Published Online
on November 14, 2005

A more recent version of this article appeared on November 22, 2005

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Submitted on April 4, 2005
Revised on August 26, 2005
Accepted on September 12, 2005

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, and 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.

^* To whom correspondence should be addressed. E-mail: pellikka.patricia{at}mayo.edu.

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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