Mitral Annulus Calcium Score in Patients With Calcific Mitral Stenosis Undergoing Invasive Hemodynamic Assessment.

BACKGROUND: Aortic valve calcium score is associated with hemodynamic severity of aortic stenosis. Whether this association is present in calcific mitral stenosis remains unknown. METHODS AND RESULTS: This study was a retrospective analysis of consecutive patients with mitral stenosis secondary to mitral annular calcification (MAC) undergoing transseptal catheterization. All patients underwent invasive mitral valve assessment via direct left atrial and left ventricular pressure measurement. Computed tomography within 1 year of cardiac catheterization and with adequate visualization of the mitral annulus was included. MAC calcium score quantification by Agatston method was obtained offline using dedicated software (Aquarius, TeraRecon, V.4). Median patient age was 66.9±11.2 years, 47% of patients were women, 50% had coronary artery disease, 40% had atrial fibrillation, 47% had prior cardiac surgery, and 33% had prior chest radiation. Median diastolic mitral valve gradient was 9.4±3.4 mm Hg on echocardiography and 8.5±4 mm Hg invasively. Invasive median mitral valve area using the Gorlin formula was 1.87±0.9 cm2. Median MAC calcium score for the cohort was 7280±7937 Hounsfield units. MAC calcium score correlated with the presence of atrial fibrillation (P=0.02) but was not associated with other comorbidities. There was no correlation between MAC calcium score and mitral valve area (r=0.07; P=0.6) or mitral valve gradient (r=-0.03; P=0.8). CONCLUSIONS: MAC calcium score did not correlate with invasively measured mitral valve gradient and mitral valve area in patients with MAC-related mitral stenosis, suggesting that calcium score should not be used as a surrogate for invasive hemodynamic parameters.

Hemodynamic implications of mitral annular calcification in patients undergoing transcatheter aortic valve implantation for severe aortic stenosis.

PURPOSES: Predicting hemodynamic changes of stenotic mitral valve (MV) lesions with mitral annular calcification (MAC) following transcatheter aortic valve implantation (TAVI) may inform clinical decision-making. This study aimed to investigate the association between the MAC severity quantified by computed tomography (CT) and changes in mean transmitral gradient (mTMG), mitral valve area (MVA) and stroke volume index (SVi) following TAVI. METHODS AND RESULTS: A total of 708 patients (median age 81, 52% male) with severe aortic stenosis (AS) underwent pre-procedural CT and pre- and post-TAVI transthoracic echocardiography. According to the classification of MAC severity determined by CT, 299 (42.2%) patients had no MAC, 229 (32.3%) mild MAC, 102 (14.4%) moderate MAC, and 78 (11.0%) severe MAC. After adjusting for age and sex, there was no significant change in mTMG following TAVI (Δ mTMG = 0.07 mmHg, 95% CI -0.10 to 0.23, P = 0.92) for patients with no MAC. In contrast, patients with mild MAC (Δ mTMG = 0.21 mmHg, 95% CI 0.01 to 0.40, P = 0.018), moderate MAC (Δ mTMG = 0.31 mmHg, 95% CI 0.02 to 0.60, P = 0.019) and severe MAC (Δ mTMG = 0.43 mmHg, 95% CI 0.10 to 0.76, P = 0.0012) had significant increases in mTMG following TAVI, with greater changes associated with increasing MAC severity. In contrast, there was no significant change in MVA or SVi following TAVI. CONCLUSION: In patients with severe AS undergoing TAVI, MAC severity was associated with greater increases in post-procedural mTMG whereas MVA or SVi remained unchanged. MAC severity should be considered for potential subsequent MV interventions if TAVI does not improve symptoms.

Predicting stroke and mortality in mitral stenosis with atrial flutter: A machine learning approach.

