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 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 usually originate in the gastrointestinal tract, but in rare instances they may arise in other organs. A patient with severe tricuspid and pulmonary regurgitation because of carcinoid syndrome successfully underwent double valve replacement using bioprostheses. The patient was finally diagnosed with carcinoid heart disease from an isolated ovarian carcinoid cancer. The diagnosis of carcinoid syndrome should be recognized as an etiology in patients with organic tricuspid and pulmonary regurgitation without left valvular disease.
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PMID:Bioprosthetic pulmonary and tricuspid valve replacement in carcinoid heart disease from ovarian primary cancer. 1913 90

Carcinoid disease arises from a low-grade neuroendocrine tumour derived from serotonin-producing enterochromaffin cells. It is the most common tumour affecting the small bowel. The majority of patients who progress to carcinoid syndrome develop cardiac disease selectively involving the right side of the heart, whereas left heart disease is unusual. The most common cause of death is dilatation and dysfunction of the right ventricle. Right ventricular dysfunction is largely secondary to pathological endocardial fibrosis of the tricuspid and pulmonary valves, presenting with regurgitation and stenosis. Average survival falls to only 11 months with the onset of symptoms, but recent evidence suggests that survival can be improved by early surgery in selected individuals. This article reviews the particular role that cardiovascular magnetic resonance imaging has in the management of carcinoid heart disease.
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PMID:Cardiovascular magnetic resonance imaging in the assessment of carcinoid heart disease. 1958 14

We present the case of a 69-year-old woman with a metastatic neuroendocrine tumour of the ileum and severe tricuspid valve regurgitation due to carcinoid valvulopathy. Carcinoid heart disease arises in the context of metastatic serotonin-producing neuroendocrine tumours and typically presents as structural and functional abnormalities of tricuspid (TV) or pulmonary valve (PV). Carcinoid heart disease arises from a low-grade neuroendocrine tumour derived from serotonin-producing enterochromaffin cells that reduces the mobility of the leaflets. Following the development of this type of heart failure, the prognosis is unfavourable and patients usually die as a result of heart failure and not because of the metastatic disease. Our patient was not considered a candidate for valvular surgery because of the progressive nature of the malignancy. However, surgical valve replacement is a therapeutic option that, although it has a significant mortality, needs to be considered in selected patients and leads to improvement in functional capacity and survival.
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PMID:Carcinoid heart disease: case and literature review. 2045 39

A 53-year-old woman is described who underwent mitral and aortic valve replacement and tricuspid valve annuloplasty for pure regurgitation at all 3 valve sites for unrecognized carcinoid heart disease without the carcinoid syndrome 22 days before death. Metastatic carcinoid was not recognized until necropsy, which disclosed a probable ovarian primary but with large hepatic metastases and left-sided cardiac involvement either greater than or equal to the right-sided involvement. Pulmonary hypertension, very unusual in carcinoid heart disease, persisted postoperatively and probably played a role in the patient's early death. Hepatic metastasis with ovarian primary is most unusual in this circumstance.
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PMID:Carcinoid heart disease without the carcinoid syndrome but with quadrivalvular regurgitation and unsuccessful operative intervention. 2131 9

Children with neurological disorders may suffer from gastroesophageal reflux disease (GERD). Typical symptoms are vomiting, regurgitation and hematemesis. Patients present with respiratory symptoms only in cases with swallowing disorders causing chronic airway aspiration. We report the case of a patient affected by chromosome 8 p deletion syndrome with mental retardation, referred to our unit for suspected GERD. Chest X-ray, performed at admission for coexisting respiratory complaints, showed left lower lobe pneumonia; esophageal pH monitoring and upper endoscopy were normal for GERD. To rule out chronic airway aspiration, gastroesophageal 99 mTc scintigraphy with lung scan 18 to 24 h after a test meal and video fluoroscopy swallowing study were performed, both negative. Two months later, a second episode of left lower lobe pneumonia occurred. A chest CT scan was performed and showed an endobronchial mass; the biopsy taken during the broncoscopy was not conclusive. Surgical excision resulted in a diagnosis of pulmonary carcinoid. Bronchial carcinoids, although rare, should be taken into consideration as a potential cause of recurrent pneumonia even in the presence of demonstrated GERD where severe respiratory infections only occur with coexisting chronic pulmonary aspiration, even in neurologically impaired people.
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PMID:Association between gastroesophageal reflux and endobronchial carcinoid: a case report. 2148 Jan 46

