Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Adrenomedullin is a novel hypotensive peptide originally isolated from human pheochromocytoma. Accumulating evidence suggests the possible involvement of adrenomedullin in the physiology of the pulmonary circulation and the pathophysiology of hypoxaemia. The aim of the present study was to investigate the pathophysiological significance of adrenomedullin in hypoxaemia caused by congenital cyanotic heart disease. Subjects were 16 patients with congenital cyanotic heart disease aged 0.8-10 years (Group C) and 12 age-matched control subjects (patients with coronary artery dilatation after Kawasaki disease; Group N). Plasma adrenomedullin concentrations were measured, using radioimmunoassay, in femoral venous, pulmonary arterial and pulmonary venous blood obtained during cardiac catheterization. Plasma adrenomedullin concentrations in Group C were significantly (3-fold) higher than those in Group N at all sampling sites. In Group C, plasma adrenomedullin concentrations in pulmonary venous blood were significantly lower than those in pulmonary arterial blood. Pulmonary uptake of adrenomedullin in Group C was significantly greater than that in Group N. Patients with congenital cyanotic heart disease showed elevated plasma adrenomedullin concentrations and an increased uptake of adrenomedullin in the pulmonary circulation, which may act to dilate pulmonary vessels and increase pulmonary blood flow to alleviate hypoxaemia. Intrinsically increased adrenomedullin levels may function as a compensatory mechanism for hypoxaemia in congenital cyanotic heart disease.
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PMID:Elevated plasma levels of adrenomedullin in congenital cyanotic heart disease. 1033 59

Cerebral infarction in children may be the result of various disease processes, including emboli from intracardiac sources, paradoxical emboli from the venous system, sickle cell disease, cyanotic heart disease, vasculitis affecting the carotid or cerebral vascular system, vascular anomalies, and prothrombotic states. We present a previously healthy adolescent who presented with the acute onset of hemiparesis. Work-up revealed a dilated cardiomyopathy with a left ventricular mural thrombus as the etiology of his cerebrovascular event. Although dilated cardiomyopathy (DCM) may predispose to the development of a mural thrombus and subsequent embolic events, there are no previous reports in pediatric-aged patients of the development of an embolic event as the presenting manifestation of DCM. Further investigation of the etiology of the DCM led to the diagnosis of a pheochromocytoma. Congestive heart failure and DCM as the presenting sign of pheochromocytoma has likewise not been reported in a pediatric-aged patient. We review this unlikely sequence of events, the diagnostic evaluation of such patients, and treatment options.
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PMID:Cerebrovascular event, dilated cardiomyopathy, and pheochromocytoma. 1186 37

Hemorrhagic necrosis of a pheochromocytoma is a rare cause of acute presentation that is often devastating to patients. A 44-year-old woman with spontaneous hemorrhage into a previously undiscovered pheochromocytoma is described. The patient presented with acute cardiogenic pulmonary edema, shock, abdominal pain, myalgia and high fever. Her cardiac function recovered with aggressive medical treatment before the tumour was removed. This case illustrates an unusual presentation of pheochromocytoma and emphasizes the importance of aggressive and appropriate medical therapy in pheochromocytoma heart disease.
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PMID:Cardiogenic shock due to acute hemorrhagic necrosis of a pheochromocytoma: a case report and review of the literature. 1271 95

A 24-year-old female rhesus macaque (Macaca mulatta) presented with a clinical history of chronic heart disease and prolonged recovery from sedation and anesthesia. At necropsy, the heart was markedly enlarged, with thinned ventricular walls, dilated chambers, and severe left atrioventricular valvular insufficiency. The ventricular walls contained numerous narrow, pale, often coalescing streaks that often extended along vessels into the deeper myocardium. Histologically, bands of interstitial fibrosis in the heart were associated with areas of myofiber atrophy, myofibril degeneration and loss, and inflammation. The left adrenal gland contained a 2 x 1 x 0.5 cm tumor with an unusual angiomatous pattern consisting largely of blood-filled sinusoids lined by one to four layers of low cuboidal to polyhedral tumor cells. In most sinusoids, the tumor cells appeared to be in direct contact with blood, although endothelial cells lined some sinusoids. Ultrastructurally, the tumor cells contained abundant electron-dense cytoplasmic granules. These granules were argyrophilic by Sevier-Munger staining and intensely immunoreactive for synaptophysin, chromogranin A, neuron-specific enolase, and S-100. These findings helped confirm the diagnosis of angiomatous pheochromocytoma. The heart lesions match those reported for catecholamine cardiomyopathy in other species.
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PMID:Cardiomyopathy associated with angiomatous pheochromocytoma in a rhesus macaque (Macaca mulatta). 1282 20

