Caspian Journal of Internal Medicine

Background: Mastocytosis is a systemic disease involving the clonal expansion of mast cells in multiple organs. Given that immune system overreaction and excessive histamine release are among the most prominent events in mastocytosis, the incidence of complications caused by immune reactions is expected to increase across various organs. While systemic manifestations of mastocytosis have been reported frequently, cardiac complications are less often discussed. These Cardiac complications can be early indicators of the disease but such uncommon features may lead to delays in diagnosis.
The significance of mast cells and histamine release in the cardiovascular system is acknowledged in prior studies.
Case Presentation: This study presents a case of recurrent prosthetic mitral valve malfunction in a 52-year-old patient with a history of cutaneous mastocytosis, who underwent mitral valve replacement three times over ten years. Despite being on appropriate anticoagulation therapy (INR: 2.5-3.5), the patient experienced recurrent prosthetic valve thrombosis. This is, to our knowledge, the first report of prosthetic mitral valve thrombosis in a patient with mastocytosis.
Conclusion: Interestingly, cardiac complications may be the first presentation of systemic mastocytosis, diagnosed long after the initial symptoms. The majority of such cases had no visible cutaneous manifestations (table 1). Regarding our case report, we recommend our colleagues to closely monitor and remain vigilant for possible cardiac symptoms of mastocytosis patients with prosthetic cardiac valves.