Degenerative Mitral Valve Research Articles

Comparison of Cardiorespiratory Fitness between Patients with Mitral Valve Prolapse and Healthy Peers: Findings from Serial Cardiopulmonary Exercise Testing

Individuals with mitral valve prolapse (MVP) have exercise intolerance even without mitral valve regurgitation. Mitral valve degeneration may progress with aging. We aimed to evaluate the influence of MVP on the cardiopulmonary function (CPF) of individuals with MVP through serial follow-ups from early to late adolescence. Thirty patients with MVP receiving at least two cardiopulmonary exercise tests (CPETs) using a treadmill (MVP group) were retrospectively analyzed. Age-, sex-, and body mass index-matched healthy peers, who also had serial CPETs, were recruited as the control group. The average time from the first CPET to the last CPET was 4.28 and 4.06 years in the MVP and control groups, respectively. At the first CPET, the MVP group had a significantly lower peak rate pressure product (PRPP) than the control group (p = 0.022). At the final CEPT, the MVP group had lower peak metabolic equivalent (MET, p = 0.032) and PRPP (p = 0.031). Moreover, the MVP group had lower peak MET and PRPP as they aged, whereas healthy peers had higher peak MET (p = 0.034) and PRPP (p = 0.047) as they aged. Individuals with MVP had poorer CPF than healthy individuals as they develop from early to late adolescence. It is important for individuals with MVP to receive regular CPET follow-ups.

Effect of Tricuspid Repair on Pacemaker Rate After Mitral Repair Is Higher in Degenerative Disease

BackgroundPermanent pacemaker (PPM) placement after mitral valve (MV) repair is affected by concomitant procedures, yet existing literature reports conflicting rates. We aimed to characterize the effect of concomitant operation on risk of need for postoperative PPM in patients who underwent MV repair. MethodsA retrospective review of a prospectively maintained institutional database (1996-2020) was conducted of consecutive patients undergoing MV repair, including concomitant procedures. Multivariable regression analysis was performed to evaluate the effect of a concomitant procedure on PPM rate. ResultsOf the 2824 patients undergoing MV repair, 6% (177/2824) required a PPM. The likelihood of PPM varied with concomitant procedures: aortic valve replacement (39/258 [15%]), coronary artery bypass grafting (86/789 [11%]), tricuspid valve (TV) repair (33/326 [10%]), and maze (27/407 [7%]). Increased PPM rate was associated with aortic valve replacement (odds ratio [OR], 2.2 [1.5-3.3]; P < .001), reduced ejection fraction (OR, 1.02 [1.01-1.04]; P < .001), and older age (OR, 1.04 [1.03-1.06]; P < .001). Concurrent TV repair was not associated with pacemaker in patients undergoing MV repair (P = .8) or MV repair for nondegenerative mitral regurgitation (P = .9). In patients with degenerative MV disease, PPM rate increased from 1.9% (21/1133) to 10% (11/109) with concomitant TV repair (P < .001), and TV repair was associated with a 3-fold increased pacemaker rate (OR, 3.1 [1.6-5.9]; P < .001). ConclusionsIn MV repair, risk of pacemaker with concomitant TV repair should be weighed more heavily in degenerative MV disease. Pacemaker risk should not discourage surgeons from performing TV repair in patients with nondegenerative MV disease, in patients already undergoing concomitant operation or with clinically significant tricuspid regurgitation.