BACKGROUND: Our study hypothesized that an intelligent gradient boosting machine (GBM) model can predict cerebrovascular events and all-cause mortality in mitral stenosis (MS) with atrial flutter (AFL) by recognizing comorbidities, electrocardiographic and echocardiographic parameters. METHODS: The machine learning model was used as a statistical analyzer in recognizing the key risk factors and high-risk features with either outcome of cerebrovascular events or mortality. RESULTS: A total of 2184 patients with their chart data and imaging studies were included and the GBM analysis demonstrated mitral valve area (MVA), right ventricular systolic pressure, pulmonary artery pressure (PAP), left ventricular ejection fraction (LVEF), New York Heart Association (NYHA) class, and surgery as the most significant predictors of transient ischemic attack (TIA/stroke). MVA, PAP, LVEF, creatinine, hemoglobin, and diastolic blood pressure were predictors for all-cause mortality. CONCLUSION: The GBM model assimilates clinical data from all diagnostic modalities and significantly improves risk prediction performance and identification of key variables for the outcome of MS with AFL.

One-year outcome of transcatheter aortic valve replacement for bicuspid aortic valve stenosis: a report from the Japanese Nationwide registry (J-TVT registry).

The outcome of transcatheter aortic valve replacement (TAVR) for patients with bicuspid aortic valve (BAV) remains unclear, particularly among Asian patients that are known to have different valvular morphology and lower body habitus. This study investigated patient characteristics, procedural and 1-year outcome of TAVR for BAV within national TAVR registry in Japan. The patient-level data were extracted from the J-TVT (Japanese Transcatheter Valvular Therapy) registry between August 2013 and December 2018; overall, there were 423 patients (2.5%) with BAV and 16,802 patients with tricuspid aortic valve (TAV). At baseline, patients with BAV were younger and had less arteriosclerotic comorbidities. There was no statistically significant difference between BAV and TAV groups in conversion to surgery (0.5% vs. 1.1%, p = 0.34) and 30-day mortality (0.5% vs. 1.3%, p = 0.18). Cumulative all-cause survival and survival from major adverse events were analyzed. Cox proportional hazard regression model was used to estimate the hazard ratio. All-cause mortality and major adverse event rate at 1 year were comparable between the two groups. Relative hazard for all-cause mortality for BAV compared to TAV was 1.01 (0.70-1.45; p = 0.96), and for major adverse event was 0.94 (0.69-1.27; p = 0.67). From the Japanese nationwide TAVR registry, procedural and 1-year outcome of TAVR in BAV was as favorable as TAVR in TAV.

What Is the Optimal Mitral Valve Repair for Isolated Posterior Leaflet Prolapse to Achieve Long-Term Durability?

Background This study assessed risk factors for mitral regurgitation (MR) recurrence or functional mitral stenosis during long-term follow-up in patients undergoing mitral valve repair for isolated posterior mitral leaflet prolapse. Methods and Results We assessed a consecutive series of 511 patients who underwent primary mitral valve repair for isolated posterior leaflet prolapse between 2001 and 2021. Annuloplasty using a partial band was selected in 86.3%. The leaflet resection technique was used in 83.0%, whereas the chordal replacement without resection was used in 14.5%. Risk factors were analyzed for MR recurrence ≥grade 2 or functional mitral stenosis with mean transmitral pressure gradient ≥5 mm Hg using a multivariable Fine-Gray regression model. The 1-, 5-, and 10-year cumulative incidence of MR ≥grade 2 was 7.8%, 22.7%, and 30.1%, respectively, whereas that of mean transmitral pressure gradient ≥5 mm Hg was 8.1%, 20.6%, and 29.3%, respectively. Risk factors for MR ≥grade 2 included chordal replacement without resection (hazard ratio [HR], 2.50, P<0.001) and larger prosthesis size (HR, 1.13, P=0.023), whereas factors for functional mitral stenosis were use of a full ring (partial band versus full ring, HR, 0.53, P=0.013), smaller prosthesis size (HR, 0.74, P<0.001), and larger body surface area (HR, 3.03, P=0.045). Both MR ≥grade 2 and mean transmitral pressure gradient ≥5 mm Hg at 1 year post surgery were significantly associated with the long-term incidence of reoperation. Conclusions Leaflet resection with a large partial band may be an optimal strategy for isolated posterior mitral valve prolapse.

