Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018801 (heart failure)
72,216 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A surgically treated case of left atrial myxoma is reported. A 66-year-old man with a history of cough and orthpnea had an echocardiographic and an MRI diagnosis of left atrial myxoma. He had the constitutional signs of myxoma including acceleration of E.S.R., positive CRP, hyperimmunoglobulinemia, loss of body weight, and so on, in addition to the symptoms of heart failure. Cardiac surgery was performed on him under extracorporeal circulation on June 12, 1990. A large myxoma with a diameter of 6.0 cm x 4.8 cm that was adhering to the fossa ovalis with a stalk was resected. Afterwards the symptoms of both heart failure and the constitutional signs disappeared, and the postoperative course was uneventful. Studies of the excised specimen demonstrated that this tumor produced Interleukin (IL-6). After operation the level of the serum IL-6 that was high before operation was normalized. This suggests that the symptoms and the laboratory results pointing to an autoimmune disease were due to the IL-6 produced from the cardiac myxoma. This is the first report that the localization of the IL-6 in the left atrial myxoma is demonstrated with immunohistochemical stain.
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PMID:[Left atrial myxoma with production of interleukin 6]. 159 79

A 40-year-old woman was admitted because of increasing exertional dyspnea. Right heart failure was suggested by the presence of hepatomegaly, pretibial edema and also echocardiographic findings. Physical examination and echocardiography showed no evidence of valvular disease or congenital heart disease except for right ventricular dilatation and tricuspid regurgitation. The ventricular septum deviated toward the left ventricle throughout the cardiac cycle, but left ventricular function was preserved. Severe pulmonary hypertension averaging 44 mmHg was revealed by cardiac catheterization. Digital subtraction angiography and pulmonary blood flow scintigraphy showed no evidence of pulmonary artery embolism, and no interstitial pulmonary lesions that might have caused pulmonary hypertension were recognized. Hypergammaglobulinemia suggested an autoimmune disorder, and signs of systemic lupus erythematosus (SLE), such as pleural effusion, proteinuria, lymphocytopenia, LE cell phenomenon and antinuclear antibodies were present. Several autoimmune diseases are known to be causative factors of pulmonary hypertension. However, only ten cases of SLE complicated by pulmonary hypertension have been reported the present one. These cases were characterized by a high incidence of Raynaud's phenomenon and positivity for anti-RNP antibody. In our present case, SLE activity was suppressed using prednisolone, but pulmonary hypertension persisted and the patient eventually died due to right cardiac failure. Judging from the clinical course of the ten reported cases of SLE-pulmonary hypertension, there seems to be no hope of improving the pulmonary hypertension once it has become established. Therefore it is important to detect and cure pulmonary hypertension as early as possible.
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PMID:[A case of lupus erythematosus preceded by right heart failure due to pulmonary hypertension]. 174 69

Recent evidence suggests that the most common form of idiopathic cardiomyopathy in our altitudes, the dilated cardiomyopathy (DCM), is a post-infectious autoimmune disease which is triggered by virus infections. In animal experiments, the development of the coxsackie virus B3 myocarditis to a congestive cardiac insufficiency resembling the clinical picture of DCM was demonstrated. In mice, species-dependent varying disease courses could be observed, which point to a genetically different behaviour of the animals' immunological reactions, either humoral or T-cell mediated immune reactions being responsible. In comparison with non-DCM patients, patients with DCM and chronic myocarditis exhibit significantly higher coxsackie virus antibody titres. Obviously, also a differently long viral persistency in the cardiac muscle plays a role, as enterovirus-specific RNA was detected in myocardial biopsies from patients with DCM. Along with myocardial fibroses, endomyocardial biopsies in DCM frequently reveal mononuclear cellular infiltrates, which, however, only in 20-25% of the cases may be regarded as chronic persisting myocarditis. The clinical and paraclinical findings in DCM and in the so-called latent cardiomyopathy are presented. In congestive heart failure, the best therapeutic results are achieved by the ACE inhibitors, along with vasodilator agents, digitalis glycosides and diuretics. Ultima ratio is the orthotopic heart transplantation, as it is only this intervention that will be able to improve the primarily bad prognosis decisively. Whether the treatment with immunosuppressive drugs exerts an influence upon the prognosis, has thus far remained an open question.
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PMID:[Dilated cardiomyopathy--heart muscle disease of unknown origin or an autoimmune disease? New aspects of etiology, pathogenesis and clinical practice]. 268 30

