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At least 217 records · Page 12Linked to original sources

PubMed1998

Progression of asymmetric pattern of left ventricular hypertrophy in patients with hypertrophic cardiomyopathy accompanied by hypertension in the elderly.

OBJECTIVE: The aim of the study was to determine the potential influence of hypertension overlapping hypertrophic cardiomyopathy (HCM) on left ventricular hypertrophy (LVH) pattern. We compared the magnitude of LVH asymmetry between younger and older patients with HCM and concomitant hypertension. MATERIAL AND METHODS: We studied 18 patients (age range from 18 to 77 years) in whom HCM was accompanied by mild-to-moderate hypertension. Patients were subdivided into two groups: (I) <50 years of age (11 patients); and (II) >50 years of age (seven patients). The thickness of ventricular septum and posterior wall was assessed by M-mode echocardiography. The pattern of LVH was determined based on the value of ventricular septum/posterior wall thickness (VS/PW) ratio and LVMI was calculated according to Devereux formula. RESULTS: The more asymmetric LVH pattern was revealed in older HCM with hypertension patients than in younger patients (VS/PW thickness ratio 2.62 +/- 0.81 vs 1.58 +/- 0.56, P < 0.05). The LVMI was similar in both groups (210 + 87 vs 191 + 30 g/m2, P > 0.5). CONCLUSION: Asymmetric pattern of LVH aggravated with increasing age in HCM with hypertension patients while LVMI was comparable between younger and older patients. Thus, the primary myocardial disease, ie, HCM, so significantly determined the asymmetric LVH pattern with aging that coexistent hypertension was not a sufficiently strong stimulus to alter LVH pattern into a more concentric one in the elderly.

Adolescent↗

PubMed1987

[Roentgenologic data patterns and hemodynamics in biopsy-proven dilated myocardial diseases].

The chest plain images of 63 patients suffering from histologically proved dilated myocardial diseases: primary cardiomyopathy (27), active and residual myocarditis (23), toxic cardiomyopathy (3), endomyocardial fibrosis (2), thesaurismotic cardiomyopathy (2), obstructive intramural fine vessel diseases (6) were analysed and correlated with invasively determined parameters: end-diastolic volume index, wall mass index, left ventricular end-diastolic pressure, pulmonary artery mean pressure, ejection fraction. The x-ray findings proved to occur in four different patterns which were defined as left heart involvement and three different patterns of bilateral left and right heart involvement representing various stages of left heart failure. This kind of image evaluation with functional classification is characterized by increased sensitivity, accuracy and information in detail in comparison with the heart lung index or other usual procedures.

Adult↗

PubMed2002

No variants in the cardiac actin gene in Finnish patients with dilated or hypertrophic cardiomyopathy.

BACKGROUND: Dilated and hypertrophic cardiomyopathies are primary myocardial diseases that cause considerable morbidity and mortality. Although these cardiomyopathies are clinically heterogeneous, genetic factors play an important role in their etiology and pathogenesis. The defects in the cardiac actin (ACTC) gene can cause both cardiomyopathies. The aim of our study was to screen for variants in the ACTC gene in patients with dilated or hypertrophic cardiomyopathy from Eastern Finland. MATERIALS AND METHODS: Altogether, 32 patients with dilated and 40 patients with hypertrophic cardiomyopathy were included in the study. Commonly approved diagnostic criteria were applied, and secondary cardiomyopathies were carefully excluded. All 6 exons of the ACTC gene were amplified with polymerase chain reaction and screened for variants with single-strand conformation polymorphism analysis. RESULTS AND CONCLUSION: We did not find any new or previously reported variants. Our results indicate that defects in the ACTC gene do not explain dilated cardiomyopathy or hypertrophic cardiomyopathy in subjects from Eastern Finland and confirm earlier results that the ACTC gene does not play an important role in the genetics of dilated or hypertrophic cardiomyopathies.

Actins↗

PubMed2002

Diagnostic value of mitral annular velocity for constrictive pericarditis in the absence of respiratory variation in mitral inflow velocity.

