Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018801 (heart failure)
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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

Carcinoids are rare endocrine tumors that can develop in several organs in the body. Clinically, patients can have a wide spectrum of signs and symptoms that range from incidental findings of a polyp during endoscopy to the carcinoid syndrome characterized by severe flushing, diarrhea, abdominal cramping, and life-threatening right-sided heart failure. Most carcinoid tumors are indolent but can metastasize to regional lymph nodes and to other organs, including the liver, bone, and the central nervous system. Treatment is determined by tumor location and by the presence of distant metastasis. Surgical resection of the tumor is advocated in patients with localized disease and can often be curative. Long-acting somatostatin analogs, including octreotide, octreotide long-acting repeatable, and lanreotide prolonged release, are effective in providing symptom relief in patients with the carcinoid syndrome. Patients with metastatic disease to the liver that is refractory to somatostatin treatment should be considered for hepatic artery occlusion. Overall, 5- and 10-year survival rates in patients with metastatic disease are favorable, although tumors can be resistant to most forms of medical or surgical therapy.
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PMID:Carcinoid tumors. 1207 68

Cytoreductive therapy is effective in the management of metastatic neuroendocrine tumors to the liver, independent of their functioning status. In functioning tumors, clinical endocrinopathies are relieved in most patients and this response usually lasts for several months. Major morbidity and mortality are not greater than the average complication rate for resection for nonneuroendocrine metastatic tumors at major centers; therefore, surgical outcomes appear to justify operative intervention. Patients whose primary tumor can be controlled, whose metastases outside the liver are limited, and who have a reasonable performance status are candidates for resection. The authors' data support the previous statements. The current mortality rate of 1.2% and major morbidity rate of 15% clearly represent the success of the operative approach in such complex cases (54% of patients received a resection of at least one lobe) [9]. A symptomatic response in the 95% range with a median response of 45 months adds many months of symptom-free survival to the lives of most patients [9]. In the literature reviewed for this article, more than half of the patients also underwent a major hepatic resection and 40% of them had concurrent resection of the primary tumor. These data confirm that resection in selected patients is not more complicated or risky than resection for other metastatic tumors. Endocrinopathies have not increased anesthetic or operative risk in this population; however, these results are the product of managing these patients over time, becoming familiar with their clinical syndromes, and being active in the prevention of life-threatening endocrine complications (i.e., carcinoid crisis). The authors have learned over time that patients with valvular disease are not good candidates for surgery. These patients develop right-sided heart failure with an increase in the central venous pressure. This condition can result in massive hemorrhage during the liver resection because of the difficulty in controlling backbleeding from the hepatic veins [26]. Correction of valvular disease is warranted for safe liver resection. The authors' current policy is to rule out valvular disease in every patient with carcinoid tumors and repair the valves prior to hepatic resection when indicated [27]. This policy clearly has decreased the complication rate. Even though liver transplantation seems to be very attractive as a means of eradicating the disease, this has not been common in clinical practice because of the shortage of allografts, and the overall costs and complications of the procedure override its benefits, especially when compared with partial hepatectomy. Current methods to detect the spread of disease that were not readily available in the past, such as MRI and indium-111 pentetreotide (Octreoscan), may expand the applications of transplantation and allow for better selection of candidates. The option of transplantation is still open for improvement and is dependent on organ availability and better staging of the disease. Metastases from neuroendocrine tumors are hypervascular, favoring the application of MRI as the single imaging method; MRI not only evaluates the location and characteristics of the lesions but also determines the relationship with major vessels and bile ducts. Spiral CT scan has been used extensively in the past with acceptable results. Indium-111 pentetreotide functions on the base of somatostatin receptors present in these tumors, but its use has not been established definitely in the work-up of these patients. Perhaps the best use of indium-111 pentetreotide is in the evaluation of disease beyond the primary and liver locations, including bone metastases; its use therefore will likely affect the preoperative work-up of candidates for transplantation [28]. Once the patient has been deemed to have resectable disease by the preoperative work-up, several steps need to be completed prior to surgery to decrease the effect of specific endocrinopathies. For patients with symptoms related to carcinoid tumors, preoperative preparation with 150 to 500 micrograms of somatostatin decreases the chances of carcinoid crisis, which is manifested by hemodynamic instability [29]. The use of this medication intraoperatively should be kept in mind because a carcinoid crisis can occur despite anesthetic premedication. For islet cell tumors, treatment of underlying endocrinopathy has been initiated before referral for surgical treatment in most patients. Surgery is appropriate for patients with metastatic neuroendocrine tumors for the following two reasons: (1) many of them still have the primary tumor in place and resection should be undertaken to avoid acute complications and (2) the addition of adjunctive ablative therapies to surgical resection accomplishes the control of greater than or equal to 90% of the bulk of the tumor. If preoperative evaluation indicates that less than 90% of the tumor is treatable, surgical therapy is contraindicated. Last, even when complete resections are performed, the recurrence rate for these tumors is extremely high. In practical terms, patients with metastatic neuroendocrine tumors are seldom cured. The best hope physicians can offer these patients is an extended survival period with minimal endocrine symptoms and decreased requirements of somatostatin analogs.
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PMID:Hepatic surgery for metastases from neuroendocrine tumors. 1273 41

A rare clinical occurrence, heart failure in the setting of tricuspid stenosis should immediately trigger a search for underlying systemic carcinoid disease. Carcinoid tumor cells can secrete a variety of vasoactive substances that result in skin erythema, excretory diarrhea, bronchospasm, and hemodynamic instability, but these manifestations are noted only in a few patients. Right heart valvular disease is common since the vasoactive noxious substances pass through the right heart unaffected and undergo metabolism in the pulmonary circulation, thereby decreasing involvement of the left-sided valves. Localization of the carcinoid tumor followed by surgically directed valvular treatment is mandatory for relief of symptoms. In nonoperative candidates, cytotoxic chemotherapy or long-term symptomatic drug treatment with somatostatin is indicated.
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PMID:Isolated tricuspid stenosis and heart failure: a focus on carcinoid heart disease. 1456 50

