Cardiac valve calcification: characteristics of patients with calcification of the mitral annulus or aortic valve

Aims—To determine whether mitral annular calcification and aortic valve calcification, with or without stenosis, are expressions of atherosclerotic disease.Methods—The incidence of atherosclerotic risk factors was analysed in patients with mitral annular calcification and aortic valve calcification and in control patients from a prospective echocardiographic database of 8160 consecutive patients; 657 patients (8%) were identified with mitral annular calcification and 815 (9%) with a calcified aortic valve, of whom 515 (6.3%) had stenosis with a minimal aortic valve gradient of 16 mm Hg. In these patients, cardiac and vascular risk factors were compared with 568 control patients using multiple logistic regression analysis.Results—Age (odds ratio (OR) varying from 5.78 to 104, depending on age class), female sex (OR 1.75), hypertension (OR 2.38), diabetes mellitus (OR 2.85), and hypercholesterolaemia (OR 2.95) were strongly and significantly associated with aortic valve calcification without stenosis, as were age (OR varying from 8.82 to 67, depending on age class), female sex (OR 2.22), hypertension (OR 2.72), diabetes mellitus (OR 2.49), and hypercholesterolaemia (OR 2.86) with mitral annular calcification. Age (OR varying from 1.11 to 7.7), hypertension (OR 1.91), and hypercholesterolaemia (OR 2.55) were strongly and significantly associated with stenotic aortic valve calcification.Conclusions—Mitral annular calcification and stenotic or non-stenotic aortic valve calcification have a high incidence of atherosclerotic risk factors, suggesting they should be considered as manifestations of generalised atherosclerosis.     

The relation between mitral annular calcification and mortality in patients undergoing diagnostic coronary angiography

To determine whether the observed association between mitral annular calcification (MAC) and mortality is independent of the severity of coronary artery disease (CAD), we analyzed data from 134 male veterans (age 63 +/- 10 years) followed for 5 years who had undergone diagnostic coronary angiography and transthoracic echocardiography within 6 months of each other. Echocardiograms were retrospectively reviewed for the presence of MAC. The relation of MAC to all-cause mortality was analyzed using logistic regression, and odds ratios (OR) were calculated. MAC was present in 49 (37%) subjects. Over the 5-year follow-up period, 38 (28%) patients expired. Five-year survival was 80% for subjects without MAC and 56% for subjects with MAC (P = 0.003). MAC (OR = 3.16, 95% confidence interval [CI]= 1.43-6.96, P = 0.003), ejection fraction (OR = 0.76, 95% CI = 0.59-0.97, P = 0.02), and left main CAD (OR = 2.70, 95% CI = 1.11-6.57, P = 0.02) were significantly associated with mortality in univariate analysis. After adjusting for left ventricular ejection fraction, number of obstructed coronary arteries and the presence of left main coronary artery stenosis, MAC significantly predicted death (OR = 2.48, 95% CI = 1.09-5.68, P = 0.03). Similarly, after adjusting for predictors of MAC, including ejection fraction, age, diabetes, peripheral vascular disease, and heart failure, MAC remained a significant predictor of death (OR = 2.38, 95% CI = 1.02-5.58, P = 0.04). MAC also predicted death independent of smoking status, hypertension, serum creatinine, low density lipoprotein cholesterol, high density lipoprotein cholesterol, and C-reactive protein levels (OR = 3.98, 95% CI = 1.68-9.40, P = 0.001). MAC detected by two-dimensional echocardiography independently predicts mortality and may provide an easy-to-perform and inexpensive way to improve risk stratification.     

Mitral annular calcification as a marker of complex aortic atheroma in patients with stroke of uncertain etiology

The aim of this study was to evaluate the presence of dense mitral annular calcification as a marker of complex aortic atherosclerosis in patients with stroke of uncertain etiology. One hundred twenty-one patients with stroke of uncertain etiology were evaluated for complex aortic atherosclerotic plaques; their presence and severity were correlated with transthoracic echocardiographic findings, demographic data, and cardiovascular risk factors. Complex plaques in the ascending aorta or aortic arch were found in 72 of the 121 patients (59.5%). The only difference seen in patients with or without plaques was the presence of dense mitral annular calcification (58.3 vs 16.3%; P < 0.001). Dense mitral annular calcification (n = 50) was associated with higher prevalence of complex aortic plaques (84.0% vs 42.3%; P < 0.001), mobile components (28.0% vs 9.9%; P < 0.01), and protruding (80.0% vs 36.6%; P < 0.001), ulcerated (16.0% vs 1.4%; P < 0.01), and multisite complex plaques (46.0% vs 9.0%; P < 0.001). Therefore, in patients with stroke of uncertain etiology dense mitral annular calcification is an important marker of aortic atherosclerosis with high risk of embolism, and this association may explain in part the high prevalence of stroke and peripheral embolism in patients with mitral annular calcification.     

