Marfan's syndrome with massive calcification of the mitral annulus at age twenty-six

Goodman, Herbert B.; Dorney, Edward R.

doi:10.1016/0002-9149(69)90440-8

Cited by 16 publications

(5 citation statements)

References 16 publications

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“…Rarely, MAC have also been described in patients with Marfan's Syndrome [9] , and in Barlow's Disease, a disease of MAC and severe mitral valve prolapse [10] . Several studies have demonstrated that although commonly seen in asymptomatic patients, MAC may be a risk factor for more significant cardiovascular pathology.…”

Section: Discussionmentioning

confidence: 99%

Caseous mitral annular calcifications: Multimodality imaging characteristics

Shriki

Rongey²,

Ghosh³

et al. 2010

WJR

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Author contributions: Shriki J served as the primary author and wrote and finalized the manuscript; Rongey C and Ghosh B researched and wrote the clinical histories of the patients presented; Daneshvar S obtained echocardiographic images and contributed to the sections of the manuscript discussing the echocardiographic appearance of caseous mitral annular calcifications; Colletti PM contributed to the discussion regarding the cardiac MR findings in the patients who were discussed; Farvid A assisted in the discussion of the clinical significance of mitral annular calcifications, and obtained IRB approval for this review; Wilcox A assisted in editing and finalizing the manuscript. AbstractThe authors report herein a series of 3 patients with caseous mitral annular calcifications (MAC). One of the patients presented with mass-like, caseous MAC as an incidental finding on a staging computed tomography (CT) for metastatic colorectal carcinoma. Another patient presented with a nodule on a chest radiograph, which was later found on CT to be due to caseous MAC. In the third patient, caseous MAC was initially detected on echocardiography, and was further evaluated with CT and cardiac magnetic resonance imaging. In all three patients, the appearances posed a diagnostic dilemma. The appearance of caseous MAC is dissimilar to non-caseous MAC and is usually seen as an ovoid, mass-like structure, with homogeneous hyperattenuation, representing a liquefied form of calcium and proteinaceous fluid. This homogeneous center is surrounded by peripheral, shell-like calcifications. Caseous MAC is likely an under-recognized entity and may present a diagnostic dilemma at CT, magnetic resonance imaging, or echocardiography.

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Section: Discussionmentioning

confidence: 99%

Caseous mitral annular calcifications: Multimodality imaging characteristics

Shriki

Rongey²,

Ghosh³

et al. 2010

WJR

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“…The disease most often occurs in elderly patients, affecting mainly women [2], especially those at risk of cardiovascular diseases (diabetes, hypertension, obesity, renal failure, ischemic heart disease, and impaired calcium metabolism) [3]. It rarely occurs in younger patients-e.g., in people with Barlow's disease [4] and Marfan's syndrome [5]. It is currently estimated that it affects 0.068% of the general population and 0.64% of all MAC cases [6].…”

Section: Introductionmentioning

confidence: 99%

The Importance of Cardiac Computed Tomography in the Diagnosis of Caseous Calcification of the Mitral Annulus—Case Reports

Gać

Cheładze²,

Poręba

2022

Diagnostics

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Mitral annular calcification (MAC) is a common pathology of the mitral valve. In rare cases, calcifications occur in the mitral annulus degenerate serous; the caseous calcification of the mitral annulus (CCMA) then develops. Detection of CCMA is often random and requires differentiation from heart tumors or an abscess. The paper presents two cases of patients with ambiguous focal lesions of the mitral valve in echocardiography. In the first case, the cardiac computed tomography (CCT) showed a spherical, slightly irregular structure measuring approximately 33 × 22 mm, which was in contact with the posterior mitral valve leaflet from the lumen of the left ventricle. The lesion was heterogeneously intense, with an average density of about 500 HU and up to 975 HU on the periphery; it was not enhanced after the administration of a contrast agent. In the second case, the CCT revealed a heterogeneous, highly calcified structure in the peripheral zone and intermediate density in the central zone in the topography of the posterior mitral valve leaf, with dimensions up to about 41 × 31 mm in the plane of the valve leaflet, passing into the lumen of the left ventricle along its inferolateral wall to a depth of about 3.5 cm. In both cases, CCT enabled the diagnosis of CCMA. In conclusion, cardiac computed tomography may be decisive in the case of suspected caseous calcification of the mitral annulus where there is ambiguous echocardiography.

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“…1 About 10% of patients with Marfan syndrome have been reported as having MAC as uncommon cases, and massive calcification of the mitral annulus has been found in young patients with mitral valve prolapse. [2][3][4][5] The pathogenesis of MAC in Marfan syndrome has been unknown. This article reports a 38-year follow-up observation on a surgical case of mitral valve replacement for Marfan syndrome in whom MAC was revealed by the preoperative examination.…”

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Mitral Annular Calcification in Marfan Syndrome: A 38-Year Follow-Up Observation

Horiuchi

2021

Thorac Cardiovasc Surg Rep

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Background Mitral annular calcification (MAC) associated with Marfan syndrome is rare in comparison with that frequently found in elderlies with valvular disease. Case Presentation A 17-year-old woman with Marfan syndrome underwent mitral valve replacement for severe mitral regurgitation. Preoperative examination showed mitral valve prolapse and a dense C -shaped MAC. We evaluated MAC and adjacent area, respectively, in postoperative 18 and 38 years, and neither progression nor expansion of calcification was observed. Conclusion MAC associated with Marfan syndrome is more likely to be caused by stresses due to valve prolapse than by connective tissue disorder.

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Marfan's syndrome with massive calcification of the mitral annulus at age twenty-six

Cited by 16 publications

References 16 publications

Caseous mitral annular calcifications: Multimodality imaging characteristics

Caseous mitral annular calcifications: Multimodality imaging characteristics

The Importance of Cardiac Computed Tomography in the Diagnosis of Caseous Calcification of the Mitral Annulus—Case Reports

Mitral Annular Calcification in Marfan Syndrome: A 38-Year Follow-Up Observation

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