Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0007095 (carcinoid)
 6,990 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The gross surgical pathologic features of the pulmonary valve were reviewed in 116 patients (63 male and 53 female) who had undergone a cardiac operation with pulmonary valve excision at our institution during the period 1973 through 1987. Although the mean age was 12 years, subjects ranged in age from 3 months to 73 years, and 25 patients, including 19 with congenital heart disease, were older than 20 years of age. Among 105 patients who had pure pulmonary stenosis, 61 (58%) had tetralogy of Fallot, 18 had isolated pulmonary stenosis, 23 had other congenital cardiac anomalies, and 3 had carcinoid heart disease. Five patients had pure pulmonary regurgitation (four with tetralogy and one with infective endocarditis), and four had combined pulmonary stenosis and regurgitation (two with congenital cardiac anomalies and two with carcinoid heart disease). In two patients, the valve was neither stenotic nor regurgitant. Thus, congenital heart disease accounted for 110 of the 116 cases (95%), and tetralogy of Fallot was the most commonly observed form (65 cases). Bicuspid pulmonary valve was the most common anomaly and was present in 58% of patients with tetralogy but in only 17% of those with isolated pulmonary stenosis.
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PMID:Surgical pathology of the pulmonary valve: a study of 116 cases spanning 15 years. 259 25

Despite recent renewed interest in the detection of tricuspid valve regurgitation by echocardiographic and Doppler techniques, little morphologic information is available on dysfunctioning tricuspid valves. This report describes 45 necropsy patients with clinical and morphologic evidence of pure (no element of stenosis) tricuspid regurgitation and provides morphometric observations (anular circumference, leaflet area) of the tricuspid valve useful in determining the etiology of pure tricuspid regurgitation. Of 45 patients, 24 (53%) had pure tricuspid regurgitation resulting from an anatomically abnormal valve (prolapse in 7, papillary muscle dysfunction in 6, rheumatic disease in 5, Ebstein's anomaly in 3, infective endocarditis in 2, carcinoid tumor in 1), and 21 (47%) had an anatomically normal valve with systolic pulmonary artery hypertension (cor pulmonale in 12, mitral stenosis in 9). Anular circumference was dilated (greater than 12 cm) in patients with various causes of pulmonary hypertension, floppy valve and Ebstein's tricuspid anomaly. Leaflet area was increased in floppy valve and Ebstein's anomaly. Of the 45 patients, 24 had pulmonary systolic artery pressure measurements available for correlation with tricuspid valve morphology. Pulmonary artery pressures accurately predicted morphologically normal from abnormal valves in 16 patients (89%). Morphologic overlap occurred in six patients with pulmonary pressures of 41 to 54 mm Hg. Of these six, the additional knowledge of normal or dilated anular circumference correctly separated valves with normal and abnormal leaflets.
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PMID:Etiology of pure tricuspid regurgitation based on anular circumference and leaflet area: analysis of 45 necropsy patients with clinical and morphologic evidence of pure tricuspid regurgitation. 395 62

Tricuspid valve disease accounts for significant morbidity and mortality in the carcinoid syndrome, but M-mode echocardiography is often insensitive in completely defining the tricuspid valve. We performed two-dimensional echocardiography (2DE) in seven patients with proven carcinoid syndrome. There were five males and two females whose ages ranged from 53 to 79 years. The carcinoid syndrome had been present by symptoms for 12 to 84 months and by 5-HIAA levels for 6 to 84 months prior to 2DE. Short, thickened, immobile tricuspid valve leaflets, fixed in a partially open position, were visualized in two patients and confirmed in one patient at surgery. Tricuspid regurgitation was demonstrated angiographically in one and by contrast 2DE in the other. A third patient had clinical evidence of tricuspid stenosis with a doming tricuspid valve on 2DE. The motion of the tricuspid value viewed in real time was clearly distinct in these two situations. Four patients had both normal M-mode and 2DE studies despite the fact that clinical and biochemical evidence of carcinoid disease had been present for equally long periods of time. The tricuspid valve was best visualized in the parasternal right ventricular long-axis and short-axis views. The apical four-chamber view was less helpful. Thus, 2DE demonstrated specific tricuspid valve abnormalities in the carcinoid syndrome with thickening, shortening, and immobility of the leaflets when valvular regurgitation was present and thickening and doming when the valve was stenotic. 2DE should be a useful method in the diagnosis and sequential evaluation of patients with carcinoid heart disease.
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PMID:Two-dimensional echocardiography in the diagnosis of carcinoid heart disease. 669 93

Little morphologic information is available on operatively excised pulmonic valves. The causes of pulmonic stenosis are limited to a few conditions: (1) rheumatic and (2) nonrheumatic (congenital, carcinoid, infective endocarditis). Congenital causes of pulmonic stenosis constitute well over 95% of these conditions. Congenital types of pulmonic stenosis include acommissural dome-shaped, dysplastic, and bicuspid. Rare acquired causes of pulmonic stenosis include carcinoid, rheumatic, and infective endocarditis. Of the acquired causes of pulmonic stenosis, carcinoid is the most common condition. In contrast, causes of pure pulmonic regurgitation are multiple. Two major categories of pure pulmonic regurgitation include (1) conditions associated with anatomically abnormal valve cusps (congenital, rheumatic, carcinoid, trauma, and infective endocarditis) and (2) conditions associated with anatomically normal cusps (elevated pulmonary artery systolic pressures, idiopathic dilated pulmonary trunk, and Marfan's syndrome).
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PMID:Pathology of pulmonic valve stenosis and pure regurgitation. 770 86

