Mitral Annulus Calcification

Introduction Mitral annulus calcification (MAC) is a chronic, non-inflammatory, degenerative mechanism of the fibrous base of the mitral valve. While MAC was originally thought to be an age-related degenerative process, there is evidence that other mechanisms, such as atherosclerosis and abnormal calcium phosphorus metabolism, also contribute to the development of MAC. Areas covered This paper summarizes, existing perception of clinically valid definition of MAC and the pathophysiological processes that lead to the development of MAC and the diagnostic implications of this disease entity. Expert opinion Minimal evidence exists on the natural history and progression of MAC. Characterization of MAC progression and identification of predisposing risk factors can help to validate hypotheses. MAC is most commonly asymptomatic and incidental finding. Echocardiography is the primary imaging modality for identification and characterization of MAC and associated mitral valve (MV) disease. For patients with an indication for MV surgery, computed tomography (CT) is a complementary imaging modality for MAC. MAC is generally recognized by its characteristic density, location, and shape on echocardiography and CT, unusual variants are sometimes confused with other lesions.

Breaking the Mold: Lithotripsy for Mitral Annular Calcification.

Background: Mitral annular calcification (MAC) is a chronic inflammatory condition that is associated with future cardiovascular events. There is limited data that explores how MAC impacts outcomes in patients with pre-existing mitral regurgitation (MR). Methods: This is a retrospective analysis using the TriNetX US Collaborative Network database. A database of hospitalized patients ≥50 years was queried using ICD-10 and TriNetX curated codes. We identified those given a discharge diagnosis of MR between 2020-2023, excluding patients with rheumatic mitral valve disease or ESRD. We then stratified by presence/absence of a new diagnosis of MAC (occurring after MR diagnosis). Cohorts were balanced for socio-demographics, body mass index, and common comorbidities including hypertension, hyperlipidemia, diabetes, and tobacco use, with a standard difference of <0.1 considered to indicate adequate balancing. Hazard ratios were calculated for outcomes, with a P<0.05 considered significant. Results: Our query yielded 11,462 patients in the MAC group and 450,474 patients in the no MAC group. Following propensity matching, each group had 11,462 patients. All targeted variables were adequately balanced. Average age was similar in both the MAC and no MAC groups (76 years vs 75.8 years). The populations were majority white (80.3% vs 81.2%) and female (56.8% vs 57.0%). BMI was also similar (29.2 vs 28.7). Among patients with pre-existing MR, subsequently diagnosed MAC was associated with increased 1-year risk of hospitalization (HR 1.22, 95%CI 1.180-1.262, p<0.0001), ICU admission (HR 1.38, 95%CI 1.250-1.524, p<0.0001), ischemic heart disease (HR 1.446, 95%CI 1.289-1.622, p<0.0001), new onset HFpEF (HR 1.286, 95%CI 1.160-1.425, p<0.0001), cerebrovascular disease (HR 1.341, 95%CI 1.190-1.512, p<0.0001), prosthetic valve replacement (HR 2.126, 95%CI 1.786-2.530, p<0.0001), and mitral stenosis (HR 3.16, 95%CI 2.490-5.234, p<0.0001). There was not a significant difference between groups for HFrEF (HR 0.955, p=0.4377), cardiogenic shock (HR 1.188, p=0.1098), or cardiac arrest (HR 1.155, p=0.1979). Conclusions: In patients with previously diagnosed MR, a subsequent diagnosis of MAC was associated with increased 1-year risk of hospitalization, new onset ischemic heart disease or HFpEF, and a variety of other adverse cardiovascular outcomes.

Minimally invasive treatment of mitral valve disease with severe mitral annular calcification: Meeting paper for Mitral Conclave 2022.