Atrial secondary mitral regurgitation: prevalence, characteristics, management, and long-term outcomes

BackgroundThe prevalence, clinical characteristics, management and long-term outcomes of patients with atrial secondary mitral regurgitation (ASMR) are not well described.MethodsWe performed a retrospective, observational study of consecutive patients with grade III/IV MR determined by transthoracic echocardiography. The aetiology of MR was grouped as being either primary (due to degenerative mitral valve disease), ventricular SMR (VSMR: due to left ventricular dilatation/dysfunction), ASMR (due to LA dilatation), or other.ResultsA total of 388 individuals were identified who had grade III/IV MR; of whom 37 (9.5%) had ASMR, 113 (29.1%) had VSMR, 193 had primary MR (49.7%), and 45 (11.6%) were classified as having other causes. Compared to MR of other subtypes, patients with ASMR were on average older (median age 82 [74–87] years, p < 0.001), were more likely to be female (67.6%, p = 0.004) and usually had atrial fibrillation (83.8%, p = 0.001). All-cause mortality was highest in patients with ASMR (p < 0.001), but similar to that in patients with VSMR once adjusted for age and sex (hazard ratio [HR] 0.81, 95% confidence interval [CI] 0.52–1.25). Hospitalisation for worsening heart failure was more commonly observed in those with ASMR or VSMR (p < 0.001) although was similar between these groups when age and sex were accounted for (HR 0.74, 95% CI 0.34–1.58). For patients with ASMR, the only variables associated with outcomes were age and co-morbidities.ConclusionsASMR is a prevalent and distinct disease process associated with a poor prognosis, with much of this related to older age and co-morbidities.Graphical

Can cartilage intermediate layer protein 1 (CILP1) use as a novel biomarker for canine myxomatous mitral valve degeneration levels or not?

BackgroundMyxomatous mitral valve degeneration (MMVD) is the most common degenerative heart disease in dogs and is associated with irreversible changes in the valve tissue. Although traditional cardiac biomarkers are efficient for diagnosing MMVD, there are limitations, therefore, it is important to find novel biomarkers. Cartilage intermediate layer protein 1 (CILP1), an extracellular matrix-derived protein, acts as a transforming growth factor-β antagonist and is involved in myocardial fibrosis. This study aimed to evaluate serum CILP1 levels in canines with MMVD. Dogs with MMVD were staged according to the American College of Veterinary Internal Medicine consensus guidelines. Data analysis was performed using the Mann–Whitney U test, Spearman’s correlation, and receiver operating characteristic (ROC) curves.ResultsCILP1 levels were elevated in dogs with MMVD (n = 27) compared to healthy controls (n = 8). Furthermore, results showed that CILP1 levels were significantly higher in stage C group dogs compared to healthy controls. The ROC curve of CILP1 and NT-proBNP were good predictors of MMVD, although no similarity was observed between the two. Left ventricular end-diastolic diameter normalized to the body weight (LVIDdn) and left atrial to aorta dimension (LA/Ao) showed a strong association with CILP1 levels; however, no correlation was observed between CILP1 levels and vertebral heart size (VHS) and vertebral left atrial score (VLAS). The optimal cut-off value was selected from the ROC curve and dogs were classified according to the cut-off value (1.068 ng/mL, sensitivity 51.9%, specificity 100%). Results showed a significant association of CILP1 with cardiac remodeling indicators, such as VHS, VLAS, LA/Ao, and LVIDdn.ConclusionsCILP1 can be an indicator of cardiac remodeling in canines with MMVD and therefore, can be used as an MMVD biomarker.

Comparative Outcomes of Mitral Valve Annuloplasty With Support Rings and Autopericardial Strip in Patients With Degenerative Diseases

Objective: To compare the outcomes of mitral valve annuloplasty with support rings and that of the current technique employing an autopericardial strip in patients with degenerative diseases (patent RU2774033C1, June 14, 2022).Materials and methods: The study included 105 patients with degenerative mitral valve disease who underwent surgery in our clinic from May 2010 to January 2020. Depending on the conduit type, the patients were divided into two groups. Group I included 53 patients who underwent mitral valve annuloplasty with an autopericardial strip. Group II included 52 patients who underwent annuloplasty with a support ring. Male patients predominated in both groups: 59.6% and 57.7%, respectively. The mean age of patients was 54.9 ± 9.5 years (36–78 years) in group I and 54.8 ± 10.05 years (32–70 years) in group II. In most cases, tri- and quadrangular resections of the P2 segment were performed, and in some cases, neochords were implanted to the A2 segment.Results: There was 1 death in group II as a result of progressive cardiovascular and multiple organ failure while in group I no hospital mortality was reported. At the discharge, 81.1% of patients in group I and 59.6% of patients in group II had no mitral regurgitation.Conclusions: The current technique of mitral valve annuloplasty with an autopericardial strip in patients with degenerative diseases allows for adequate stabilization of the annulus fibrosus and improves the surgical outcomes in selected patients. With our proposed method of mitral valve annuloplasty, the suitable strip length can be chosen for each patient.

