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 diseaseare poorly understood. We conducted a retrospective study toidentify such factors.
Methods Our sample included 71 patients with the carcinoid syndromewho underwent serial echocardiographic studies performed morethan one year apart and 32 patients referred directly for surgicalintervention after an initial echocardiographic evaluation.A score for carcinoid heart disease was determined on the basisof an assessment of valvular anatomy and function and the functionof the right ventricle. An increase of more than 25 percentin the score between studies was considered suggestive of diseaseprogression. Tumor progression was assessed on the basis ofabdominal computed tomographic scans and changes in the levelof urinary 5-hydroxyindoleacetic acid (5-HIAA), a metaboliteof serotonin.
Results Of the patients with serial echocardiographic studies,25 (35 percent) had an increase of more than 25 percent in thecardiac score. As compared with patients whose score changedby 25 percent or less, these patients had higher urinary peak5-HIAA levels (median, 265 mg per 24 hours [interquartile range,209 to 593] vs. 189 mg per 24 hours [interquartile range, 75to 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 receivedchemotherapy (13 of 25 vs. 10 of 46, P=0.009). Logistic-regressionanalysis showed that a higher peak urinary 5-HIAA level andprevious chemotherapy were predictors of an increase in thecardiac score that exceeded 25 percent (odds ratio for eachincrease in 5-HIAA of 25 mg per 24 hours, 1.08 [95 percent confidenceinterval, 1.03 to 1.13]; P=0.009); odds ratio associated withchemotherapy, 3.65 [95 percent confidence interval, 1.74 to7.48]; P=0.001).
Conclusions Serotonin is related to the progression of carcinoidheart disease, and the risk of progressive heart disease ishigher in patients who receive chemotherapy than in those whodo not.
Source Information
From the Divisions of Cardiovascular Diseases (J.E.M., H.M.C., J.B.S., K.M., P.A.P.) and Medical Oncology (J.R.), Mayo Clinic, Rochester, Minn.
Address reprint requests to Dr. Pellikka at the Echocardiography Laboratory, Gonda 6-138 NW, Mayo Clinic, 200 First St. SW, Rochester, MN 55905, or at pellikka.patricia{at}mayo.edu.
Carcinoid Heart Disease
van der Horst-Schrivers A. N.A., Wymenga A. N.M., de Vries E. G.D., Zuetenhorst J. M., Taal B. G., Møller J. E., Connolly H. M., Pellikka P. A.
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N Engl J Med 2003;
348:2359-2361, Jun 5, 2003.
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