Prognosis of carcinoid heart disease: analysis of 200 cases over two decades.
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BACKGROUND: The long-term prognosis of patients who develop carcinoid heart disease and the effect of cardiac surgery on outcome are not well established. METHODS AND RESULTS: In this retrospective study, we identified 200 patients with carcinoid syndrome referred for echocardiography in whom the diagnosis of carcinoid heart disease was confirmed. Patients were divided into 3 groups of similar size according to the date from first diagnosis of carcinoid heart disease. Group A comprised patients diagnosed from 1981 through June 1989; group B, diagnosed July 1989 through May 1995; and group C, June 1995 through 2000. The end point was all-cause mortality. Median survival was significantly lower in group A (1.5 years, 95% CI 1.1 to 1.9 years) compared with groups B (3.2, 95% CI 1.3 to 5.1 years) and C (4.4, 95% CI 2.4 to 7.1 years; P=0.009). In a multivariate model adjusted for treatment and clinical characteristics, the risk of death in groups B (hazard ratio 0.67, 95% CI 0.46 to 0.99, P=0.04) and C (hazard ratio 0.61, 95% CI 0.39 to 0.92, P=0.006) was significantly reduced relative to group A. Cardiac surgery was performed in 87 patients. When cardiac surgery was included as a time-dependent covariate in a multivariate analysis, it was associated with a risk reduction of 0.48 (95% CI 0.31 to 0.73, P<0.001), whereas the time period of diagnosis was no longer significant. CONCLUSIONS: The prognosis of patients with recognized carcinoid heart disease has improved over the past 2 decades at our institution. This change in survival may be related to valve replacement surgery. (+info)
A most unusual acute coronary syndrome.
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A 60-year-old man, known for stable coronary artery disease, was admitted for suspected unstable angina. In the previous month, the patient presented with progressive dyspnea on light exertion. In the preceding four months, he had experience occasional episodes of flushing and diarrhea, and had inexplicably lost 22.7 kg. Night sweats and fever were absent. ST segment elevation in the inferior leads and ST segment depression in the precordial leads were documented during an episode of chest pain. The coronary angiogram showed diffuse disease with 70% stenosis of the left anterior descending coronary artery and 50% stenosis on the second diagonal (D(2)). An echocardiogram showed a patent foramen ovale. Balloon angioplasty and stenting were performed on the two lesions. Two days later, prolonged chest pain recurred. Cardiac catheterization was repeated and showed occlusive thrombus within the stent on the D(2). Angioplasty was repeated. Symptoms recurred 36 h later, with the electrocardiogram showing ST segment elevation. The first angiogram was reviewed and vasospasm was suspected on a branch of the D(2), on the second marginal and in the distal circumflex artery. The diagnosis of vasospastic angina was retained. Beta-blockers were replaced by high doses of a calcium channel blocker with an excellent clinical response. The case described is of a patient with an acute coronary syndrome, vasospastic angina, in-stent thrombosis and carcinoid disease. Coronary vasospasm was attributed to serotonin, which was secreted by the carcinoid tumour that reached an atherosclerotic coronary vasculature through a patent foramen ovale, thereby avoiding pulmonary inactivation. (+info)
Echocardiographic diagnosis of carcinoid disease: exceptional evolution after ovarian tumor resection without cardiac surgery.
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There are very few cases described in literature with carcinoid heart disease caused by a pure ovarian tumor. Right heart failure remains the major cause of morbidity and mortality. Cardiac surgery is the only definitive treatment when cardiac symptoms become severe but with high perioperative mortality rates. We present a 68-year-old woman with a pure ovarian carcinoid tumor complicated with heart disease alive 7 years later after ovarian resection, without valve replacement and with regression of tricuspid and pulmonary injury. Echocardiography was the diagnostic clue. (+info)
Carcinoid crisis and reversible right ventricular dysfunction after embolization in untreated carcinoid syndrome.