Obstruction to left ventricular inflow secondary to combined mitral annular calcification and idiopathic hypertrophic subaortic stenosis

We present an elderly woman with idiopathic hypertrophic subaortic stenosis (IHSS) and obstruction to left ventricular inflow. The inflow obstruction is thought to be due to massive mitral annular calcification. The association of IHSS and inflow obstruction is rare and may be overlooked unless careful hemodynamic measurements are obtained.     

Atrial extension of mitral annular calcification mimicking intracardiac tumor

Liquefaction necrosis of mitral annular calcification occurs in 3% of autopsied cases. The liquified material can spread intramurally in various directions creating suspicious appearing mass lesions on chest x-ray or echocardiography. An incidential finding of an echogenic left atrial calcified mass in a 57-year-old woman was suspected to be an intracardiac tumor for which she underwent exploratory cardiotomy. Instead, culture-negative liquified pasty material was removed from the posterior left atrial wall adjacent to the calcified mitral annulus. This case illustrates that liquefaction necrosis of mitral annular calcification may spread to contiguous structures and masquerade as an intracardiac tumor. This relatively unknown aspect of mitral annular calcification should be considered in the differential diagnosis of echogenic left atrial mass lesions.     

Abnormal distortion of aortic corevalve bioprosthesis with suicide left ventricle,aortic insufficiency,and severe mitral regurgitation during transcatheter aortic valve replacement

We present a patient with critical degenerative aortic stenosis, mitral annular and aortomitral continuity calcification, and senile sigmoid septal hypertrophy who underwent transcatheter aortic valve replacement using the CoreValve bioprosthesis. Immediately after predilation of the aortic valve (18‐mm balloon), the patient developed severe hypotension and dynamic left ventricular outflow tract (LVOT) obstruction with systolic anterior motion of the anterior mitral leaflet, causing severe mitral regurgitation. After deployment of a 26‐mm bioprosthesis, a transesophageal echocardiogram and left ventriculogram showed that the frame of the bioprosthesis appeared distorted and underexpanded. On the mitral side of the aorta (side of the aortomitral curtain between 12:00 and 3:00, echo short axis view), we found moderate periprosthetic aortic insufficiency with worse mitral regurgitation. The left ventricle was small and hyperdynamic (ejection fraction >85%). The patient soon developed complete heart block, atrial fibrillation, and ventricular tachycardia. She was resuscitated with aggressive intravenous fluids, vasopressors, and an emergently placed atrioventricular sequential pacemaker. We postdilated the 26‐mm bioprosthesis with a 22‐mm Z‐Med balloon and subsequently with a 25‐mm balloon. Each balloon was inflated to its nominal volume and pressure and conformed the nitinol frame of the valve to the net circular shape and expected diameter. However, as soon as each balloon was deflated, the surrounding aortic root anatomy visibly recoiled and the frame returned to its smaller diameter with a distorted shape. A second 26‐mm CoreValve bioprosthesis was then deployed in a “valve‐in‐valve” configuration. Soon after, the patient's hemodynamics improved, her clinical condition stabilized, and she completely recovered. © 2016 Wiley Periodicals, Inc.     

Intraoperative transesophageal echocardiographic imaging of double valve repair for aortic and mitral stenosis

An intraoperative echocardiographic evaluation to determine the feasibility and adequacy of the valve repair procedure is crucial for a successful repair. However, aortic valve repair in severe aortic stenosis (AS) is very limited and, consequently, its intraoperative echocardiographic evaluation has not been described well. Here, we describe an intraoperative transesophageal echocardiographic evaluation of a double-valve repair procedure for a patient with severe AS, moderate aortic insufficiency, and severe mitral stenosis.     

The echocardiographic association of mitral valve prolapse and mitral anulus calcification

Mitral anulus calcification (MAC) and mitral valve prolapse (MVP) are frequently diagnosed conditions. We studied two patients with mild or moderate mitral regurgitation who demonstrated both MAC and MVP on angiography and echocardiography. M-mode echocardiography is probably the definitive test for confirming the presence of MVP. Echocardiography is moderately sensitive in the diagnosis of cardiac calcification, such as MAC, but M-mode echocardiography may not detect the MAC in the majority of patients with both MVP and MAC demonstrated by angiography.     

Mitral inflow and mitral annular motion velocities in patients with mitral annular calcification: evaluation by pulsed Doppler echocardiography and pulsed Doppler tissue imaging.

