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Results for “Cardiomyopathy, Restrictive”

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At least 991 records · Page 55Linked to original sources
PubMed1987

Construction of cosmid genomic libraries for the normal and myopathic Syrian hamsters.

Cosmid genomic libraries from both normal and myopathic Syrian hamsters have been constructed. MboI was used to generate 35- to 50-kilobase DNA fragments which were isolated from a 5-25% NaCl gradient. The 35- to 50-kilobase DNA fragments were ligated to the cosmid vector pCV108 and packaged into Escherichia coli DK1. Approximately 3 X 10(5) - 4 X 10(5) clones were obtained per microgram of ligated DNA. Thirteen clones have been isolated from 2 X 10(5) colonies using a cardiac myosin heavy chain clone as a probe. Restriction maps of two of these clones are presented here.

Animals↗
PubMedDate not supplied

Hypertrophic obstructive cardiomyopathy in an infant of a diabetic mother: support by extracorporeal membrane oxygenation and treatment with beta-adrenergic blockade and increased intravenous fluid administration.

In infants with hypertrophic obstructive cardiomyopathy (HOCM) on extracorporeal membrane oxygenation (ECMO), conventional treatment with inotropes, fluid restriction, and diuretics may be detrimental. This case reports an infant of a diabetic mother with HOCM on ECMO illustrating beneficial effects of beta adrenergic blockade and increased fluid administration.

Adrenergic beta-Antagonists↗
PubMed2000

Polymorphisms of tumor necrosis factor-alpha and interleukin-10 genes in Japanese patients with idiopathic dilated cardiomyopathy.

Various cytokines play important roles in the pathogenesis of congestive heart failure. TNF-alpha is one of the pro-inflammatory cytokines, and IL-10 has anti-inflammatory actions. The -308 (G / A) polymorphism of the TNF-alpha gene (TNFA1 and A2) and the single base -1082 (G / A) polymorphism of the IL-10 gene (IL-10 1*G and 1*A) have been identified as causing alterations to the in vivo production of TNF-alpha and IL-10, respectively. We examined TNF-alpha and IL-10 gene polymorphisms using a polymerase chain reaction-restriction fragment length polymorphism technique in 48 Japanese patients with idiopathic dilated cardiomyopathy. The frequency of these polymorphisms was compared with 50 healthy Japanese. The clinical courses, such as disease onset, left ventricular function, progression during the follow up period and hospitalization from congestive heart failure, were also analyzed. Serum TNF-alpha levels were measured using an enzyme-linked immunosorbent assay (ELISA) technique in the patients with idiopathic dilated cardiomyopathy to reveal the correlation with genotypes. Patients with ischemic cardiomyopathy or other secondary cardiomyopathies were excluded from this study. The allele frequency of TNFA2 in idiopathic dilated cardiomyopathy was significantly higher than that of the healthy group (13.5% and 3.0%, respectively, p = 0.0084). There was no difference in the allele frequency of the IL-10 gene between the two groups. Polymorphism of the TNFA2 gene was not associated with the clinical course. Serum TNF-alpha levels were elevated in the patient group compared with the healthy group. There were no differences in serum TNF-alpha levels between the patients with TNFA1 and those with TNFA2. In conclusion, the TNFA2 allele may be linked to the pathogenesis of idiopathic dilated cardiomyopathy in Japanese patients.

Adult↗
PubMed2004

The mitochondrial superoxide dismutase A16V polymorphism in the cardiomyopathy associated with hereditary haemochromatosis.

The A16V mitochondrial targeting sequence polymorphism influences the antioxidant activity of MnSOD, an enzyme involved in neutralising iron induced oxidative stress. Patients with hereditary haemochromatosis develop parenchymal iron overload, which may lead to cirrhosis, diabetes, hypogonadism, and heart disease. The objective of this study was to determine in patients with haemochromatosis whether the presence of the Val MnSOD allele, associated with reduced enzymatic activity, affects tissue damage, and in particular heart disease, as MnSOD knockout mice develop lethal cardiomyopathy. We studied 217 consecutive unrelated probands with haemochromatosis, and 212 healthy controls. MnSOD polymorphism was evaluated by restriction analysis. The frequency distribution of the polymorphism did not differ between patients and controls. Patients carrying the Val allele had higher prevalence of cardiomyopathy (A/A 4%, A/V 11%, V/V 30%, p = 0.0006) but not of cirrhosis, diabetes, or hypogonadism, independently of age, sex, alcohol misuse, diabetes, and iron overload (odds ratio 10.1 for V/V, p = 0.006). The frequency of the Val allele was higher in patients with cardiomyopathy (0.67 v 0.45, p = 0.003). The association was significant in both C282Y+/+ (p = 0.02), and in non-C282Y+/+ patients (p = 0.003), and for both dilated (p = 0.01) and non-dilated stage (p = 0.04) cardiomyopathy, but not for ischaemic heart disease. In patients with hereditary haemochromatosis, the MnSOD genotype affects the risk of cardiomyopathy related to iron overload and possibly to other known and unknown risk factors and could represent an iron toxicity modifier gene.

