Caseous calcification of the mitral annulus. A review of six cases.

Caseous calcification of the mitral annulus is a rare form of mitral annular calcification, whose etiology is not completely understood and which can lead to an erroneous diagnosis of intracardiac tumor. The authors describe the cases of six patients, five of them female, mean age 74.8 +/- 6.4 years (65-81). Four patients presented with heart failure, two with atrial fibrillation and five with hypertension. Round, echogenic images, 18-26 mm in their largest diameter with a central echolucent area, were identified by transthoracic echocardiography on the lateral and posterior segments of the mitral annulus. Severe mitral regurgitation was also found in four patients. Only three patients with severe mitral regurgitation and heart failure were operated on, and one patient refused surgical treatment. A caseous mass, similar to toothpaste, was obtained from the mitral annulus zone during surgery.     

Volumosa Calcificação Caseosa do Anel Mitral em Localização Atípica

Mitral annular calcification (MAC) is a commonly observed chronic and degenerative fibrotic process of the base of the mitral valve, usually deemed as an incidental finding. Although initially, MAC was thought to be a consequence of an age-related degenerative process, recent findings suggest other independent contributive mechanisms, such as atherosclerosis and abnormal calcium-phosphorus metabolism.Caseous calcification of the mitral annulus (cMAC) is a rarely described variant of MAC, characterized by an ovoid, focal mass with internal caseous fluid-like calcifications and debris.Differentiating a cMAC from other cardiac masses attached to the mitral annulus may be challenging. A single imaging modality, such as transthoracic echocardiography, may not be sufficient for a clear diagnosis. Therefore, a multimodal imaging approach is necessary, including cardiac tomography computerized imaging and cardiac magnetic resonance (CMR).MAC and cMAC typically affect the posterior mitral annulus, with very few cases in the literature describing the involvement of the anterior annulus. We present a rare case of an anterior mitral annulus caseous calcification found in a CMR performed to evaluate a left atrial mass identified on a transthoracic echocardiogram.     

Cardiac imaging in the evaluation of mitral annulus caseous calcification

The current case report illustrates a comprehensive assessment with different imaging techniques, as echocardiography, magnetic resonance and computer tomography, of a rare cardiac mass known as caseous calcification of mitral annulus. Transesophageal echocardiography was able to identify and characterize the caseous calcification of mitral annulus.     

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.     

Caseous calcification of the mitral annulus, a rare echocardiographic finding

Caseous calcification of mitral annulus, also known as caseoma, is a rare echocardiographic finding. Because it often presents asymptomatically, the diagnosis is usually incidental. The condition arises due to caseous degeneration in the internal material of the mitral annulus calcification. On echocardiography, the calcification is a round mass with a central echolucent area, typically located at the base of the posterior leaflet, and can be mistaken for cardiac tumors or abscesses. We describe 3 patients with the condition, one of whom required surgical removal of the mass to obtain a definitive diagnosis.Full English text available from: www.revespcardiol.org     

Traitement chirurgical d’une insuffisance mitrale fonctionnelle aggravée par l’effort avec insuffisance cardiaque à fraction d’éjection préservée : une étude de cas

We here report the case of a 67-year-old woman with moderate mitral regurgitation without significant structural abnormalities that get worse during severe recurrent heart failures and preserved ejection fraction with concomitant paroxysmal atrial fibrillation. Atrial fibrillation became permanent and despite a well-controlled cardiac frequency, new heart failure episodes occurred. Exercise doppler echocardiography showed that the mechanism of this mitral regurgitation was a two leaflet mitral tenting. We discuss here the different mechanisms that could induce these kinds of mitral regurgitation with excessive tenting. We emphasize the interest of early detection by exercise doppler echocardiography even when a triggering factor like atrial fibrillation seems to be involved. We also discuss the interest of mitral valve replacement for these patients.     

