Cardiac disease and midgut carcinoid syndrome

Abstract

Abstract Background Valvular heart disease is a well known complication of the midgut carcinoid syndrome but its impact on survival and effects of modern tumour treatment remain to be analysed. Methods In a prospective study cardiac abnormalities and clinical outcome were analysed in 64 consecutive patients with high urinary excretion of the serotonin metabolite 5-HIAA and carcinoid tumours with liver metastases (mean(s.d.) follow-up 119(7) months). A tumour reduction programme by radical surgery alone (including hemihepatectomy) or with hepatic artery embolization following primary surgery (in bilobar liver disease) was offered to all patients. At entry into the study, patients underwent Doppler echocardiography, exercise electrocardiography, Holter monitoring and urinary 5-HIAA determination. Results At follow-up, patients were grouped according to clinical outcome. Twenty-four patients were alive, 11 after radical surgery alone (group 1) and 13 after embolization plus octreotide (group 2). Forty patients had died during follow-up (group 3), three with radical surgery (unrelated disease) and 27 patients with embolization plus octreotide. Ten deceased patients had medical therapy alone after primary surgery owing to complicating diseases. The mean 5-HIAA levels before treatment were lower in group 1 (185 mol per 24 h) than in group 2 (956 (mol per 24 h) or group 3 (566 (mol per 24 h). Tricuspid valve structural abnormalities (TSA) were found in 13, 38 and 33 per cent, and holosystolic tricuspid regurgitation (TR) in 36, 54 and 60 per cent of patients in groups 1, 2 and 3 respectively. No patient had pulmonary stenosis, but 9 per cent (group 1), 15 per cent (group 2) and 13 per cent (group 3) had holodiastolic pulmonary regurgitation. A novel index (TSA–TR) of TSA (0–4) and TR (0–4) was formed. Patients with TSA–TR (3 had significantly (P = 0·025) higher 5-HIAA levels than patients with an index (1. Patients with 5-HIAA levels above 500 (mol per 24 h demonstrated a significantly (P = 0·04) higher late-to-early peak ratio (A/E) of diastolic tricuspid Doppler flow than patients with 5-HIAA levels less than 500 (mol per 24 h. Of the six patients who suffered cardiac death, five died from ischaemic heart disease or sudden death and one from advanced right heart failure. Conclusion Tricuspid abnormalities were common findings in patients with the midgut carcinoid syndrome and severe findings were related to high urinary excretion of 5-HIAA. The highest initial hormone levels were seen in one of the surviving groups. Sudden or ischaemic heart death was more common than death from right-sided heart failure.