<b><i>Objectives:</i></b> Myocardial fibrosis causes deterioration of myocardial function in carcinoid intestinal disease (CID). We assessed the ability of myocardial function and various biomarkers to predict mortality in patients with CID. <b><i>Methods:</i></b> A total of 71 patients with small intestinal carcinoid tumours were included, and underwent echocardiography at baseline. Systolic function was assessed by two-dimensional speckle tracking echocardiography as left ventricular (LV) and right ventricular (RV) strain, and as mitral annular displacement (MAD), by tissue Doppler imaging. Parameters of diastolic function, the amount of liver metastases, and various biomarkers were also analysed. <b><i>Results:</i></b> During 1,274 ± 368 days of follow-up, 18 patients (25%) died. Of the 53 survivors, 46 patients (87%) were available for follow-up echocardiography. Baseline LV strain and MAD was reduced in those who died compared to those who survived (p < 0.001). Baseline plasma levels of activin A were markedly higher in patients who died during follow-up than those who survived (p = 0.001). In multivariate Cox hazard models (A, B, C and D), LV strain, age, the amount of liver metastases, MAD, and activin A were independent predictors of mortality. <b><i>Conclusion:</i></b> Assessment of myocardial function by echocardiography, and the biomarker activin A, can be very useful in the risk stratification of patients with CID.