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Results for “Cardiomyopathy, Restrictive”

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At least 721 records · Page 40Linked to original sources
PubMed1993

Long-term myocardial function after heart transplantation.

With an increasing number of heart transplantations and an improved survival rate a characterization of the long-term hemodynamic profile of the transplanted heart is necessary. 222 cardiac catheterizations were performed in 71 heart recipients under cyclosporine A immunosuppression during a 7-year follow-up after transplantation. Hemodynamic evaluation revealed a non-progressive arterial hypertension due to an increased systemic vascular resistance, leading to a mild increase of left-ventricular chamber stiffness. Parameters of systolic myocardial function were found to be normal and did not deteriorate during the 7 year period. The prevalence of allograft coronary angiopathy increased from 13% one year to 54% five years after transplantation. Myocardial function was neither adversely influenced by the presence of coronary angiopathy nor by the number or severity of former rejection episodes. Unchanged annual pressure-volume loops excluded the development of a dilated or restrictive posttransplantation cardiomyopathy. Thus, not the myocardial function, but the coronary angiopathy represents the most critical cardiac long-term problem after transplantation.

Adolescent↗
PubMedDate not supplied

[Cardiomyopathies in childhood].

Primary cardiomyopathies are heart muscle diseases of unknown cause. In hypertrophic cardiomyopathy (HCM) a hypertrophy of unknown aetiology results either in asymmetric septal hypertrophy (mostly with obstruction) or in concentric hypertrophy of the left ventricle (mostly without obstruction). This condition is rare in childhood, partly inherited and sometimes observed already in newborns. Sudden cardiac death is a main complication in the second decade. In non-obstructive HCM endomyocardial biopsy is the tool to differentiate secondary forms. Dilated cardiomyopathy is recognized by aetiologically unclear dilatation of left or right or both ventricles. In childhood this condition has to be differentiated from coronary anomalies, endocardial fibroelastosis, and myocarditis. Also rare secondary forms have to be considered because of possible therapeutic consequences. From the very few histologically proven reports a reliable prognosis for this disease in childhood cannot be deduced.

Cardiomyopathies↗
PubMed2003

A death at Duke.

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ABO Blood-Group System↗
PubMed2007

Dramatic accumulation of triglycerides and precipitation of cardiac hemodynamic dysfunction during brief caloric restriction in transgenic myocardium expressing human calcium-independent phospholipase A2gamma.

Previously, we identified calcium-independent phospholipase A2gamma (iPLA2gamma) with multiple translation initiation sites and dual mitochondrial and peroxisomal localization motifs. To determine the role of iPLA2gamma in integrating lipid and energy metabolism, we generated transgenic mice containing the alpha-myosin heavy chain promoter (alphaMHC) placed proximally to the human iPLA2gamma coding sequence that resulted in cardiac myocyte-restricted expression of iPLA2gamma (TGiPLA2gamma). TGiPLA2gamma mice possessed multiple phenotypes including: 1) a dramatic approximately 35% reduction in myocardial phospholipid mass in both the fed and mildly fasted states; 2) a marked accumulation of triglycerides during brief caloric restriction that represented 50% of total myocardial lipid mass; and 3) acute fasting-induced hemodynamic dysfunction. Biochemical characterization of the TGiPLA2gamma protein expressed in cardiac myocytes demonstrated over 25 distinct isoforms by two-dimensional SDS-PAGE Western analysis. Immunohistochemistry identified iPLA2gamma in the peroxisomal and mitochondrial compartments in both wild type and transgenic myocardium. Electron microscopy revealed the presence of loosely packed and disorganized mitochondrial cristae in TGiPLA2gamma mice that were accompanied by defects in mitochondrial function. Moreover, markedly elevated levels of 1-hydroxyl-2-arachidonoyl-sn-glycero-3-phosphocholine and 1-hydroxyl-2-docosahexaenoyl-sn-glycero-3-phosphocholine were prominent in the TGiPLA2gamma myocardium identifying the production of signaling metabolites by this enzyme in vivo. Collectively, these results identified the participation of iPLA2gamma in the remarkable lipid plasticity of myocardium, its role in generating signaling metabolites, and its prominent effects in modulating energy storage and utilization in myocardium in different metabolic contexts.

Animals↗
PubMed1986

Cardiomyopathy. 2. Hypertrophic and restrictive/obliterative types.