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A 59-year-old white male with carcinoid tumor and hepatic metastases underwent hepatic artery embolization. The patient developed carcinoid crisis and a subsequent transthoracic echocardiogram showed classic findings of carcinoid heart disease along with a dilated hypertrophied right ventricle and severely depressed right ventricular ejection fraction. After treatment with octreotide the patient's clinical condition improved and a repeat transthoracic echocardiogram showed a significant improvement and normalization of right ventricular systolic function. Serotonin levels showed a progressive decline that correlated well with the patient's improved clinical condition. These findings suggest that the acute right ventricular dysfunction was secondary to acute carcinoid crisis and resolution resulted in a significant improvement of both right ventricular systolic function and clinical condition. (+info)
Profound hypoxaemia corrected by PFO closure device in carcinoid heart disease.
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A 66-year-old man with known metastatic carcinoid tumor presented with increasing dyspnoea, right heart failure and marked hypoxaemia which did not correct with oxygen. Echocardiography demonstrated severe tricuspid regurgitation, moderate pulmonary regurgitation and marked right heart dilatation. The inter-atrial septum was aneurysmal, with a large patent foramen ovale (PFO) with continuous right to left shunting. Cardiac catheterization demonstrated oxygen saturations of 96% in the pulmonary veins and 74% in the left atrium with a significant right to left shunt. During percutaneous closure of the PFO, anaesthetic induction resulted in marked systemic hypotension and worsening hypoxia related to systemic vasodilatation and increased shunting. PFO flow was temporarily obstructed with a sizing balloon resulting in a rapid increase in arterial oxygen saturation from 60% to >90%, but marked systemic hypotension due to acute left ventricular preload reduction, requiring volume replacement and adrenaline. Following deployment of a PFO occluder device, prominent pulsatile splaying of the right and left discs was noted due to the severe tricuspid regurgitation, resulting in some residual inter-atrial shunting. Arterial oxygen saturation was 83%, increasing to 92% at day 4 post-procedure as tissue organization occurred within the device, and the patient reported improvement in dyspnoea. (+info)
Description of patients with midgut carcinoid tumours: clinical database from a Danish centre.
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BACKGROUND: We have initiated a clinical database of patients with neuroendocrine tumours (n = 132). Data on patients with well-differentiated endocrine carcinoma (WHO classification) previous classified as midgut carcinoid patients, are presented. PATIENTS AND METHODS: Retrospectively, 56 patients with midgut carcinoid tumours were evaluated with respect to symptoms, primary tumour size, metastases, tumour markers, treatment and survival. RESULTS: Flushing was described in 29%, diarrhoea in 52%, abdominal pain in 34%, bronchial constriction in 2% and carcinoid heart disease in 4% of the patients. Fifty-two percent had liver metastases at referral. Twenty-seven percent were considered to have had radical surgery. Patients not considered for radical surgery and patients with liver metastases had significantly higher tumour marker levels (serum chromogranin A (CgA), serum serotonin and urinary 5-hydroxyindolic acid (5-HIAA)) compared to radically-operated patients and to patients without liver metastases (p<0.05, respectively). For all the midgut carcinoid tumour patients the overall 5-year survival rate was 72%. The radically-operated patients had a 5-year survival rate of 100% (other death causes excluded). The patients with normal CgA or <5 liver metastases at referral had a 100% 5-year survival rate. The patients with <5 liver metastases had a significantly better 5-year survival rate compared to patients with multiple liver metastases (100% vs. 50%, p<0.05). CONCLUSION: This group of patients exhibited the same characteristic clinical features with similar survival as reported from other specialised centres. Radical surgery, normal CgA level and <5 liver metastases indicated a good prognosis and patients with <5 liver metastases had a significantly better survival compared to patients with multiple liver metastases. (+info)
Management of patients undergoing multivalvular surgery for carcinoid heart disease: the role of the anaesthetist.
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Bioprosthetic pulmonary and tricuspid valve replacement in carcinoid heart disease from ovarian primary cancer.
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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. (+info)