Outcomes of transapical mitral valve repair with neochordae implantation.

Transapical off-pump beating-heart neochord implantation (NC) has shown encouraging early results in patients with degenerative mitral regurgitation. The aim of this study was to evaluate clinical and echocardiographic 5-year outcomes of patients who underwent NC. All patients who underwent NC at our institution from November 2013 to March 2016 were included. Indications were severe symptomatic degenerative mitral regurgitation due to leaflet prolapse/flail. Patients were classified as having favorable anatomy (FA) and unfavorable anatomy (UA) on the basis of the extent and severity of mitral valve disease. All patients underwent clinical and echocardiographic follow-up at 1, 3, 6, and 12months, and annually thereafter. Data were prospectively collected and retrospectively analyzed. Outcomes were on the basis of the Mitral Valve Academic Research Consortium guidelines. One hundred consecutive patients were included in the analysis (FA: 81%; UA: 19%). Median age was 66years (interquartile range, 58-76) and median European System for Cardiac Operative Risk Evaluation (EuroSCORE) II was 1.4% (interquartile range, 0.7-2.3). Technical and procedural success were 98% and 94%, respectively. Thirty-day mortality was 2%. Device success was 94%, 92%, and 78%, at 30days, 1-year, and 5years, respectively. Patient success at 1year was 92%. Median follow-up was 5.1years. At 5years, overall survival was 83% with no difference between FA and UA patients. Cumulative incidence of severe mitral regurgitation recurrence at 5years was 14% (95% CI, 6.5%-22.8%) in FA patients and 63% (95% CI, 39.7%-86.2%) in UA patients, respectively (P<.001). Patients with FA compared with UAhad a lower incidence of reintervention (14.7% vs 43.4%; P<.001). Transapical off-pump beating heart NC might represent an acceptable option in patients with degenerative mitral valve disease and FA.

A Retrospective Study of Radiographic Measurements of Small Breed Dogs with Myxomatous Mitral Valve Degeneration: A New Modified Vertebral Left Atrial Size

A Retrospective Study of Radiographic Measurements of Small Breed Dogs with Myxomatous Mitral Valve Degeneration: A New Modified Vertebral Left Atrial Size

Longitudinal Outcomes Following Surgical Repair of Primary Mitral Regurgitation

Degenerative mitral valve (MV) disease is the most common cause of organic mitral regurgitation (MR) in developed countries. Surgical mitral valve repair is the gold standard treatment for primary MR. Surgical mitral valve repair is associated with excellent outcomes in terms of survival and freedom from recurrent MR. As well, innovations in surgical repair techniques, including thoracoscopically and robotically assisted approaches, further reduce morbidity. Emerging catheter-based therapies may also provide advantages in select patient groups. Although the outcomes following surgical mitral valve repair are well described in the literature, longitudinal follow-up is heterogenous. Indeed, longitudinal follow-up and long-term data are vital to better advise treatment and counsel patients.

Mitral degenerative valve leaflets suspected as primary valvular tumor: a case report

BackgroundEchocardiography plays an important role in the diagnosis of heart disease. Sometimes, however, it may also provide misdiagnosis information.Case presentationWe report a rare case of a misdiagnosis of primary mitral valvular tumor with severe regurgitation by preoperative echocardiography. During the surgery, the true lesion was found to be mitral valve leaflet prolapse due to degenerative mitral valve disease.ConclusionFor individual patient, the best clinical decision not only needs the extensive application of echocardiography, but also needs the combination of clinical symptoms and more auxiliary examination.