The functional classification of the three types of cardiomyopathy has been helpful in organizing practitioners' thinking. The past three decades have witnessed tremendous advances in understanding of the pathophysiology, clinical and laboratory diagnosis, and treatment of these disorders. New doors of exploration are opening to answer such nagging questions as whether medical therapy alters long-term survival rates in dilated (congestive) cardiomyopathy, whether immunologic therapy will alter the course of dilated or restrictive/obliterative cardiomyopathy, and whether genetic manipulation or in utero therapy will prevent hypertrophic cardiomyopathy. The goals of effective treatment in all these disorders remain the same: amelioration of symptoms, prevention of sudden death, and constraint or resolution of the basic disease process. Achieving these goals requires a multidisciplinary approach, involving cardiologists, surgeons, immunologists, geneticists, cell biologists, virologists, and pathologists. Prospects are that one day treatment will no longer be an imprecise, palliative art but a definitive, curative science.

Adrenergic beta-Antagonists↗
PubMed1997

Epidemiology of idiopathic cardiomyopathies in children and adolescents. A nationwide study in Finland.

Although idiopathic cardiomyopathies are prognostically important and are a common indication for cardiac transplantation in all age groups, the incidence and age distribution of idiopathic cardiomyopathies in a well-defined pediatric population have been poorly characterized. A retrospective study was carried out in Finland in 1980-1991 to obtain information on the epidemiology of childhood cardiomyopathies. The medical records of all patients aged birth to 20 years with cardiomyopathy from the five university hospitals and 16 central hospitals covering the entire country were reviewed. Moreover, data on causes of death from the Finnish National Census Bureau were examined. Of the 808 potential cases screened, 118 infants, children, and adolescents, representing an average age-specific population of 1.4 million, were definitely identified as having idiopathic cardiomyopathy. The average annual occurrence of new cases was 0.65 per 100,000 population (95% confidence interval (CI) 0.53-0.79). If the 15 cases diagnosed only after death during the 12-year study period were included, the occurrence increased to 0.74 per 100,000 population per year. Fifty-six new cases of dilated cardiomyopathy and 40 new cases of hypertrophic cardiomyopathy were diagnosed during the study period, giving average annual occurrences of 0.34/100,000/year (95% CI 0.26-0.44) and 0.24/100,000/year (95% CI 0.17-0.33) for new cases of dilated and hypertrophic cardiomyopathies, respectively. At the end of 1991, the prevalence of dilated cardiomyopathy was 2.6/100,000 (95% CI 1.8-3.6) and that for hypertrophic cardiomyopathy was 2.9/100,000 (95% CI 2.0-4.0). The number of new cases of dilated cardiomyopathy per year increased over the study period, whereas the annual occurrence of hypertrophic cardiomyopathy remained relatively constant. Marked variability was seen in occurrence among the different age groups of children with dilated cardiomyopathy, suggesting that different pathophysiologic mechanisms, and possibly etiologies, may exist in different age groups.

Adolescent↗
PubMed2007

Is arterial stiffening in Alström syndrome linked to the development of cardiomyopathy?

BACKGROUND: Alström syndrome (AS) is a rare autosomal recessive condition characterized by retinal degeneration, childhood obesity, and severe insulin resistance. Dilated cardiomyopathy of unknown aetiology is a well-recognized and potentially lethal complication. The aim of this study was to investigate the relationship between vascular function, hyperinsulinaemia and cardiac performance in AS. MATERIALS AND METHODS: Fifteen subjects with AS (mean age 21 years, range 10-35) were studied and compared with age-, sex-, and blood pressure-matched healthy controls. Large artery stiffness and wave reflections were assessed in both groups by measuring aortic and brachial pulse wave velocity (PWV) (carotid-femoral and carotid-radial) and augmentation index (AIX) (Sphygmocor). In AS subjects, left ventricular function was assessed by echocardiography and metabolic parameters including fasting insulin, glucose, lipids and brain natriuretic peptide were also measured. RESULTS: Comparing AS subjects vs. controls (mean +/- SD), AIX was elevated in AS subjects (18 +/- 9% vs. 3 +/- 11%, P < 0.0001). No significant changes in brachial PWV (8.1 +/- 1.3 m s(-1) vs. 7.3 +/- 1.1 m s(-1), P = 0.14) or aortic PWV (6.5 +/- 1.1 m s(-1) vs. 6.0 +/- 1.0 m s(-1), P = 0.26) were observed. AS subjects were hyperinsulinaemic and had disturbances in lipid profiles relative to controls. No correlations were observed between vascular, metabolic and echocardiographic parameters. CONCLUSIONS: In AS there are alterations in the shape of the central arterial pressure waveform associated with augmented aortic systolic pressure and indicative of increased wave reflection. Unfavourable central arterial haemodynamics in AS may contribute to the development of cardiomyopathy but other aetiological factors are probably involved.

Adolescent↗