AIMS: We evaluated the relationship between the mitral inflow velocities by pulsed Doppler echocardiography and mitral annular motion velocities by pulsed Doppler tissue imaging in patients with mitral annular calcification.METHODS AND RESULTS: Fifty-three patients with mitral annular calcification were divided into two groups: severe mitral annular calcification (n=15, mitral annular calcification bigger than or equal 5mm in width) and mild mitral annular calcification (n=38, mitral annular calcification <5mm in width). In addition, 20 patients with hypertensive heart disease (HHD group) and mild left ventricular hypertrophy but no mitral annular calcification and 30 normal individuals (normal group) were studied. The early diastolic mitral inflow velocity (E) was higher in the severe mitral annular calcification group (0.75+/-0.26 m/s) than in the HHD and normal groups (mild mitral annular calcification, 0.65+/-0.21; HHD, 0.57+/-0.24; normal, 0.55+/-0.15m/s), and the late diastolic mitral inflow velocity (A) was higher in the severe mitral annular calcification group (1.24+/-0.23 m/s) than in the other three groups (mild mitral annular calcification, 0.96+/-0.20; HHD, 0.84+/-0.23; normal, 0.75+/-0.13 m/s). In contrast, the early and late diastolic annular velocities (Ea, Aa) were lower in the severe mitral annular calcification group (Ea: 5.7+/-2.2; Aa: 11.9+/-4.4 cm/s) than in the other three groups (Ea: mild mitral annular calcification, 8.3+/-2.5; HHD, 7.7+/-2.2; normal, 9.0+/-1.8 cm/s; Aa: mild mitral annular calcification, 14.2+/-4.1; HHD, 14.3+/-2.8; normal, 14.2+/-2.1cm/s). Mitral valve area was smaller in the severe mitral annular calcification group (2.6+/-1.0 cm(2)) than in the other three groups (mild mitral annular calcification, 3.1+/-0.7; HHD, 4.1+/-0.7; normal, 4.2+/-0.9 cm(2)). In the mitral annular calcification and normal groups, the A correlated inversely with mitral valve area (r=-0.67, P<0.01) and directly with severity of mitral annular calcification (r=0.65, P<0.01), and the Ea correlated inversely with left ventricular wall thickness (r=-0.37, P<0.01) and severity of mitral annular calcification (r=-0.45, P<0.01).CONCLUSION: Patients with severe mitral annular calcification have higher mitral inflow velocities due to mitral annular restriction and lower mitral annular velocities caused by decreased mitral annular motion and abnormal left ventricular relaxation.     

The impact of mitral stenosis on outcomes of aortic valve stenosis patient undergoing surgical aortic valve replacement or transcatheter aortic valve replacement

Background

The concomitant presence of mitral stenosis (MS) in the setting of symptomatic aortic stenosis represent a clinical challenge. Little is known regarding the outcome of mitral stenosis (MS) patients undergoing transcatheter aortic valve replacement (TAVR) or surgical aortic valve replacement (SAVR). Therefore, we sought to study the outcome of MS patients undergoing aortic valve replacement (AVR).

Method

Using weighted data from the National Inpatient Sample (NIS) database between 2011 and 2014, we identified patients who were diagnosed with MS. Patients who had undergone TAVR as a primary procedure were identified and compared to patients who had SAVR. Univariate and multivariate logistic regression analysis were performed for the outcomes of in‐hospital mortality, length of stay (LOS), blood transfusion, postprocedural hemorrhage, vascular, cardiac and respiratory complications, permanent pacemaker placement (PPM), postprocedural stroke, acute kidney injury (AKI), and discharge to an outside facility.

Results

A total of 4524 patients were diagnosed with MS, of which 552 (12.2%) had TAVR and 3972 (87.8%) had SAVR. TAVR patients were older (79.9 vs 70.0) with more females (67.4% vs 60.0%) and African American patients (7.7% vs 7.1%) (P < 0.001). In addition, the TAVR group had more comorbidities compared to SAVR in term of coronary artery disease (CAD), congestive heart failure (CHF), chronic lung disease, hypertension (HTN), chronic kidney disease (CKD), and peripheral vascular disease (PVD) (P < 0.001 for all). Using Multivariate logistic regression, and after adjusting for potential risk factors, TAVR patients had lower in‐hospital mortality (7.9% vs 8.1% adjusted Odds Ratio [aOR], 0.615; 95% confidence interval [CI], 0.392–0.964, P = 0.034), shorter LOS. Also, TAVR patients had lower rates of cardiac and respiratory complications, PPM, AKI, and discharge to an outside facility compared with the SAVR group.

