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

We describe two female infants with Hurler syndrome (mucopolysaccharidosis I) whose deaths are attributed to cardiac failure with associated, autopsy-confirmed endocardial fibroelastosis. One infant had confirmed alpha-L-iduronidase deficiency in cultured dermal fibroblasts, and the other infant had histologic evidence of tissue mucopolysaccharide accumulation at autopsy and a sibling with confirmed alpha-L-iduronidase deficiency and the Hurler syndrome phenotype. Clear cells ("Hurler" cells) were identified within the myocardium and endocardium of both infants. We propose that the ventricular mural accumulation of mucopolysaccharides induced extensive proliferation of elastic or collagen fibers within the endocardium. Cardiac failure may precede recognition of clinical and roentgenographic features of Hurler syndrome. Our findings and a literature review suggest that certain heritable storage disorders, including mucopolysaccharidosis I, should be considered when infants have clinical electrocardiographic and echocardiographic findings consistent with endocardial fibroelastosis or have autopsy-documented endocardial fibroelastosis.
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PMID:Mucopolysaccharidosis I presenting with endocardial fibroelastosis of infancy. 250 Aug 43

The myocardium consists of three compartments: cardiac muscle cells; a nutritive microcirculation; and an extracellular matrix composed largely of fibrillar collagen. The hypertrophic remodeling of the myocardium, which involves each of these compartments, can be adaptive or pathologic. This article reviews the remodeling process and the current state of our knowledge regarding the pathogenetic features of pathologic hypertrophy with heart failure.
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PMID:Pathogenesis of heart failure. 252 40

The accumulation of collagen within the myocardium is termed fibrosis. In left ventricular pressure overload a reactive interstitial fibrosis, having distinctive biochemical and structural features, is seen. This reactive fibrosis occurs in the absence of myocyte necrosis, is progressive in nature, and initially is an adaptive response that preserves the force generating capacity, or active (systolic) stiffness, of the hypertrophied myocardium. Later in hypertrophy a reparative (or replacement) fibrosis occurs in response to cell loss, the pathogenesis of which is not clear. Nevertheless, independently of cell loss, interstitial fibrosis can have a detrimental influence on the diastolic and systolic stiffness of the myocardium and can result in pathologic hypertrophy with heart failure. In established hypertrophy with disproportionate collagen matrix remodeling (ie, interstitial heart disease), it would be desirable to retard the continued formation of collagen and, if necessary, degrade collagen fibers that are responsible for impeding the stretching and shortening of muscle fibers. Prevention of interstitial fibrosis in pressure overload hypertrophy with pharmacologic agents with both antihypertensive and antifibrotic properties must also be considered. Future research should address these issues with a view toward developing corrective and preventative forms of therapy. Such advances will require a better understanding of cardiac fibroblast growth, collagen synthesis and the regulation of collagen gene expression in the heart.
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PMID:Myocardial collagen remodeling in pressure overload hypertrophy. A case for interstitial heart disease. 253 16

The interstitium of the myocardium is composed of predominantly type I collagen; type III collagen is present to a lesser extent. The fibrillar collagens serve as tethers between muscle cells, muscle fibers, and blood vessels while also providing a scaffolding that supports the muscular and vascular compartments. In pressure overload hypertrophy, a continuous structural remodeling of the fibrillar collagen matrix is seen. What is initially an adaptive process that enhances tensile strength can eventuate in pathologic hypertrophy with muscle fiber entrapment, cell loss, and abnormal diastolic and systolic stiffness of the myocardium. Morphologically distinct patterns of myocardial collagen accumulation, or fibrosis, have been identified based on the alignment of thick and thin collagen fibers to one another and to cardiac muscle. Each pattern, representing either a reactive (without necrosis) or reparative process, can alter stiffness in a unique manner. The manner in which the interstitium regulates the nature and proportion of fibrillar collagen formation is unknown and deserving of further study. Such information may lead to the development of antifibrotic agents that counteract, prevent or modify disproportionate collagen remodeling in pressure overload hypertrophy. These agents may thereby ultimately represent corrective forms of therapy for the management of heart failure.
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PMID:Patterns of myocardial fibrosis. 253 37

The hypertensive heart is an example of myocardial adaptation to a chronic mechanical overload. In this model, the myocardium adapts by quantitative mechanisms such as hypertrophy and by qualitative processes at sarcomere and cell membrane level. Cardiac failure occurs when these mechanisms become inadequate although other phenomena such as collagen, coronary resistances, adverse hormonal effects and associated pathologies also play a role. This report describes an experimental model: uninephrectomised rats were made hypertensive by administering a DOCA-salt diet. The resulting cardiac hypertrophy was reduced and the hypertension corrected by propranolol. Rilmenidine, a new antihypertensive agent, has no effect on cardiac hypertrophy but it does reduce myocardial collagen concentrations significantly which has potentially important beneficial consequences.
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PMID:[The heart in patients with hypertension: effects of rilmenidine on experimental cardiac hypertrophy of hypertensive origin in rats]. 253 68

The safety of 738 high-risk patients treated with enalapril under various clinical programs was evaluated. High risk was defined as the presence of a collagen vascular disease; a renal disease, including renovascular hypertension; or either hypertension or refractory cardiac failure with serum creatinine greater than or equal to 1.7 mg/dl at baseline. Essential hypertension was the primary diagnosis in most of these patients. Treatment with enalapril in these patients usually continued without interruption for the length of the particular protocol. The incidence of adverse reactions resulting in discontinuation of treatment was comparable to that observed with other standard antihypertensive therapies in patients with milder forms of disease. No enalapril-related neutropenia, proteinuria, dysgeusia or ageusia were reported in these high-risk patients. The incidence of discontinuation due to rash was less than 0.5%. Resolution and/or improvement of captopril-related adverse effects was observed in many patients crossed over to treatment with enalapril. In patients with collagen vascular diseases and those with severe impairment of renal function (serum creatinine greater than or equal to 3.0 mg/dl), the incidence of discontinuation due to adverse experiences or death as well as the profile of reported adverse experiences was similar to those for the total group of high-risk patients. The data suggest that enalapril is efficacious and well tolerated by the high-risk patients.
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PMID:High-risk patients treated with enalapril maleate: safety considerations. 253 44