Background There is a paucity of data about the prevalence and natural history of mitral annular calcification (MAC). Purposes In a large cohort of patients undergoing clinically-indicated echocardiography, we aimed to evaluate the prevalence and the natural history of MAC, and the frequency of associated mitral valve dysfunction (MVD). Methods Retrospective review of all patients who underwent echocardiographic evaluation at our institution between January 1st and December 31st 2015. The patients were stratified into 3 groups: MAC with MVD, MAC without MVD, no MAC. MVD was defined as moderate or greater mitral regurgitation (MR) or stenosis (MS). The 3 groups were compared for baseline clinical and echocardiographic characteristics and all-cause mortality during follow-up. Results Of 24,415 evaluated patients, 5478 (22%) had MAC. Patients with MAC were older (75±10 vs. 60±16 years, p<0.01) and more often female (46% vs. 43%, p<0.01). MVD was more frequently observed in patients with MAC (16% vs. 7%, p<0.01); moderate or greater MR was found in 10% of patients with MAC vs. 6% without MAC (p<0.01) and MS in 7% vs. 0.5% (p<0.01). In patients with MAC, those with MVD were more often female and more frequently had creatinine ≥2mg/dl and moderate or greater aortic stenosis (AS) than those without MVD (Fig. A). Kaplan-Meier survivals at 1 year were 75% in patients with MAC and MVD, 87% in those with MAC without MVD, and 92% in those without MAC (Fig B). Adjusted for age, sex, diabetes, creatinine ≥2 mg/dl, coronary artery disease, left ventricular ejection fraction <50%, and AS, MAC was associated with higher all-cause mortality (adjusted hazard ratio1.4, 95% confidence interval: 1.3–1.5, p<0.01); combined MAC and MVD was associated with the highest mortality during follow-up of 2.5±1.8 years. Conclusion In a large cohort of patients undergoing echocardiographic evaluation, the prevalence of MAC was 22%. MV dysfunction was more than twice as prevalent in patients with MAC as without. Patients with MAC had higher all-cause mortality than patients without MAC. Among patients with MAC and mitral valvular dysfunction, adjusted mortality was two-fold higher compared to those without MAC. Funding Acknowledgement Type of funding source: None

To compare all-cause mortality in patients with mitral annulus calcification (MAC) and severe mitral valve dysfunction (MVD) who received standard mitral intervention versus no intervention. Patients with MAC often have high surgical risk due to advanced age, comorbidities, and technical challenges related to calcium. The impact of a mitral intervention on outcomes of patients with MAC and severe MVD is not well known. Retrospective review of patients with MAC by transthoracic echocardiography (TTE) in 2015 at a single institution. Patients with severe mitral stenosis (MS) or regurgitation (MR) were analyzed and stratified into two groups: surgical or transcatheter intervention performed <1 year after the index TTE, and no or later intervention. The primary endpoint was all-cause mortality. Of 5502 patients with MAC, 357 had severe MVD (MS = 27%, MR = 73%). Of those, 108 underwent mitral intervention (surgery = 87; transcatheter = 21). They were younger (73 ± 11 vs. 76 ± 11 years, p < 0.01) and less frequently had cardiovascular diseases compared with no-intervention. Frequency in women was similar (45% vs. 50%, p = 0.44). During median follow-up of 3.2 years, the intervention group had higher estimated survival than those without intervention (80% vs. 72% at 1 year and 55% vs. 35% at 4 year, p < 0.01). Adjusted for age, eGFR, LVEF < 50%, and pulmonary hypertension, mitral intervention was an independent predictor of lower mortality (hazard ratio = 0.66, 95% confidence interval 0.43-0.99, p = 0.046). Patients with MAC and severe MVD who underwent mitral intervention <1 year from index TTE had lower mortality than those without intervention. Mitral intervention was independently associated with lower mortality.