Non-invasive evaluation of pulmonary capillary wedge pressure using the left atrial expansion index in mitral valve stenosis, prosthesis and repair.

Pulmonary capillary wedge pressure (PCWP) non-invasive evaluation is limited in patients with mitral valve (MV) stenosis, prosthesis, and surgical repair. This study aimed to assess the left atrial expansion index (LAEI) measured through transthoracic echocardiography (TTE) as a novel parameter for estimating PCWP in these challenging cardiac conditions. We performed a retrospective, cross-sectional study, including chronic cardiac patients receiving within 24h a clinically indicated right heart catheterization (RHC) and transthoracic echocardiographic (TTE) exam. PCWP measured during RHC was used as the reference. TTE measurements were performed offline, blinded to RHC results. LAEI was calculated as LAEI = [(LAmaxVolume-LAminVolume)/LAminVolume] × 100. We included 167 patients (age = 73 ± 11.5years; PCWP = 18 ± 7.7mmHg) with rheumatic mitral valve (MV) stenosis (16.2%), degenerative MV stenosis (51.2%), MV prosthesis (18.0%), and MV surgical repair (13.8%). LAEI correlated logarithmically with PCWP, and the log-transformed LAEI (lnLAEI) showed a good linear association with PCWP (r = -0.616; p < 0.001). lnLAEI was an independent PCWP determinant, providing added predictive value over conventional clinical (age, atrial fibrillation, heart rate, MV subgroups) and echocardiographic variables (LVEF, MV effective orifice area, MV mean gradient, net atrioventricular compliance, and pulmonary arterial systolic pressure). lnLAEI identified PCWP > 12mmHg with AUC = 0.870, p < 0.001; and PCWP > 15mmHg with AUC = 0.797, p < 0.001, with an optimal cut-off of lnLAEI < 3.69. The derived equation PCWP = 36.8-5.5xlnLAEI estimated the invasively measured PCWP ± 6.1mmHg. In this cohort of patients with MV stenosis, prosthesis, and surgical repair, lnLAEI resulted in a helpful echocardiographic parameter for PCWP estimation.

Differences in clinical and echocardiographic features and outcomes between atrial functional mitral regurgitation patients with and without posterior mitral leaflet bending

BackgroundPosterior mitral leaflet (PML) bending is a cause of atrial functional mitral regurgitation (AFMR). We aimed to investigate differences in clinical and echocardiographic features and outcomes between AFMR patients with and without PML bending. MethodsWe retrospectively examined 118 AFMR patients with atrial fibrillation (AF), mild or greater MR without degenerative mitral valve changes, and left ventricular ejection fraction ≥50 %. Patients were classified by the presence of PML bending: PML bending (n=24) and no PML bending (n=94). PML bending was defined as PML-to-anterior mitral leaflet angle ratio ≥3.1 calculated using receiver operating characteristics analysis for eccentric MR jet toward left atrial posterior wall. The study endpoint was a composite of cardiac death, admission for heart failure, and mitral valve surgery. ResultsOverall, a total of 88 patients (75 %) had mild MR. There were no between-group differences in clinical and echocardiographic characteristics including AF duration and cardiac cavities size except for the length of inward bending of the left ventricular posterobasal wall and the mitral annular area. The 36-month event-free survival for the composite endpoint was significantly lower in the PML bending group (63 % vs. 78 %; Log-rank p=0.047). In multivariate analysis, PML bending was also associated with the composite outcome. ConclusionsAFMR patients with PML bending may have worse outcomes than those without PML bending despite similar clinical features.