Respiratory variation of 25% or more in transmitral early diastolic filling (E) velocity is a well-recognized diagnostic feature of constrictive pericarditis (CP) that is useful for distinguishing it from restrictive cardiomyopathy. However, a subset of patients with CP do not exhibit the typical respiratory change. Recent data showed that mitral annular (E') velocity measured by Doppler tissue echocardiography (DTE) is markedly reduced in patients with restrictive cardiomyopathy whereas E' velocity is well-preserved in CP. This study evaluated the role of DTE for the diagnosis of CP when there is no characteristic respiratory variation of E velocity. From September 1999 to March 2001, 19 patients (17 men, 2 women; mean age, 57 +/- 13 years) with surgically confirmed CP underwent comprehensive echocardiography preoperatively, including pulsed wave and DTE examination with simultaneous recording of respiration. Nine (47%) of the 19 patients had less than 25% respiratory variation in E velocity. There was no significant difference in mitral inflow peak velocity, deceleration time, early-to-late ventricular filling ratio, and E' velocity between patients with and patients without respiratory variation of E velocity of 25% or more. Regardless of the presence or absence of a significant respiratory variation of E velocity, E' velocity was relatively normal (mean, 12 +/- 4 cm/s) in all patients with CP. In conclusion, E' velocity is well preserved in patients with isolated CP even when there is no characteristic respiratory variation of E velocity. Thus, when the respiratory variation in Doppler E velocity is blunted or absent during the evaluation of suspected CP in patients with restrictive mitral inflow velocity, preserved E' velocity shown by DTE should support the diagnosis of CP over a primary myocardial disease.

Adult↗

PubMed1999

Cardiac involvement in Coffin-Lowry syndrome.

Coffin-Lowry syndrome is an X-linked recessive syndrome of mental retardation, characteristic facies and skeletal anomalies. In one patient with the syndrome, we observed early recurrent episodes of congestive heart failure with intercurrent normalization and the late development of mitral insufficiency due to annular dilation and congenital abnormalities of the valve apparatus. This unusual course of cardiac involvement, the non-adaptation of the left ventricular contractility to the aggravation of the mitral insufficiency and the postoperative persistence of the ventricular dysfunction, underline the possible role of an associated primary myocardial disease. This clinical observation demonstrates clearly that a mitral valve malformation can occur in patients with the syndrome, but also the role of a dilated cardiomyopathy, which can be secondary to the mitral regurgitation, but is more likely a myocardial disorder occurring as part of the syndrome.

Abnormalities, Multiple↗

PubMedDate not supplied

Aortic regurgitation and pigmentation - unusual features of Noonan syndrome.

A patient with typical features of Noonan's syndrome showed aortic regurgitation and widespread diffuse hyperpigmentation, features not previously associated with this syndrome. Detailed endocrine and dermatological studies failed to delineate the cause of hyperpigmentation. In addition to mild aortic regurgitation, cardiac catheterization revealed mild left ventricular dysfunction, probably due to primary myocardial disease. Other interesting findings included lymphedema and sexual infantilism despite normal testosterone levels.

Adult↗

PubMed1980

Fatal myocarditis associated with abortion in early pregnancy.

Primary myocardial disease in early pregnancy is a rare phenomenon. We describe four abortion-related deaths in the United States from 1975 through 1978 which were attributed at postmortem examination to myocarditis in the first trimester of pregnancy. Three of the four deaths were associated with conditions which have a presumed immunologic mechanism.

Abortion, Induced↗

PubMed1970

Cardiomyopathy in the Southwest American Indian.

Six Southwestern American Indian patients are reported to have primary myocardial disease or congestive failure of unknown cause. Two are Navajo, three are Laguna, and one is Isleta. Clinical and laboratory features are discussed. This disease appears in culturally traditional Indians and is not associated with contact with American civilization. It occurs in probably adequately nourished Indians in the majority of whom excessive alcohol consumption is not found. No familial cases were noted. This syndrome is probably not uncommon in the American Indian.

Adult↗

PubMed1999

Simultaneous onset of idiopathic dilated cardiomyopathy in identical middle-aged twins.

