Prediction of prognosis by echocardiography in patients with midgut carcinoid syndrome

The association between malignant midgut carcinoid tumours and right-sided cardiac lesions is well known, but the pathogenetic link between tumour sec retion and valvular disease is still obscure. The purpose of this investiga tion was to describe the morphological and functional changes of valvular h eart disease in a large patient series and to correlate these findings with hormonal secretion and prognosis. Of 64 consecutive patients with the midgut carcinoid syndrome followed betw een 1985 and 1998, valvular heart disease was evaluated in 52 patients by t wo-dimensional echocardiography, Doppler estimation of valvular regurgitati on and flow profiles. A majority was also evaluated with exercise electroca rdiography and spirometry. Structural and functional abnormalities of the tricuspid valve were found i n 65 per cent of patients, while only 19 per cent had pulmonary valve regur gitation. Long-term survival was related to excessive urinary excretion of 5-hydroxyindole acetic acid of over 500 mu mol in 24 h, but the main predic tor of prognosis was the presence of severe structural and functional abnor malities of the tricuspid valve. Although advanced tricuspid abnormalities were prevalent in this series, only one patient died from right ventricular heart failure. Tricuspid valvular disease is a common manifestation of the midgut carcinoi d syndrome and advanced changes are associated with poor long-term survival . Active surgical and medical therapy of the tumour disease reduced the hor monal secretion and, combined with cardiological surveillance, made right v entricular heart failure a rare cause of death in these patients.