Cardiovascular outcomes evaluation in a cohort of patients with neuroendocrine tumors and carcinoid syndrome

Abstract Introduction Neuroendocrine neoplasms (NENs) are a rare entity, usually late- diagnosed condition, which may be related to the presence of carcinoid syndrome explained by hormonal activity, usually derived from the action of serotonin or derivates, which generates a clinical picture of flushing, diarrhea and bronchoespasm. In these patients, a high probability of carcinoid heart disease (CHD) has been detected, that usually affects the right heart valves and has a serious prognostic impact. Purpose To define the cardiovascular clinical outcomes of a cohort of patients with gastroenteropancreatic neuroendocrine tumors, associated with the presence of carcinoid syndrome by clinical presentation or elevation of 5-hydroxyindoleacetic acid levels. Methods In a high-complexity oncology institution, a descriptive analysis of patients with NENs was performed, based on the institutional macro-project for evaluating real-world evidence, from July 2022 to July 2024. Based on symptoms of carcinoid syndrome and/or levels of chromogranin A and 5-hydroxyindoleacetic acid, patients were identified for monitoring of cardiovascular clinical variables, baseline and follow-up echocardiographic variables during the observation period. Results Forty-seven patients with gastroenteropancreatic NENs were identified in the two years of inclusion, with the presence of carcinoid syndrome found in 7 patients (15.5%), the latter being analyzed based on their cardiovascular symptoms, echocardiographic and clinical variables, finding 3 cases of carcinoid heart disease (42.8%). The three patients had an institutional echocardiographic evaluation, 2 with tricuspid insufficiency described as moderate and 1 as severe, with progression of the two moderate insufficiencies to severe in approximately 6 months of re-evaluation, mild valvular thickening and restriction in tricuspid valvular motility. Peptide values were only performed in the three patients with a confirmed diagnosis of carcinoid heart disease (CHD) and taken late, so their average values were 3612 pg/ml (982 – 8495 pg/ml) and highest level was found in the patient who died. Two of the patients with a confirmed diagnosis of carcinoid heart disease required surgical management for tricuspid valve replacement and one died from right heart failure attributable to carcinoid heart disease. All seven patients with carcinoid syndrome were referred for evaluation and clinical and echocardiografic follow-up in the institutional Cardio-Oncology service. Conclusions Patients with NENs and carcinoid syndrome identified by symptoms or by elevated levels of 5-HIAA, had an incidence of carcinoid heart disease of 42% in our study and require close follow-up by Cardio-Oncology to define earlier the benefit of valvular surgical intervention or regular screening with peptide levels.Carcinoid heart Disease, tricuspid valve CHD in carcinoid Syndrome