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Query: UMLS:C0018801 (heart failure)
 72,216 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We evaluated left ventricular function in 10 scleroderma patients with signs and symptoms suggestive of congestive heart failure. M-mode and two-dimensional echocardiography demonstrated normal to increased systolic function in all patients. The presence of pulmonary venous congestion on the chest radiograph was not useful in assessing left ventricular systolic function. Five of nine patients with normal to increased left ventricular ejection fraction (LVEF) had increased cardiothoracic ratios and increased pulmonary vascular markings. Left ventricular hypertrophy was associated with a worse New York Heart Association functional class, more pulmonary vascular congestion, and greater left atrial size. In the presence of normal systolic function and ventricular hypertrophy, diminished left ventricular diastolic compliance may account for the cardiac dysfunction in these patients. Cold pressor testing induced peripheral Raynaud's phenomenon in nine of nine patients; however, no ST segment changes or chest pain was provoked. In seven of nine patients there was no abnormal fall in LVEF. The mechanism for the fall in ejection fraction seen in two patients may be related to an increase in afterload or myocardial ischemia secondary to coronary atherosclerosis. We found little to suggest that a myocardial Raynaud's phenomenon affects left ventricular perfusion or systolic function. Clinical signs and symptoms of congestive failure as well as chest radiographs are poor indicators of impaired systolic function in scleroderma patients. Based on these findings, it appears that evaluation of left ventricular systolic function should include echocardiographic or angiographic study before such patients are treated for heart failure with inotropic agents.
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PMID:Left ventricular function at rest and during Raynaud's phenomenon in patients with scleroderma. 650 43

A 53-year-old man with scleroderma, pulmonary fibrosis, cardiac decompensation and secondary polycythaemia, but no arterial hypertension, developed central retinal vein occlusion (CRVO) in the left eye. 1.5 years later, during the treatment with systemic steroids and anticoagulants, he developed CRVO in the right eye, and a further half year later, secondary glaucoma in the left eye and loss of the visual acuity to counting fingers at 2.5 m in the right eye and at 0.5 m in the left. Retinal vascular changes, pulmonary and cardiac insufficiency and secondary polycythemia, symptoms of scleroderma, most probably contributed to the development of bilateral CRVO.
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PMID:Bilateral central retinal vein occlusion in a patient with scleroderma. 726 1

Although pulmonary disease is now the main cause of mortality in systemic sclerosis, there is a subset of patients with early rapidly progressive disease and a high risk of renal and cardiac failure and death. Recognition of risk factors (clinical and laboratory, including autoantibody profiles and tissue types) will help identify patients whose disease outcome may be improved with early and more aggressive treatment. The frequency of cancer has increased in scleroderma patients. Serious cardiac disease may be present in the absence of symptoms, whereas not all lung manifestations (reduced diffusion capacity) are necessarily progressive. More detailed descriptions of various manifestations and disease associations have been published. The development of noninvasive procedures will allow us to evaluate and follow up pulmonary and cardiac manifestations more objectively in such patients. The evolution of eosinophilic fasciitis into local scleroderma in children is of interest.
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PMID:Clinical aspects of systemic and localized sclerosis. 811 42

