Prevalence of carcinoid heart disease in patients with gastrointestinal neuroendocrine tumors in a single-center prospective registry

Carcinoid heart disease (CHD) caused by neuroendocrine tumors (NET) is associated with increased morbidity and mortality due to valvular dysfunction and right-sided heart failure. This study aims to assess the prevalence and one-year incidence of CHD in NET patients. Tumor characteristics, laboratory measurements, and echocardiographic findings were examined to identify predictors of CHD development.

The study was an investigator-initiated, monocentric, prospective trial. Patients with gastroenterological NET without previously diagnosed CHD were included and underwent oncological and cardiological diagnostics. Transthoracic echocardiography (TTE) and blood test were performed at baseline.

Thirty-nine NET patients (mean age 67 ± 11 years) were enrolled into the study and received TTE. A total of four patients exhibited signs of CHD (mean age 69 ± 8.8 years). Four patients showed classical right sided carcinoid heart disease with high-grade tricuspid regurgitation. Two of these patients also exhibited mild to moderate right ventricular dysfunction. One NET patient exhibited aortic stenosis and another had a history of an Ebstein Anomaly. In such cases, the differentiation between preexisting or concomitant valvular disease and its interaction with NET and carcinoid heart disease is difficult.

NT-proBNP was significantly elevated in CHD patients compared to NET patients without CHD (810.25 pg/mL ± 651.7 vs. 175.15 pg/mL ± 149.3, p = 0.02) as was 5-Hydroxyindoleacetic acid (5-HIAA) in 24h urine (67 mg ± 26 vs. 11 mg ± 8, p < 0.01) and Troponin T (21 ng/L ± 5 vs. 10.6 ng/L ± 4, p < 0.01). Chromogranin A showed no significant difference (889.25 ng/mL ± 1040.93 vs. 584.54 ng/mL ± 1066.76, p = 0.64) between groups.

10.26% (n = 4) of NET patients showed signs of CHD, all of which showed right-sided carcinoid heart disease. CHD patients exhibited significantly elevated cardiac biomarkers such as proBNP and Troponin T, as well as increased 5-HIAA in 24h urine. The comparatively mild severity of CHD in our patient collective might be due to improvements in the pharmacological treatment of NETs. One CHD patient underwent TriClip implantation with a good result, highlighting the possibility of interventional treatment in these patients.

TTE and TEE in CHD