A rare coexistence: Hammock mitral valve and aortopulmonary window.

Congenital mitral stenosis is a broad-spectrum pathology in which blood flow to the left ventricle is obstructed both functionally and anatomically. Hammock mitral valve, also known as anomalous mitral arcade, is a rare congenital anomaly particularly in infants and children. Hammock mitral valve may not be suitable for repair regarding the advanced dysplastic mitral valve structure. Aortopulmonary window is an unusual cardiac anomaly which is defined as a communication between the main pulmonary artery and the ascending aorta. As a result of the excessive left-to-right shunt, early intervention and surgical closure deemed mandatory to avoid development of severe pulmonary hypertension and its consequences. All patients with an aortopulmonary window necessitates prompt repair immediately. In this brief report, mitral valve replacement with a mechanical valve and repair of aortopulmonary window with a Dacron patch were performed simultaneously in a 5-month-old patient with a hammock mitral valve and accompanying aortopulmonary window.

Surgical treatment of caseous calcification of the mitral annulus causing mitral stenosis and mimicking the clinical picture of infective endocarditis: A case report.

Among masses involving the mitral valve and annulus, caseous calcification of the mitral annulus (CCMA) is a rare disease. CCMA accounts for .63% of all mitral annular calcification (MAC) cases. The pathophysiology is still unknown. The correct diagnosis and treatment of this disease is very important to prevent complications. We present a case of giant CCMA with advanced mitral stenosis and hypertrophic cardiomyopathy, presenting with symptoms of infection and therefore a preliminary diagnosis of infective endocarditis. Because of these features, we wanted to share our case as it is the 1st case in the literature.

Concomitant transcatheter transfemoral double native valve replacement for severe aortic stenosis and severe mitral stenosis with mitral annular calcification.

Concomitant mitral and aortic valve stenosis in a patient with mitral annular calcification and porcelain aorta poses a unique problem to the surgical team. Transcatheter aortic and mitral valve replacements in native valves offer a viable option for such selected group of patients. We present the case of a 54-year-old male who presented with severe aortic stenosis (AS) and severe mitral stenosis (MS) but was deemed high risk for surgery owing to intense calcification of the aorta and mitral annular calcification, and successfully underwent transcatheter double native valve replacement.

Mitral Valve Replacement for Mitral Stenosis Secondary to Massive Mitral Annular Calcium.

Mitral annular calcium (MAC) may produce mitral stenosis (MS) if its quantity is massive. We define massive MAC as the presence of a huge quantity of calcium underlying the posterior mitral leaflet and extending across all or nearly all of the ventricular aspect of the anterior mitral leaflet. This report was prompted to emphasize the hazards of performing mitral valve replacement in patients with MS secondary to massive MAC. The clinical data and morphology of the operatively excised mitral valves from the 11 patients who had mitral valve replacement for MS secondary to massive MAC are described. Of the 11 patients, 6 died postoperatively, 5 of whom had 4+/4+ MAC. The high mortality in these patients suggests that the decision to perform mitral valve replacement needs to be carefully considered if the quantity of MAC is massive.

Use of Mechanical Circulatory Support to Demonstrate Pulmonary Hypertension Reversibility in Mitral Stenosis.

A 56 year old female with a history of rheumatic mitral stenosis (MS) presented with severe pulmonary edema. Transthoracic echocardiogram demonstrated severe MS (mean valve area 0.5 cm 2 , mean gradient of 16 mm Hg) with preserved left ventricular ejection fraction. Right heart catheterization demonstrated elevated pulmonary artery (PA) pressures of 110/80 mm Hg and a wedge pressure of 40 mm Hg. Mechanical circulatory support (MCS) was initiated with extracorporeal left atrial to femoral artery bypass. MCS allowed preoperative unloading of the left atrium. The volume status and lung congestion were optimized before surgery. In addition, pulmonary hypertension reversibility was demonstrated with significantly lower PA pressures after initiation of MCS. Intraoperatively, the MCS left atrial inflow cannula was pulled back into the right heart and used as a venous cannula for cardiopulmonary bypass. Successful mitral valve replacement was performed. Postoperatively, the mitral valve mean gradient was 3 mm Hg.

