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As the general population ages, the incidence of degenerative mitral stenosis (MS) among patients has increased. Percutaneous mitral valvuloplasty (PMV) has emerged as a well-established option for mitral rheumatic stenosis with specific characteristics. However, a blank therapeutic space must be filled with the treatment options for degenerative or rheumatic mitral stenosis in patients with many comorbidities and contraindication for valvuloplasty. We here present a comprehensive overview of the current possibilities, despite their scarce success. That is the reason why we propose a case series to facilitate a better understanding of our innovative technique in this challenging clinical context.

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We report a 73-year-old female who underwent mitral valve replacement for degenerative mitral stenosis (DMS) and aortic valve replacement for aortic valve stenosis. She was transferred to our hospital because of congestive heart failure. Transthoracic echocardiogram demonstrated severe mitral valve stenosis and aortic valve stenosis. Transesophageal echocardiogram (TEE) revealed severe mitral annular calcification (MAC) and calcification of the anterior mitral leaflet without commissure fusion. The diagnosis of DMS associated with MAC and aortic valve stenosis was made. Since she did not have other significant comorbidities except diabetes mellitus and hypertension, open-heart surgery for double valve replacement was scheduled by our heart team. Complete resection of the calcium bar and annulus reconstruction with an autologous pericardium allowed safe mitral valve replacement with a mechanical valve. Concomitant aortic valve replacement with a mechanical valve was carried out for aortic valve stenosis. Intraoperative TEE demonstrated good left ventricular function without perivalvular leakage in both mitral and aortic prosthetic valves. The postoperative course was uneventful, and the patient was discharged from the hospital. Surgical intervention may be one of the alternative treatments for elderly patients with degenerative mitral stenosis and MAC. Learning objective: Degenerative mitral stenosis (DMS) associated with mitral annular calcification (MAC) is a severe heart valve disease in the elderly population in developed countries. The prognosis of patients with severe DMS is poor, and open-heart surgery for elderly patients with MAC is especially challenging from a surgical point of view. We report a surgical treatment for a patient with DMS and aortic valve stenosis considering the patient's comorbidities and extent of MAC.

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PURPOSE: Degenerative mitral stenosis (DMS) is associated with a poor prognosis. Although mean transmitral gradient (TMG) has shown a good correlation with outcome, little is known about the association between other echocardiographic parameters and prognosis in patients with DMS. The current study aimed to evaluate the prognostic value of left atrial volume index (LAVI) in patients with DMS. METHODS: A total of 155 patients with DMS (72[63-80] years, 67% female) were included. The population was divided according to LAVI: normal-sized LAVI (LAVI ≤ 34 ml/m2); and enlarged LAVI (> 34 ml/m2). RESULTS: Patients with enlarged LAVI had a higher left ventricular mass index (120[96-146] vs. 91[70-112] g/m2 p < 0.001), as well as a higher prevalence of significant mitral regurgitation and severe aortic stenosis (23% vs. 10% p = 0.046 and 38% vs. 15% p=0.001, respectively) compared to patients with normal-sized LAVI. During a median follow-up of 25 months, 56 (36%) patients died. Patients with enlarged LAVI had worse prognosis compared to patients with normal-sized LAVI (p = 0.026). In multivariable Cox regression model, an enlarged LAVI was independently associated with all-cause mortality (HR 2.009, 95% CI 1.040 to 3.880, P = 0.038). CONCLUSION: An enlarged LAVI (> 34 ml/m2) is significantly associated with excess mortality in patients with DMS. After adjusting for potential confounders, an enlarged LAVI was the only parameter that remained independently associated with prognosis.

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Background: Atrial fibrillation (AF) is associated with an increased risk of stroke and the combination of AF and mitral stenosis (MS) is associated with a higher risk. In developed nations, degenerative mitral stenosis (DMS) constitutes a sizeable proportion of patients with MS. Current international guidelines do not offer recommendations regarding anticoagulation in these patients. The objective of this study was to describe the incidence of stroke or systemic embolism in patients with DMS with or without prevalent AF. Methods: A cohort study of DMS patients from 1997 to 2018, using data from the Danish health registries. The cohort was stratified based on AF prevalence and prior ischemic stroke. The primary outcome was a diagnosis of ischemic stroke or systemic embolism after 1 year of follow-up from time of DMS diagnosis. Results: The study included 1162 patients with DMS, of which 421 had prevalent AF. The incidence rate of stroke or systemic embolism after 1 year of follow-up was highest in the DMS without AF group (7.58 vs. 6.63 per 100 person-years). In both groups, DMS without AF and DMS with AF, the incidence rate was highest in patients with prior thromboembolic events (29.61 vs. 5.15 and 19.53 vs. 5.15, respectively). Conclusions: The incidence rate of stroke or systemic embolism was highest in DMS patients without AF. Current Danish guidelines recommend DMS patients should be treated with anticoagulation only with concurrent AF, yet our results call for additional research to establish if DMS patients without AF could benefit from stroke prevention therapy.

