Progress in the medical and surgical management of patients with carcinoid disease has resulted in improved symptoms and survival. Carcinoid heart disease remains a major cause of morbidity and mortality among patients with malignant carcinoid syndrome. Limited medical treatment options are available for patients with symptomatic carcinoid heart disease. At the Mayo Clinic (Rochester, MN), we have taken an aggressive approach to severe valvular dysfunction from carcinoid heart disease. Patients with severe carcinoid heart disease currently are referred for cardiac operation when they develop cardiac symptoms, ventricular dysfunction, or (rarely) in anticipation of hepatic surgery. Surgical outcome depends on patient age and functional class at the time of cardiac surgery. Despite metastatic disease that limits longevity, cardiac surgical survivors usually demonstrate dramatic improvement in functional capacity. Cardiac surgery should be considered early for patients with symptomatic carcinoid heart disease and controlled carcinoid symptoms. An experienced medical, surgical, and anesthetic team approach to the patient with carcinoid heart disease is critical in order to provide state of the art management.