Cardiomyopathies↗
PubMed1996

Early identification of anthracycline cardiomyopathy: possibilities and implications.

The number of survivors of childhood cancer affected by anthracycline cardiomyopathy is steadily increasing, despite efforts to limit cardiotoxicity by dose restriction. Cardiac function was evaluated prospectively in 125 children during treatment to attempt to identify individual susceptibilities to cardiotoxicity and hence any potential for treatment modification. Left ventricular shortening fraction was used as an index of cardiotoxicity. Shortening fraction declined as cumulative anthracycline dose increased, at an average rate of 1% per 100 mg/m2. Six patients (5%) developed heart failure. Twenty four patients (19%) had abnormal shortening fraction (< 30%) by the end of treatment, and their rate of fall of shortening fraction was significantly steeper throughout treatment than in patients finishing with normal function (shortening fraction > or = 30%). This differential susceptibility to cardiotoxicity was apparent from very early in treatment, interquartile ranges of the two shortening fraction groups separating at doses > 200 mg/m2. Patients at high risk of risk of important anthracycline cardiotoxicity may be identifiable early in treatment by regular and careful monitoring of shortening fraction. However, frequent assessment is required and this has significant resource implications.

Antibiotics, Antineoplastic↗
PubMed2001

Effects of alcohol on the heart.

Some evidence suggests that light to moderate alcohol consumption protects against cardiovascular diseases. However, this cardioprotective effect of alcohol consumption in adults is absent at the population level. Approximately 20 to 30% of patients admitted to a hospital are alcohol abusers. In medical practice, it is essential that patients' levels of consumption are known because of the many adverse effects that might result in the course of routine care. Ethanol damage to the heart is evident if alcohol consumption exceeds 90 to 100 g/d. Heavy ethanol consumption leads to increased risk for sudden cardiac death and cardiac arrhythmias. In patients with coronary heart disease, alcohol use was associated with increased mortality. An early response to drinking was an increased ventricular wall thickness to diameter ratio, possibly proceeding with continuous drinking to alcoholic cardiomyopathy, which had a worse outcome compared with idiopathic dilative cardiomyopathy if drinking was not stopped or at least reduced (< 60 g/d). In the ICU, patients with chronic alcoholism have more cardiac complications postoperatively. These complications probably are caused by biventricular dysfunction, particularly with the occurrence of severe infections or septic shock, events that are three to four times more frequent among chronic alcoholics than occasional drinkers or nondrinkers. To prevent further complications from drinking and for long-term management of drinking, patients with alcohol abuse and heart failure should be treated in brief intervention and follow-up programs. Prognosis is good even in patients with New York Heart Association class IV heart failure caused by cardiomyopathy if complete abstinence is accomplished. Noncompliance to smoking and alcohol restrictions, which are amenable to change, dramatically increases the risk for hospital readmissions among patients with heart failure.

Alcohol Drinking↗
PubMed1997

Monensin toxicity in a flock of ostriches.

A total of 42 birds from a flock of 104 farmed ostriches showed signs of toxicity after the accidental inclusion of monensin in their concentrate ration. The initial clinical signs were muscle weakness and ataxia which progressed to recumbency, dyspnoea and death, despite intensive supportive therapy. The serum activity of the enzymes creatine kinase, aspartate aminotransferase and lactate dehydrogenase was high in the affected birds, indicating significant muscle pathology. Few gross lesions were identifiable postmortem, but widespread lesions of degenerative myopathy were present at the histopathological level. However, these degenerative changes were restricted to the skeletal muscle and there was no evidence of cardiomyopathy in any of the birds examined. The birds were fed a ration which contained 215 to 224 ppm monensin for 13 days. New clinical cases ceased to occur shortly after the withdrawal of the source of monensin, but all the individuals which showed clinical signs of toxicity died or were euthanased on humane grounds.

Animal Feed↗
PubMed1992

[Non-invasive assessment of left ventricular diastolic function disorder using Doppler echocardiography].

Symptoms of a variety of cardiac diseases may well be caused predominantly by diastolic dysfunction, which appears to be one of the first detectable cardiac alterations. Diastole represents a complex interplay of multiple factors. Doppler echocardiography offers a noninvasive and relative simple method to assess left ventricular volumetric filling rates. The pulsed-wave Doppler method enables us to measure mitral flow velocities. Two distinct mitral flow velocity patterns have been recognized for situations with diastolic dysfunction. The pattern of delayed relaxation was reported in primary and in secondary left ventricular hypertrophy, myocardial ischemia and infarction and in dilated cardiomyopathy. The pattern of increased stiffness was described in constrictive pericarditis, restrictive processes and in heart transplant rejection. Doppler echocardiography has shown to be a helpful diagnostic tool, but a number of factors influencing mitral flow measurements have to be considered in each case, as there are age, heart rate, loading conditions and mitral regurgitation. Further studies are needed with respect to future control of therapeutic interventions and prognostic statements.

