Caseous Calcification Of The Mitral Annulus Research Articles

Multi-Modality Imaging in Caseous Calcification of the Mitral Annulus: Case Series and Clinical Implications

Multi-Modality Imaging in Caseous Calcification of the Mitral Annulus: Case Series and Clinical Implications

Caseous Calcification of the Mitral Annulus: Calcified Toothpaste of the Heart.

Caseous calcification of the mitral annulus (CCMA) is a rare variant of mitral annular calcification (MAC) usually described as an antemortem finding. We report a case of sudden cardiac arrest in a 39-year-old male with end-stage renal disease undergoing hemodialysis with a history of Fabry disease by kidney biopsy. Autopsy revealed significant circumferential annular calcification in both mitral and aortic valves with a caseous gross appearance. Histologically, these areas consisted of amorphous basophilic material accompanied by a surrounding granulomatous-appearing infiltrate. Von Kossa staining on non-decalcified tissue revealed strong positive staining, confirming CCMA diagnosis. While identifiable, the atrioventricular node was displaced and distorted by caseous deposits. Toluidine blue staining of myocardium showed osmophilic accumulations, and electron microscopy (EM) showed myeloid/zebra bodies, consistent with Fabry disease. We posit that Fabry disease leads to end-stage kidney disease, altering calcium phosphate metabolism, a proposed mechanism for CCMA. This case highlights the multifactorial nature of sudden cardiac death in decedents with various structural cardiac changes and potential renal-disease-induced electrolyte imbalances. We aim to bring awareness to this rare entity, its potential role in a sudden cardiac death, and to highlight the need to use non-decalcified tissue when staining for calcium to establish the diagnosis.

A rare case of caseous necrosis of mitral valve

Caseous calcification of the mitral annulus, an uncommon kind of intracardiac mass, is a form of degenerative disease that afflicts the mitral valve causing liquefactive or caseous necrosis. Caseous calcification of mitral annulus is rarely encountered in practice; however, it should be on the list of differential diagnosis of any intracardiac masses located close to the mitral annulus.

A Report of Two Cases with Caseous Annular Calcification of the Mitral Valve.

Introduction: Caseous calcification of the mitral annulus (CCMA) is a rare variant of mitral annulus calcification, a chronic and degenerative mitral valve fibrous ring process. It usually carries a benign prognosis. The prevalence of CCMA is about 0.06%-0.07% of the population. CCMA is generally diagnosed incidentally and can be confused with other intracardiac masses such as cardiac tumors, abscesses, vegetation, or calcified thrombi. Multimodality imaging, including TEE, cardiac computed tomography, and cardiac magnetic nuclear resonance, can easily differentiate CCMA from other masses and help avoid unnecessary surgery. CCMA is typically located in the basal area of the posterior mitral valve, and the calcification seems like a round, large, soft mass with a central echo-dense location. CCMA may have a benign course, but it may sometimes be complicated with mitral valvular dysfunction, systemic embolization, or conduction abnormalities in the scenarios mentioned above, as well as when the diagnosis is unclear, surgery is indicated. Mitral valve replacement should be preferred compared to mitral valve repair.
 Conclusions: It is important to note that the decision for mitral valve replacement, including CAC cases, should be individualized based on various factors, including the patient's clinical condition, symptoms, severity of valve disease, and associated comorbidities.
 As scientific understanding and research progress, there may be ongoing developments and refinements in the conservative and surgical management of CCMA.

Caseous calcification of the mitral annulus

Caseous calcification of the mitral annulus (CCMA) is a rare form of degenerative lesion of the mitral valve fibrous ring. It’s a biochemical transformation of calcification area accompanied with formation of masses of a curdled consistency (caseosis). It is usually located in the area of the posterior mitral valve. In most cases, it is asymptomatic or minimally symptomatic, and is an incidental finding during imaging studies. The causes and pathogenesis of CCMA are unknown, however, there is a connection with disorders of calcium and phosphorus metabolism and there is no connection with an valve infection. There is no specific treatment. In cases where CCMA leads to significant disturbances of cardiac hemodynamics and/or there is a high probability of ebolism, it needs to be treated surgically.Differential diagnosis is carried out with other cardiac mass.

Caseous Calcification of the Mitral Annulus.

Caseous Calcification of the Mitral Annulus.

Caseous Calcification of the Mitral Annulus: Mimicking A Cardiac Mass.

Caseous calcification of the mitral annulus is a rare condition.An important differential diagnosis is cardiac tumours and myocardial abscesses.Cardiac computed tomography and cardiac magnetic resonance imaging are important in confirming the diagnosis, and assessing the extent and location of annular calcium.

