Carcinoid Heart Disease: A Rare Complication of Primary Ovarian Carcinoid Tumor
Document Type
Article
Abstract
Background: Carcinoid heart disease is a rare manifestation of an exceedingly rare neuroendocrine malignancy. Pathological cardiovascular changes in carcinoid syndrome occur secondary to the release of vasoactive neuroendocrine mediators. It is thought that these cause the formation of fibrous lesions along the tricuspid and pulmonary valves which subsequently precipitate right heart failure. Specificity of the pathology to the right heart is due to the ability of the lungs to inactivate these mediators prior to reaching the left heart.
Case: A 56 year old female without significant past medical history presented with severe pelvic pain, renal failure and congestive heart failure. Physical examination demonstrated significant jugular venous distention, a grade II/VI holosystolic murmur at the left sternal border and bilateral lower extremity edema. Serum Creatinine was 3.19 mg/dL, Chromogranin A was 42,980 ng/mL and whole blood Serotonin was 3,640 ng/mL. Computed tomography without contrast demonstrated a large pelvic mass. Echocardiography demonstrated severe dilatation of the right atrium and ventricle, dilated tricuspid annulus with malcoaptation of the tricuspid leaflet, torrential tricuspid regurgitation, moderate pulmonary regurgitation, moderate pulmonary hypertension and ejection fraction of 65%.
Decision-making: This case highlights the importance of maintaining carcinoid heart disease on the differential for patients presenting with right heart failure in the setting of an ovarian mass. Exploratory laparotomy with right and left salpingo-oopherectomy was performed and right heart failure was managed with pharmacotherapy. While ovarian carcinoid tumors are rare, valvular heart disease occurs in 20% of patients that present with ovarian carcinoid. Valve replacement must always be considered in patients with carcinoid mediated cardiovascular pathology.
Conclusion: Clinicians must work expeditiously to identify the etiology of right heart failure, as management differs based on the causative factors and appropriate intervention can alleviate symptomology and prolong life.
First Page
2911
DOI
10.1016/S0735-1097(20)33538-5
Publication Date
3-24-2020
Recommended Citation
Joshi A, Kurtz E, Vadhar S, Sheikh T, Hess K, Boozel T, Rajeswaran Y. CARCINOID HEART DISEASE: A RARE COMPLICATION OF PRIMARY OVARIAN CARCINOID TUMOR. Journal of the American College of Cardiology. 2020 Mar 24;75(11_Supplement_1):2911-.