title: Factors associated with progression of carcinoid heart disease.
abstract: BACKGROUND
  By releasing vasoactive substances into the circulation, carcinoid tumors can cause right-sided valvular heart disease. Factors associated with the progression of carcinoid heart disease are poorly understood. We conducted a retrospective study to identify such factors.
  
  
  METHODS
  Our sample included 71 patients with the carcinoid syndrome who underwent serial echocardiographic studies performed more than one year apart and 32 patients referred directly for surgical intervention after an initial echocardiographic evaluation. A score for carcinoid heart disease was determined on the basis of an assessment of valvular anatomy and function and the function of the right ventricle. An increase of more than 25 percent in the score between studies was considered suggestive of disease progression. Tumor progression was assessed on the basis of abdominal computed tomographic scans and changes in the level of urinary 5-hydroxyindoleacetic acid (5-HIAA), a metabolite of serotonin.
  
  
  RESULTS
  Of the patients with serial echocardiographic studies, 25 (35 percent) had an increase of more than 25 percent in the cardiac score. As compared with patients whose score changed by 25 percent or less, these patients had higher urinary peak 5-HIAA levels (median, 265 mg per 24 hours [interquartile range, 209 to 593] vs. 189 mg per 24 hours [interquartile range, 75 to 286]; P=0.004) and were more likely to have biochemical progression (10 of 25 patients vs. 9 of 46, P=0.05) and to have received chemotherapy (13 of 25 vs. 10 of 46, P=0.009). Logistic-regression analysis showed that a higher peak urinary 5-HIAA level and previous chemotherapy were predictors of an increase in the cardiac score that exceeded 25 percent (odds ratio for each increase in 5-HIAA of 25 mg per 24 hours, 1.08 [95 percent confidence interval, 1.03 to 1.13]; P=0.009); odds ratio associated with chemotherapy, 3.65 [95 percent confidence interval, 1.74 to 7.48]; P=0.001).
  
  
  CONCLUSIONS
  Serotonin is related to the progression of carcinoid heart disease, and the risk of progressive heart disease is higher in patients who receive chemotherapy than in those who do not.
authors.affiliation: Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minn 55905, USA.
authors.forename: Jacob E
authors.initials: JE
authors.lastname: Møller
authors.forename: Heidi M
authors.initials: HM
authors.lastname: Connolly
authors.forename: Joseph
authors.initials: J
authors.lastname: Rubin
authors.forename: James B
authors.initials: JB
authors.lastname: Seward
authors.forename: Karen
authors.initials: K
authors.lastname: Modesto
authors.forename: Patricia A
authors.initials: PA
authors.lastname: Pellikka
mesh_terms: D000368:Aged; D002275:Carcinoid Heart Disease / Q000145:classification / Q000000981:diagnostic imaging / Q000652:urine; D018450:Disease Progression; D015150:Echocardiography, Doppler; D005260:Female; D006349:Heart Valve Diseases / Q000000981:diagnostic imaging; D006801:Humans; D006897:Hydroxyindoleacetic Acid / Q000652:urine; D016015:Logistic Models; D008137:Longitudinal Studies; D008297:Male; D008303:Malignant Carcinoid Syndrome / Q000000981:diagnostic imaging; D008875:Middle Aged; D011859:Radiography; D012189:Retrospective Studies
chemical_list: D006897:Hydroxyindoleacetic Acid