Caseous calcification of the mitral annulus: a multi-modality imaging perspective

Mitral annulus calcification is a common echocardiographic finding, particularly in the elderly and in end-stage renal disease patients under chronic dialysis. Caseous calcification or liquefaction necrosis of mitral annulus calcification is a rare evolution of mitral annular calcification. Early recognition of this entity avoids an invasive diagnostic approach, since it is benign and, unlike intracardiac tumors and abscesses, has a favorable prognosis. The authors present the case of an 84-year-old woman with a suspicious large, echodense mass at the level of the posterior mitral leaflet with associated severe mitral regurgitation. Cardiac magnetic resonance imaging demonstrated a hypoperfused mass with strong peripheral enhancement 10 minutes after gadolinium administration. Multislice computed tomography showed the calcified nature of the mass. A multi-modality imaging approach confirmed the diagnosis of caseous calcification of the posterior mitral annulus. The patient refused surgical treatment.     

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.     

Severe mitral annular calcification in rheumatic heart disease: A rare presentation

Severe mitral annular calcification(MAC)is frequently seen in patients with advanced age and chronic kidney disease,but it is rare in rheumatic heart disease(RHD).We hereby report a case of 45-year-old female with chronic RHD,who had severe MAC and mitral regurgitation.Fluoroscopy revealed a "crown"-like severe calcification of the mitral annulus.Autopsy of the heart revealed a calcified posterior mitral annulus,fused commissures,and calcified nodules at the atrial aspect of the mitral valve.     

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.     

Intracardiac pseudotumor caused by mitral annular calcification.

The current report describes a rare case of a pseudotumor in the left ventricle. Transthoracic and transesophageal echocardiography demonstrated a round, echodense, mobile mass attached to the posterior mitral leaflet and annulus. At surgical exploration caseous annular calcification of the posterior mitral leaflet was diagnosed. After resection of the mass, successful mitral valvular plasty was performed. Review of the literature indicated that mitral annular calcification is associated with an increased risk of stroke. Optimal treatment may be surgery, especially when valve plasty can be performed, although randomized trials are currently lacking.     

Images in Cardiovascular Medicine: Caseous Calcification of the Mitral Annulus

A 78‐year‐old woman was admitted to our emergency department for subarachnoid hemorrhage. Since a month ago, she was taking warfarin after diagnosis, on transthoracic echocardiogram (TTE), of a suspected large atrial thrombus. The patient, referred to our institution for further investigation, presented asymptomatic; electrocardiogram showed sinus rhythm. TTE revealed an echo dense spherical mass located in the mitral periannular posterior region with moderate mitral regurgitation. Transesophageal echocardiography and cardiac computed tomography confirmed a calcified round mass (2.0 × 2.9 cm) with central areas of echolucency‐like liquefaction surrounded by a hyperechogenic structure without systolic flow inside the cavity. The mass was diagnosed as caseous calcification of the mitral annulus (CCMA), a rare finding associated with a benign prognosis, requiring surgery only in the presence of mitral valve dysfunction. The diagnosis of CCMA is, often, misconstrued as thrombus, tumor, or abscess, leading to unnecessary investigations or interventions. Our patient was discharged after discontinuation of warfarin.     

Multimodality imaging assessment of a caseous calcification of the mitral valve annulus

Caseous calcification of the mitral annulus (CCMA) is a rare echocardiographic finding. It is commonly misdiagnosed as an abscess, tumor or infective vegetation on the mitral valve. Since it is a benign process, differentiating it from malignant intra-cardiac mass is primordial to avoid unnecessary surgery. Various imaging modalities can be complimentary for definitive diagnosis. We present a case of CCMA in a 71-year-old female patient. Her medical history revealed hypertension, diabetes mellitus, hyperlipidaemia and coronary artery disease. She was referred to our department for coronary catheterization because of angina symptoms upon minimal exertion. The lesion was detected during echocardiography and was defined as a mass of heterogeneous content with calcification points, located at the posterior side of the mitral valve annulus. Restricted motion of the posterior leaflet and the mass effect caused only minimal mitral regurgitation. To establish the correct diagnosis, we performed the full spectrum of noninvasive cardiac imaging modalities. Transesophageal echocardiography identified well-organized, composite lesion with regular edges, markedly calcified margins and more echolucent central portion. A computed tomography (CT) was performed, showing a hyperdense mass with hypodense center and a calcified peripheral rim located at the posterior mitral ring. Cardiac magnetic resonance imaging (MRI) showed that the mass was hypointense with respect to the myocardium in the T1 and T2-weighted sequences and only presented late-phase enhancement in the surrounding capsule. Based on the CT and MRI findings, the diagnosis of CCMA was established. The patient was managed conservatively.     

