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(Circulation. 2005;112:3320-3327.)
© 2005 American Heart Association, Inc.

Valvular Heart Disease

Prognosis of Carcinoid Heart Disease

Analysis of 200 Cases Over Two Decades

Jacob E. Møller, MD, PhD; Patricia A. Pellikka, MD; Alain M. Bernheim, MD; Hartzell V. Schaff, MD; Joseph Rubin, MD; Heidi M. Connolly, MD

From the Division of Cardiovascular Diseases (J.E.M., P.A.P., A.M.B., H.M.C.), Division of Cardiovascular Surgery (H.V.S.), and Division of Medical Oncology (J.R.), Mayo Clinic, Rochester, Minn.

Correspondence to Patricia A. Pellikka, MD, Mayo Clinic, 200 First St SW, Rochester, MN 55905. E-mail pellikka.patricia{at}mayo.edu

Received April 4, 2005; revision received August 26, 2005; accepted September 12, 2005.

Background— The long-term prognosis of patients who develop carcinoid heart disease and the effect of cardiac surgery on outcome are not well established.

Methods and Results— In this retrospective study, we identified 200 patients with carcinoid syndrome referred for echocardiography in whom the diagnosis of carcinoid heart disease was confirmed. Patients were divided into 3 groups of similar size according to the date from first diagnosis of carcinoid heart disease. Group A comprised patients diagnosed from 1981 through June 1989; group B, diagnosed July 1989 through May 1995; and group C, June 1995 through 2000. The end point was all-cause mortality. Median survival was significantly lower in group A (1.5 years, 95% CI 1.1 to 1.9 years) compared with groups B (3.2, 95% CI 1.3 to 5.1 years) and C (4.4, 95% CI 2.4 to 7.1 years; P=0.009). In a multivariate model adjusted for treatment and clinical characteristics, the risk of death in groups B (hazard ratio 0.67, 95% CI 0.46 to 0.99, P=0.04) and C (hazard ratio 0.61, 95% CI 0.39 to 0.92, P=0.006) was significantly reduced relative to group A. Cardiac surgery was performed in 87 patients. When cardiac surgery was included as a time-dependent covariate in a multivariate analysis, it was associated with a risk reduction of 0.48 (95% CI 0.31 to 0.73, P<0.001), whereas the time period of diagnosis was no longer significant.

Conclusions— The prognosis of patients with recognized carcinoid heart disease has improved over the past 2 decades at our institution. This change in survival may be related to valve replacement surgery.

Key Words: carcinoid • echocardiography • prognosis • surgery • valves

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