Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P61278 (somatostatin)
 22,083 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In this case report the typical echocardiographic features of carcinoid heart disease are presented. Newer treatments such as the use of a somatostatin analogue, interferon and hepatic de-arterialisation have improved the prognosis in patients with carcinoid syndrome. Nevertheless this syndrome portends a poor prognosis in patients with cardiac involvement. Cardiac lesions are mainly located in the right side of the heart. Regurgitation and stenosis of the tricuspid and pulmonary valve, leading to right heart failure, are the most common cardiac manifestations of the disease. Elevated levels of serotonin are probably responsible for the development of these cardiac lesions. Despite treatment resulting in significant reductions of urinary levels of 5-HIAA, regression of the cardiac manifestations in carcinoid syndrome has not been observed. Two-dimensional and Doppler echocardiography are the main tools to establish the diagnosis and severity of carcinoid heart disease. Cardiac surgery for carcinoid heart disease might improve symptoms and longevity, but the scarce data report on early mortality of over 35%.
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PMID:Echocardiographic diagnosis in carcinoid heart disease. 1216 98

The carcinoid syndrome is usually evident when enterochromaffin (EC) cell-derived neuroendocrine tumors (carcinoids) metastasize to the liver. In addition to carcinoid symptomatology, about 40% of patients exhibit carcinoid heart disease (CHD) with fibrotic endocardial plaques and associated heart valve dysfunction. The mechanism behind CHD development is not fully understood, but serotonin (5-HT) is considered to be a major initiator of the fibrotic process. Most patients present with right-sided heart valve dysfunction since pulmonary and tricuspid valves lesions are the most common (>95%) cardiac pathology. Left-sided valvular involvement, and angina associated with coronary vasospasm occur in ~10% of subjects with CHD. Pathognomonic echocardiograpic features include immobility of valve leaflets and thickening and retraction of the cusps most commonly resulting in tricuspid valve regurgitation and pulmonary stenosis. Therapeutic options include cardioactive pharmacotherapy for heart failure and, in selected individuals, cardiac valve replacement. Previously valve replacement was reserved for advanced disease due to a perioperative mortality of >20% however in the last decade, technical advances as well as an earlier diagnosis have decreased surgical mortality to <10% and valve replacements are undertaken more frequently. A recent analysis of 200 cases demonstrated an increase in median survival from 1.5 years to 4.4 years in the last two decades. Although the improved prognosis might also reflect the increased use of surgical cytoreduction, hepatic metastatic ablative therapies and somatostatin analogs a robust correlation between diminution of circulating tumor products and an increased long-term survival in CHD has not been rigorously demonstrated.
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PMID:Carcinoid heart disease. 1857 Dec 50

Carcinoid tumors are rare, indolent neuroendocrine tumors that are often associated with a syndrome characterized by episodic flushing, secretory diarrhea, bronchospasm, and hypotension-the carcinoid syndrome. Cardiac involvement occurs in one-half to two-thirds of patients with carcinoid syndrome and is associated with a worse clinical outcome. Carcinoid heart disease is characterized by endocardial plaque-like deposits found predominantly on right-sided heart valves, leading to the combination of valvular stenosis and regurgitation. Left-sided cardiac involvement can also occur in <10% of patients. Somatostatin analogs form the therapeutic cornerstone in the medical management of these patients. Cytotoxic chemotherapy has had only limited success in the treatment of metastatic carcinoid tumors. Hepatic resection or palliative cytoreduction may be of benefit in patients with limited hepatic disease. Hepatic artery embolization is usually applied if a patient is not eligible for surgical debulking. The development and progression of carcinoid heart disease are associated with an unfavorable outcome. In those patients having severe cardiac involvement and well-controlled systemic disease, valve replacement surgery has been found to be an effective treatment that can both relieve intractable symptoms and contribute to improved clinical outcomes.
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PMID:Carcinoid heart disease. 2231 45