Carcinoid Heart Disease Associated with a Primary Ovarian Stromal Carcinoid Tumor: A Case Report and a Review of the Literature

Introduction: Carcinoid heart disease (CHD) is a rare cardiac manifestation of neuroendocrine tumors, typically associated with hepatic metastases. Primary ovarian carcinoid tumors represent an uncommon source of CHD and may lead to cardiac involvement in the absence of metastatic disease. Case Presentation: A 51-year-old Iranian woman presented with progressive exertional dyspnea, bilateral lower extremity edema, and episodes of facial flushing. She had undergone total abdominal hysterectomy and bilateral salpingo-oophorectomy for a primary ovarian stromal carcinoid tumor 3 months prior. Transthoracic echocardiography revealed severe tricuspid regurgitation with thickened, retracted valve leaflets and impaired coaptation, while the pulmonary valve was spared. Laboratory findings were within normal limits except for a markedly elevated 24-h urinary 5-hydroxyindoleacetic acid (5-HIAA) level (128 mg/24 h). Cross-sectional imaging, including chest CT and abdominal-pelvic MRI, showed no evidence of hepatic or pulmonary metastases. A diagnosis of isolated CHD secondary to a primary ovarian carcinoid tumor was established. The patient declined surgical intervention and was discharged against medical advice. Conclusion: This case highlights a rare presentation of CHD originating from a primary ovarian carcinoid tumor without metastatic involvement. Clinicians should consider this diagnosis in patients with unexplained right-sided valvular disease and features of carcinoid syndrome, even in the absence of liver metastases.