Cardiac surgery for carcinoid heart disease.

Carcinoid is a rare tumor originating from neuroendocrine cells, which are generally localized in the gastrointestinal tract. It has an annual incidence of 1.5 persons per 100 000. Usually, only carcinoid tumors that invade the liver result in pathological changes to the heart. The cardiac manifestations are caused by paraneoplastic effects of vasoactive substances such as serotonin, bradykinin, tachykinins, histamine, and prostaglandins that are released by the malignant cells, rather than by direct cardiac metastatic involvement. Carcinoid heart disease is manifested by structural changes in the right heart secondary to severe fi brotic endocardial plaques resulting from elevated blood concentrations of these vasoactive substances. Therefore, carcinoid heart disease is a rare but important cause of intrinsic tricuspid and pulmonary valve disease, leading to signifi cant morbidity and mortality secondary to right heart failure. Mitral or aortic valve dysfunction is less frequent and presents usually as regurgitation. Because the vasoactive substances that are produced by the tumor are metabolized in the lungs, the presence of left heart disease usually refl ects the presence of a persistent foramen Received: 27 January 2011 © The Japanese Association for Thoracic Surgery 2011