Coaptation Length as Predictor of Recurrent Mitral Regurgitation After Surgical Repair for Degenerative Mitral Valve Disease: Meta-Analysis of Reconstructed Time-to-Event Data

Coaptation Length as Predictor of Recurrent Mitral Regurgitation After Surgical Repair for Degenerative Mitral Valve Disease: Meta-Analysis of Reconstructed Time-to-Event Data

Impact of pulmonary arterial systolic pressure on patients with mitral valve disease combined with atrial fibrillation.

To determine whether different changes of pulmonary artery systolic pressure (PASP) after surgeries have an impact on the long-term outcomes in patients with rheumatic and degenerative mitral valve (MV) disease and atrial fibrillation. Between 2004 and 2016, 1,188 patients with rheumatic and degenerative MV disease undergoing MV and Cox-Maze procedure were identified. Clinic outcomes, as well as rhythm state and echocardiography indices in long-term follow-up were recorded. Patients were grouped by the changes of PASP (persistently normal, persistently increased, increased, and decreased) from preoperative estimation to follow-up. A complete echocardiography was performed at baseline and after 5 years. During follow-up, free of death and atrial fibrillation (AF) off antiarrhythmic drugs was 90 and 61%, 78 and 41% at 5 and 10 years, respectively. Survival rate was higher in patients with persistently normal and became worse in patients with persistently increased and increased PASP (log-rank 166.0, P < 0.0001). Moreover, the patients with persistently normal PASP had a lowest risk of recurrent AF (SHR: 0817; CI: 0.765-0.872; P < 0.0001) after considering death as a competing risk. A persistently normal PASP at follow-up and degenerative MV disease were associated with improved survival and sinus rhythm (SR) maintenance at multivariable Cox regression analysis (P < 0.05). Patients with degenerative MV disease or have persistently normal PASP during follow-up have better survival and SR maintenance rate than patients with either rheumatic MV disease or persistently abnormal PASP.

Decreased serotonin transporter activity in the mitral valve contributes to progression of degenerative mitral regurgitation.

Degenerative mitral valve (MV) regurgitation (MR) is a highly prevalent heart disease that requires surgery in severe cases. Here, we show that a decrease in the activity of the serotonin transporter (SERT) accelerates MV remodeling and progression to MR. Through studies of a population of patients with MR, we show that selective serotonin reuptake inhibitor (SSRI) use and SERT promoter polymorphism 5-HTTLPR LL genotype were associated with MV surgery at younger age. Functional characterization of 122 human MV samples, in conjunction with in vivo studies in SERT-/- mice and wild-type mice treated with the SSRI fluoxetine, showed that diminished SERT activity in MV interstitial cells (MVICs) contributed to the pathophysiology of MR through enhanced serotonin receptor (HTR) signaling. SERT activity was decreased in LL MVICs partially because of diminished membrane localization of SERT. In mice, fluoxetine treatment or SERT knockdown resulted in thickened MV leaflets. Similarly, silencing of SERT in normal human MVICs led to up-regulation of transforming growth factor β1 (TGFβ1) and collagen (COL1A1) in the presence of serotonin. In addition, treatment of MVICs with fluoxetine not only directly inhibited SERT activity but also decreased SERT expression and increased HTR2B expression. Fluoxetine treatment and LL genotype were also associated with increased COL1A1 expression in the presence of serotonin in MVICs, and these effects were attenuated by HTR2B inhibition. These results suggest that assessment of both 5-HTTLPR genotype and SERT-inhibiting treatments may be useful tools to risk-stratify patients with MV disease to estimate the likelihood of rapid disease progression.

