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)

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. 2243 50

We describe the case of a 76-year-old woman, diagnosed with a neuroendocrine tumor of the cecum in 2004, with liver metastases and carcinoid syndrome since September 2010. The patient had been treated intermittently with chemotherapy cycles, and remained symptomatic, with worsening secondary lesions. In June 2011 she began to present signs and symptoms of right heart failure and was hospitalized in September 2011. Transthoracic two- and three-dimensional echocardiography revealed enlarged right atrium and ventricle and thickened and fixed tricuspid and pulmonary valve leaflets, causing severe tricuspid regurgitation and mild pulmonary regurgitation and stenosis, suggestive of carcinoid heart disease. The authors discuss the clinical importance of transthoracic echocardiography, and the more recent three-dimensional echocardiography, as the diagnostic exam of choice in these cases, as it is especially suitable for assessing the valves and subvalvular apparatus.
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PMID:[Carcinoid heart - case report]. 2298 41

A 66-year-old woman was diagnosed with hepatic metastasized carcinoid tumor of the ileocecal junction resulting in elevated plasma chromogranin A levels and urinary 5-hydroxyindoleacetic acid (5-HIAA) levels. Further examination showed right-sided heart failure with severe tricuspid valve regurgitation. Carcinoid tumors produce serotonin which leads to flushing, secretory diarrhea, bronchospasm and hypotension, known as carcinoid syndrome. Serotonin is metabolized to 5-HIAA, which is inactive, in the liver and the lungs. However, hepatic metastases may result in direct exposure of the heart to serotonin, which induces plaque-like deformities on the tricuspid valve, and in turn induces valve regurgitation. This condition is known as carcinoid heart disease. Tricuspid valve regurgitation may induce risk of massive blood loss in case of liver surgery through high-volume backflow in the hepatic veins. This report shows the clinical relevance of carcinoid heart disease in the perioperative setting.
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PMID:Tricuspid valve regurgitation as a presenting symptom of metastasized carcinoid tumor. 2313 56

Isolated tricuspid valve disease is rare. We report the case of a 65-year-old female patient who presented with right heart failure. Initial echocardiographic evaluation revealed a giant right atrium (RA) with severe tricuspid inflow obstruction with regurgitation. A comprehensive two- and three-dimensional echocardiographic evaluation revealed a dysplastic tricuspid valve resulting in the inflow obstruction and regurgitation. Rheumatic and carcinoid etiologies were ruled out by relevant tests. It is very rare for dysplastic tricuspid valve to present in late adulthood. The classic echocardiographic findings are discussed followed by a brief review of the literature.
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PMID:Giant right atrium due to congenital dysplastic tricuspid valve in an elderly female patient. 2348 67

A best evidence topic in cardiac surgery was written according to a structured protocol. We addressed the following question: in patients who are diagnosed with carcinoid heart disease (CHD), do valvular surgeries improve their prognosis? Fifty percent of the patients with clinically diagnosed carcinoid syndrome had cardiac involvement which was present either as valvular dysfunction or as cardiac metastases. These patients often require surgery due to their heightened risk of cardiac disease. Altogether 217 relevant papers were identified as a result of the below-mentioned search, of which 10 papers represented the best evidence to answer the question. The author, journal, date and country of publication, patient group studied, study type, relevant outcomes, results and study weaknesses were tabulated. Of the patients who were identified to have carcinoid heart disease in different studies, 193 patients had valve procedure, mainly replacements at tricuspid, mitral and aortic valve positions and either valvuloplasty or replacement at pulmonary valve. Tricuspid and pulmonary valves represented the majority of the excised valves among patients undergoing valvular surgery for CHD. The pathology of carcinoid valve was attributed to the presence of plaque, causing thickening and retraction. Pure regurgitation was the most common finding in all the valves except pulmonary valve which had both stenosis and insufficiency. Thirty-day mortality was 17% (range 1-63%) and long-term survivors were reported to be alive at an average of 58 months (28-80 months) after the valve surgery. The evidence demonstrates that surgical intervention can lead to improved prognosis and reduce the symptoms of heart failure. Postoperative mortality was mainly due to the carcinoid disease itself and not as a complication of the surgery. Therefore, surgery could be considered for symptomatic palliation in carefully selected individuals.
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PMID:Prognosis of patients with carcinoid heart disease after valvular surgery. 2481 31