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BACKGROUND: In our institute, the causes of mitral stenosis (MS) are generally categorized into three main etiologies-rheumatic MS (RMS), degenerative MS with annular and leaflet calcification, and post-clip MS as a consequence of transcatheter mitral valve repair with clips for treating mitral regurgitation. However, clinical differences among the three etiologies are uncertain. METHODS: We retrospectively assessed 293 consecutive patients (53 with RMS, 118 with degenerative MS, and 122 with post-clip MS) who had a three-dimensional (3D) transesophageal echocardiography (TEE) derived mitral valve orifice area (MVA) of ≤1.5 cm2 , and a mean transmitral pressure gradient of ≥5 mmHg on transthoracic echocardiography. RESULTS: Although there was no difference in 3D-TEE-derived MVA among the three groups, patients with post-clip MS had a significantly lower mean transmitral pressure gradient compared to those with either of the other two types of MS (10.8 ([7.9-15.2] mmHg vs. 9.6 [7.3-12.5] mmHg vs. 6.9 [6.0-9.2] mmHg; p < .001). Patients with RMS had a higher prevalence of dyspnea. The independent determinants of dyspnea were pressure half time in RMS, 3D-TEE-derived MVA and estimated right atrial pressure in degenerative MS, and left ventricle ejection fraction in post-clip MS. CONCLUSIONS: Patients with post-clip MS had the lowest mean transmitral pressure gradient, and patients with RMS had the highest prevalence of dyspnea, despite having a similar 3D-TEE-derived MVA. The determinants of dyspnea were different among the three etiologies of MS.

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Determining the severity of stenosis in degenerative mitral stenosis (DMS) is fraught with challenges. Neither a high trans-mitral gradient nor a small valve area calculation is sufficiently diagnostic for DMS due to variable left atrial and left ventricular compliance in the setting of diastolic dysfunction, and the variable flow seen in patients with chronic kidney disease (i.e., high flow state) and elderly women (low flow state). Three-dimensional measurement of mitral valve area may be underestimated due to shadowing from basal calcium, and mitral valve annulus (MVA) by continuity equation (CEQ) or dimensionless mitral valve index can be erroneous in the presence of significant regurgitation of left-sided valves. The proposed dimensionless mitral stenosis index (DMSI) can be an easy echocardiographic tool to use in daily practice but needs further validation and is limited in the setting of significant regurgitation of left sided valves. Mean trans-mitral gradients > 8 mmHg and pulmonary artery pressure > 50 mmHg are independent predictors of mortality in those with MVA < 1.5 cm 2 derived by CEQ. In patients who have symptoms that are out of proportion to the degree of stenosis reported, exercise stress testing may help determine the physiologic effects of the stenotic valve. A combination of MVA by CEQ or DMSI and mean transmitral gradient at a given left ventricle stroke volume (flow) should be evaluated in larger studies.

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As the life expectancy of the population continues to increase, mitral annular calcification has emerged as an important cause of mitral stenosis (MS), commonly referred to as calcific or degenerative MS. Mitral annular calcification results in valvular stenosis when calcification extends into the base of the mitral leaflet(s) and displaces the mitral valve hinge point(s) into the left ventricular inlet. Echocardiographic determination of mitral vale area is fraught with difficulties and often precludes using planimetry or the Hatle formula. Given the numerous confounders that affect transmitral flow in calcific MS, evaluation of lesion severity should incorporate flow-independent methods such as the continuity equation and the mitral valve dimensionless index. In light of the significant risks entailed, there is little enthusiasm for mitral valve replacement in patients with calcific MS. Transcatheter mitral valve replacement is generally offered on a compassionate use basis to patients deemed to be at high surgical risk.

