Carcinoid heart disease and recurrent hypertensive crises in a patient with primary high-grade ovarian neuroendocrine carcinoma

Background: Carcinoid tumors compose approximately 0.1% of all ovarian carcinomas and can cause carcinoid syndrome and valve disease without metastases due to systemic venous drainage bypassing liver metabolism. Carcinoid valve disease can result in significant clinical heart failure despite it being typically well tolerated clinically. Hypertensive crisis is a potential presentation of carcinoid syndrome, but it is not well described in the literature. Case: We present a case of a 41-year-old African American female who developed carcinoid heart disease from a primary high grade left ovarian neuroendocrine carcinoma. The patient was referred to our cardiology clinic for new onset, refractory hypertension to multiple anti-hypertensive agents as well as 30-pound weight gain and symptoms of carcinoid syndrome and congestive heart failure. Echocardiogram revealed moderate tricuspid leaflet and annulus thickening with reduced tricuspid opening and turbulent high-velocity diastolic flow across the tricuspid valve. Serum serotonin was markedly elevated at 2,237. The patient underwent surgical resection of the left adnexal mass found on pelvic computed tomography scan. Frozen section showed stage 1A, grade 3 neuroendocrine ovarian carcinoma. On follow-up, the patient experienced resolution of her preexisting uncontrolled hypertension, carcinoid syndrome and heart failure symptoms. Her valvular disease has not progressed on serial clinical and echocardiographic follow-up. Conclusion: Carcinoid syndrome is an extremely rare cause of secondary hypertension, particularly in patients without hepatic metastases. In this case, we present a patient with primary ovarian neuroendocrine carcinoma with presenting symptoms most notable for hypertensive crises, which to the best of our knowledge has not been reported. Carcinoid tumors should be considered in patients with new-onset, poorly controlled hypertension, particularly in females where ovarian tumors’ ability to bypass portal circulation can lead to rapid progression of disease.