Features of patients with severe mitral stenosis with respect to atrial rhythm. Atrial fibrillation in predominant and tight mitral stenosis.

The aim of this study was to assess the features of patients with severe mitral stenosis in relation to atrial rhythm. Six hundred and fifty patients (pts) with severe mitral stenosis (MS) (valve area less than or equal to 1.5 cm2) who underwent percutaneous balloon commissurotomy (n = 600) or surgery (n = 50) were classified into 3 groups according to their atrial rhythm (AR): group A: sinus rhythm (SR) (n = 379), group B: SR with episodes of transient atrial fibrillation (AF) (n = 65), group C: permanent AF (n = 206). Uni- and multivariate analysis of clinical, echocardiographic and hemodynamic parameters with respect to the atrial rhythm was performed. Some parameters were comparable in all 3 groups: sex, pulmonary, right and left atrial pressures, mitral valve area, incidence of associated aortic valve disease. Nine parameters were different: mean age, NYHA class III or IV, previous commissurotomy, previous embolism, cardiac index, mitral regurgitation, tricuspid regurgitation, left atrium diameter, mitral calcification. Multivariate analysis, identified age, left atrial diameter and presence of mitral calcification as independent predictors of atrial fibrillation. Transoesophageal echocardiography was performed in 167 cases. A spontaneous echo contrast was recorded in 106 cases (63.5%) and was significantly correlated with a history of embolism and or left atrial thrombi detected by echocardiography. Atrial fibrillation, size of left atrium, severity of mitral stenosis and cardiac index were found to be independent predictive factors of spontaneous echo contrast.     

Unusual tumor-like calcification of the mitral annulus: diagnosis and tissue characterization by ultrasound, computed tomography and magnetic resonance imaging.

Calcification of the mitral annulus is a common echocardiographic finding in the elderly, particularly in females. Calcium deposits are generally located in the posterior mitral ring, sometimes extending to the whole mitral annulus and involving the mitral valve apparatus. The present report refers to 2 patients with a very atypical mass-like calcification of the mitral annulus resembling a cardiac tumor. A detailed evaluation of the mass was obtained at transthoracic and transesophageal echocardiography; the differential diagnosis with other intracardiac masses was aided by the use of computed tomography and magnetic resonance imaging. To our knowledge there has been no prior report of such a lesion evaluated at cardiac magnetic resonance imaging.     

Study of calcification of the mitral annulus. Importance of echocardiographic analysis

Mitral annulus calcification is a common finding in old people. In order to know the association of mitral annulus calcification with other pathologic conditions, 25 patients were studied by echocardiography, 20 females and 5 males. No significant differences _ere observed with respect to sex. The average age was 67 +/- 15 years. Mitral annulus calcification was associated with aortic sclerosis in 100% of the cases, to arterial hypertension in 19 (76%), to obstructive pulmonary disease in 8 (32%), to diabetes mellitus in 5 (20%), and to peripheral arterial insufficiency in 5 (20%). Mitral regurgitation was found in 14 cases, atrial fibrillation in 2 (8%). Complete A-V block was not observed, but there was enlargement of the left ventricle in 23 (92%) and of the left atrium in 17 (68%). The chest X-ray showed mitral annulus calcification in 5 (20%) with a sensitivity of 25% and a specificity of 100%. The M-mode echocardiogram showed LA-Ao ratio of 1.4 +/- 0.3, the mitral valve D-E excursion was reduced 11.9 +/- 3.1 mm. and also the E-F slope 28.6 mm/seg +/- 16.7 with appearance of mitral stenosis, but the two-dimensional study demonstrated that this was false. In all patients the left ventricle was dilated and fractional shortening was diminished. Echocardiography not only is a good diagnostic method for mitral annulus calcification, it also allow us to evaluate the hemodynamic consequences of this pathology which occurs in older patients and is often associated with other common illnesses of advanced age. In our study 56% of mitral annulus calcification cases were associated with mitral regurgitation.     