Idiopathic dilated cardiomyopathy is a primary myocardial disease which is characterised by left ventricular, or biventricular, dilatation and impaired contractility. The precise aetiology is unknown and the relative contribution of genetic and environmental factors is debated. We report two identical male twins of Caucasian origin with idiopathic dilated cardiomyopathy who presented within a few months of each other.

Atrial Fibrillation↗

PubMed1992

Respiratory chain defect of myocardial mitochondria in idiopathic dilated cardiomyopathy of Doberman pinscher dogs.

Idiopathic dilated cardiomyopathy (IDCM) is a primary myocardial disease of unknown cause. We tested the hypothesis that IDCM was associated with a myocardial metabolic defect by determining a comprehensive biochemical profile of metabolite concentrations and enzyme activities for the major metabolic pathways of the myocardium. We used the Doberman pinscher breed as a naturally occurring canine model of IDCM and compared its myocardial profile with that of healthy adult mongrels. Compared with controls, myocardium in IDCM had markedly reduced mitochondrial electron transport activity and myoglobin concentration, in association with acidosis and energy depletion following anoxic challenge: 60% decreased NADH dehydrogenase and 50% decreased ATP synthetase activities; 90% decreased myoglobin concentration; and 30% reduced ATP and 50% increased lactate and proton concentrations. Sarcoplasmic reticulum Ca(2+)-transport ATPase was decreased by 42%. There was a 15% compensatory increase in fatty acid oxidation and Krebs cycle activity. Other biochemical changes were mild by comparison with the mitochondrial defects. We conclude that IDCM is associated with a marked impairment of mitochondrial production of ATP, arising from decreased activity of the mitochondrial electron transport system, including myoglobin. These changes may be secondary to an underlying genetic defect or may indicate a deficiency of the mitochondrial respiratory chain that predisposes this breed to heart failure.

ATP Synthetase Complexes↗

PubMed1985

Ajmaline-induced torsade de pointes.

A patient with primary myocardial disease and left bundle-branch block who developed marked QT prolongation and torsade de pointes following an intravenous injection of ajmaline during an electrophysiologic study is reported. The patient could be resuscitated successfully 1 h after the onset of tachycardia.

Ajmaline↗

PubMed1998

Dilated cardiomyopathy in transgenic mice expressing a dominant-negative CREB transcription factor in the heart.

Idiopathic-dilated cardiomyopathy (IDC) is a common primary myocardial disease of unknown etiology characterized by progressive biventricular failure, cardiac dilatation, and premature mortality. Here we show that transgenic mice expressing a dominant-negative form of the CREB transcription factor (CREBA133) under the control of the cardiac myocyte-specific alpha-MHC promoter develop dilated cardiomyopathy that closely resembles many of the anatomical, physiological, and clinical features of human IDC. Between 2 and 20 wk of age, these mice develop four chamber cardiac dilatation, decreased systolic and diastolic left ventricular function, and attenuated contractile responses to the beta-adrenergic agonist, isoproterenol. Histologically, the CREBA133 hearts demonstrated both atrophic and hypertrophied fibers as well as significant interstitial fibrosis. These anatomical and hemodynamic changes were associated with hepatic congestion and peripheral edema, intracardiac thrombi, and premature mortality. Taken together, these results implicate CREB as an important regulator of cardiac myocyte function and provide a genetic model of dilated cardiomyopathy which should facilitate studies of both the pathogenesis and therapy of this clinically important disorder.

Animals↗

PubMed1996

Two necropsy cases of hypertrophic cardiomyopathy in Holstein cattle.

Two cases of hypertrophic cardiomyopathy in Holstein dairy cows are presented. At necropsy, the hearts revealed proportionate hypertrophy of the entire ventricles. The cut surface showed relatively large areas of myocardial scarring scattered throughout the ventricular walls including the septum. Microscopic examination revealed marked disorganization of cardiac muscle cells, intramural coronary arteries with thickened walls and narrowed lumina, and pronounced myocardial fibrosis. These features resemble those of hypertrophic cardiomyopathy in humans, suggesting the presence of a similar primary myocardial disease in cattle.

Animals↗

PubMed1995

An echocardiographic evaluation of patients with idiopathic heart failure.