For the first time, we performed an epidemiological study of SSc in Japan to study the factors influencing prognosis, survival rate and cause of death of Japanese SSc patients and to compare our data with those from foreign countries. Prognosis of 496 Japanese patients with progressive systemic sclerosis (SSc) was analyzed based on clinical data described in case cards provided by the members of the Scleroderma Research Committee of the Japanese Ministry of Health and Welfare. The essential observation period was from 5 to 20 years, at ending in 1994. Ninety patients died (males 11, females 79). The age of onset of the deceased patients was significantly higher than that of surviving patients (deceased, 45.6 yrs, surviving 41.3 yrs). Statistically significant factors for a poor prognosis were as follows: Barnett type III > type II > type I, positive for anti-Scl-70 antibody, negative for anti-centromere antibody. The survival rate at 5 years after the onset of the disease was 0.937, followed by 0.82 at 10 years, 0.567 at 20 years and 0.40 at 30 years after the onset. Sex was not a predictor for prognosis, although male patients died at an earlier stage of the disease. The most common causes of death were heart failure, pulmonary insufficiency, lung fibrosis, and renal failure. Twenty-four patients had cancer of which 13 were lung cancers. The current status of the survival rate and prognostic factors of 496 Japanese SSc patients is summarized. In future, more well-controlled studies using the same criteria should be performed for the better understanding and management of SSc.
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PMID:Epidemiological analysis of prognosis of 496 Japanese patients with progressive systemic sclerosis (SSc). Scleroderma Research Committee Japan. 897 32

Mixed connective tissue disease (MCTD) was first reported 25 years ago. This report provides an assessment of the course of juvenile (J) MCTD in 224 patients available in the literature until 1996, including our own 33 patients. Most patients improved and remissions were observed in 3-5% (up to 27%). Among the long-term problems, a loss in joint function was seen in up to 29% of the cases, renal involvement in up to 47%, restrictive lung disease in up to 54% and gastrointestinal manifestations consisting of oesophageal dysmotility in up to 29%. Cerebral involvement was rare but severe. Cardiovascular problems observed include cardiomyopathy, myopericarditis and pulmonary hypertension. Among other long-term problems were Raynaud's phenomenon and scleroderma-like skin changes in up to 86% of the patients. Seventeen of the 224 patients had died (7.6%) because of sepsis or infection (7), cerebral complications (3), heart failure (2), pulmonary hypertension (2), renal failure (2) or gastrointestinal bleeding (1). The mortality rate of JMCTD seems to be in the same range as that of juvenile systemic lupus erythematosus, dermatomyositis and scleroderma. When compared with the other connective tissue diseases, however, mainly minor long-term problems are seen in the surviving patients.
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PMID:Course of mixed connective tissue disease in children. 945 80

The renin-angiotensin system is central to the pathophysiology of a number of cardiovascular disorders. Most obviously this is so with renin secreting tumours, but the system is of central importance in other disorders such as scleroderma renal crisis and most cases of malignant hypertension. Activation of the renin-angiotensin system in unilateral renal artery stenosis is pivotal to the development of hypertension and the disturbances in electrolyte and volume balance -- most particularly in the hyponatraemic-hypertensive syndrome. Likewise, stimulation of the renin-angiotensin system is an important contributor, amongst many other systems, to the pathophysiology of cardiac failure. In diabetic nephropathy, the renin-angiotensin system is often suppressed as gauged by circulating levels of renin, yet it appears to make an important contribution to the progressive decline in renal function. Much less clear is the role of the renin-angiotensin system in essential hypertension insofar as it contributes to the level of blood pressure, to the development of left ventricular hypertrophy, and in the evolution of complications such as stroke and myocardial infarction. Blockade of the renin-angiotensin system with angiotensin-converting enzyme inhibitors has contributed to our understanding of the role of this system in cardiovascular disease. The advent of selective angiotensin II type-1 receptor blockers will further increase knowledge in this area.
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PMID:The importance of the renin-angiotensin system in cardiovascular disease. 965 50

The renin angiotensin system (RAS) is now recognized as the body's most powerful hormone system for controlling renal hemodynamics and sodium excretion and, therefore, body fluid volumes and arterial pressure. The discovery of angiotensin converting enzyme inhibitors (ACEi) was a keystone for the understanding of the significance of the RAS since ACEi proved to be effective in controlling hypertension and heart failure and in preventing the development of the vascular injury of chronic diseases like scleroderma and diabetes mellitus. The success of ACEi stimulated the research into inhibitors of other actors of the RAS like renin or angiotensin receptor antagonists. It is not often realized that the discovery of ACEi owes a great deal to basic research in which the venom of a Brazilian viper, Bothrops Jararaca, was instrumental for the discovery of bradykinin by Rocha e Silva and the bradykinin potentiating factor. This article reviews the contribution of the converting enzyme inhibitors for the demonstration of the relevance of the RAS to several human pathologies.
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PMID:Angiotensin converting enzyme: history and relevance. 1070 50