Carcinoid is a rare neuroendocrine tumor typically arising in the gastrointestinal tract that can cause heart valve involvement. We report two patients with carcinoid syndrome and tricuspid/pulmonary valve lesions. A 33-year-old male presenting with fatigue and weight loss: A tumor in the tail of the pancreas was found on an abdominal CAT scan. The percutaneous biopsy was informed as a carcinoid tumor. A trans-esophageal echocardiogram showed a tricuspid and pulmonary valve involvement, which was replaced surgically. The biopsy informed an extensive fibrous and myxoid degeneration of the valves. A 35-year-old male with a carcinoid syndrome and cardiac failure: An echocardiogram showed a severe tricuspid stenosis and severe pulmonary regurgitation. The patient was subjected to a double surgical valve replacement. The pathology report of the excised valve showed a deforming fibrous and myxoid valvulopathy.
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PMID:[Tricuspid and pulmonary valve involvement in carcinoid syndrome. Report of two cases]. 2542 26

Hedinger syndrome refers to carcinoid valvular heart disease. The disease is believed to be triggered by vasoactive substances that result in valvular fibrosis. It classically occurs in patients with metastatic carcinoid and preferentially involves the right sided cardiac valves. Affected valves become thickened and retracted, exhibiting regurgitation and sometimes, stenosis. Echocardiography is recommended in patients with carcinoid syndrome and a follow up study is advisable in those who develop a murmur or other symptoms or signs of valvular heart disease. For appropriately selected patients, valve replacement surgery appears to improve outcomes.
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PMID:Carcinoid heart disease: Diagnosis and management. 2697 51

Carcinoid heart disease presents as right-sided heart failure attributable to the dysfunction of the tricuspid and pulmonary valves. Although surgical valve replacement is the mainstay of treatment when patients become symptomatic, it is associated with substantial perioperative mortality rates. We present a case of severe pulmonary valve stenosis secondary to carcinoid heart disease, treated successfully with percutaneous valve replacement. A 67-year-old man with severe pulmonary valve stenosis was referred to our center for pulmonary valve replacement. The patient had a history of metastatic neuroendocrine tumor of the small bowel with carcinoid syndrome, carcinoid heart disease, and tricuspid valve regurgitation previously treated with surgical valve replacement. Because of the patient's severe chronic obstructive pulmonary disease and hostile chest anatomy seen on a computed tomographic scan dating from previous cardiothoracic surgery, we considered off-label percutaneous valve replacement a viable alternative to open-heart surgery. A 29-mm Edwards Sapien XT valve was successfully deployed over the native pulmonary valve. There were no adverse sequelae after the procedure, and the patient was discharged from the hospital the next day. This case report shows that percutaneous valve replacement can be a valid option in carcinoid heart disease patients who are not amenable to surgical valve replacement.
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PMID:Transcatheter Pulmonary Valve Replacement in a Carcinoid Heart. 2754 48

Carcinoid heart disease (CHD) complicates approximately 25% of patients with a carcinoid tumor and carcinoid syndrome and leads to heart valve degeneration with mixed-stenotic and regurgitation pathology and consequent heart failure (HF) leading to significant morbidity and mortality. Cardiac surgery in symptomatic, severe CHD leads to significantly better functional capacity and prolonged survival when compared to medical treatment alone. Recent studies have shown improvement in postoperative outcomes of patients undergoing surgery for CHD over the last decades. The trend for early diagnosis and application of surgery prior to the manifestation of HF symptoms, which tended to develop during the previous years, does not seem justifiable based on the findings of recent studies. Therefore, the optimal timing of intervention in CHD and the type of valve that should preferably be used remain issues of controversy. This review comprehensively examines the existing literature on the treatment options for patients with CHD, with a special focus on short- and long-term survival after cardiac surgery, and discusses the selection of the exact patient profile and intervention timing that are more likely to optimize the benefit-to-risk ratio for surgical intervention.
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PMID:Cardiac Surgery for Carcinoid Heart Disease: A Weapon Not to Be Misused. 2783 43

Carcinoid syndrome is the constellation of symptoms mediated by humoral factors produced by some carcinoid tumors. It consists primarily of vasomotor symptoms, gastrointestinal hypermotility, hypotension, and bronchospasm, due to the production and release of vasoactive substances. Carcinoid heart disease occurs in more than 50% of patients with carcinoid syndrome; in some cases, it represents the initial manifestation of the disease. We report the case of a 75-year-old woman with a metastatic neuroendocrine tumor admitted to the emergency room for fatigue and heart failure. Transthoracic echocardiography showed severe tricuspid and pulmonic regurgitation suggesting carcinoid heart disease. A hypervascular retroperitoneal mass was found on abdominal computed tomography, which seemed to arise from the mesenteric artery, anteriorly to the abdominal aorta. Unfortunately, our patient was neither a candidate for mass resection nor for cardiac surgery due to advanced metastatic disease and poor clinical condition. Additionally, we performed a systematic literature review of carcinoid heart disease focusing on typical echocardiographic findings.
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PMID:Carcinoid Heart Disease: Starting From Heart Failure. 2863 93


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