Endocrine Abstracts

Case History: We present the case of a 77 year old female, who presented to the cardiology team with progressive shortness of breath and signs of heart failure. Echocardiogram showed pulmonary hypertension, tricuspid, pulmonary and aortic regurgitation and atrial septal defect with a bidirectional shunt. She also reported a 5 year long history of diarrhoea, flushing and wheezing. The Echocardiographic features together with the chronic symptoms raised suspicion for carcinoid syndrome and carcinoid heart disease (CHD). Abdominal computed tomography showed a 6 cm left adnexal mass without hepatic metastasis suspicious of ovarian carcinoid.

Results and Treatments: 24 H Urine 5HIAA level was elevated at 781 umol/l (0-50 umol/l). A Gallium 68-DOTATATE Scan demonstrated high SSTR expression in the left adnexal mass, consistent with ovarian carcinoid and no features of hepatic or distant metastases. Short acting somatostatin analogue therapy (Octreotide 100 mg three times a day subcutaneously) was commenced while awaiting cardiac surgery and intravenous Octreotide as a bolus followed by a continuous infusion was given perioperatively in line with our local guidance. She underwent multivalve replacement (tricuspid, aortic and pulmonary), repair of the septal defect and a pacemaker insertion. Histopathology confirmed features in keeping with CHD. Post operatively, long acting subcutaneous somatostatin analogue therapy (Lanreotide 120 mg every 28 days) was started. Three months later, she underwent a laparoscopic total abdominal hysterectomy and bilateral salpingo-oophorectomy. Histopathology and post operative 24H Urine 5 HIAA levels are awaited.

Conclusion and Point of Discussion: CHD can occur as a complication of carcinoid syndrome, which is typically associated with carcinoid tumour of gastrointestinal tract(GIT)with hepatic metastasis. GIT carcinoids secrete large amount of vasoactive peptides such as serotonins which are metabolised by the liver before entering into the systemic circulation. Ovarian carcinoids are rare accounting for <0.1% of ovarian cancers and only 1% of carcinoid tumours. CHD caused by ovarian carcinoids without liver metastasis is a rare form of CHD. In ovarian carcinoid, the drainage of the ovary bypasses the liver on route to the inferior vena cava allowing vasoactive peptides to be secreted into the circulation. Left sided valve disease in this case is likely due to the ASD. The case is a rare case of CHD due to ovarian carcinoid with both right and left sided valvular disease who has been successfully managed through curative cardiac and gynecological surgery