Transcatheter mitral valve intervention (TMVI) has emerged as a potential alternative to surgery for patients with mitral annular calcification (MAC)-associated mitral valve disease, a population often deemed high-risk due to clinical and anatomic complexities. However, MAC also presents significant challenges to TMVI itself. In transcatheter edge-to-edge repair (TEER), procedural success may be limited by fibrotic and short leaflets, reduced mitral valve area, and subvalvular calcium protrusion. Transcatheter mitral valve replacement (TMVR) poses further anatomical hurdles, including a small, eccentric, or non-conforming annuli; insufficient MAC to ensure anchoring; narrow aortomitral angle; septal hypertrophy; elongated anterior mitral leaflet predisposing to left ventricular outflow tract (LVOT) obstruction; and heterogeneous MAC morphology leading to paravalvular leak or suboptimal fixation. Mechanical injury to adjacent structures such as the circumflex artery and atrioventricular groove is also a concern. Balloon-expandable valves, used off-label for TMVR, may be delivered via transapical, transfemoral, transseptal, or hybrid transatrial approaches. While early data show high mortality for valve-in-MAC (ViMAC) procedures, a shift toward transfemoral transseptal access has improved outcomes. The hybrid transatrial approach offers advantages including resection of the anterior leaflet, septal myectomy, and reinforcement of the valve skirt with Teflon felt. Among dedicated self-expanding TMVR systems, only Tendyne has both CE mark and US Food and Drug Administration (FDA) approval and is delivered transapically. New transfemoral transseptal systems—such as Intrepid, AltaValve, and Cephea—are under active investigation. Techniques like LAMPOON (Laceration of the Anterior Mitral leaflet to Prevent Outflow ObstructioN) and BATMAN (Balloon-Assisted Translocation of the Mitral Anterior Leaflet) have been developed to mitigate the risk of fatal LVOT obstruction. Despite progress, mid-term mortality after ViMAC TMVR remains high. Continued innovations in technique, device design, and patient selection are critical to establishing TMVI as a viable treatment for MAC-associated mitral valve disease.

Introduction: Mitral annular calcification (MAC) has been associated with age, hemodialysis (HD) status, coronary disease, and mortality. While MAC is seen frequently in pts undergoing percutaneous edge-to-edge repair of mitral regurgitation (MR) its impact on survival is unknown. Hypothesis: MAC is not associated with mortality after percutaneous MR repair. Methods: Pts who underwent MitraClip repair of MR between Apr 2009 and May 2014 were included. Kaplan-Meier survival was plotted between pts with no MAC and pts with MAC. A multivariate Cox regression of all-cause mortality was performed simultaneously adjusting for MAC, age, HD status, and prior myocardial infarction (MI). Results: 173 pts were included. Mean age at percutaneous repair was 76.9 ± 12.6 yrs and 40.8% were females. MAC was present in 86/173 (49.7%) of pts, and was mild in 61/86 (70.9%) cases and moderate or severe in the remaining cases. In pts with MAC prevalence of prior MI was higher 28/86 (32.6%) vs. 16/87 (18.4%) p=0.037 but ejection fraction was similar 51.8 ± 17.1 vs. 50.4 ± 18.6 % p=0.603. Age was increased in pts with MAC 79.9 ± 10.1 vs. 74.3 ± 14.0 yrs p=0.003. MR was moderate-to-severe in 35/173 (20.2%) of pts and severe in 138/173 (79.8%) of pts. MR improved by 2 grades or more in 165/173 (95.4%) of pts after repair. Survival was decreased in pts with MAC as compared to pts with no MAC by Log Rank p=0.049 (Figure 1A). However, in a multivariate Cox regression model of all-cause mortality, MAC, after adjusting for age, HD status, and prior MI, was not associated with mortality with a hazard ratio of 1.166 (95% CI 0.707 - 1.922) p=0.548 (Figure 1B). In this model age and prior MI were associated with mortality with hazard ratios of 1.048 (95% CI 1.021 - 1.077) p=0.001 and 1.798 (95% CI 1.052 - 3.075) p=0.032, respectively. Conclusions: The presence of MAC was not associated with mortality after adjusting for age, HD status, and prior MI and should not preclude the MitraClip procedure. Procedural success was equally high.

