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Query: UMLS:C0018801 (heart failure)
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Although lupus cardiomyopathy is thought to be clinically uncommon, we encountered 6 patients with systemic lupus erythematosus (SLE) over a 10 year period who had severe left ventricular dysfunction and showed remarkable improvement in their cardiac function after cytotoxic therapy. All patients met the American College of Rheumatology criteria for classification of SLE and presented with signs of severe biventricular failure relatively early in their disease. Concurrent manifestations of SLE at the time of cardiomyopathy included rash, arthritis, myalgias, pleuritis, pericarditis, and nephritis. Four of the 6 patients were taking prednisone 20 mg/day at the time heart failure developed. In all cases the CPK were normal. Evaluation of cardiac function by echocardiogram and/or radionuclide gated blood pool scintigraphy revealed a severe depression of ventricular function with initial left ventricular ejection fraction (LVEF) ranging from 11 to 34% (mean 19%). Within 6 months of initiation of cytotoxic treatment all patients showed a dramatic response: the post-treatment LVEF ranged from 25 to 55%. This series of patients suggests that cardiomyopathy may be a more common complication of SLE than previously reported. Cardiomyopathy occurs relatively early in the course of SLE, may lead to severe cardiac dysfunction despite corticosteroid therapy, and appears to be responsive to cytotoxic therapy.
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PMID:Dramatic improvement of left ventricular function after cytotoxic therapy in lupus patients with acute cardiomyopathy: report of 6 cases. 1052 51

Mechanical cardiac assistance can be critical in saving the lives of patients with acute cardiac failure. However, currently used methods of ventricular assistance require advanced technical knowledge and equipment, and only small numbers of patients can be provided with them. Our aim was to develop new cannulas to construct a less invasive biventricular assist system that would permit easy application without thoracotomy. We prepared 2 centrifugal pumps and 4 uniquely shaped cannulas and conducted experiments to investigate the potential and problems of the system. In the first experiment, the system was attached to 6 dogs with ventricular fibrillation to confirm whether our system could maintain cardiac output. In the second experiment, the system was installed for 3 days in 3 goats, and changes in aminotransferases, BUN, creatinine, and plasma free hemoglobin levels were examined. In Experiment 1, it was demonstrated that the system was able to maintain circulation in dogs. In Experiment 2, although the flow rate of the pumps was maintained over 3 days, increased hemolysis and deteriorating renal function were observed. Although these problems need to be solved, the system was still helpful in the management of acute biventricular failure for short periods and may be clinically useful in the near future.
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PMID:Potentialities and problems of a novel bilateral ventricular assist system without thoracotomy. 1071 68

The clinical diagnosis of heart failure is based upon history and physical examination. Careful questioning and examination requires understanding of the pathophysiology of this systemic disorder. Symptoms and signs of congestive heart failure need to be differentiated from the manifestations of the underlying cardiovascular disorder. Only then will the specific signs and symptoms be unraveled. Symptoms arise from pulmonary congestion and peripheral or organ-underperfusion. Findings related to congestion can be found over the lungs (rales, pleural effusion), or at the jugular veins displaying either frank central venous pressure elevation or paradoxic inspiratory venous pressure rise (Kussmaul sign), or the more discrete sign of right, left of biventricular failure, the hepatojugular reflux. Dilatation and hypertrophy of the cardiac chambers can clinically easily and reliably be assessed by careful palpation. Galopprhythm, right and/or left ventricular in origin, is a particularly reliable sign of a failing ventricle. While a presystolic, atrial sound indicates merely elevated resistance to ventricular filling, i.e. the presence diastolic dysfunction or increased chamber filling, is the ventricular diastolic galopp a reliable sign of ventricular failure. Especially the appearance of a quadruple rhythm or a summation galopp can be considered both highly specific as well as prognostically dubious. Relative mitral and/or tricuspid insufficiency as a sign of ventricular dilatation needs to be differentiated from organic valve disease. This requires often echocardiography. Oedema of cardiac origin is symmetric and more pronounced in the evening. It arises both from left and from right ventricular failure. History and physical examination are both reliable tools in the initial diagnosis, as well as during follow-up and for control of therapeutic measures. Technical methods, such as chest x-ray, echocardiography or else are used for quantification and documentation. Properly applied and utilized they allow the physician to sharpen his clinical acumen, thus allowing for both a reliable diagnosis and a semi-quantitative estimation of ventricular size, enddiastolic and atrial, as well as pulmonary pressures and valve function.
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PMID:[Clinical diagnosis of heart failure]. 1085 88