Management of Rheumatic Mitral Stenosis With Annular Calcification During HeartMate 3 Implantation.

There is a lack of data-driven consensus on the treatment of mitral stenosis at the time of left ventricular assist device implantation. The presence of severe mitral annular calcification further complicates mitral valve intervention. This case report presents a 72-year-old woman with severe mitral stenosis and severe annular calcification with end-stage ischemic cardiomyopathy who underwent HeartMate 3 (Abbott Cardiovascular) implantation. The mitral valve pathology was successfully managed with concomitant open balloon valvuloplasty and surgical commissurotomy on a fibrillating heart without aortic cross-clamp. This approach avoided the need for mitral valve replacement and the potential risks associated with annular decalcification and reconstruction. Longer follow-up is needed to determine its effectiveness over time.

Lutembacher syndrome presenting as heart failure in a young Nigerian at Obafemi Awolowo University Teaching Hospitals Complex (OAUTHC), Ile-Ife: a case report.

Lutembacher syndrome (LS) is a rare syndrome comprising a combination of atrial septal defect (ASD) and mitral stenosis. We present the case of a 28-year-old man, who presented with progressively worsening dyspnea of 2 months associated with orthopnea, paroxysmal nocturnal dyspnea, bilateral leg swelling and productive cough. Chest X-ray revealed plethoric lung fields with prominent pulmonary conus and cardiomegaly. Transthoracic echocardiography revealed a large ostium secundum ASD with left to right shunt, mild mitral stenosis, severe mitral and tricuspid regurgitations and pulmonary hypertension. A diagnosis of Lutembacher syndrome in heart failure with pulmonary hypertension was made. The patient was managed conservatively, but declined surgery primarily because of financial reasons. This rare case of LS presenting with heart failure and complicated by pulmonary hypertension is the first reported case in our centre and our region. The patient's inability to afford the cost of definitive care posed a significant problem in his management.

Prevalence and Natural History of Mitral Annulus Calcification and Related Valve Dysfunction.

OBJECTIVE: To evaluate the prevalence and natural history of mitral annulus calcification (MAC) and associated mitral valve dysfunction (MVD) in patients undergoing clinically indicated echocardiography. METHODS: A retrospective review was conducted of all adults who underwent echocardiography in 2015. Mitral valve dysfunction was defined as mitral regurgitation or mitral stenosis (MS) of moderate or greater severity. All-cause mortality during 3.0 (0.4 to 4.2) years of follow-up was compared between groups stratified according to the presence of MAC or MVD. RESULTS: Of 24,414 evaluated patients, 5502 (23%) had MAC. Patients with MAC were older (75±10 years vs 60±16 years; P<.001) and more frequently had MVD (MS: 6.6% vs 0.5% [P<.001]; mitral regurgitation without MS: 9.5% vs 6.1% [P<.001]). Associated with MS in patients with MAC were aortic valve dysfunction, female sex, chest irradiation, renal dysfunction, and coronary artery disease. Kaplan-Meier 1-year survival was 76% in MAC+/MVD+, 87% in MAC+/MVD-, 86% in MAC-/MVD+, and 92% in MAC-/MVD-. Adjusted for age, diabetes, renal dysfunction, cancer, chest irradiation, ejection fraction below 50%, aortic stenosis, tricuspid regurgitation, and pulmonary hypertension, MAC was associated with higher mortality during follow-up (adjusted hazard ratio, 1.40; 95% CI, 1.31 to 1.49; P<.001); MVD was associated with even higher mortality in patients with MAC (adjusted hazard ratio, 1.79; 95% CI, 1.58 to 2.01; P<.001). There was no significant interaction between MAC and MVD for mortality (P=.10). CONCLUSION: In a large cohort of adults undergoing echocardiography, the prevalence of MAC was 23%. Mitral valve dysfunction was more than twice as prevalent in patients with MAC. Adjusted mortality was increased in patients with MAC and worse with both MAC and MVD.