We report two cases of pheochromocytoma combined with tetralogy of Fallot who showed different clinical courses. Case 1 was a 45-year-old woman with a history of radical operation for tetralogy of Fallot at 20 years of age. She presented with sudden hypertensive attack, and was diagnosed with pheochromocytoma of the left adrenal gland. She was treated surgically, and her high plasma noradrenaline level normalized. Case 2 was a 41-year-old woman who had been suffering from severe cyanosis due to tetralogy of Fallot throughout her life. A palliative operation had been performed at 7 years of age, but a radical operation had not been performed. She has had resistant hypertension since 38 years of age. She was diagnosed as having pheochromocytoma of the left adrenal gland at 41 years of age, but surgery was not performed. She was pharmacologically treated with doxazosin, followed by bisoprolol. Her symptoms somewhat improved, although she continued to have high plasma levels of noradrenaline and adrenomedullin. The combination of pheochromocytoma with tetralogy of Fallot or cyanotic congenital heart disease is rare; however, pheochromocytoma and congenital heart disease might be related through chronic hypoxia and/or gene abnormalities. The presence of pheochromocytoma worsens the hemodynamic state in patients with congenital heart disease regardless of whether radical surgery for congenital heart disease had been performed. Differential diagnosis of pheochromocytoma could be paramount in congenital heart disease patients who show unexpected or unusual symptoms.
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PMID:Two cases of pheochromocytoma associated with tetralogy of Fallot. 1288 36

It is widely, but mistakenly, believed that ischemic heart disease (IsHD) and its complications are the sole and direct result of reduced coronary blood flow by obstructive coronary artery disease (CAD). However, cardiac angina, acute myocardial infarction (AMI), and sudden cardiac death (SCD) occur in 15%-20% of patients with anatomically unobstructed and grossly normal coronaries. Moreover, severe obstructive coronary disease often occurs without associated pathologic myocardiopathy or prior symptoms, ie, unexpected sudden death, silent myocardial infarction, or the insidious appearance of congestive heart failure (CHF). The fact that catecholamines explosively augment oxidative metabolism much more than cardiac work is generally underappreciated. Thus, adrenergic actions alone are likely to be more prone to cause cardiac ischemia than reduced coronary blood flow per se. The autonomic etiology of IsHD raises contradictions to the traditional concept of anatomically obstructive CAD as the lone cause of cardiac ischemia and AMI. Actually, all the signs and symptoms of IsHD reflect autonomic nervous system imbalance, particularly adrenergic hyperactivity, which may by itself cause ischemia as in rest angina. Adrenergic activity causing ischemia signals cardiac pain to pain centers via sympathetic efferent pathways and tend to induce arrhythmogenic and necrotizing ischemic actions on the cardiovascular system. This may result in ischemia induced metabolic myocardiopathy not unlike that caused by anatomic or spasmogenic coronary obstruction. The clinical study and review presented herein suggest that adrenergic hyperactivity alone without CAD can be a primary cause of IsHD. Thus, adrenergic heart disease (AdHD), or actually adrenergic cardiovascular heart disease (ACVHD), appears to be a distinct entity, most commonly but not necessarily occurring in parallel with CAD. CAD certainly contributes to vulnerability as well as the progression of IsHD. This vicious cycle, which explains the frequent parallel occurrence of arteriosclerosis and IHD, an association that appears to be linked by the same cause, comprises a common vulnerability to deleterious adrenergic actions on the myocardium, lipid metabolism, and vascular system alike, rather than viewing CAD and IsHD as having a putative cause and effect relationship as commonly thought. Adrenergic actions can also cause the abnormal lipid metabolism that is associated with CAD and IsHD by catecholamine-induced metabolic actions on lipid mobilization by activation of phospholipases. This may also be part of toxic catecholamine hypermetabolic actions by enhancing deleterious cholesterol and lipid actions in damaging coronary vessels by plaque formation as well as inducing obstructive coronary spasm and platelet aggregation. This may also cause direct toxic necrosis on the myocardium as well as atherosclerosis in blood vessels. In fact, drugs that inhibit adrenergic actions like propranolol, reserpine, and guanethidine all inhibit arteriosclerosis induced by hypercholesterolemia in experimental animals and prevent carotid vascular disease (associated with stroke) in humans. The concomitant development of myocardiopathy and coronary vascular lesions or coronary and carotid artery intimal medial thickening by catecholamine toxicity is reflected by the frequent primary presentation of patients with catecholamine-secreting pheochromocytoma with cardiovascular disease, ie, hypertension arrhythmias, AMI, SCD, CHF, and vascular disease, which represents a clear example of the primary deleterious impact of catecholamines on the entire cardiovascular system causing adrenergic cardiovascular disease. Thus, like myocardiopathy, CAD and atherosclerosis in general may be the consequences of or a complication of catecholamine actions rather than its putative cause. This report shows how prophylactic bretylium not only prevents arrhythmias but prevents myocardial necrosis, shock, CHF, maintains or restores normal contractility, and lowers mortality in AMI patients by inducing adrenergic blockade.
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PMID:Prevention of ventricular fibrillation, acute myocardial infarction (myocardial necrosis), heart failure, and mortality by bretylium: is ischemic heart disease primarily adrenergic cardiovascular disease? 1535 32