Arrhythmogenic right ventricular cardiomyopathy is a rare heart muscle disease characterized by right and often left ventricular myocardial atrophy and fibrofatty replacement. Heart failure, arrhythmias and sudden death are characteristic complications. We observed a female in whom arrhythmogenic right ventricular cardiomyopathy was diagnosed due to presyncopes and dyspnea on exertion. A left ventricular thrombus was found echocardiographically, which disappeared with oral anticoagulation. Subsequently, however, extensive thrombus formation in the dilated akinetic right ventricle occurred which was resistant to combined treatment with heparin and oral anticoagulation. Thrombophilia screening showed a mutant prothrombin 20210A allele which is an inherited coagulopathy associated with increased plasma levels of prothrombin and increased risks of mainly venous thrombosis. The patient developed endstage biventricular heart failure and underwent heart transplantation within 3 months after thrombus formation in the right ventricle was diagnosed. In the explanted heart, the thrombus in the right ventricle was impressively large and calcified. In patients with unusual thrombus formation in the heart, coagulopathy may be associated and should be excluded.
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PMID:Extensive thrombus formation in the right ventricle due to a rare combination of arrhythmogenic right ventricular cardiomyopathy and heterozygous prothrombin gene mutation G20210 A. 1089 19

The hemodynamic effects of cardiomyoplasty (CMP) have been investigated in many centers, but the question of whether it is necessary to stimulate the latissimus dorsi muscle (LDM) 24 hours a day has not been answered. The main goal of our investigation was to determine whether hemodynamic results after CMP were impaired when continuous electrical stimulation (ES) was off for 12 hours a day. A model of chronic heart failure was created in 12 sheep by performing an arteriovenous anastamosis and administering doxorubicin. Two weeks after the anastomosis, CMP was performed in eight sheep (experimental series); ES training was begun at 2 weeks after CMP. After completion of the initial ES conditioning (8 weeks after CMP), one group of sheep continued to receive ES 24 hours daily. Another group of sheep had only 12 hours of ES daily. Hemodynamic parameters were investigated 2 weeks later with the stimulator turned on and then off. With doxorubicin administration, arteriovenous anastamosis created a stable model of biventricular heart failure (right atrial pressure 20 +/- 3 mmHg vs 6 +/- 2 mmHg at baseline; pulmonary capillary wedge pressure 18 +/- 3 mmHg vs 9 +/- 2 mmHg; left ventricular end-diastolic area 15.2 +/- 1.2 cm2 vs 6.4 +/- 0.7 cm2; left ventricular ejection fraction 0.38 +/- 0.6 vs 0.65 +/- 0.7). Cardiomyoplasty improved hemodynamic status in all eight experimental sheep. However, when the investigation was performed with the stimulator off, this improvement was statistically insignificant. With stimulation on, there was decreased right atrial pressure, pulmonary capillary wedge pressure, left ventricular end-diastolic volume, and increased left ventricular ejection fraction. With the stimulator turned off for 12 hours daily, hemodynamic measurements did not differ from data with continuous ES for 24 hours daily. Because hemodynamic results do not seem to be impaired, we recommend daily, periodic cessation of stimulation to prevent damage to the LDM after CMP.
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PMID:Effects of electrical stimulation postcardiomyoplasty in a model of chronic heart failure: hemodynamic results after daily 12-hour cessation versus a nonstop regimen. 1091 64

Cardiogenic shock and apnoea appeared in a 78-year-old woman with a history of biventricular heart failure, following ingestion of a single 80 mg verapamil tablet. She was resuscitated with artificial ventilation, dobutamine, norepinephrine and calcium gluconate. Toxicological analysis revealed unexpectedly high plasma verapamil concentration, which was attributed to the patient's liver failure. In patients with advanced heart failure a single oral therapeutic dose of verapamil may have a severe toxic effect.
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PMID:Cardiogenic shock following a single therapeutic oral dose of verapamil. 1121 25

