Carcinoid Heart Disease

Overview

Demonstrated are eight cases of carcinoid heart disease (7 females, 1 male; average age 63.4 y.).The primary tumor was localized in the ileum in 5, and in the cecal area in 3 patients, with livermetastases in each case. The characteristic heart lesion appears as an endocardial plaque composedof loose intercellular matrix with scattered smooth muscle cells, covered by an endotheliallayer. The plaques are apposed on an otherwise normal valvular, or mural endocardium of theright heart. The resulting combined valvular lesions show a predominance of tricuspid incompetence,or of pulmonary stenosis. In one case with a widely patent foramen ovale there was involvementof left-heart valves in addition. Local effect of serotonin appears to play the main role inpathogenesis of the endocardial plaques.

Key words:
carcinoid – carcinoid syndrome – tricuspid incompetence – pulmonary stenosis