Carcinoid heart disease is characterized by heart valve dysfunction as well as carcinoid symptomatology. We report a case of carcinoid heart disease associated with a primary ovarian tumor. A 60-year-old woman presented for dyspnea evaluation with a history of facial flushing, telangiectatic skin changes, and pitting edema of both lower extremities. Chest radiography showed cardiomegaly, and echocardiography revealed an isolated, severe tricuspid regurgitation without left-sided valvular dysfunction. The tricuspid leaflets were severely retracted and shortened, resulting in poor coaptation. Furthermore, mild pulmonary valve stenosis and moderate regurgitation were found along with this deformation. The 24-hour urine analysis revealed an increased level of 5-hydroxyindoleacetic acid, and an ovarian tumor was apparent on computed tomography images. The mass was surgically removed, and the patient was diagnosed as having a primary ovarian carcinoid tumor. She was treated with chemotherapy and regularly followed-up with supportive treatments, deferring surgical correction.
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PMID:Ovarian tumor-associated carcinoid heart disease presenting as severe tricuspid regurgitation. 2151 94

Carcinoid heart disease is a rare cause of heart failure with or without right valvular heart impairments. In this study, we showed a case of carcinoid tumour with hepatic metastases inducing carcinoid heart disease. Neuroendocrine heart involvement happens for severe tricuspid valve insufficiency and plaques on right ventricular (RV) walls produced by a release of serotonin (5-HT). A patient affected by primitive ileal tumour with 5-HT-secernent hepatic metastases inducing tricuspid insufficiency is showed. Transthoracic 2-D echocardiography showed tricuspid valve regurgitation and both right atrium, RV-walls plaques and RV dilation. Continue-wave Doppler showed a characteristic "dagger shaped" spectrum of tricuspid systolic flow. RV function was evaluated with 3-D transthoracic echocardiography. In particular, RV volumes, RV ejection fraction and stroke volume were defined by this technique. 2, 3-D echocardiography and Doppler method are useful techniques to show heart valves' derangements and RV function to non-invasively detect RV impairments in carcinoid heart disease.
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PMID:Carcinoid Heart Disease: A Rare Cause of Right Ventricular Dysfunction Evaluation by Transthoracic 2D, Doppler and 3-D Echocardiography. 2186 Jul 26

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

We present an adult with metastatic carcinoid disease affecting the heart, in whom live/real time three-dimensional transthoracic echocardiography (3DTTE) provided incremental value over two-dimensional transthoracic echocardiography (2DTTE). Initial 2DTTE was able to demonstrate severe pulmonic and tricuspid regurgitation, but was unable to visualize the posterior leaflet of the tricuspid valve or the right (right anterior) leaflet of the pulmonic valve. Further analysis with 3DTTE demonstrated thickening, restricted mobility, and noncoaptation of all three leaflets of both the tricuspid and the pulmonary valves. En face viewing of tricuspid and pulmonary regurgitation vena contractas permitted more reliable quantification of regurgitation severity. In addition, localized, linear, echogenic areas consistent with carcinoid deposits were noted along the inner walls of the right atrium, atrial septum, and inferior vena cava. To the best of our knowledge, endocardial carcinoid deposits have never been reported by 2D or 3D echocardiography. En face viewing of these deposits by 3DTTE enabled measurement of their dimensions and areas. Subcostal examination also identified large circumscribed hepatic lesions consistent with metastatic disease. Neither the carcinoid deposits nor the metastatic lesions were detected by 2DTTE. This case demonstrates the usefulness of 3DTTE as a supplement to 2DTTE in more comprehensively assessing carcinoid involvement of the heart.
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PMID:Live/real time three-dimensional transthoracic echocardiographic assessment of the involvement of cardiac valves and chambers in carcinoid disease. 2296 95


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