Carcinoid Disease
I. Heart Damage
II. Diagnosis
III. Treatment
IV. Clinical Study
GLOSSARY
endocardium internal lining of the heart.
malignant tumor the tumor that invades and spreads to adja¬cent and distant organs.
tricuspid regurgitation tricuspid valve leaks and blood is pro¬pelled backwards from the right ventricle into the right atrium and into the neck veins.
CARCINOID HEART DISEASE MAY OCCUR IN patients with carcinoid syndrome. The main symptoms of flushing, diarrhea, and occasional wheezing are caused mainly by 5-hydroxytryptamine or serotonin, that is lib¬erated from carcinoid tumors that originate from chro-maffin cells (neuroendocrine cells) of the terminal ileum. These tumors of the small intestine contain neurosecre-tory granules that release a variety of biogenic amines that include serotonin, histamine, bradykinins, tachykinins, and prostaglandins. Involvement of the heart occurs in about half of carcinoid syndrome cases. It is seen mainly in patients with malignant tumors that have metastasized to the liver.
- I. HEART DAMAGE
Bioactivity amines, principally, serotonin, liberated from a malignant tumor causes deformation of the tricuspid valve that leads to tricuspid regurgitation. The pulmonary valve becomes deformed by the plaque-like material resulting in a leaky, incompetent valve (pulmonary regurgitation) or a tight, stenotic valve (pulmonary stenosis). In a clinical study of carcinoid heart disease, 97% of patients had right-sided valvular involvement; severe tricuspid valve regurgitation occurred in all patients and severe pulmonary valve regurgitation in 72%. - II. DIAGNOSIS
Carcinoid tumors are rare. They arise from enterochro-maffin cells typically located in the gastrointestinal tract. At the time of diagnosis, more than 30% of patients have disseminated disease characterized by cutaneous vasomotor flushing, secretory diarrhea, and mild bronchospasm. - III. TREATMENT
There are no specific treatments for carcinoid heart disease. The noncardiac symptoms may be controlled with soma-tostatin, but the action of this drug is only minutes. Octreotide has been shown to be much more effective in reducing flushing diarrhea and urinary levels of 5-HIAA. - BIBLIOGRAPHY
Howard, R. J., Drobac, M., Rider, W. D. et al. Carcinoid heart disease: Diagnosis by two-dimensional echocardiography. Circulation, 66:1059–65, 1995.