P271 CASEOUS CALCIFICATION OF THE MITRAL ANNULUS: ROLE OF 3D ECHOCARDIOGRAPHY

Abstract The caseous calcification of the mitral annulus (CCMA) is a rare degenerative condition of the mitral valve (0.067% prevalence in the general population) that generally affects older women with hypertension, chronic kidney disease and dyslipidemia. Usually CCMA is asymptomatic and it is associated with mild to moderate mitral regurgitation and it can cause severe mitral valve dysfunction that requires surgical treatment. CASE 1: A 84–year–old woman, with arterial hypertension, was evaluated in our clinic to perform a transthoracic echocardiogram (TTE) for a previous finding of heart murmur. TTE reveled at the level of the posterior mitral annulus a hyperechoic roundish formation with a central eco–shiny area with a maximum diameter of 20x20 mm. A tridimensional trans–esophageal echocardiography (3D TEE) allowed a better characterization of the mass showing well–defined contours, adhered to the posterior mitral annulus, systolic dislocation of the anterior mitral leaflet, with slight mitral regurgitation. Considering the morphological characteristics of the mass we concluded for a presumptive diagnosis of CMAC. We recommended regular follow–up to the patient. After 6 and 12 months the mass did not change in size and echostructure. CASE 2: An 88–year–old woman with chronic kidney disease, high blood pressure and chronic ischemic heart disease was admitted to our ward for acute heart failure. TTE showed a large an echodense mass (43 × 19 mm) involving the posterior mitral annulus, with a central echo–lucent area. A 3D TEE was performed with better mass characterization: involvement of the entire posterior mitral ring, reduced movement of the posterior mitral flap, anterior mitral flap with normal morphology and normal mobility. In consideration of the echocardiographic characteristics we concluded for the presence of CMAC. We discharged the patient with indication for a regular clinical and echocardiographic follow–up. Conclusions The 3D TTE provides adequate morphostructural information that allows in most cases to characterize the CCAM. When the transthoracic approach is difficult due to poor acoustic windows, 3D TEE can be used. Only in rare cases it is necessary to use second–level diagnostic tests such as CT and Cardiac Magnetic Resonance Imaging.

Surgery for mitral annular caseous calcification-related calcified amorphous tumor: a case report

BackgroundA calcified amorphous tumor (a non-neoplastic tumor) with caseous calcification of the mitral annulus is a rare pathology that causes severe embolic events. We present a rare case of mitral valve surgery for a mitral annular caseous calcification-related calcified amorphous tumor found in cerebral infarction.Case presentationA 69-year-old man was diagnosed with a mitral valve calcified amorphous tumor with mitral annular caseous calcification found in cerebral infarction. He was admitted because of acute multiple embolic cerebral infarctions. A search for the embolic source through transesophageal echocardiography revealed a mitral valve tumor raised from the posterior mitral valve leaflet on the side of the left ventricle. Computed tomographic cardiac angiography revealed a calcified mitral valve tumor invading the posterior mitral valve annulus and left ventricular muscle. Intraoperative findings revealed a mitral annular calcification-related calcified amorphous tumor with caseous calcification of the posterior leaflet and annulus, which was suspected. Therefore, we performed radical debridement of the mitral annular calcification and bioprosthetic mitral valve replacement with patch repair of the posterior mitral valve annulus 2 weeks after the onset of cerebral infarction. The patient recovered well post operation, without any embolic events.ConclusionsA calcified amorphous tumor with caseous mitral annulus calcification may be highly associated with embolic events. In this case, mitral valve replacement with annular patch repair may be a favorable procedure for preventing embolic events.

Intracardiac pseudotumor: Caseous calcification of the mitral annulus on multimodality imaging.

Intracardiac pseudotumor: Caseous calcification of the mitral annulus on multimodality imaging.

A case of caseous calcification of mitral annulus resulting in multiple cerebral infarctions

The patient is a 73-year-old woman. She presented with dysarthria, and a head MRI revealed multiple acute cerebral infarctions in the bilateral cerebral hemisphere and cerebellar hemisphere. Transesophageal echocardiography after admission revealed a 16mm large mobile calcification of the mitral annulus (caseous calcification of the mitral annulus; CCMA) on the posterior apex of the mitral valve annulus. Since the CCMA had a high risk of relapse, and a new infarction was detected on the 8th day, resection of the mass and mitral valve replacement surgery were performed. CCMA is a subtype of mitral annular calcification (MAC). When calcification progresses from the MAC state to form a mass, it is called a calcified amorphous tumor; CAT. Reports of embolic cerebral infarction caused by CAT are rare, but this is a rare report of an embolic cerebral infarction from CCMA presenting as CAT.

