Endocrine Abstracts

Carcinoid heart disease (CHD) affects about 20-50% of patients with carcinoid syndrome (CS) and is the main cause of mortality in this group of patients. The 3-year survival rate in patients with CHD is 31% compared to 68% in those without cardiac involvement. The cause of the development of CHD is the flow of serotonin-rich blood, and the lesions affect the right heart (localizing mainly in the endocardium and inner membrane of large vessels). The study aim was to present a single centre experience with CHD. Ten patients with CHD selected from the group of 162 patients with disseminated and/or inoperable neuroendocrine tumours treated with long-acting somatostatin analogues were included in a retrospective analysis. The study group consisted of 2 females (median age 72 years) and 8 males (median age 61,5 years). There were 2 patients with NET G1 and 6 patients with NET G2. In 8/10 patients the primary site was small intestinal NET (in 100% of these patients the primary site was resected), in 2 cases the primary site was unknown. Elevated serum chromogranin A (CgA) concentration with a median value 105,5 nmol/l (range 42,5-255) [reference value 0,0 - 6,0] was observed in all patients, and 5-hydroxy indoleacetic acid (5-HIAA) excretion in 24-hour urine collection was also elevated in all patients - median value 71,4 mg/24h (range 21,3-766) [reference value 0 - 8,2]. N-terminal pro-B-type natriuretic peptide (NT-proBNP) concentrations were above the upper limit of normal in 9/10 patients: median value 390 pg/ml (range 245-11162) [reference value < 125]. Two patients presented symptoms of heart failure in NYHA I class, 3-NYHA II, 3-NYHA III and 2-NYHA IV. In all patients tricuspid regurgitation was confirmed in a transthoracic two-dimensional echocardiography (in 6 cases moderate, and in 4 severe). Two patients underwent a cardiac surgery for tricuspid valve replacement (under the cover of a short-acting somatostatin analogue). Two patients were treated with Peptide Receptor Radionuclide Therapy (PRRT) receiving 4 cycles of [177Lu]Lu-DOTATATE. Six patients from the study group are alive, of whom five have CS symptoms well controlled with a long-acting somatostatin analogue. CHD is a life-threatening complication of CS. CgA, 5-HIAA and NT-proBNP are screening tools to diagnose this state and transthoracic two-dimensional echocardiography is essentialfor imaging CHD. Multidisciplinary approach is mandatory to offer a patient a proper treatment.