Invasive Hemodynamic Predictors of Survival in Patients With Mitral Stenosis Secondary to Mitral Annular Calcification.

Background The aim of this study was to establish prognostic hemodynamic parameters in patients with mitral stenosis secondary to mitral annular calcification. Methods and Results A retrospective cohort of 105 patients undergoing transseptal catheterization for hemodynamic evaluation of mitral annular calcification-related mitral stenosis between 2004 and 2020 was studied. Mitral valve gradient (MVG) and mitral valve area (MVA; calculated by the Gorlin formula) were measured using direct left atrial and left ventricular pressures. The median age of the patients was 70.3 years (58.4-76.7 years), and 53.3% were women. The median MVA was 1.7 cm2 (1.3-2.3 cm2) and MVG was 7.3 mm Hg (5.3-10.3 mm Hg); left ventricular end-diastolic pressure was 17.6±28.3 mm Hg. During a median of 2.1 years (0.7-4.5 years), there were 63 deaths; 1- and 5-year survival were 76% and 40%, respectively. There was no association between left ventricular end-diastolic pressure and survival. After adjusting for age and comorbidities, both MVA (hazard ratio [HR], 0.50 per cm2; 95% CI, 0.34-0.73) and MVG (HR, 1.1 per mm Hg; 95% CI, 1.05-1.20) were independent predictors of death. Atrial fibrillation was also independently associated with mortality. When added to a combined model, MVA remained associated with death (HR, 0.51 per cm2; 95% CI, 0.33-0.79) while MVG was not. Conclusions In patients with mitral annular calcification-related mitral stenosis, survival was poor. MVA and MVG were independently associated with death, but MVA was a better predictor of outcomes.

Early and late results of mitral valve repair with anterior leaflet patch augmentation.

OBJECTIVES: The aim of this study was to determine the long-term results of mitral valve (MV) repair with anterior leaflet patch augmentation. METHODS: Between 2012 and 2015, 45 patients underwent MV repair using the anterior leaflet patch augmentation technique at our institution. The mean age of the patients was 65.9 ± 13.0 years (16 males). We reviewed the MV pathology and the surgical techniques used and assessed the early and late results. RESULTS: In terms of MV pathology, 43 patients (95.6%) had pure mitral regurgitation (MR) and 2 patients (4.4%) had mixed mitral stenosis and MR. Rheumatic changes were seen in 18 patients (40.0%). Postoperative echocardiography showed that 95.6% of patients had none to mild MR. During a median follow-up period of 5.5 years (range 0.1-8.3 years), there were 8 late deaths. Nine patients (20%) required reoperation. The mean interval between the initial operation and redo operation was 3.7 ± 3.1 years (range: 0.4-7.8 years). The causes of reoperation included patch dehiscence (n = 4), progression of mitral stenosis (n = 2), band dehiscence (n = 1), patch enlargement (n = 1) and unknown (n = 1). Eight patients underwent MV replacement and 1 underwent repeat MV repair. The freedom from reoperation at 3 and 5 years was 85.7 ± 6.7% and 81.2 ± 7.7%, respectively. CONCLUSIONS: Anterior leaflet patch augmentation can provide excellent early results in the majority of the patients even in the presence of rheumatic pathology; however, we observed late reoperation in 20% of patients. Thus, this technique should be used with caution and careful follow-up with serial echocardiography is essential.

Clinical Outcomes of Mitral Valve Disease With Mitral Annular Calcification.

