Caseous Calcification of the Mitral Annulus: Calcified Toothpaste of the Heart.
| Autor: | Jorgenson KM; From the University of Rochester Medical Center, Rochester, NY., McMullen PD, Goldman BI; From the University of Rochester Medical Center, Rochester, NY., Jacob-Leonce M; From the University of Rochester Medical Center, Rochester, NY. |
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| Jazyk: | angličtina |
| Zdroj: | The American journal of forensic medicine and pathology [Am J Forensic Med Pathol] 2024 Jul 18. Date of Electronic Publication: 2024 Jul 18. |
| DOI: | 10.1097/PAF.0000000000000971 |
| Abstrakt: | Abstract: 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. Competing Interests: Sources of support: The authors have no funding, financial relationships, or conflicts of interest to disclose. (Copyright © 2024 The Author(s). Published by Wolters Kluwer Health, Inc.) |
| Databáze: | MEDLINE |
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