Atypical presentation of carcinoid heart disease (case report and literature’s review)
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Abstract:
Carcinoid disease is a rare neoplasm but it's an important etiology of intrinsic right heart valve disorders that leads to right heart failure. carcinoid heart involvement is a common complication of disease that effects on morbidity and mortality of the patient.Keywords:
Carcinoid Heart Disease
Presentation (obstetrics)
Etiology
Rare disease
Carcinoid Heart Disease
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The carcinoid syndrome is rare but it is associated with carcinoid heart disease in more than a half of the cases. Carcinoid heart disease is typically characterised by morphological and functional modifications of right-sided valves. Its aetiology is probable multifactorial but serotonin appears to play a key role in the development of this valvular disease. Unlike gastrointestinal neuroendocrine tumours, ovarian neuroendocrine tumours can present with carcinoid syndrome and carcinoid heart disease in the absence of liver metastases; such ovarian neuroendocrine tumours are a unique clinical entity. The additional burden of cardiac impairment in these patients represents a significant reduction in survival. Early recognition and surgical valve replacement before advanced heart failure is established may improve the clinical outcome. We report the case of a woman with an ovarian neuroendocrine tumour and highly symptomatic carcinoid heart disease who was submitted to tumour resection followed by valvuloplasty. She demonstrated an outstanding clinical improvement and has remained free of tumour and symptomatology.A síndrome carcinoide é rara mas encontra-se associada, em cerca de metade dos casos, a doença carcinoide cardíaca. Esta patologia cardíaca caracteriza-se tipicamente por alterações morfo-funcionais das válvulas direitas. A sua etiologia é provavelmente multifatorial mas, aparentemente, a serotonina parece exercer um papel preponderante no desenvolvimento desta doença valvular. Ao contrário dos tumores neuroendócrinos gastrointestinais, os tumores neuroendócrinos do ovário podem apresentar síndrome carcinoide e doença carcinoide cardíaca na ausência de metastização hepática, tornando os tumores neuroendócrinos do ovário uma entidade clínica única. Estas alterações cardíacas acarretam uma diminuição adicional da sobrevivência destes doentes. Portanto, o reconhecimento precoce desta entidade e a substituição valvular prévia ao estabelecimento da insuficiência cardíaca avançada podem melhorar a evolução clínica destes doentes. Os autores apresentam o caso de uma mulher com tumor neuroendócrino do ovário e doença carcinoide cardíaca muito sintomática que foi submetida a resseção tumoral seguida de valvuloplastia. A doente demonstrou uma marcada melhoria clínica e tem permanecido livre de doença e assintomática.
Carcinoid Heart Disease
Carcinoid tumour
valvular heart disease
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Carcinoids are rare tumors originating from neuroendocrine cells. A large proportion of these tumors produce serotonin and other biologically active hormones which may produce carcinoid syndrome characterized by flushing, diarrhoea and bronchospasm. Carcinoid heart disease, a rare complication of carcinoid syndrome, may itself have a great impact on life expectancy of patients with carcinoid syndrome. The authors present a case history of a patients with carcinoid heart disease and they review the symptoms, diagnosis and therapeutic options of this rare complication of carcinoid syndrome. Orv. Hetil., 2013, 154, 546–550.
Carcinoid Heart Disease
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Introduction: Carcinoid syndrome is the most frequent functional syndrome of neuroendocrine neoplasia. It is characterized by flushing, diarrhea, wheezing, hypotension, and exanthema and may cause carcinoid heart disease. Methods: We assessed clinical characteristics and prognosis of patients with carcinoid syndrome and carcinoid heart disease in 276 patients from 3 referral centers. Results: Carcinoid syndrome patients had a mean age of 57 years (range 21–84) and a normal BMI of 24.9 (SD 4.5; range 13.8–39.6). Most primaries were of small bowel or unknown primaries with distant metastasis in 94.6%. Flushing was the most frequent symptom in 74.3% of patients, followed by diarrhea in 68.8%, and wheezing in 40.9%. Pain was described by 45.3%, weakness by 23.5%, and weight loss of >10% in 6 months by 30.1% of patients. Carcinoid heart disease was diagnosed in 37.3% of patients (n = 104) by echocardiography and involved predominantly in the tricuspid valve. Combinations with other valve defects were common. Somatostatin analogs were taken by 80.4% of patients and 17% needed additional loperamide/opium tincture. Surgery and peptide receptor radiotherapy were most frequent treatments. The median survival of patients with carcinoid syndrome after diagnosis was 9 years. Prognosis was significantly impaired by male sex and diagnosis of carcinoid heart disease but surprisingly significantly increased by the presence of symptoms flushing and weakness. Discussion/Conclusion: Carcinoid syndrome is associated with extensive disease and primaries in small bowels or of unknown primary. Weight loss, weakness, and pain are frequent, and carcinoid heart disease is diagnosed in more than one-third of patients.
Carcinoid Heart Disease
Carcinoid tumors
Loperamide
Carcinoid tumour
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Carcinoid heart disease is a frequent manifestation of carcinoid syndrome. It results from the release of a large amount of serotonin and subsequently fibrosis of right sided heart valves, that is, tricuspid and pulmonic valve. This article reviews the pathogenesis, clinical symptoms, diagnosis, treatment and prognosis of carcinoid heart disease. Recent developments in treating carcinoid heart disease have improved the poor prognosis associated with the disease.
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A 39-year-old lady presented in early 2011 with Carcinoid syndrome due to Ileal Carcinoid with liver metastases and Carcinoid valvular heart disease with Right heart failure. Liver resection combined with ethanol injection of Liver metastases was carried out after reduction of Central Venous Pressure by an initial Tricuspid valve replacement (TVR), in a staged manner, with resolution of symptoms. This case highlights the necessity of TVR prior to major Liver resection for Liver metastases in Carcinoid heart disease with Tricuspid regurgitation as well as the value of treating Carcinoid heart disease aggressively.
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valvular heart disease
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A carcinoid tumorok neuroendokrin sejtekből szarmazo ritka daganatok. E daganatok jelentős resze szerotonint es mas biologiailag aktiv hormonokat termel, amelyek lilaspiros bőrpirral, hasmenessel es bronchospasmussal jaro tunetegyuttest, carcinoid szindromat okoznak. A carcinoid szindroma ritka szovődmenye a carcinoid szivbetegseg, ami onmagaban lenyegesen befolyasolja a carcinoid tumoros betegek eletkilatasait. A szerzők carcinoid szivbetegsegben szenvedő ferfi kortortenetet ismertetik es attekintik e ritka szovődmeny jellegzetessegeit. Orv. Hetil., 2013, 154, 546–550.
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Carcinoids are rare tumors originating from neuroendocrine cells. A large proportion of these tumors produce serotonin and other biologically active hormones which may produce carcinoid syndrome characterized by flushing, diarrhoea and bronchospasm. Carcinoid heart disease, a rare complication of carcinoid syndrome, may itself have a great impact on life expectancy of patients with carcinoid syndrome. The authors present a case history of a patients with carcinoid heart disease and they review the symptoms, diagnosis and therapeutic options of this rare complication of carcinoid syndrome. Orv. Hetil., 2013, 154, 546–550.
Carcinoid Heart Disease
Carcinoid tumors
Carcinoid tumour
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Carcinoid Heart Disease
Chromogranin A
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