cases and compared them to 54 age‐ and sex‐matched normal mitral valves. Results: Age of death was 39 ± 16 years, with 59 females and 61 males. History of mild MV disease was present in 19 (16%). Eleven (9%) died associated with exertion. Left ventricular hypertrophy was present in nine (15%) females and 10 (16%) males. Both MV leaflets showed thickening and ballooning in all individuals. MVP showed highly significantly increased annular circumference, elongation and thickening of both leaflets as well as increased MAD (all P < 0.001). Left ventricular fibrosis was present in 108 (90%), with interstitial fibrosis in the posterolateral wall and papillary muscle in 88 (81%) and coexisting replacement fibrosis in 40 (37%). Conclusion: This is the largest MVP associated with SCD series highlighting a young cohort with equal representation of males and females. There is involvement of both leaflets with significant annular dilatation, elongation and thickening of both leaflets with MAD. Left ventricular fibrosis explains arrhythmia. Our quantitative measurements should serve as a reference for pathologists assessing post‐mortem hearts for MVP. [ABSTRACT FROM AUTHOR]

Mitral valve prolapse is a well-known condition that is generally benign, but it can be associated with cardiac arrhythmias, particularly malignant ventricular arrhythmias and sudden cardiac death. This association and its outcome have been described in medical literature, but the low incidence leads to a lack of evidence regarding its management and stratification.The case of a young woman is presented, whose initial manifestation was syncope, with subsequent development of severe mitral insufficiency, followed by severe mitral regurgitation and the discovery of frequent ventricular extrasystoles. She underwent surgical repair with mitral valve plasty and cryoablation of the anterior papillary muscle. The clinical outcome was favorable, as evidenced by an improvement in symptoms and control of arrhythmia burden. [ABSTRACT FROM AUTHOR]

A retrospective cohort analysis was conducted on patients who were receiving treatment at a single center for rheumatic Mitral Stenosis (MS). 50 patients with mild to moderate MS who were at least 30 years old at the time of initial echocardiography were included. From a computerized database, demographic information, medical history, echocardiographic reports spanning a minimum of ten years, and pertinent complications were extracted. Results: During a duration of 13.4 ± 2.51 years, the severity of stenosis did not progress significantly in 34 patients. The ultimate echocardiographic evaluation identified two cohorts distinguished by a statistically significant disparity in mean valvular pressure gradient (7.38 ± 3.63 vs. 9.6 ± 2.75 mm Hg, p < 0.05) and mitral valve area (1.73 ± 0.67 vs. 1.2 ± 0.34 cm2, p < 0.05). When comparing patients with quiescent MS (group A) to patients with progressive disease (group B), it was observed that group B contained a greater proportion of patients (OR 9.147, p = 0.024). There were no notable disparities observed in the remaining parameters. Despite their prevalence, complications such as atrial fibrillation, cerebral ischemic events, and impaired right ventricle function did not differ significantly between the groups from a statistical standpoint. In our investigation, we identified an indolent natural progression of rheumatic MS. Notwithstanding this discovery, it retains the potential for adverse consequences. [ABSTRACT FROM AUTHOR]

Preoperative left ventricular (LV) ejection fraction (PreLVEF) and preoperative LV end-systolic diameter (PreESD) are known predictors for postoperative LV dysfunction after mitral valve repair (MVR). Fragmented QRS (fQRS) evaluated in 12-derivation electrocardiography has widely been accepted as a sign of myocardial fibrosis. In the present study, we aimed to evaluate the relationship between fQRS in preoperative 12‑lead electrocardiography (ECG) and postoperative LV dysfunction that develop after MVR in patients with severe primary mitral regurgitation (MR) due to mitral valve prolapse (MVP). From 2019 to 2022, 49 patients who had undergone successful MVR surgery for severeMR caused by MVP were enrolled in the study. The preoperative and postoperative echocardiographic data were collected retrospectively. We analyzed the demographic, echocardiographic, operative and postoperative parameters to assess the relationship between fQRS and early postoperative LV dysfunction, defined as an LVEF<60%. PreLVEF of all patients were ≥ %65. A total of 22 patients had fQRS (44.9%) and postoperative LV dysfunction was found to be 36.7%. A significantly higher rate of fQRS was observed in the group with postoperative LV dysfunction compared to the group without (12 (66.7%) vs 10 (32.3%), p: 0.036). In multivariate analysis for fQRS, PreESD, preoperative pulmonary artery systolic pressure (PrePASP), preoperative atrial fibrillation (PreAF), and male gender, only fQRS was found to be a significant predictor of postoperative LV dysfunction (p: 0.003, OR: 4.28, 95% CI (1.15–15.96). fQRS was found to be a predictor of postoperative LV dysfunction in the early period after MVR. fQRS may be a readily available and cost-effective test that can be used in clinical practice to predict postoperative LV dysfunction in patients undergoing MVR. [ABSTRACT FROM AUTHOR]