Bradykinin is formed by the interaction of factor XII, prekallikrein, and high-molecular-weight kininogen on negatively charged inorganic surfaces (silicates, urate, and pyrophosphate) or macromolecular organic surfaces (heparin, other mucopolysaccharides, and sulfatides) or on assembly along the surface of cells. Catalysis along the cell surface requires zinc-dependent binding of factor XII and high-molecular-weight kininogen to proteins, such as the receptor for the globular heads of the C1q subcomponent of complement, cytokeratin 1, and urokinase plasminogen activator receptor. These 3 proteins complex together within the cell membrane, and initiation depends on autoactivation of factor XII on binding to gC1qR (the receptor for the globular heads of the C1q subcomponent of complement). There is also a factor XII-independent bypass mechanism requiring a cell-derived cofactor or protease that activates prekallikrein. Bradykinin is degraded by carboxypeptidase N and angiotensin-converting enzyme. Angioedema that is bradykinin dependent results from hereditary or acquired C1 inhibitor deficiencies or use of angiotensin-converting enzyme inhibitors to treat hypertension, heart failure, diabetes, or scleroderma. The role for bradykinin in allergic rhinitis, asthma, and anaphylaxis is to contribute to tissue hyperresponsiveness, local inflammation, and hypotension. Activation of the plasma cascade occurs as a result of heparin release and endothelial-cell activation and as a secondary event caused by other pathways of inflammation.
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PMID:Pathways for bradykinin formation and inflammatory disease. 1184 87

Scleroderma renal crisis occurs most often during the first years of the disease, in patients with systemic sclerosis and evolving cutaneous lesions. Clinically, it is responsible for severe hypertension, sometimes associated with cardiac failure or neurological symptoms. Laboratory tests disclose rapidly progressive renal failure, and often signs of thrombotic microangiopathy. If performed (which is rarely the case), renal biopsy shows scleroderma-induced chronic vascular lesions, but also vascular lesions that are secondary to malignant hypertension. The cornerstone of treatment is blood pressure control using angiotensin converting enzyme inhibitors, often in association with other antihypertensive agents. It has to be started as early as possible, in order to optimise vital and renal prognosis.
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PMID:[Renal involvement in scleroderma]. 1253 68

We describe a 40-year-old man with limited scleroderma who presented with acute heart failure following a flu-like illness. He was known to have incomplete left anterior bundle branch block, initial isolated pulmonary hypertension with enlarged right atrium, and no pulmonary fibrosis. He received therapy for acute heart failure and was transferred to a scleroderma centre for specific treatment of scleroderma cardiomyopathy. Investigations showed raised inflammatory markers and diffuse hyperechogenic thickening of the myocardium on echocardiography. Contrast-enhanced (Gd-DOTA) cardiovascular magnetic resonance imaging (CV-MRI) showed multiple areas of non-homogeneous delayed hyperenhancement in the left ventricle, suggestive of myocarditis. Antiadenovirus IgM antibodies were detected with a titer consistent with recent infection. Six weeks later a repeat Gd-DOTA CV-MRI showed an almost complete resolution of the areas of hyperenhancement and there was a significant reduction in the adenovirus antibody titer with serological conversion to IgG. To our knowledge this is the first report of viral myocarditis in scleroderma. Infections are important causes of morbidity and mortality in this disease and should always be included in the differential diagnosis of cardiac symptoms. We propose that contrast-enhanced CV-MRI is valuable in a non-invasive diagnosis of heart disease in patients with scleroderma.
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PMID:Acute myocarditis associated with adenoviral infection in a patient with scleroderma. 1467 36


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