Conclusion

In patients with severe aortic stenosis and concomitant mitral stenosis, TAVR is a safe and attractive option for patients undergoing AVR with less complications compared with SAVR.

     

Acute fulminant hemolysis after transcatheter mitral valve replacement for mitral annular calcification

Transcatheter mitral valve replacement (TMVR) is emerging as an alternative treatment strategy to surgery for patients with severe mitral annular calcification (MAC) who are not candidates for traditional mitral valve surgery. Paravalvular leak (PVL) is common following TMVR for severe MAC and can lead to heart failure symptoms and/or intravascular hemolysis, the latter of which usually is clinically stable. We report the case of a 67‐year‐old woman with symptomatic severe aortic stenosis and mitral stenosis with MAC in the setting of prior chest irradiation who was treated initially with transcatheter aortic valve replacement followed by TMVR at a later date (Sapien S3 system; Edwards Lifesciences). Immediately following TMVR, she developed acute profound hemolysis which manifested with hemoglobinuria, transfusion‐dependent anemia, and acute renal failure requiring renal replacement therapy. She was treated with post‐dilation balloon valvuloplasty after failed transcatheter PVL closure 10 days following TMVR with resulting improvement in the PVL. The hemolytic anemia resolved and renal function recovered without the need for continued hemodialysis 2 months later and stabilization of glomerular filtration rate at 6 months. This case highlights a potential severe complication of TMVR in MAC and suggests that improvement in hemolysis and late recovery of renal function may occur following treatment of PVL.     

Predicting progression of aortic stenosis by measuring serum calcification propensity

BackgroundThe aim of this prospective, double‐blinded study in patients with aortic sclerosis was to determine whether a new calcification propensity measure in the serum could predict disease progression.MethodsWe included 129 consecutive patients with aortic sclerosis as assessed during a routine clinical echocardiographic exam. Clinical, echocardiographic, and serum laboratory parameters were collected, including a new blood test providing an overall measure of calcification propensity by monitoring the maturation time of calciprotein particles (T50 test). The echocardiographic exam was repeated after 1 year. Multiple regression analysis was performed to identify independent predictors of the annual increase of peak transvalvular Doppler velocity (∆vmax). Furthermore, the accuracy of the T50 test to detect patients with the most marked stenosis progression was assessed by receiver operating characteristic (ROC)‐analysis.ResultsMean age was 75 ± 9 years, 79% were men. The T50 was 271 ± 58 min. Overall, there was no significant stenosis progression between baseline and follow‐up (∆vmax 3.8 ± 29.8 cm/s, p = ns). The T50 test was not found to be an independent linear predictor in multivariate testing. By ROC‐analysis, however, a T50‐value ≤ 242 min was able to significantly detect a ∆vmax above the 90th percentile (∆vmax ≥ 43 cm/s, AUC = 0.67, p = .04, Sensitivity = 69%, Specificity = 70%).ConclusionsThe T50 test showed a modest but significant ability to identify a pronounced aortic stenosis progression in patients with aortic sclerosis. The test could not be established as an independent linear predictor of disease progression, possibly due to the low valvular disease burden and short follow‐up interval.     

An extensive caseous calcification of the mitral annulus complicated with severe mitral regurgitation

Caseous calcification of the mitral annulus (CCMA) is considered a rare variant of mitral annular calcification (MAC) due to caseous transformation of the inner material and prevalence of CCMA, about 0.63% of all MAC cases and 0.06%–0.07% of the population. The precise pathophysiology of CCMA is still unknown. It is a chronic degenerative disorder common in the elderly, particularly in women. Caseous calcification of the mitral annulus (CCMA) is a soft peri‐annular extensive calcification, resembling cardiac tumors, abscesses, vegetation, or calcified thrombus, which is composed of an admixture of calcium, fatty acids, and cholesterol with a toothpaste‐like texture. In the characteristic appearance on echocardiography, the calcification is a round, large, soft mass with a central echo dense area, typically located at the basal area of the posterior mitral valve. It usually carries a benign prognosis but rarely may be complicated with mitral valve dysfunction (valve stenosis/mitral regurgitation) or systemic embolization. The current data suggest conservative medical therapy and clinical follow‐up for management of CCMA unless there is a need of cardiac surgery for severe mitral stenosis and/or regurgitation. We present a case, complicated with severe mitral regurgitation, and need surgical resection of the CCMA because of the CCMA‐induced symptomatic severe mitral regurgitation despite optimal medical therapy. Macroscopic and microscopic examination of caseous material also demonstrated the characteristic appearance of CCMA.