A woman who had carcinoid syndrome and carcinoid heart disease underwent tricuspid and pulmonary valve replacements with a xenograft and a cryopreserved allograft, respectively. Within 3 months of the operation severe pulmonary regurgitation and pulmonary hypertension refractory to medical therapy developed. Autopsy found the biomechanical tricuspid valve to be free of disease but the allograft in the pulmonary position was involved by carcinoid heart disease in a fashion similar to the excised native pulmonary valve.
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PMID:Carcinoid heart disease: early failure of an allograft valve replacement. 794 71

Right-sided valvular heart disease is a common complication of metastatic carcinoid tumors. On the contrary, left valve involvement is a rare finding. In our report we describe a patient in whom the subsequent involvement of right and left heart valves was documented by 2D and Doppler echocardiography. The patient was a 46-year-old woman who, in the last three years, complained of face flushing, postprandial diarrhea and shooting epigastric pain lasting for hours. She came at our observation for increasing dyspnoea, peripheral edemas and asthenia. 2D-echocardiography and Color Doppler revealed a severe tricuspid regurgitation and a turbulent blood flow across the pulmonary valve. An Angiographic examination confirmed the severe tricuspidal regurgitation and disclosed a mild pulmonary stenosis. These findings were associated with high 5 hydroxyndole acetic acid (5-HIAA) urinary excretion and the presence of a liver metastasizing ileal carcinoid tumor. Some months later, a new 2D echo-Doppler examination showed thickening and reduced motion of the posterior mitral leaflet, associated with regurgitation and an aortic insufficiency without evident structural valvular abnormalities. Even if carefully investigated no right-to-left shunt was found.
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PMID:[Left valvular involvement in carcinoid: description of a case]. 805 18

This report describes findings present on magnetic resonance (MR) imaging and computed tomography (CT) in a patient with carcinoid heart disease. Major abnormalities included thickening and immobility of the tricuspid valve leaflets and disturbed flow patterns indicative of tricuspid stenosis and regurgitation demonstrated with cine MR. While echocardiography is usually the initial imaging modality in patients with carcinoid heart disease, other modalities such as MR and CT may provide correlative or supplemental information.
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PMID:MR and CT diagnosis of carcinoid heart disease. 843 73

Serotoninergic appetite-suppressant drugs, fenfluramine and dexfenfluramine, were withdrawn from the market in September 1997 on account of two major cardiopulmonary complications: primary pulmonary hypertension and valvular regurgitation. The valvular heart diseases involve mainly left-sided valves, and contrary to physiological valvular regurgitations, they appear mostly on the aortic valve. Prolonged exposure (> 3 months) appears to confer a higher risk of cardiac valve involvement. Pathological features are similar to carcinoid or ergot alkaloid-induced valve diseases, and suggest a common pathophysiological mechanism which would also explain pulmonary hypertension by the toxic effect of high levels of circulating serotonin. After the first reports documenting a dramatically high prevalence of valvular side effects (up to 33% according to the Food and Drug Administration), recent studies reported a lower prevalence and severity. The long-term outcome and the real incidence are unknown and require further research and epidemiological data. A clinical survey of the patients exposed to serotoninergic appetite-suppressants is necessary, to be repeated 6 to 8 months later in the absence of an initial cardiac murmur. Doppler echocardiographic examination should be performed after prolonged exposure (> 3 months) or a high dosage of these drugs, in circumstances such as the presence of cardiovascular symptoms, a cardiac murmur, or an uncertain cardiac examination because of weight of patients.
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PMID:[Appetite suppressants and heart valve disorders]. 1053 70

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 report on the case of a 34-year-old male patient suffering from end-stage carcinoid heart disease with severe tricuspid, pulmonary and mitral valve regurgitation. In addition, a persisting foramen ovale was present. The primary carcinoid tumor was never discovered. However, urine 5-hydroxy-indole-acetic-acid (5-HIAA) were consistently elevated after the first diagnosis of carcinoid disease and after eight years of medication with Octreotide and Interferon alpha-2b our patient developed significant cardiac insufficiency mainly due to severe valvular dysfunction. Ultimately, mechanical tricuspid, mitral and pulmonary valve replacement was performed. Twelve hours following the operation the patient had to be returned to the operating room for persisting intrathoracic hemorrhage. He recovered uneventfully and was discharged from hospital on day 37. Twelve months following triple valve replacement the cardiac status recovered from preoperative NYHA-IV to NYHA-I.
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PMID:Triple valve replacement in a patient with severe carcinoid heart disease. 1204 40


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