Carcinoid tumors are associated with the production of a number of bioactive substances that might deposit on the endocardium (endocardial fibroelastosis) and produce cardiac failure. Carcinoid heart disease usually presents as right-sided valvular heart disease. Even if the diagnosis is frequently made by transthoracic echocardiography, cardiac magnetic resonance imaging adds valuable information when evaluating cardiac structures that might be difficult to analyze on echocardiography.
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PMID:Magnetic resonance imaging diagnosis of carcinoid heart disease. 1627 47

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 heart disease (CHD) develops in serotonin-producing neuroendocrine tumours (NET) due to fibrotic endocardial plaques with associated valve dysfunction leading most often to right-sided heart failure. The classical carcinoid syndrome usually occurs when serotonin-producing NET metastasize to the liver. Up to 50% of those patients will exhibit carcinoid heart disease. The pathophysiological process is not yet completely understood: serotonin is considered to be a major initiator of the fibrotic process, but other tumour secreted factors may contribute to the pathogenesis. Histopathology reveals intact valvular cusps with superimposed fibrotic plaques, leading to thickening and retraction of the valves, causing valvular dysfunction. A high index of clinical suspicion to diagnose CHD is needed since symptoms can be rather non-specific. Transthoracic echocardiography is the gold standard for diagnosis and should probably be performed at the time of diagnosing serotonin-producing NET and then repeated annually. On the other hand, when diagnosing right-heart failure, the presence of CHD and underlying serotonin-producing NET should be taken into account. Therapeutic options include pharmacotherapy for heart failure, control of the systemic carcinoid disease and in selected individuals cardiac valve replacement. The elucidation of the pathologic process is necessary to develop targeted antifibrotic therapeutic agents since CHD seems to be irreversible and associated with poor prognosis.
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PMID:Carcinoid heart disease--a hidden complication of neuroendocrine tumours. 1940 69

Cardiac surgery for symptomatic carcinoid heart disease in conjunction with adjunctive therapy is being increasingly shown to improve the long-term outlook of patients with carcinoid heart disease. Herein is reported the case of a female patient with heart failure, secondary to carcinoid heart disease affecting all four cardiac valves, who successfully underwent quadruple valve replacement in the same sitting. The patient made an uneventful postoperative recovery, and had an excellent quality of life at the one-year follow up examination.
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PMID:Quadruple valve replacement for carcinoid heart disease. 1945

Endocrine tumors could be defined by their ability to produce structural proteins or hormones common to nervous and endocrine cells. They might induce physiological transforms or outcome adverse events which should be well known in order to prevent or treat them early. The goal of this review was to describe these changes, to describe preoperative assessment, and to discuss intraoperative monitoring and drugs choice based on the literature from the last 30 years. As an example, it should be noticed that: (1) preoperative blood pressure control is essential to prepare phaeochromocytoma for surgery. It should be followed during anaesthesia by intensive fluid load, reversible anaesthetic drugs and rational cardiovascular medications use (as for example remifentanil, sevoflurane, calcium channel blockers and esmolol), and after surgery by narrow clinical and biological monitoring; (2) after medullar thyroid cancer, main adverse events include cervical compressive haematoma and recurrent laryngeal nerve injury as for any thyroid surgery; (3) during pituitary surgery, air embolism might be expected, whereas water dysregulation (diabetes insipidus), corticotroph insufficiency, cerebrospinal fluid (CSF) leak might occur postoperatively. In acromegaly, difficult endotracheal intubation is possible whereas severe Cushing's syndrome may be complicated with hypertensive cardiac failure, infections, thrombosis, delayed cicatrisation; (4) somatostatine analogs are a keystone in carcinoid tumors preoperative and anaesthetic management.
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PMID:[Anaesthesia for endocrine tumor removal]. 1946 26

Carcinoids are the most common neuroendocrine tumours. They are usually slowly growing, located in the small intestine, secrete serotonin, and are characterized by long survival of patients, so prognosis is generally good. The most frequently encountered clinical presentations of carcinoids are intermittent abdominal pain and carcinoid syndrome (diarrhoea and flushing). Metastases worsen the prognosis and limit the survival of the patients. We report a case of carcinoid tumour with primary focus in the ileum, with an appendix infiltration, in a thirty-two-year-old woman with acute appendicitis symptoms only. Carcinoid was diagnosed postoperatively by histopathological examination. Nowadays, twenty-five years after the surgery, there is evidence of nearly asymptomatic numerous metastases. Only intermittent abdominal pain for about 1-2 years was reported. Partial metastases resection was performed, followed by chemotherapy, (90)Y-DOTATATE and then long-acting release octreotide analogue therapy. In the meantime, severe chronic heart failure (NYHA IV) due to tricuspid combined valvular heart disease and pulmonary hypertension was diagnosed. Combined therapy, typical for chronic heart failure, together with long-acting octreotide analogue highly improved the patient's heart sufficiency and reduced carcinoid syndrome symptoms. The only adverse events of octreotide therapy were hyperbilirubinaemia and itching. Long-term survival is typical for carcinoids, but 30-years survival has not been described in the literature yet.
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PMID:Long-term survival and nearly asymptomatic course of carcinoid tumour with multiple metastases (treated by surgery, chemotherapy, (90)Y-DOTATATE, and LAR octreotide analogue): a case report. 1988 12


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