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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Time domain heart rate variability measurements and echocardiographic studies were performed in 35 patients with carcinoid syndrome. Carcinoid heart disease was present in 18 patients (51%). Heart rate variability parameters (standard deviation of all normal RR intervals, percentage of the number of pairs of adjacent normal RR intervals differing by >50 ms) were significantly reduced in patients with than in those without carcinoid heart disease.
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PMID:Heart rate variability in carcinoid heart disease. 1007 2

Tumor debulking can greatly improve quality of life for patients with malignant carcinoid syndrome, but hepatic cytoreduction is confounded by carcinoid heart disease, which can cause postsinusoidal portal hypertension, thereby increasing the risk of death from hemorrhage during hepatic resection. We describe a patient with metastatic carcinoid syndrome and carcinoid heart disease who had repair of his carcinoid heart disease and, after improvement of right-sided heart function, had successful hepatic debulking of carcinoid metastases.
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PMID:Carcinoid heart disease and carcinoid syndrome: successful surgical treatment. 1019 87

A 76-year-old woman presenting with right heart failure is reported. The investigation showed an ileal carcinoid tumour with multiple metastases and tricuspid regurgitation caused by a cardiac carcinoid lesion. Echocardiographic study and histopathological examination revealed typical characteristics of carcinoid heart disease. Nevertheless, except for the cardiac symptomatology, the patient did not show any other clinical manifestation of the carcinoid syndrome. Isolated right heart failure as the first sign of a carcinoid syndrome is rare; only two other cases have been described in the literature.
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PMID:[Atypical presentation of a carcinoid syndrome]. 1032 57

Carcinoid tumors are endocrine malignancies that are often associated with a characteristic syndrome, the malignant carcinoid syndrome, which is most common in patients with small bowel tumors and liver metastases. In the rare instances when the syndrome is present without liver metastases the primary tumor is usually localized to the bronchus or ovary and secretes hormones directly into the systemic circulation. About two thirds of patients with carcinoid syndrome have evidence of carcinoid heart disease. We report on a case of a primary ovarian carcinoid tumor with an unusual clinical presentation.
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PMID:Primary carcinoid tumor of the ovary: report of an unusual case. 1077 76

A 70-year-old man with metastatic liver tumors showed carcinoid syndrome with clinical symptoms of facial flushing, palpitation, dyspnea, and an itching sensation. Regurgitation of the tricuspid and aortic valves was observed by echocardiography. An elevated serum level of serotonin and a high urine excretion of serotonin metabolites were confirmed. Autopsy confirmed a serotonin-immunoreactive 1 cm ileal carcinoid tumor with metastasis to the liver, bone and peritoneum. The tumor cells were argyrophilic but not argentaffin, and showed erythrophagocytosis in the primary lesion. All the four heart valves and bilateral atrial endocardium showed fibromyxoid thickening, indicating the association of carcinoid heart disease. Desmoplastic reaction with deposition of sulfated acid mucopolysaccharides was also observed in the retroperitoneal space (secondary retroperitoneal fibrosis). Stenosis of the bilateral ureters and inferior mesenteric artery provoked hydronephrosis and lethal ischemic necrosis of the left-sided colon, respectively. Rarity of functioning ileal carcinoid tumor and pathogenesis of systemic fibroplasia are discussed.
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PMID:Ileal carcinoid tumor complicating carcinoid heart disease and secondary retroperitoneal fibrosis. 1084 30

We describe a patient with carcinoid heart disease secondary to a primary ovarian carcinoid tumor who initially presented with unexplained right-sided heart failure. The patient did not exhibit any of the typical clinical manifestations of the carcinoid syndrome. Echocardiography demonstrated typical features of carcinoid heart disease and played an important role in the detection and management of a condition that was previously unrecognized.
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PMID:Carcinoid heart disease in association with a primary ovarian carcinoid tumor: diagnostic role of echocardiography. 1100 May 93

