Carcinoid Heart Disease: Classical Echocardiographic Features.
Carcinoid tumors are rare neuro endocrine tumors arising in 1.2 to 2.1% per 1000,000
people in the general population per year. They most commonly originate from the gastrointestinal tract (foregut) or the bronchus (midgut) and usually clinically asymptomatic until metastases are present. The foregut carcinoids metastasize to the liver or abdominal lymph nodes and may present with bowel obstruction. In 20-30% cases they may present with manifestations secondary to the release of vasoactiveamines (serotonin, bradykinin and histamine) which are referred to as Carcinoid Syndrome (CS). The classical triad of CS is flushing, diarrhea and bronchospasm. 50% cases of CS develop Carcinoid Heart Disease (CHD) a major cause of morbidity and mortality in such patients especially when hepatic metastases is present.
The tricuspid and pulmonary valves are commonly regurgitant and less commonly stenotic. Pulmonic stenosis is commoner than tricuspid stenosis as the pulmonary valve orifice is smaller and the plaques on the valves, in the annulus and sinuses lead to a narrowed pulmonic root.2,4 Left sided lesions occur in <10% cases and may present with mitral or aortic regurgitation. Myocardial metastasis are rare and may occur in 4% cases5 while pericardial effusion occurs in 14% cases and patient for a men ovale (PFO) in 7% cases.
The stenotic right sided valves may be subject to balloon valvuloplasty in selected cases but symptom recurrence rates are high. In some cases with extensive structural defects valve surgery may be the only definitive treatment. Patients with carcinoid heart disease usually die as a result of severe tricuspid regurgitation rather than carcinomatosis. An aggressive approach with surgery soon after the onset of cardiac symptoms may be the answer, as delay can result in worsening right ventricular failure and increase the risk of surgery.
Heart Res Open J. 2016; 2(5): 134-136.doi: 10.17140/HROJ-2-124