Diastole↗
PubMed1992

[Enzymatic activities of the mitochondrial respiratory chain in child cardiomyopathies. 34 cases prospectively studied by endomyocardial biopsy].

The arguments in favour of mitochondrial pathology of certain childhood cardiomyopathies (multi tissue involvement, lactic acidosis, histochemical abnormalities of skeletal muscle) are indirect and may be absent in isolated cardiomyopathy. The authors therefore set up a prospective study of enzyme activity of the mitochondrial respiratory chain directly by endomyocardial biopsy. Fifty children aged 2 months to 16 years were included. Thirty four had cardiomyopathy which was dilated and hypokinetic with thin walls in 21 cases, restrictive in 2 cases, and hypertrophic in 11 cases; the remaining 16 children had either normal hearts (13 catheterised for other reasons) or myocardial hypertrophy due to pulmonary or aortic stenosis (3 cases). Both ventricles were evaluated in 3 cases; macro-surgical biopsies were obtained in 6 cases and skeletal muscle biopsy was obtained in 9 cases. The results indicate the method to be reliable with no difference between the micro and macro biopsies. The absolute values of enzyme activity were too variable to serve as quantitative parameters but some ratios of activity were remarkably stable and allowed a qualitative assessment which was all the more accurate when identical values were obtained in the myocardium, skeletal muscle and liver. The mitochondrial respiration was independent of ventricular pressures and of the type (right or left) of ventricle. Enzyme activity was nearly always normal in dilated cardiomyopathy (20/21) which suggests that it was unaffected by dilatation of the heart and by abnormal myocardial contractility. The results could be normal in myocardial hypertrophy and in valvular stenosis and in over half the cases of hypertrophic cardiomyopathy.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1992

[Coronary arteriopathy associated with cardiomyopathy in an adolescent with AIDS].

The purpose of this paper is to inform the case of a 15 years old male patient who died as a consequence of acquired immunodeficiency syndrome (AIDS) complications. The postmortem examination showed a coronary lesion which, to the best of our knowledge, has not been previously described. This vasculopathy was restricted to the coronary arteries; myocardial changes similar to those described in AIDS-associated dilated cardiomyopathy were also present. The coronariopathy was indistinguishable from that described in the blood vessels of the brain in patients with AIDS-related cerebral arteriopathy. We also reviewed the autopsy material from another 14 children who died of AIDS, an in none of them exhibited similar changes in the coronary arteries. In addition we present the clinical findings and some theoretical considerations regarding the pathogenesis of the lesions.

Acquired Immunodeficiency Syndrome↗
PubMed2000

Sleeve lobectomy of the middle lobe for hilar lung cancer with accompanying cardiomyopathy and actinomycosis.

The indication for sleeve lobectomy of the middle lobe is limited, because the frequency of cancer occupying both the middle lobe and its bifurcation is low and the more commonly used middle and lower lobectomy is an alternate procedure. However, if a patient has accompanying cardiopulmonary restriction, lung preservation is paramount. We present a patient with hilar lung cancer (TisN0M0) associated with progressive cardiomyopathy, who underwent sleeve lobectomy of the middle lobe; the cancer took a favorable course.

Actinomycosis↗
PubMed2004

Development of a simple restriction fragment length polymorphism assay for subtyping of coxsackie B viruses.

Coxsackie B viruses (genus, Enterovirus; family, Picornaviridae) can cause aseptic meningitis, encephalitis, pleurodynia, myocarditis and are implicated in the pathogenesis of dilated cardiomyopathy. The differentiation of the group B coxsackieviruses into their subtypes has potential clinical and epidemiological implications. In the present study, a simple restriction fragment length polymorphism (RFLP) assay was developed for typing of group B coxsackieviruses into subtypes 1-6. It is a two step process, first, virus isolation and identification by virus neutralization assay, using pools of polyclonal antisera, second, the reverse transcription polymerase chain reaction (RT-PCR) using a single primer pair selected from the conserved 5'-untranslated region (5'-UTR) of enterovirus genome followed by RFLP. A 440 bp product was amplified from the reference strains of each subtype of group B coxsackievirus and 29 clinical isolates (positive for group B coxsackieviruses by neutralization assay). The amplified products were subjected to restriction endonuclease digestion by enzyme BsaJI. The assay was able to distinguish all six serotypes of coxsackie B viruses. The results were comparable to serotyping and showed that due to the relatively conserved nature of 5'-UTR in enterovirus genome, this region can be used for subgeneric molecular identification of enteroviruses.

5' Untranslated Regions↗