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a heart muscle disease of unknown etiology characterized by the peculiar right ventricular (RV) involvement. Distinctive pathologic features are myocardial atrophy and fibro-fatty replacement of the RV free wall, and clinical presentation is usually related to ventricular tachycardias with a left bundle branch block pattern or ventricular fibrillation leading to cardiac arrest, mostly in young people and athletes. Later in the disease evolution, progression and extension of RV muscle disease and left ventricular involvement may result in right or biventricular heart failure. The diagnosis of ARVC may be difficult because of several problems with specificity of ECG abnormalities, different potential etiologies of ventricular arrhythmias with a left bundle branch morphology, assessment of RV structure and function, and interpretation of endomyocardial biopsy findings. Therefore, standardized diagnostic criteria have been proposed by the Study Group on ARVC of the European Society of Cardiology. According to these guidelines, the diagnosis of ARVC is based on the presence of major and minor criteria encompassing electrocardiographic, arrhythmic, morphofunctional, histopathologic, and genetic factors. Since the assessment of sudden death risk in patients with ARVC is still not well established, there are no precise guidelines to determine which patients need to be treated and what is the best management approach. The therapeutic options include beta-blockers, antiarrhythmic drugs, catheter ablation, and implantable cardioverter defibrillator (ICD). The ICD is the most effective safeguard against arrhythmic sudden death. However, its precise role in changing the natural history of ARVC by preventing sudden and nonsudden death needs to be evaluated by a prospective study of a large series of patients. In patients in whom ARVC has progressed to severe RV or biventricular systolic dysfunction with risk of thromboembolic complications, treatment consists of current therapy for heart failure including anticoagulant therapy. In cases of refractory congestive heart failure, patients may become candidates for heart transplantation.
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PMID:Arrhythmogenic right ventricular cardiomyopathy: current diagnostic and management strategies. 1152 Apr 49

Ventricular assist systems have become an important tool in the surgical management of acute and chronic heart failure. While indications for left ventricular support ar e well established, criteria for right ventricular support are much less defined. This report summarizes current knowledge on the implantation of right ventricular support in clinical settings of isolated right heart failure, biventricular failure, postcardiotomy failure and post-LVAD right heart failure.
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PMID:When should we consider right ventricular support? 1216 69

Continuous ambulatory peritoneal dialysis (CAPD) offers a new modality for use in the treatment of refractory congestive heart failure (CHF). Although the use of peritoneal dialysis to treat refractory heart failure is not new, improved understanding of the physiology and technique of this system has allowed its use over prolonged periods of time. The application of continuous peritoneal dialysis to the cardiac patient changes its scope considerably. When combined with current medical treatment, it removes fluid volume effectively and safely providing the best possibility for prolonged medical improvement. Continuous ambulatory peritoneal dialysis should be considered in patients with biventricular failure who have associated comorbidities like pulmonary hypertension and progressive renal insufficiency. We report the management of two such patients with NYHA Class IV heart failure who had become unresponsive to maximum pharmacological management and developed prerenal dialysis and during the 6 month follow up remained on dialysis without developing any significant complications and improved to NYHA class I. (c)1999 by CHF, Inc.
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PMID:Difficult cases in heart failure: Role of continuous ambulatory peritoneal dialysis in refractory heart failure. 1218 98

Beriberi (BB), thiamine deficiency, has been described in the Asian literature in the 17th century and is characterized by peripheral neuropathy and muscle weakness, also called "dry" beriberi (BB) to differentiate it from "wet" BB, with essentially cardiovascular manifestations. Wet can be either "classic" wet BB in which signs and symptoms of right-sided heart failure with normal or high cardiac output are the presenting features or the "shoshin" BB variant with severe biventricular failure and metabolic acidosis, which must be treated early to prevent the rapid development of low cardiac output failure and sudden death. In this case, we report a 58 year old alcoholic woman who developed dyspnea, oliguria, edema, cardiac failure with high output, metabolic acidosis, renal tubular dysfunction and serum lactate level of 5.6 mEq/L. Neurological examination revealed peripheral neuropathy in the lower legs and cognitive alteration. She was treated with a loading dose of 100 mg of intravenous thyamine and responded with a marked increase in urine output, correction of acidosis, reduction in pulmonary-capillary wedge pressure and a change of the hemodynamic pattern. We conclude that shoshin-BB is uncommonly encountered but not widely recognized. In lactic acidosis and/or hyperdynamic circulation without any other apparent etiology in patients with possible vitamin B1 deficiency, the diagnosis of BB must be considered and thiamine should be administered.
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PMID:[Acute cardiovascular beriberi (shoshin-beriberi)]. 1232 90


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