Clinical, electrocardiographic and echocardiographic profile of dogs with chronic degenerative mitral valve disease, treated at a private service of cardiology and respiratory diseases

Clinical, electrocardiographic and echocardiographic profile of dogs with chronic degenerative mitral valve disease, treated at a private service of cardiology and respiratory diseases

Болезнь Барлоу на примере клинического наблюдения

Barlow's disease (BD) is one of the dominant forms of degenerative mitral valve (MV) disease characterized by morphological changes of the connective tissue triggering the mechanism of mitral valve leaflet coaptation failure. The disease is characterized by MV prolapse driven by chordal rupture or elongation, leaflet area enlargement and annular ectasia. Typical BD symptoms are found in 24% of patients with mitral valve prolapse (MPV). It should be noted that BD is a heritable disorder. Patients with BD are typically young women (less than 40-year-old), who remain asymptomatic for a long time. BD is often diagnosed incidentally, during the screening examination. Radical correction of such MV dysfunction can be achieved only by using surgical techniques. Based on echocardiography findings, two types of Barlow's disease are distinguished: a classical one with MV leaflet thickening and a non-classical — without its thickening (presumably associated with dysplasia of the connective tissue). This article is focused on the foundations of semiotics and the main principles of BD patient management. It provides a review of MVP and mitral incompetence causes and describes a clinical case of surgical BD treatment. KEYWORDS: mitral regurgitation, plastic surgery of the mitral valve, Barlow's disease, mitral valve prolapse, heritable disorders of connective tissue, dysplasia. FOR CITATION: Reznik E.V., Golubev Yu.Yu., Yurtaeva N.V. et al. Barlow's disease illustrated by a clinical case. Russian Medical Inquiry. 2023;7(1):41–48 (in Russ.). DOI: 10.32364/2587-6821-2023-7-1-41-48.

Sex Differences in the Outcomes of Degenerative Mitral Valve Repair.

This study explored the sex differences in the outcomes of degenerative mitral valve repair (MVr). From 2010 to 2019, 1069 patients who underwent MVr due to degenerative mitral disease at Beijing Anzhen Hospital were analyzed. The average patient follow-up was 5.1 years (interquartile range: 5-7 years). The primary endpoint was overall survival. Secondary endpoints were freedom from reoperation and recurrent mitral regurgitation. A propensity-matched analysis was used to compare the outcomes of males and females. Females were older, had a higher prevalence of atrial fibrillation and moderate-to-severe tricuspid regurgitation, and had smaller left atrial, left ventricular end-diastolic, and left ventricular end-systolic diameters. Males were more likely to undergo concomitant coronary artery bypass grafting and had longer cardiopulmonary bypass and aortic cross-clamp times. The in-hospital mortality was <1% (10/1,069). After propensity score matching of 331 pairs of patients, most variables were well balanced. Before and after propensity score matching, the long-term survival and freedom from reoperation rates were similar. Males had higher durability after surgery compared with females. Females were referred to surgery later and had more complications than males. Long-term survival and freedom from reoperation rates were not significantly different between the sexes.

Association of Paraoxonase-1 and NT-proBNP with Clinical, Clinico-Pathologic and Echocardiographic Variables in Dogs with Mitral Valve Disease

The objective of the present study was to measure the concentration of Paraoxonase-1 (PON-1) and N-terminal-prohormone-B-type natriuretic peptide (NT-proBNP), in the serum of dogs with degenerative Mitral Valve Disease (MVD), in order to identify their association with the clinical stage and specific clinico-pathologic and echocardiographic findings.Eighty dogs diagnosed with MVD and staged according to the ACVIM (American College of Veterinary Internal Medicine) consensus statement (B1, B2, C and D), based on their clinical, radiographic, and echocardiographic findings, were included in the study. NT-proBNP was measured only in stage B1 and B2 dogs. Clinical stage did not have a significant effect on PON-1 concentrations (p = 0.149), but NT-proBNP levels were lower in B1 dogs (p = 0.001). A significant correlation between PON-1 and total plasma proteins (p = 0.001), albumin (p = 0.003) and white blood cell count (p = 0.041) was detected, whereas there was no significant correlation (p = 0.847) between PON-1 and NT-proBNP concentrations. PON-1 showed a significant but weak negative correlation with normalized left ventricular internal diameter at diastole (LVIDdn) (p = 0.022) and systole (LVIDsn) (p = 0.012), as well as mitral valve E to A wave velocity ratio (MV E/A) (p = 0.015), but not with Left Atrial to Aortic root ratio (LA/Ao) (p = 0.892) or fractional shortening (FS%) (p = 0.944). PON-1 seems to be an insensitive marker of clinical stage and disease severity in MVD, but can be indicative of some clinico-pathological and echocardiographic changes. NT-proBNP changes are independent of oxidative stress.

