Carcinoid Heart Disease: Review of Current Knowledge

Abstract

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.

Carcinoid Syndrome: A Review

“…In many patients, the cause of death is attributed directly to cardiac disease. 5-HT is inactivated in the lungs so left-heart involvement is rare and may indicate an intra-atrial shunt [14][15]. Uncommonly, CS may cause bronchoconstriction.…”

“…Somatostatin receptor scintigraphy alone or in combination with other imaging modalities led to the detection of more tumor sites than any combination of imaging studies [33]. Echocardiography (TTE): TTE should be performed in all patients with carcinoid syndrome and a high suspicion for cardiac involvement consistent with clinical features such as cardiac murmurs, raised brain natriuretic peptide (BNP), or pro-b-type natriuretic peptide (proBNP) with or without thickening and retraction of immobile tricuspid valve leaflets and pulmonary valve cusps with associated tricuspid regurgitation [34]. Transesophageal echocardiography provides a better assessment of the degree of valvular involvement and the atrial septal anatomy.…”

Surgery and Perioperative Management in Small Intestinal Neuroendocrine Tumors

“…Carcinoid heart disease is the most feared complication of carcinoid syndrome. It is characterized by a fibrotic degeneration of the valves, particularly affecting the right heart chambers-isolated tricuspid valve regurgitation in up to 90% of cases-leading to a deterioration of the right ventricular function [38,44,45]. Carcinoid heart disease is detected in approximately 40% of patients with a carcinoid syndrome [44].…”

Pheochromocytoma: Another Neuroendocrine Tumor that Substantially Affects the Heart

“…The February 2019 issue of the Journal included an excellent review of carcinoid heart disease (HD) by Ram and colleagues. 1 Practitioners may not be aware that pheochromocytoma, another neuroendocrine tumor, can also cause substantial cardiac injuries. There are important differences between cardiomyopathy in carcinoid HD and pheochromocytoma-induced cardiomyopathy.…”

“…Carcinoid HD usually involves only the tricuspid valve and right ventricle. 1 Pheochromocytoma manifests itself in a much wider spectrum of cardiac abnormalities, such as myocarditis, congestive heart failure, left ventricular thrombus, arrhythmia, and cardiac arrest; however, the cardiac valves are spared. [2][3][4] Carcinoid HD is a late manifestation of carcinoid tumor after extensive liver metastasis; therefore, the prognosis is poor.…”