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PURPOSE: Degenerative mitral stenosis (DMS) is an increasingly recognized cause of mitral stenosis. The goal of this study was to compare echocardiographic differences between DMS and rheumatic mitral stenosis (RMS), identify echocardiographic variables reflective of DMS severity, and propose a dimensionless mitral stenosis index (DMSI) for assessment of DMS severity. METHODS: This is a single-center, retrospective cohort study. We included patients with at least mild MS and a mean transmitral pressure gradient (TMPG) ≥4 mm Hg. Mitral valve area by the continuity equation (MVACEQ ) was used as an independent reference. The DMSI was calculated as follows: DMSI = VTILVOT / VTIMV. All-cause mortality data were collected retrospectively. RESULTS: A total of 64 patients with DMS and 24 patients with RMS were identified. MVACEQ was larger in patients with DMS (1.43 ± 0.4 cm2 ) than RMS (0.9 ± 0.3 cm2 ) by ~0.5 cm2 (P = <.001), and mean TMPG was lower in the DMS group (6.0 ± 2 vs 7.9 ± 3 mm Hg, P = .003). A DMSI of ≤0.50 and ≤0.351 was associated with MVACEQ ≤1.5 and MVACEQ ≤1.0 cm2 (P < .001), respectively. With the progression of DMS from severe to very severe, there was a significant drop in DMSI. There was a nonsignificant trend toward worse survival in patients with MVACEQ ≤1.0 cm2 and DMSI ≤0.35, suggesting severe stenosis severity. CONCLUSION: Our results show that TMPG correlates poorly with MVA in patients with DMS. Proposed DMSI may serve as a simple echocardiographic indicator of hemodynamically significant DMS.

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Mitral stenosis (MS) is a common valvular disease characterized by narrowing of the mitral valve orifice and a reduction in mitral valve area (MVA). While rheumatic MS (RMS) is frequently encountered in young individuals in developing countries, degenerative MS (DMS) is seen in the elderly in developed countries and its prevalence is increasing. DMS is usually a late presentation of mitral annular calcification (MAC). Accurate assessment of MVA in patients with MAC is challenging due to the alterations in the atrial and valvular structures as well as the presence of other comorbidities in this aging population. We will review the epidemiology, etiology, pathophysiology, diagnostic assessment, and management of DMS and compare the findings with RMS. The latest therapeutic approaches, including medical, surgical, and transcatheter valvular interventions, will be discussed.

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PURPOSE OF REVIEW: Mitral annular calcification (MAC) and associated calcific mitral stenosis (MS) are frequent in the aging population, although optimal management remains debated and outcomes are poor. This article summarizes challenges in the diagnosis and therapy of calcific MS, the indications for valve intervention, procedural concerns, and emerging treatment options. RECENT FINDINGS: Surgical mitral valve replacement is the procedure of choice in symptomatic patients at acceptable surgical risk, with transcatheter mitral valve replacement (TMVR) being evaluated in clinical trials as an alternative for patients at prohibitive surgical risk. Significant challenges exist with the currently available technology and outcomes have been suboptimal. Optimizing the patient-selection process by using multimodality imaging tools has proven to be essential. MAC and calcific MS is an increasingly prevalent, challenging issue with poor outcomes. While surgical valve replacement can be performed in patients with acceptable surgical risk, TMVR can be considered for patients at higher risk. Clinical trials are underway to optimize outcomes. Dedicated device designs and techniques to minimize risk of left ventricular outflow tract obstruction, paravalvular leakage, and device embolization are to be awaited.

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Transcatheter mitral valve replacement (TMVR) is a novel approach for treatment of severe mitral regurgitation. A number of TMVR devices are currently undergoing feasibility trials using both transseptal and transapical routes for device delivery. Overall experience worldwide is limited to fewer than 200 cases. At present, the 30-day mortality exceeds 30% and is attributable to both patient- and device-related factors. TMVR has been successfully used to treat patients with degenerative mitral stenosis (DMS) as well as failed mitral bioprosthesis and mitral repair using transcatheter mitral valve-in-valve (TMViV)/valve-in-ring (ViR) repair. These patients are currently treated with devices designed for transcatheter aortic valve replacement. Multicenter registries have been initiated to collect outcomes data on patients currently undergoing TMViV/ViR and TMVR for DMS and have confirmed the feasibility of TMVR in these patients. However, the high periprocedural and 30-day event rates underscore the need for further improvements in device design and multicenter randomized studies to delineate the role of these technologies in patients with mitral valve disease.

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