Carcinoid Heart Disease (CHD) is an uncommon presentation of carcinoid syndrome, but it ultimately occurs in most of these patients and is the major cause of morbidity and mortality in patients with CHD. CHD is primarily attributable to excessive levels of serotonin produced by carcinoid tumor, a neuroendocrine neoplasm usually located in the gastrointestinal or genitourinary tract. High levels of serotonin stimulate fibrosis resulting in fibrotic changes in the right-sided cardiac valves, causing tricuspid and pulmonic regurgitation and, to a lesser degree, stenosis of these valves. The disease is monitored by following urine levels of 5-hydroxy indole acetic acid, the major metabolite of serotonin. The mortality of patients with CHD and heart failure is very high but recent advances in medical and cardiac interventional therapy have provided the potential for an improved outlook of patients with this disease.
Carcinoid heart disease; Carcinoid syndrome; Carcinoid tumor; Endocrine disorder; Neuroendocrine tumor
Carcinoid heart disease (CHD) is an uncommon manifestation of carcinoid syndrome, which is a rare endocrine disorder attributed primarily to the effects of serotonin (5-hydroxytryptamine) secreted by carcinoid tumors (Table 1) . Few of these neoplasms are malignant. The incidence of carcinoid tumors is 1-5 of 100,000 persons [1-3]. Carcinoid tumors typically arise in the gastrointestinal and genitourinary tracts. The appropriate term for carcinoid neoplasm is a neuroendocrine tumor (NET) (Table 1). The major clinical manifestations of CHD are tricuspid and pulmonic regurgitation and, to a lesser degree, stenosis of these valves [1-3].