Carcinoid syndrome originates from metastatic carcinoid tumors localized in the gastrointestinal system, pancreas, biliary vessels, bronchi, ovaries, and testes; it is characterized by flushing, telangiectasias, diarrhea, bronchoconstriction, and fibrous endocardial plaques in the heart. Cardiac involvement is detected by echocardiography in over 50% of patients with this syndrome. Right-sided valvular heart disease occurs frequently in patients with carcinoid syndrome, involving most commonly the tricuspid and pulmonary valves. Involvement of the left-sided valves rarely occurs. Medical therapy for carcinoid heart disease includes digitalis and diuretics for congestive heart failure symptoms; the effects of carcinoid syndrome can be treated with the use of somatostatin analogues. Conventional chemotherapy has been of little beneficial value, with response rates of only 10% to 30%. The use of octreotide, a long-acting and potent somatostatin analogue, is a major advance in the management of carcinoid tumors. In addition to providing effective symptom relief in malignant carcinoid syndrome, octreotide can also be used for diagnostic purposes. Despite its expense, octreotide is the current agent of choice for the treatment of this condition. Analogues with different receptor specificities and pharmacokinetics hold promise for the future. Valve surgery is the only definitive treatment for intractable right-sided heart failure. Although cardiac surgery carries high perioperative mortality, marked symptomatic improvement occurs in survivors. Surgical intervention therefore should be considered in the appropriate patients when cardiac symptoms become severe. Balloon valvulotomy in patients with severe pulmonary artery stenosis often results in palliation of symptoms; however, surgery still is required often in these patients.
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PMID:Carcinoid Heart Disease. 1109 44

Carcinoid tumors usually present as diagnostic dilemmas due to obscure or nonspecific symptomatology. Advances in molecular biology are allowing the investigation of molecular markers of aggressiveness, better serum tumor markers, as well as the molecular pathogenesis of carcinoid heart disease. Somatostatin receptor scintigraphy (SRS) and whole body positron emission tomography (PET) are providing much improved sensitivity in localization of both primary and metastatic tumors. Long acting depot somatostatin analogues are combining effectiveness and ease of use for medical management of carcinoid syndrome. An additional benefit may be tumor growth suppression.
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PMID:Recent advances in carcinoid pathogenesis, diagnosis and management. 1147 88

Gastroenteropancreatic (GEP) neoplasms originate from any of the various cell types belonging to the neuroendocrine system. A general characteristic of GEP endocrine tumours is that the vast majority produce and secrete a multitude of peptide hormones and amines. Many patients with malignant metastasising tumours present clinical symptoms related to hormone hyperproduction. These include the so-called carcinoid syndrome, characterised by flushing, diarrhoea, wheezing and right heart disease, which is predominantly associated with the serotonin- and tachykinins-producing carcinoids of the midgut. Several types of syndrome associated with GEP endocrine tumors are caused by overproduction of a specific hormone. For instance, the well-known Zollinger-Ellison syndrome is gastrin-mediated. The so-called 'insulinoma syndrome' depends on excessive production of insulin and proinsulin, resulting in hypoglycemia. The 'glucagonoma syndrome' is characterised by necrolytic migratory erythema, diabetes and diarrhoea. The Verner-Morrison syndrome, which is brought about by high circulating levels of vasointestinal peptide (VIP). produces severe secretory diarrhoea. Finally the 'somatostatinoma syndrome' involves gallbladder dysfunction and gallstones, diarrhoea with or without steatorrhea, and impaired glucose tolerance. The biochemical diagnosis of endocrine digestive tumors is based on general and specific markers. The best general markers are chromogranin A (CgA) and pancreatic polypeptide (PP). Specific markers for endocrine tumors include insulin, gastrin, glucagon, vaso intestinal polypeptide (VIP), somatostatin and the primary cathabolic product of serotonin, 5-hydroxyndoleacetic acid (5-HIAA). Localisation procedures commonly applied, in the diagnosis of endocrine tumours include ultrasound (US), computed tomography (CT) and somatostatin receptor scintigraphy (SRS).
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PMID:Epidemiology, clinical features and diagnosis of gastroenteropancreatic endocrine tumours. 1176 60

Carcinoid heart disease occurs in about one third of patients with carcinoid syndrome, especially in those with ileal carcinoid and hepatic metastases. Patients with primary ovarian carcinoid tumor are extremely rare. In these circumstances, typical carcinoid cardiac lesions may develop unassociated with hepatic metastases, due to the venous drainage from the ovaries into the inferior vena cava of vasoactive released substances such as serotonin. The present report describes a woman with unrecognized primary ovarian carcinoid tumor, unexpectedly exhibiting heart failure. Diagnosis was performed on the basis of echocardiographic findings, occurrence of diarrhea and increased levels of 5-hydroxy-indoleacetic acid (5-HIAA). After complete surgical removal of the tumor, the patient was maintained under therapy with ACE-inhibitors and diuretics. Levels of 5-HIAA are still within normal range, there is regression of heart failure and echocardiographic findings are stabilized. These data confirm the importance of prompt diagnosis for a favorable prognosis of carcinoid heart disease.
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PMID:[Carcinoid heart disease and primary ovarian tumor]. 1202 91


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