Mitral repair of myxomatous valves with simple annuloplasty: a follow-up up to 12 years

Diffuse myxomatous mitral valve degeneration (DMD) represents a challenge in the reparative mitral valve surgery. A subgroup of patients with symmetrical DMD can be effectively treated with a simple band-annuloplasty with good early and mid-term results. Here, we evaluate the long-term outcomes in terms of freedom from reoperation, recurrence of moderate or severe mitral regurgitation (MR) and overall survival. Between April 2006 and December 2020, patients with DMD causing severe MR and the echocardiographic features of symmetrical bileaflet prolapse, central regurgitant jet(s), annular dilation and no chordal ruptures were treated using a simple annuloplasty with a semi-rigid band. These patients were prospectively collected and retrospectively analysed. Seventy-five patients were enrolled. The mean clinical follow-up time was 104 [standard deviation (SD): 43] months, and echocardiographic follow-up time was 95 (SD: 43) months. The mean age was 54 (SD: 15) years, and 56% were females. Long-term overall survival was 98.2% [standard error (SE): 1.8], 93.7% (SE: 4.7) and 93.7% (SE: 4.7) at 4, 8 and 12 years, respectively. The freedom from reoperation was 100% at 4 and 8 years and 94.1% (SE: 5.7) at 12 years. The freedom from recurrent moderate or severe MR was 98.3% (SE: 1.7), 98.3% (SE: 1.7) and 92.8% (SE: 5.5) at 4, 8 and 12 years, respectively. Mitral repair with the simple band-annuloplasty for the treatment of MR due to symmetrical DMD seems to be stable and effective in the long term.

Mapping of the myxomatous mitral valve: The three-dimensional extension of mitral annular disjunction in surgically repaired mitral prolapse.

This study aimed to describe the heterogeneous extension of mitral annular disjunction (MAD) and assess the hypotesis that different phenotypes of disjunction are not associated with increased surgical challenges. Mitral regurgitation (MR) is the most common end-stage scenario of degenerative mitral valve disease (DMVD). Few data exist on the three-dimensional extension and geometry of MAD, as well as for its role in valvular dynamic and coaptation. A total of 85 consecutive subjects, who underwent elective mitral valve repair (MVR) for MMVD at our Institution between November 2019 and October 2021, were studied retrospectively. The extension and geometry of MAD was assessed using the digitally stored volumetric datasets of real-time 3D transesophageal echocardiography (TEE). Annular phenotypes and surgical repair techniques were analyzed. Mitral annular disjunction was diagnosed in 50 out of 85 patients (59%) with Barlow disease (BD). A detailed analysis of MAD extension was conducted on 33 patients. Two pattern of disjunction were identified: a bimodal shape was highlighted in 21 patients, while a more uniform distribution of the disjuncted annulus was observed in 12 patients. The bimodal pattern was characterized by lower disjunction distance (DD) at the 140°-220° arch (3.6 ± 2.2 mm), while a more regular DD was measured in the remaining patients. All patients successfully underwent MVR. Triangular leaflet resection was performed in 58% of the cases, neochordae implantation in 9%, and notably a 27% received an isolated annuloplasty. Rather than a binary feature, MAD should be taken into account in its complex and heterogeneous morphology, where two major phenotypes can be identified. Despite its anatomical complexity, MAD was not associated with an increased surgical challenge; conversely a peculiar subgroup of patient was successfully treated with an isolated annuloplasty.