Carcinoid heart disease is the collective term for all cardiac manifestations in patients who have carcinoid syndrome. Carcinoid heart disease has a multifactorial pathophysiology, and the right side of the heart is usually involved. Symptoms and signs vary depending upon the affected cardiac components; most typical is right-sided heart failure secondary to diseased tricuspid and pulmonary valves. Despite no single ideal diagnostic test, strong suspicion, coupled with serologic and imaging results, usually enables diagnosis. Advances in imaging, such as speckle-tracking echocardiography and cardiac magnetic resonance, have improved the diagnostic yield. Treatment is challenging, warrants a multidisciplinary approach, and can be medical or surgical depending on the cardiac manifestations. Investigators are exploring the therapeutic use of monoclonal antibodies and new somatostatin analogues. In this review, we cover current knowledge about the pathophysiology, diagnosis, and treatment of carcinoid heart disease.
Keywords: Biomarkers/blood; carcinoid heart disease/blood/diagnostic imaging/physiopathology/therapy/urine; carcinoid tumor/complications/diagnosis/epidemiology/mortality/pathology/surgery; disease progression; heart valve diseases/complications; malignant carcinoid syndrome/pathology; neuroendocrine tumors/pathology; pulmonary valve insufficiency/etiology; serotonin/metabolism; tricuspid valve insufficiency/etiology.