Background Mitral Annular Calcification (MAC) is a significant predictor of cardiovascular events and mitral valve dysfunctions (MVD), including mitral regurgitation and mitral stenosis. This study investigates the prevalence of MAC and its severity across different age groups and genders, and examines its association with MVD in a single tertiary care center. Methods A retrospective analysis was conducted on an echocardiography database of 48,912 consecutive patients. The prevalence of MAC, including both any MAC and moderate to severe MAC was assessed. Patients were categorized into different age groups: &amp;lt;55 years, 55-65 years, 66-75 years, and &amp;gt;75 years. The study also analyzed the prevalence of MVD in these patients and examined gender differences. Chi-square tests were used to evaluate statistical significance in gender and age group differences in association with MVD. Results The overall prevalence of any MAC was 12.5% (6,104), while moderate or severe MAC was observed in 2.2% (1,104) of the patients. When stratified by age, the prevalence of any MAC was 3% in patients &amp;lt;55 years, 15.0% in patients 55-65 years, 28.6% in patients 66-75 years, and 40.9% in patients &amp;gt;75 years. The prevalence of moderate or severe MAC followed a similar trend, with 0.2%, 1.9%, 5.5%, and 10.7% in the respective age groups. Gender distribution analysis revealed a significant female predominance among patients with moderate or severe MAC (59.5% vs. 40.5%), p &amp;lt; 0.0001. Patients with moderate or severe mitral annular calcification (MAC) had a more than four-fold increased risk of mitral valve disease (MVD) compared to those with mild MAC (57.9% vs. 13.1%, p &amp;lt; 0.0001).Age group was not a significant factor in MVD prevalence. Conclusion The age-related prevalence of mitral annular calcification (MAC), especially in older women, represents a significant clinical concern given its association with increased risk of mitral valve disease (MVD). This highlights the importance of vigilant MAC screening and proactive MVD management in this population. Future research should focus on identifying modifiable risk factors and developing effective screening strategies to mitigate the progression of MAC and its associated valvular complications.

Mitral Annular Calcification in Obstructive Hypertrophic Cardiomyopathy: Prevalence and Outcomes

Mitral annular calcification in hypertrophic cardiomyopathy

Mitral annular calcification (MAC) is usually considered an incidental, benign, age-related finding without serious complications in patients evaluated for cardiovascular or pulmonary disease with imaging studies that may result in mitral regurgitation or stenosis when severe. Therefore, it is usually not considered a significant alteration. However, there is accumulating evidence that it is associated with a higher risk of cardiovascular events, such as atherosclerotic coronary artery disease, aortic artery disease, carotid artery disease, peripheral artery disease, stroke, atrial fibrillation, atrioventricular and/or intraventricular conduction disturbance, systemic embolization, infective endocarditis, heart failure and mortality. The presence of MAC also significantly influences the outcome of mitral valve transcatheter and surgical interventions. Several conditions may predispose to MAC. MAC is strongly related to cardiovascular risk factors, such as hypertension, diabetes, smoking and cardiovascular atherosclerosis, and inflammation may also play a role in the pathogenesis of MAC. Also, conditions that increase mitral valve stress, such as hypertension, aortic stenosis and hypertrophic cardiomyopathy, predispose to accelerated degenerative calcification of the mitral annulus area. Congenital disorders, e.g., Marfan syndrome and Hurler syndrome, are also associated with MAC, due to an intrinsic abnormality of the connective tissue composing the annulus.