We studied the conduction system of 65 cases of proven active or healed myocarditis and related diseases among 7120 autopsy samples. For this purpose, we prepared serial sections by Lev's method. The pathological diagnoses were idiopathic acute myocarditis (5), giant cell myocarditis (3), chronic myocarditis (13), healed myocarditis (22), sarcoidosis (4), collagen or autoimmune disease (13) and complication of cachexia (5). Among all the autopsy cases, Fiedler's myocarditis was found in only one case, but myocarditis was revealed in 19 out of 30 cases of dilated cardiomyopathy, and 15 out of 25 cases of sick sinus syndrome. Conduction system lesions were divided into two groups. In older cases manifesting mainly arrhythmia, the SA node, atrial muscle and AV node were involved concomitantly with perimyocarditis. In younger cases mainly showing heart failure, the RBB, LBB and Purkinje fibers were damaged by endomyocarditis. Histologically, interstitial myocarditis was observed in the former group and parenchymatous myocarditis in the latter.
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PMID:Myocarditis and arrhythmia: a clinico-pathological study of conduction system based on serial section in 65 cases. 271 70

Graves' disease, which is an organ-specific autoimmune disorder, is the most frequent cause of thyrotoxicosis. Females are more often affected than males. The clinical pattern varies, however, and nearly every organ system may be involved. This case study describes a formerly healthy young woman with Graves' disease who was admitted to the hospital with cardiac failure, convulsions and generalized lymphoid hyperplasia. A reversible thyrotoxic cardiomyopathia combined with hyperdynamic circulation may result in cardiac failure even in younger patients, and the cardiac function normalizes in the euthyroid state. Seizures may occur in association with hyperthyroidism. High doses of propranolol may precipitate the condition by lowering the threshold for seizures.
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PMID:[Heart failure and convulsions in thyrotoxicosis. A young woman with Graves' disease]. 797 5

We have implanted cardiac pacemaker (PM) in 3 early neonates ranging from 5 hours old to 7 days old of age with congenital complete or incomplete AV block. They were in advanced stage of rapidly developed cardiac failure with resistance to drug therapy and with ventricular tachycardia or fibrillation. Cardiac failure was improved immediately in the 3 cases after the operation and the effectiveness by the pacing was recognized for a long term observation (4 months to 2 years). There appeared PM implantation in-14 neonates, including our cases, in Japanese literatures. In review of the 14 neonates, we discussed the autoimmune disease, indication of the pacing, implantation site of the PM generator and prognosis. An immediate PM implantation after birth in neonate with cardiac failure complicated by congenital AV block is treatment of choice. The generator as well as the electrode design for neonates are still yet to be developed.
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PMID:[Pacemaker implantation in 7 days or less old of early neonates]. 930 62

Autoimmune diseases, especially autoimmune thyroid disease, frequently develop after delivery due to the immune rebound mechanism. Most cases have transient dysfunction of affected organs. Cardiac dysfunction developed after delivery is called postpartum or peripartum cardiomyopathy. However, the aetiology of the disease is not clarified yet. Here we report three cases that developed acute heart failure in the postpartum period. One was complicated with an atrioventricular block and postpartum autoimmune thyroiditis. All patients recovered to normal cardiac function or pre-attack condition after 1 month of therapy with conventional drugs and bed rest. All three had positive antiheart antibody detected by indirect immunofluorescence assay, and one had antibody to heart myosin detected by enzyme-linked immunosorbent assay. Moreover, one of two patients examined revealed lymphocytic infiltration by endomyocardial biopsy. Antibodies to 26 viruses were not elevated significantly during the first 2 weeks after admission in any case. It is strongly suggested that heart failure is induced by postpartum autoimmune myocarditis, and thus clinicians should be aware of this disease.
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PMID:Postpartum onset of acute heart failure possibly due to postpartum autoimmune myocarditis. A report of three cases. 1008 23