Anatomy, mechanics, and pathophysiology of the mitral annulus

The mitral annulus plays an important role in leaflet coaptation, in unloading mitral valve closing forces, and in promoting left atrial and left ventricular filling and emptying. Perturbations of annular mechanics figure prominently in a number of disorders including functional and ischemic mitral regurgitation, mitral valve prolapse, atrial fibrillation, mitral annular calcification, and annular submitral aneurysm. This review discusses the role of annular dysfunction in the pathogenesis of these disorders.     

An autopsy case of endocardial cushion defect (ECD) in an 82-year-old female]

In an 82-year-old female case of endocardial cushion defect (ECD), a systolic regurgitant murmur was heard at the apex, and her ECG showed atrial fibrillation without right bundle branch block or left axis deviation. An echocardiogram demonstrated atrial septal defect (ASD) and a cleft of the anterior mitral leaflet with calcification. She died of refractory congestive heart failure. Autopsy revealed ECD (intermediate type) with mitral and tricuspid cleft, and ASD (ostium primum type, 2.0 x 1.0 cm in diameter). In addition, mitral ring calcification and calcification of the cleft mitral valve was disclosed, causing mitral stenosis in addition to mitral regurgitation due to the cleft mitral valve. This was the second oldest Japanese autopsy case of ECD. We concluded that echocardiographic examinations, including color flow imaging, in aged patients with heart murmur are necessary to confirm the diagnosis of congenital heart disease in the aged.     

Percutaneous valve repair for mitral regurgitation using the Carillon Mitral Contour System. Description of the method and case report

Mitral regurgitation may result from left ventricular dilatation and cause progression of heart failure. Percutaneous techniques for mitral valve repair are under development. Techniques utilizing a trans-coronary venous approach exploit the anatomical relationship between the mitral annulus and the venous system of the heart. The coronary sinus, great cardiac vein and the origin of the anterior interventricular vein surround the posterior mitral annulus. This enables percutaneous approaches to annuloplasty for mitral regurgitation. Devices can be implanted into the coronary veins that modify the shape and size of the mitral annulus. We present a case of ischaemic mitral regurgitation successfully treated by use of a percutaneous approach, the Carillon Mitral Contour System. Significant reduction of the mitral regurgitation jet was observed. The patient was discharged 4 days after the procedure. During the follow-up visits, the patient showed an improved general condition and increased exercise capacity. Procedural steps are shown in detail and the current status of the coronary sinus based technique is discussed. Percutaneous techniques for mitral valve repair may be an attractive alternative to cardiac surgery in heart failure patients with secondary mitral regurgitation. The Carillon Mitral Contour System is under ongoing clinical evaluation in the AMADEUS trial.     

Cardiac mass in a patient with colorectal cancer:“Not all that glitters is gold!”

When performing echocardiography in a 74-year-old woman admitted with non ST elevation myocardial infarction and concomitant colorectal cancer(CC),a dense calcification of the mitral annulus was detected. Differential diagnosis between secondary metastasis and other etiologies of cardiac masses was essential for staging and therapeutic decision-making.Multimodality imaging with echocardiography alongside a computed tomography scan and cardiac magnetic resonance was crucial for the final diagnosis of caseous calcification of the mitral annulus(CCMA).CCMA is briefly reviewed and some possible explanations for the previously undescribed association of CC with CCMA are suggested.