Caseous calcification of the mitral annulus

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501 CASEOUS CALCIFICATION OF THE MITRAL ANNULUS AND THE IMPORTANCE OF ITS IDENTIFICATION IN THE CLINICAL PRACTICE: A REPRESENTATIVE CASE

Abstract 1. Introduction Caseous Calcification of the Mitral Annulus (CCMA) is a rare condition characterized by a liquefaction degeneration that usually involves posterior mitral ring and it is considered a variant of mitral annulus calcification. Its prevalence is very low, but it is possible that it is often underdiagnosed or misdiagnosed. This condition is related with increased age, female sex, chronic kidney disease and calcium metabolism disorders. The lesion can be detected using imaging techniques and its management is conservative in most cases. 2. Case Study A 72-year-old man hospitalized for bilateral pneumonia complicated with ARDS has come to our attention. The patient had a history of stage IV renal failure, type 2 diabetes mellitus and calcific degenerative aortic valve disease. During its stay in the Intensive Care Unit a routine Transthoracic Echocardiogram showed a vegetation suspected for infective endocarditis that involved the posterior mitral leaflet. However, the clinical presentation did not support endocarditis diagnosis as no microorganisms were isolated at multiple blood cultures and other Duke Criteria were negative. Given the limited acoustic viewing of transthoracic echocardiogram it was necessary to perform a Transesophageal Echocardiogram (TEE) for a better characterization of the valve lesion. TEE showed the presence of a round lesion, about 2×2 cm in size, with smooth borders, located over the mitral annulus with extension up to the posterior mitral leaflet and with a partially mobile portion. The lesion had a corpuscular appearance inside with less echogenicity and without evidence of flow. No significant functional alterations of the mitral valve were detected (anterograde transmitral flow Mean Gradient 4-5 mmHg; Mild regurgitation). We therefore concluded for Caseous Calcification of the Mitral Annulus as the most likely diagnosis and decided for a conservative management. A new TEE was performed as a control after one week showing no significant changes on the valve lesion. 3. Conclusions It is important for echocardiographers to be familiar with CCMA and to know how to distinguish it from other valve lesions such as infective vegetation, abscesses or tumors. An integration with other cardiac imaging techniques, patient past medical history and clinical-laboratory data can help the physician in the correct characterization of valve lesions and subsequent most appropriate therapeutic approach for the patient.

Bulky Mitral Annulus Caseous Calcification in an Atypical Location.

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.

Non-invasive imaging of ventricular–atrial fistulization secondary to infective rupture of caseous calcification of the mitral annulus

Non-invasive imaging of ventricular–atrial fistulization secondary to infective rupture of caseous calcification of the mitral annulus

Caseous calcification of the mitral annulus mimicking benign cardiac tumour of the mitral valve

Caseous calcification of the mitral annulus (CCMA) is a rare subtype of mitral annular calcification. It usually appears as a large, round, mass-like calcification with an echolucent core, which may be misdiagnosed as an intracardiac mass, cyst, thrombus or abscess of the mitral valve. We present a case report of CCMA that was misdiagnosed by echocardiography as a benign tumour due to its atypical imaging. The mitral valve mass was resected and it was pathologically confirmed to be a myxoid change with calcification. Echocardiography is the preferred initial diagnostic tool. Myocardial contrast echocardiography (MCE) is used to evaluate the vascularity of intracardiac masses or mass-like lesions, but neither echocardiography nor MCE is reliable for identifying atypical lesions. Cardiac computed tomography is helpful in establishing a diagnosis by showing dense calcifications, while cardiac magnetic resonance imaging is used primarily as a credible tool. We therefore recommend that a diagnosis should be made based on various imaging modalities, if necessary, and operators should be skilled to avoid misdiagnosis.

Caseous Calcification of the Mitral Annulus leading to symptomatic mitral stenosis.

Caseous calcification of the mitral annulus (CCMA) is a rare variant of mitral annular calcification, which can be difficult to diagnose and can be misdiagnosed as a cardiac tumour or abscess. Multimodality imaging is important for the diagnosis of this entity and to avoid unnecessary surgical treatment.

A Giant Caseous Calcification of the Mitral Annulus with Uncommon Presentation

scholarly articles on cardiology Journal of Cardiology & Cardiovascular Therapy is an Open Access, international peer reviewed journal launched from Juniper Publishers. Cardiology is a branch of medicine deals with the disorders of heart that keeps cardiologists up to date with advances in the diagnosis and treatment of cardiovascular diseases.

Caseous Calcification of the Mitral Annulus Presenting with Complete Heart Block

Caseous Calcification of the Mitral Annulus (CCMA) is a rare variant of Mitral Annular Calcification (MAC), which is usually a benign condition that is associated with people above the age of 85. It is usually incidentally diagnosed on echocardiography. This is a case of a relatively young patient who presented to hospital with chest discomfort and dizziness due to CCMA that had infiltrated the basal septum resulting in an infra-Hisian atrioventricular (AV block).

A Case of Caseous Calcification of the Mitral Annulus Leading to Rupture into Pericardium and Cerebral Infarction

An 84-year-old woman presented with respiratory distress, left hemiplegia, and chest pain for the past few days. Chest Computed Tomography (CT) revealed a hemopericardium with the Caseous Calcification of the Mitral Annulus (CCMA) reduced to a C-shaped structure.