Carcinoid Heart Disease

Abstract

Carcinoid is a rare neuroendocrine tumor that typically originates in the gastrointestinal tract and can result in a constellation of symptoms, mediated by vasoactive substances, referred to as carcinoid syndrome. Carcinoid valve and heart disease is characterized by the plaque-like, endocardial fibrous tissue deposits, primarily affecting the right heart endocardium and valves, which result as a consequence of the disease process. Potential mechanisms for the carcinoid valve disease include the complex role of excess serotonin and its interaction with serotonin receptors and transporters. Carcinoid valve and heart disease is a frequent occurrence in patients with carcinoid syndrome and is accountable for substantial morbidity and mortality. Cardiac surgery remains the most effective treatment option for carcinoid valve disease and a multidisciplinary approach at an experienced center is recommended for patients with metastatic carcinoid and carcinoid heart disease. This chapter provides an overview of the pathophysiology, clinical presentation and treatment of carcinoid heart disease as well as early and late outcomes of valve replacement in this condition.