The primary myocardial disease idiopathic dilated cardiomyopathy (IDCM) is not clearly defined in the literature. The description is both morphologic and etiologic. We examined consecutive patients with congestive heart failure (CHF) of unknown cause to identify possible cases of IDCM and to give a detailed description of echocardiographic data and possible diastolic dysfunction in this group. The hospital records of patients aged 16 to 65 years hospitalized due to CHF or IDCM during a 6-year period (N = 2,711) were evaluated in a defined region of western Sweden. Twenty-two percent (584/2,711) of these records contained no plausible cause of CHF or IDCM, and among patients being alive, obvious cause was lacking in 411 of 1,516 (27%). These 411 patients were offered a diagnostic investigation, including echocardiography, and they were compared with a randomly selected control group (n = 103) from the general population. Of 411 patients, 293 accepted investigation. From the control group, we defined the reference level for left ventricular (LV) dilatation to be > 32 mm/m2, and reduced ejection fraction according to Teichholz formula to be < 50%. Applying these borderlines, we identified LV dilatation and systolic dysfunction to be present in 30%, either dilatation or systolic dysfunction in 36%, and neither in 34%. In patients without any signs of systolic dysfunction 44% (26/59) showed signs of diastolic dysfunction. In a multivariate analysis, LV dimension was not independently correlated to disease, although LV dimension was univariately correlated to ejection fraction (EF) (r = -0.59; p < 0.0001). However, EF (p < 0.0001), left atrial dimension (p < 0.0001), and the first third filling fraction (p < 0.0001) were the constellation of parameters that most accurately separated patients from controls. By using these three parameters, a positive and negative predictive accuracy of 98% and 61%, respectively, was achieved. Thus, in a consecutive group of patients with idiopathic CHF recruited from a nonselected group of hospitalized patients with CHF, all grades of ventricular function were found. In this group, 30% were identified as having IDCM. We give reference values for the diagnosis of idiopathic IDCM and a simple tool to identify patients with systolic and diastolic dysfunction.

Adult↗

PubMed1975

Relationships of left ventricular systolic time intervals with hemodynamic variables in intact and failing hearts.

Deviation of systolic time intervals (STI) from the regression lines obtained from 122 normal subjects was studied in 22 healthy adults (Group 1), 18 N.Y. functional class I cardiac patients (Group II) with ischemic (IHD) or primary myocardial disease (PMD), and 15 similar patients (pts) but N.Y. functional class II with prior heart failure (Group III). STIc (corrected for heart rate) were normal in Groups I and II. Supine exercise caused shortening of pre-ejection period PEPc and prolongation of left ventricular ejection time ETc in both groups. Group III pts had a significantly longer PEPc and shorter ETc at rest. Supine exercise caused further prolongation of PEPc and a slight prolongation of ETc in this group. In 8 mongrel dogs, the effect of controlled changes of hemodynamic variables on STI was studied with intact hearts and repeated after myocardial impairment has been induced by pentobarbital. Doubling of venous return while the heart was intact produced changes in STI similar to the effects of supine exercise in Groups I and II human subjects. With myocardial impairment, comparable increase in venous return had an effect on STI similar to the effect of supine exercise Group III patients. Controlled increase in each of heart rate or blood pressure, with other hemodynamic variables kept constant, produced changes in STI different from the effect of supine exercise on human subjects. The study suggests that the value of supine exercise induced changes in STI in reflecting left ventricular performance is attributable primarily to increased volume load. In this resepct supine exercise is probably superior to other forms of exercise in disclosing impaired left ventricular performance.

Adolescent↗

PubMed1988

Postmenopausal estrogen use and coronary atherosclerosis.

