Endocrine Abstracts

Introduction: Carcinoid Heart Disease (CHD) can occur in up to 50% of patients with long standing carcinoid syndrome as a result of metastatic neuroendocrine tumour (NET). It is associated with poor prognosis due to the development of right side heart failure. High circulating levels of serotonin cause cardiac fibrosis leading to tricuspid and pulmonary valve regurgitation and poor ventricular function. Cardiac surgery with valve replacement in combination with cytoreductive surgery and control of hormonal symptoms may offer patients improved long term survival.

Methods: We describe our series of patients who presented to the regional NET multidisciplinary meeting over a 5 year period who underwent cardiac surgery for CHD.

Results: Between 2011 and 2016, eleven patients (9 female), median age 62 years with metastatic NET and CHD underwent valve replacement. The commonest tumour site was midgut (10) and 9 patients had metastatic liver disease. Tumour grade 1(7), grade 2(2) and unknown in 2 patients. Urinary 5HIAA (median 795, range 187–2690) and chromogranin A (median 1015, range 229–4047) were elevated in all patients. Octreotide treatment was commenced in all patients. Median time from diagnosis to cardiac surgery was 15 (range 3–144) months. All patients had a tissue valve replacement: 8 tricuspid and pulmonary valves; 3 tricuspid valve only; and 1 with closure of a patent foramen ovale. All underwent additional treatment (embolization or surgery), although the exact order and type varied between patients. Median follow-up after cardiac surgery was 9(range 3–65) months. There were 4 early deaths at 3,4,4&12 months all as a result of cardiac failure from progressive (3) or untreated (1) disease. Of the remaining patients, at median follow-up 23(range 4–56) months, 5 of 7 have undergone complete cytoreductive surgery and have stable disease, 1 awaiting surgery, 1 non surgical treatment.

Conclusion: Cardiac surgery is a therapeutic option for treatment of CHD. It is associated with high mortality in the presence of untreated or progressive metastatic disease. Good outcomes are achievable when patients have undergone successful cytoreductive surgery leading to good disease control.