ECHOCARDIOGRAPHIC AND BIOCHEMICAL EVALUATION OF THE DEVELOPMENT AND PROGRESSION OF CARCINOID HEART-DISEASE

Objectives. To study the applicability of a newly developed echocardio graphic scoring system in the assessment of carcinoid valvular heart d isease. Background. We investigated prospectively the development, pro gression and regression of carcinoid valvular heart disease in patient s,vith carcinoid syndrome by serial echocardiography, correlating thes e features with urinary 5-HIAA levels and clinical data collected duri ng therapy with somatostatin analog. Methods. Twenty-three patients wi th carcinoid syndrome underwent serial echocardiographic examinations. An echocardiographic carcinoid valvular heart disease (CVHD) % score was determined from points assigned for tricuspid and pulmonary valve structure and function. Results. Fifteen patients had no CVHD at study entry (group 1), while 8 patients had findings of CVHD (group 2). Fiv e patients in group 1 developed new CVHD (1B), while one demonstrated progression of CVHD (2B). The remaining patients did not develop (1A) or had no progression of CVHD (2B). Despite major declines in 5-HIAA l evels during therapy in most patients, CVHD did not regress. There wer e significantly lower levels of median baseline 5-HIAA (98.8 vs. 256 m g/24 h), posttreatment 5-HIAA (50.3 vs. 324 mg/24 h) and posttreatment 5-HIAA time integral (37.3 vs. 192 g/24 h days) in group A vs. B (p < 0.05). However, only posttreatment 5-HIAA levels independently predi cted the development or progression of CVHD by multiple step-wise regr ession analysis (p < 0.005), with a threshold observed in the 100 mg/2 4 h range. Conclusions. We designed a new echocardiographic scoring sy stem to evaluate CVHD. Correlating echocardiographic scores with bioch emical and clinical markers showed that only posttreatment 5-HIAA leve ls independently predicted the development or progression of CVHD. Thi s study strengthens the association between serotonin secretion and CV HD, as well as introducing a new technique for serial follow-up of the se patients. (C) 1998 by the American College of Cardiology.