Abstract

Carcinoid heart disease was first described in 1954. It develops in over 70% of carcinoid syndrome patients. Up to 20% of patients with carcinoid syndrome have pronounced carcinoid heart disease at diagnosis. Cardiac involvement is usually well tolerated by patients. Symptoms are associated with secretion of serotonin, tachykinin and other vasoactive substances in the systemic circulation. Deposition of fibrous tissue along the tricuspid annulus leads to ring constriction and is the basis of tricuspid stenosis. Primary insular carcinoid tumor of the ovary is a very rare tumor that accounts for less than 1% of all carcinoid cases. We present a case of a 62-year-old woman, demonstrating shortness of breath and fatigue, diarrhea, redness and progressive swelling on both lower legs. After single-photon emission computed tomography we found pathologically increased somatostatin expression in a tumor formation in the pelvis. After removal of the primary focus, the patient received a prosthetic tricuspid valve with good clinical results.

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