STUDY OBJECTIVE: To determine whether estrogen replacement therapy affects the prevalence of severely obstructive coronary arterial lesions defined by selective coronary arteriography. DESIGN: Case-control study. SETTING: Large, urban, university-affiliated referral hospital. PATIENTS: From a consecutive sample of 6452 women having coronary arteriography between 1972 and 1984, 2188 patients were eligible for study; others were excluded because they were nonmenopausal, had congenital heart defects, valvular heart disorders, primary myocardial disease, or no more than mild to moderate coronary artery disease. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Hospital nurses routinely obtained medication histories. Staff invasive cardiologists interpreted coronary arteriograms. Clinical, laboratory, and angiographic data were abstracted from the cardiac catheterization reports and entered into a computerized registry. Postmenopausal estrogen use for 1444 cases of coronary artery disease (70% stenosis) was compared to that 744 controls (0% stenosis). The odds ratio estimate of the risk of coronary artery disease for estrogen users relative to the risk of coronary artery disease for nonusers was 0.44 (95% confidence interval, 0.29 to 0.67) after adjustment for age, cigarette smoking, diabetes, cholesterol, and hypertension. Postmenopausal estrogen replacement was a significant independent protective factor for coronary artery disease in a multivariate logistic regression model (P = 0.037). CONCLUSION: The data suggest that postmenopausal estrogen treatment reduces the risk for angiographically significant coronary artery disease.

Age Factors↗

PubMed1998

Genetic heterogeneity for familial hypertrophic cardiomyopathy in Chinese: analysis of six Chinese kindreds.

OBJECTIVE: Familial hypertrophic cardiomyopathy (FHCM) is a primary myocardial disease characterized by unexplained ventricular hypertrophy. The application of the techniques of reverse genetics has identified at least five chromosomal loci as the major causes for FHCM in diverse ethnic populations, suggesting substantial genetic heterogeneity for FHCM. Recently, the defective gene loci of two Chinese families with FHCM have been mapped to chromosome 11 and 14q1, respectively. For further understanding of the molecular basis of FHCM in Chinese, we analyzed the linkage between four other Chinese kindreds and DNA markers from chromosome 14q1. METHODS: Six unrelated Chinese families with FHCM, including two previously reported, were studied. Totally 90 family members were included for analysis. DNA from 80 individuals was extracted and polymerase chain reactions were performed using the primers designed according to the sequences derived from the alpha and beta myosin heavy chain gene. Totally four polymorphisms were studied, including three polymorphic microsatellite sequences and one single strand conformation polymorphism. Genetic linkage analysis were performed using the Linkage program. RESULTS: In the six studied families, 39 of the 90 family members were found to be affected diagnosed either by echocardiography or by clinical evaluation. The pattern of inheritance in all six studied families was most consistent with an autosomal dominant trait with a high degree of penetrance. Genetic linkage analysis using polymorphisms on the alpha and beta MHC genes showed a combined maximal lod score of 6.2 for trinucleotide repeat polymorphism AMHC-I 15 at theta = 0.00 for three studied families without recombination. Exclusion of linkage to the chromosome 14q1 location was noted in two of three other families with the maximal lod score of -2 or less. CONCLUSIONS: These results provide further evidence that FHCM in Chinese is genetically heterogeneous. Chromosome 14q1 locus, probably the beta myosin heavy chain gene, is important as the molecular basis for FHCM in Chinese.

Adolescent↗

PubMed1975

Maximal rate of fall of left ventricular pressure in cardiomyopathy and constrictive pericarditis.

The maximal rate of fall of left ventricular pressure (peak negative dp/dt) was measured in 4 patients with congestive cardiomyopathy (primary myocardial disease), in 5 patients with constrictive pericarditis and in 3 controls. Measurements were made at rest, with leg raising, after a bolus of 6 mug intravenous isoprenaline, and in patients with constrictive pericarditis during pulsus paradoxus. Peak negative dp/dt was 1810 +/- 234 mmHg/sec in controls; it was reduced in patients with constrictive pericarditis (1337 +/- 514 mmHg/sec) and greatly decreased in patients with congestive cardiomyopathy (812 +/- 190 mmHg/sec). There was close linear correlation between resting peak positive and peak negative dp/dt and there was little change with leg raising. Isoprenaline caused an increase in peak positive dp/dt, but there was only a small change in peak negative dp/dt. In patients with constrictive pericarditis, peak negative dp/dt varied during pulsus paradoxus: the linear relationship to peak positive dp/dt was maintained throughout the respiratory cycle. Peak negative dp/dt may be a useful index of myocardial function.

Adolescent↗