Establishing the diagnosis of drug-induced pneumonitis is always difficult and requires that the following criteria be met: administration of the drug on a long-term basis; knowledge that the drug is able to induce pulmonary disorders; occurrence during therapy of interstitial pneumonitis with clinical, radiological and functional characteristics of this type of lung disease; exclusion of all other causes of interstitial pneumonitis (cardiac failure, infections, collagen vascular diseases, malignancies); bronchoalveolar lavage specimen, revealing lymphocytosis with an inverted CD4/CD8 lymphocyte ratio, isolated or associated with neutrophil and/or eosinophil alveolitis; finally, full recovery within several weeks or months after drug withdrawal unless irreversible pulmonary fibrosis has occurred. Certain specific characteristics correspond to the therapeutic class of the drug, i.e. antimicrobial, cardiovascular, antiinflammatory, neurological, metabolic, antiallergy or some other drugs.
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PMID:[Drug-induced pneumopathies (excluding cytostatic drugs)]. 261 Apr 52

To quantify the myocardial catecholamine content in heart failure patients and to assess the regional distribution of catecholamines, we investigated norepinephrine and dopamine concentrations in explanted hearts from 34 patients in end-stage heart failure. 28 patients with cardiomyopathy were compared with six patients with coronary artery disease. In comparison with the right atria of a control group without heart failure, reduced myocardial norepinephrine contents (in pg/micrograms non-collagen protein (NCP] were found in all areas of the explanted hearts: controls: right atrium 17.6 +/- 6.6; cardiomyopathy: right atrium 7.1 +/- 7.9, right ventricle 4.4 +/- 2.7, septum 3.8 +/- 1.5, left ventricle 3.5 +/- 1.4. Coronary artery disease: right atrium 7.0 +/- 6.9, right ventricle 4.2 +/- 2.6, septum 3.6 +/- 1.4, left ventricle 3.4 +/- 1.4. Highest norepinephrine levels were measured in the right atrium. Right ventricle, septum, base and midventricular portion of the left ventricle had lower concentrations and were not different from each other. In contrast to reduced norepinephrine (NE) levels in all patients, dopamine (Dop) was inhomogenously elevated (only in a subgroup of 44%). Catecholamine contents in any two arbitrarily selected areas correlated significantly (NA: r = 0.53-0.77; Dop: r = 0.81-0.93, p less than 0.05 in all cases). The patients with heart failure due to dilated cardiomyopathy and to coronary artery disease did not differ in myocardial catecholamine levels or distribution. In end-stage heart failure a significant loss of myocardial norepinephrine independent from the underlying disease is found. It affects all areas of the hearts but does not equalize catecholamine content in ventricles and atria.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Myocardial catecholamine content in heart failure--I: Regional distribution in explanted hearts. Comparison between dilated cardiomyopathy and coronary heart disease]. 262 16

To enable the assessment of a possible gradual loss of myocardial catecholamines in heart failure, we determined control values in endomyocardial biopsies from normal human right ventricular myocardium. The reproducibility of the determinations and its dependence on the reference system, wet weight (wwt) or non-collagen-protein (NCP), was investigated in explanted hearts. Parallel determinations of norepinephrine in several samples from 1-2 mg in the same heart yielded a variability of about 20%. To obtain reproducible values, catecholamine concentrations had to be related to non-collagen-protein. Non-collagen-protein content was higher in the ventricles (138 +/- 16 micrograms/mg wwt) than in the atria (102 +/- 15 micrograms/mg wwt). Norepinephrine levels in normal human myocardium, measured in right ventricular endomyocardial biopsies were 10.3 +/- 3 pg/micrograms NCP. If they were compared with norepinephrine levels in right atrial samples from 11 patients without heart failure, obtained at open heart surgery (17.6 +/- 6 pg/micrograms NCP), an atrioventricular gradient, with ventricular norepinephrine content being 58% of right atrial levels was calculated for healthy human hearts. This gradient was almost identical with that found in heart failure, patients, where right ventricular norepinephrine amounted to 60% of right atrial levels. This implicates a percentually homogeneous loss of norepinephrine in heart failure, which, however, does not equalize ventricular and atrial levels. Thus, to interpret myocardial catecholamine content in cardiac diseases, normal values in corresponding areas are mandatory.
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PMID:[Myocardial catecholamine content in heart failure--II: Measurement in endomyocardial biopsies, reference systems, normal values]. 262 17

A number of experimental and clinical studies have indicated that the process of aging and diabetes mellitus may result in alterations of cardiac function and composition. These appear to be independent of myocardial ischemia. Left ventricular diastolic compliance is diminished in both situations associated with interstitial collagen accumulation. There is also a reduction in the relaxation rate of the ventricle. In the subclinical state, the aged heart cell undergoes enlargement, but this has not been described in diabetes. In an unknown portion of patients with subclinical abnormalities, the process may advance to abnormalities of systolic function, heart failure, and arrhythmias. There is no convincing evidence that intramural small vessel disease can account for the diffuse cardiomyopathy of these two states. Management requires a particularly cautious use of cardioactive agents.
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PMID:Cardiac disease in the older diabetic: management considerations. 265 Dec 18


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