The prevalence of mitral valve disease with mitral annular calcification (MAC) and its clinical outcomes remain uncertain. This study sought to evaluate the prevalence of significant mitral disease due to MAC, and the impact of intervention on the clinical outcomes in these patients. All patients who underwent transthoracic echocardiography (TTE) between January 2014 and December 2015 in our health care system were reviewed and identified for having MAC with significant mitral valve disease (i.e., either≥moderate mitral regurgitation (MR) or mitral stenosis (MS)). The primary endpoints of the study were all-cause mortality and a composite outcome of mortality or heart failure hospitalization at 3-year follow-up. Of 41,136 patients who underwent TTE, MAC was identified in 2,855 (6.9%) patients, including 434 (1.1% of total) patients who had significant MR and/or MS (median age [IQR], 80 [73 to 87] years; 63% women). MAC predominately involved the posterior annulus (95%), with the majority having calcification of both trigones (55%), the leaflets (71%), and circumferential involvement (67%). During 3-year follow-up, 59 (14%) patients underwent surgical or transcatheter MV intervention. Patients who did not undergo mitral intervention had higher all-cause mortality (HR 2.80, 95% CI 1.60 to 4.92; p <0.001) and a greater risk of the composite outcome (HR 1.43, 1.00 to 2.04; p = 0.05) than those treated. Survival at 3-year follow-up was markedly greater in those with mitral intervention (78% vs 50%; p <0.001). This survival benefit remained after multivariable adjustment. In conclusion, MAC affects approximately % of patients who undergo echocardiography. Those with significant mitral valve disease due to any degree of MAC have poor survival, which may be ameliorated with transcatheter or surgical intervention.

Anticoagulation in Atrial Fibrillation Associated with Mitral Stenosis.

Rheumatic valve disease is present in 0.4 % of the word population, mainly in lowincome countries. Rheumatic mitral stenosis affects more women and between 40 to 75 % of patients may have atrial fibrillation (AF), more frequently in upper-middle income countries. This rhythm disturbance is due to increased atrial pressure, chronic inflammation, fibrosis, and left atrial enlargement. There is also an increase in the prevalence of AF with age in patients with mitral stenosis. The risk of stroke is 4 % per year. Success rates for cardioversion, Cox-Maze procedure, and catheter ablation are low. Therefore, anticoagulation with vitamin K antagonist is mandatory for Evaluated Heart valves, Rheumatic or Artificial (EHRA) classification type 1. However, this anticoagulation is used by less than 80 % of those eligible and less than 30 % have the international normalized ratio in the therapeutic range. The safety and efficacy of using rivaroxaban, a direct factor Xa inhibitor anticoagulant, were demonstrated in the RIVER trial with a sample of 1005 patients with AF and bioprosthetic mitral valve. The indication for valve replacement, that is, if severe mitral stenosis or severe mitral regurgitation, was not specified. A randomized, open-label study (DAVID-MS) is underway to compare the effectiveness and safety of dabigatran and warfarin therapy for stroke prevention in patients with AF and moderate or severe mitral stenosis. Thus, the applicability of the use of direct anticoagulants in patients with AF and mitral stenosis and also in those undergoing mitral bioprostheses surgery will be the subject of further studies. The findings may explain if specific atrial changes of mitral stenosis even after the valve replacement will influence thromboembolic events with direct anticoagulants.

Bioprosthetic mitral valve stenosis secondary to hypereosinophilic syndrome.

Herein we present a case of hypereosinophilic syndrome (HES) with recurrent involvement of mitral valve. The patient developed mitral regurgitation secondary to HES. Surgical mitral valve replacement was performed successfully. The prosthetic valve dysfunction occurred 3 years later and echocardiography showed severe mitral valve stenosis. Extensive mural thrombi were discovered on both sides of the stenotic valve with eosinophilic infiltration. The patient underwent a repeated mechanical prosthesis replacement and recovered uneventfully.

Rapid progression of mitral stenosis after atrial septal defect closure in a Lutembacher's syndrome patient.

The combination of the atrial septal defect (ASD) and mitral stenosis (MS) is an unusual clinical entity called Lutembacher's syndrome. The hemodynamic interaction between the two cardiac malformations modifies the disease progression of each other. The symptom and progression of MS were thought to be slowed because of the existence of a left-to-right shunt that relived the blood flow through the mitral orifice. There is no consensus about caring this patient population for now. Here, we present a 58-year-old female with mild MS and coexistent ASD experiencing rapid progression of mitral valve lesions after percutaneous ASD closure. This case might identify the effect of ASD on delaying MS progression. From this point of view, MS and coexisting ASD should be evaluated and treated as a whole.