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Background  Patients with carcinoid syndrome (CS) may present carcinoid heart disease (CHD) but prognostic factors are not entirely understood.    Patients and Methods  Retrospective study of patients with metastatic neuroendocrine tumours (NETs) and CS and/or abnormal 24-hour-urinary 5-hydroxiindolacetic acid. CHD was defined as moderate to severe tricuspid or pulmonary regurgitation in the echocardiogram.    Results  The frequency of CHD among 42 patients was 38% (95% confidence interval [CI]: 23%–54%). CHD was associated with higher volume of liver metastases (odds ratio [OR] 13.86, 95% CI: 2.57–74.68,  p  = 0.002). Time from CS symptoms to NET diagnosis was borderline significant ( p  = 0.08). When CHD was defined as at least mild tricuspide regurgitation, the frequency of CHD was 45% and it was associated with cardiovascular comorbidities (OR: 6.58, 95% CI: 1.09; 39.78,  p  = 0.040).    Conclusion  CHD was frequent among patients with CS, significantly associated with high liver tumour burden, and likely linked to the history of cardiovascular disease and longer time of CS.

High tumour burden, delayed diagnosis and history of cardiovascular disease may be associated with carcinoid heart disease