Severe tricuspid regurgitation as first manifestation of a primary ovarian carcinoid tumor

Abstract

A 52-year-old woman presented with a 1 year-history of reduced exercise tolerance and fatigue. Physical examination revealed a grade II/VI systolic murmur at the left sternal border. Transthoracic echocardiography showed thickened tricuspid leaflets with retraction and restricted movement, resulting in a severe tricuspid regurgitation. The characteristic pattern aroused the suspicion of a systemic or hormonal etiology. 24-Hour urine analysis revealed an increased level of 5hydroxyindoleacetic acid and abdominal computed tomography (CT) showed a large tumor in the pelvis; however, without the presence of liver metastasis. During uncomplicated trans-abdominal surgery a large mass in the ovary was removed, and pathology showed a primary ovarian carcinoid tumor. One year after the diagnosis, patient remains clinically stable without signs of right heart failure and an excellent exercise tolerance. This case report shows that isolated tricuspid valve pathology in the absence of left valvular disease may raise the suspicion for an atypical cause of valvular dysfunction, in which carcinoid tumor is included.