We present a technique for mitral valve (MV) replacement that preserves the anterior mitral leaflet (AML) using complete chordal-sparing mitral valve replacement (CCS-MVR), which maintains the architecture of the left ventricle. This technique is beneficial for patients with functional, degenerative, and infective MV disease who were unable to undergo MV repair. The objective of this study is to ascertain the most efficacious treatment for MV disease. Furthermore, the study will contribute to international guidelines for this procedure and provide data on its efficacy in treating MV disease. We performed MV replacement surgery on 161 patients, maintaining the integrity of the entire subvalvular chordae tendineae apparatus. Of these cases, 92 (57.1%) were degenerative, 58 (36.0%) were functional, 9 (5.6%) were infective, and 2 (1.2%) were rheumatic. Of the 93 patients with complicated MV disease, massive calcification was observed in 59 cases (36.6%) and mitral annular disjunction (MAD) was observed in 25 cases (15.5%) while endocarditis with posterior annular abscess was observed in 9 cases (5.6%). The anterior leaflet is detached from its connection to the annulus along the entire extent from the posterior commissure to the anterior commissure. In cases of excess tissue, a section of the free edge of the translocated leaflet must be removed. This must preserve a small section of the edge and the entire chordae tendineae unit. A CCS-MVR requires 24 to 28 individual sutures in routine cases. The AML was reinserted using 12 to 14 single sutures in this specific order: posterior annulus, posterior mitral leaflet, AML, and prosthetic valve. The 30-day mortality rate was 8.7%, based on 14 cases. Ten patients, representing 5% of the total, required reoperation after the initial procedure. Out of 140 patients,74 (52.8%) experienced LV reverse remodeling at the six-month follow-up. Female patients exhibited a significantly higher rate of LV reverse remodeling (71% vs. 29%, P<0.001) compared to male patients. Patients with mitral annular calcification and MAD demonstrated significantly higher rates of left ventricular reverse remodeling compared to patients with coronary artery disease (CAD). We can conclude with certainty that male gender, CAD, atrial fibrillation, and preoperative left ventricular end-diastolic volume indexed (LVEDVI) are significant predictors of LV remodeling. We found that the median survival time after CCS-MVR was 39.5 months. The results of the multivariable analysis revealed that patients with MAD had significantly worse survival rates than those without. For patients presenting with severely calcified posterior mitral annulus, complicated Barlow disease with MAD, or endocarditis involving the posterior annulus, the AML is consolidated with the posterior mitral leaflet to form a neo-annulus. This procedure is safe and effective for a wide range of simple and complex MV pathologies. Its efficacy is unquestionable. It preserves the complete architecture of the left ventricle, avoiding dilatation and promoting reverse remodeling.

BackgroundMany previous studies have evaluated the impact of mitral valve (MV) deformity scores on the percutaneous transvenous mitral commissurotomy (PTMC) outcome in patients with mitral stenosis; however, the relationship between mitral annulus calcification (MAC) and the PTMC result has not yet been established. The current study aimed to investigate whether MAC could independently influence the immediate result of PTMC.MethodsOf all patients undergoing PTMC in our institution between April 2005 and November 2009, we included 87 patients (28.7%male, mean ± SD age = 42.8 ± 12.6 years) with rheumatic mitral stenosis who had additional data on the echocardiographic evaluation of MAC along with MV leaflets morphology. Echocardiographic assessments were repeated up to six weeks after PTMC to evaluate the immediate PTMC outcome. The frequency of the optimal PTMC result (secondary MV area > = 1.5 cm2 with > = 25% increase and without final mitral regurgitation grade > 2) was compared between two groups of patients with MAC (n = 17) and those without MAC (n = 70).ResultsThe optimal result was obtained in 55 (63.2%) patients, whereas the result was suboptimal in 32 (36.8%) patients due to insufficient MV area increase in 31(96.9%) subjects and post-procedure mitral regurgitation grade > 2 in 1(3.1%). The rate of optimal PTMC results was less in patients with MAC in comparison to those without MAC (29.4% vs.71.4%). After adjustments for possible confounders such as age and leaflets morphological subcomponents (thickening, mobility, calcification, and subvalvular thickening), MAC remained a significant negative predictor of a suboptimal PTMC result (odds ratio = 0.154; 95%CI = 0.038-0.626, p value = 0.009) together with leaflet thickening (odds ratio = 0.214; 95%CI = 0.060-0.770, p value = 0.018).ConclusionsMAC appeared to independently influence the immediate result of PTMC; therefore, mitral annulus evaluation may be considered in the echocardiographic assessment of the mitral apparatus prior to PTMC.

Mitral and aortic annular calcification is an age-related degenerative process that can result in severe mitral and/or aortic stenosis and/or regurgitation. Annular calcification not only increases the surgical complexity but also increases the risk of complications. In this case report, we present the innovative use of the Sonopet ultrasonic surgical aspirator for aortic and mitral annular decalcification in a patient with hypertrophic obstructive cardiomyopathy, mild aortic stenosis and moderate mitral regurgitation in the presence of mitral annular calcification (MAC) and aorto-mitral curtain calcification.