Pheochromocytoma is associated with intense physiologic effects of alpha- and beta-adrenergic stimulation from catecholamine secretion. Perioperative management for these patients includes alpha-adrenergic receptor blockade, intravascular volume replacement, and, if necessary, beta-adrenergic receptor blockade. Significant perioperative changes in preload and afterload, fluid status, heart rate and rhythm, and inotropy can occur and may be contrary to anesthetic management goals for patients with certain conditions of congenital heart disease. We report the perioperative management with doxazosin of a patient with single ventricle physiology and cavo-pulmonary and aorto-pulmonary lung perfusion who presented for resection of a pheochromocytoma.
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PMID:The perioperative management of a patient with complex single ventricle physiology and pheochromocytoma. 1578 8

Adrenomedullin (AM) is a potent, long-lasting vasoactive peptide originally isolated from human pheochromocytoma. Since its discovery, serum and tissue AM expression have been shown to be increased in experimental models and in patients with cardiac hypertrophy, myocardial infarction and end-stage heart failure with several beneficial effects. Considerable evidence exists for a wide range of autocrine, paracrine and endocrine mechanisms for AM which include vasodilatory, anti-apoptotic, angiogenic, anti-fibrotic, natriuretic, diuretic and positive inotropic. Thus, through regulation of body fluid or direct cardiac mechanisms, AM has additive and beneficial effects in the context of heart disease. Notable molecular mechanisms of AM include cyclic adenosine monophosphate, guanosine-3',5'-monophosphate, PI3K/Akt and MAPK-ERK-mediated cascades. Given the endogenous and multifunctional nature of AM, we consider this molecule to have great potential in the treatment of cardiovascular diseases. In agreement, early experimental and preliminary clinical studies suggest that AM is a new and promising therapy for cardiovascular diseases.
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PMID:Adrenomedullin: molecular mechanisms and its role in cardiac disease. 1658 14

A 23-year-old female patient with malignant pheochromocytoma was admitted to the Tokyo Women's Medical University. The patient had been clinically diagnosed with Holt-Oram syndrome at birth. Since she had complex congenital heart disease, chronic heart failure, and severe hypoxia, the risk surrounding surgery to remove the primary tumor was predicted to be very high, and subsequently, chemotherapy was performed. The patient was not able to continue chemotherapy due to adverse effects. However, for one year, both her hypertension and catecholamine-dependent symptoms were well controlled by an alpha-adrenergic and beta-adrenergic receptor blockade, although the patient did experience high plasma norepinephrine levels. To our knowledge, this is the first report of a patient with the combination of malignant pheochromocytoma and Holt-Oram syndrome. A correlation between chronic hypoxia and pheochromocytoma has been reported. This instructive case reminds us to consider the possibility of pheochromocytoma with congenital heart disease when these types of unexpected or unusual symptoms are encountered.
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PMID:A case of malignant pheochromocytoma with Holt-Oram syndrome. 1825 May 43

Surgical treatment of pheochromocytoma is associated with high hemodynamic risk, which is even higher in patients with complex congenital heart disease. Nowadays, patients with cyanotic congenital heart disease are living longer and an increased incidence of pheochromocytoma has been reported in this population. We demonstrate the feasibility and importance of minimally invasive surgery in the management of pheochromocytoma in a 45-year-old woman with complex congenital heart disease and Eisenmenger's syndrome. A successful laparoscopic resection of the tumor was performed in association with multidisciplinary management during hospitalization.
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PMID:Pheochromocytoma in Eisenmenger's syndrome: a therapeutic challenge. 2142 42


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