Congenital atrioventricular block is defined (Yater) as a documented conduction defect in a young subject with unquestionable bradycardia in the absence of a history of infection which might have caused the condition after birth. It is a rare condition (1 out of 20,000 births) and may be isolated or associated with another congenital malformation. Four cardiac malformations are classically associated: endomyocardial fibrosis, morphological abnormalities close to the conduction system: corrected transposition, left isomerism; patent ductus arteriosus and atrial septal defect apparently unrelated but the most common; the association of mitral regurgitation in adults, although the significance is not very clear. Isolated block is often observed in patients with mothers suffering from autoimmune disease, often clinically latent. Anti Ro/SS-A and La/SS-B antibodies cross into the foetal circulation and cause inflammation of the conduction tissues but the causal mechanism is not known. The diagnosis of the conduction defect is sometimes made during foetal life by echocardiography. After birth, the diagnosis is made by electrocardiography but the block is not always complete or permanent, its degree often increasing with time. In addition, in advanced degrees of block, the escape rhythm tends to slow down. Long-term follow-up studies have revised the previously considered good prognosis of isolated congenital atrioventricular block but advances in cardiac pacing provide satisfactory treatment. In the foetus, isolated atrioventricular block is usually associated with an escape rhythm > 60/min and enables normal vaginal delivery; a low heart rate < 55/min and anasarca carry a poor prognosis. In the neonate, pacing is indicated in babies with cardiac failure and a heart rate < 55/min. Follow-up by Holter monitoring, exercise testing and echocardiography is justified in children and adolescents; the patients may become symptomatic at any age. Pacing is essential in symptomatic cases (malaise, ventricular dysfunction) and useful in cases with long QT intervals, frequent ventricular extrasystoles and wide ventriculogrammes. Pacing is not always easy in children. Epicardial pacing by thoracotomy or an epigastric approach is possible but endocavitary pacing is to be preferred using thin pacing catheters introduced via the subclavian vein and small pacemakers implanted in a sub or prepectoral site. A pacing mode which restores the normal atrioventricular sequence is theoretically superior to single ventricular pacing even if rate responsive frequency.
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PMID:[Congenital atrioventricular block]. 1032 58

Dilated cardiomyopathy (DCM) is a major cause of heart failure in younger individuals. Its prognosis is poor with 40-50% of patients dying within 2 years after diagnosis. Although the etiology of DCM is poorly understood, there is increasing evidence that DCM may represent an autoimmune disease in a significant subset of patients. In order to identify candidate antigens in DCM, we applied a molecular strategy which combines recombinant expression cloning and autoimmunological screening procedures. A left ventricle from a male DCM patient was explanted at heart transplantation and a human DCM left ventricular cDNA-expression library was constructed. 2 x 10(6) clones were immunologically screened with serum collected from the same patient prior transplantation. Subsequent rounds of screening and purification allowed isolation of a positive clone which was sequenced and identified as Recombination Signal Binding Protein-jkappa (RBP-jkappa). RBP-jkappa is an already identified transcription factor, e.g., involved in Epstein-Barr-virus-induced immortalization processes. Radioactively labeled RBP-jkappa protein was synthesized via in vitro translation using the isolated RBP-jkappa cDNA. This RBP-jkappa protein was used for immunoprecipitation reactions to screen sera of healthy controls and patients suffering of DCM for the presence of RBP-jkappa autoantibodies. Analysis revealed that only 31% (n = 16) of healthy but 70.6% of DCM patients (n = 17) carry an autoantibody against RBP-jkappa. Patients suffering from ischemic cardiomyopathy showed a prevalence of 22% of RBP-jkappa autoantibodies. Western analysis with an monoclonal antibody raised against RBP-jkappa showed that RBP-jkappa was overexpressed to 488 +/- 140% in DCM hearts compared to non-failing controls (n = 8). Autologous immunological screening of a cDNA expression library is a powerful and novel technology to gain insights into the etiology of human idiopathic DCM. Human DCM displays an autoimmune response against RBP-jkappa and an overexpression of RBP-jkappa. Since RBP-jkappa is involved in cellular immortalization and exerts antiapoptotic effects, the increased RBP-jkappa autoantibody level during DCM may inhibit this growth-regulating feature of RBP-jkappa. In this setting, enhanced myocardial RBP-jkappa expression could represent a compensatory but ineffective response to counteract the increased rate of apoptosis in DCM. Furthermore, RBP-jkappa may be a useful diagnostic marker for DCM.
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PMID:Enhanced expression and autoimmunity of recombination signal binding protein-jkappa in human dilated cardiomyopathy. 1060 May 20

Hepatitis C virus (HCV) infection is frequently associated with autoimmune disease. We present here a case of dermatomyositis manifested as heart failure in which HCV was detected from an endomyocardial biopsy sample. HCV infection may have contributed to the left ventricular dysfunction in this patient with dermatomyositis.
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PMID:Hepatitis C virus infection in a patient with dermatomyositis and left ventricular dysfunction. 1095 60


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