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At least 19 recordsLinked to original sources
PubMed2005

Review of dilated cardiomyopathies. Dilated cardiomyopathies and altered prothrombotic state: a point of view of the literature.

Heart failure is an enormously important clinical problem that, if not faced, may overwhelm health care resources. Primary and secondary cardiomyopathies cause the majority of cases of clinical heart failure, which is thus better approached from the utility point of view of myocardial failure. Furthermore, the risk of thromboembolic complications presenting in such disease may be higher than in ischemic cardiomyopathy. Intracardiac thrombi and mural endocardial plaques (from the organization of thrombi) are present at necropsy in more than 50% of patients with dilated cardiomyopathy (DCM). Several studies have shown that systemic and pulmonary emboli are more frequent in patients with ventricular thrombi or plaques. Dilated cardiomyopathy has been associated with left ventricular thrombosis which leads to substantial morbidity and mortality as a site for peripheral emboli. There are some studies on patients with dilated cardiomyopathy showing altered hemostasis and platelet behavior despite sinus rhythm. Platelet activation, thrombin activation and fibrinolytic activity are increased in patients with DCM compared to normal subjects. However, these markers reflecting coagulation activation in patients with left ventricle thrombus are comparable to those in patients without thrombus in the left ventricle. The pathophysiology and clinical issues concerning the susceptibility to develop left ventricular (LV) thrombosis and its complications like cerebrovascular disease in patients with DCM are summarized and the most recent articles present in the medical literature are reviewed.

Animals↗
PubMed1991

[Arrhythmogenic potential of cardiomyopathies. Dilated cardiomyopathies].

Sixty-nine cases of non-ischemic dilated cardiomyopathy were studied prospectively from February 1983 to February 1989 (52 men: 53 +/- 13.5 years of age). There were 6 cases of sustained ventricular tachycardia. Thirty-seven patients were in Class III or IV of the NYHA Classification. In addition to echocardiography, radionuclide studies, cardiac catheterisation and coronary angiography, they all underwent 24 hour Holter monitoring, signal-averaged electrocardiography and, in 46 cases, endocavitary electrophysiological investigations. Holter monitoring showed ventricular extrasystoles greater than or equal to Grade 3 of Lown's classification in 72% of patients (26% had nonsustained ventricular tachycardia) and these patients had a significantly lower cardiac index. Twenty five per cent of patients had late ventricular potentials (versus 2% in 50 normal subjects; p less than 0.02); this proportion rose to 32% in those patients with greater than or equal to Grade 3 ventricular extrasystoles and to 66% in the patients with spontaneous ventricular tachycardia; the cardiac index was lower in patients with late ventricular potentials (2.3 vs 2.8 l/min/m2; p less than 0.01) and they had a higher incidence of greater than or equal to Grade 3 ventricular extrasystoles (94% vs 65% in patients without late ventricular potentials, p less than 0.05). Programmed ventricular stimulation induced sustained or nonsustained monomorphic ventricular tachycardia and ventricular fibrillation in 15% of cases. The 6 cases of induced sustained ventricular tachycardia were only observed in the 6 patients with spontaneous sustained ventricular tachycardia and they had the same electrocardiographic appearances.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1982

An animal model of congestive (dilated) cardiomyopathy: dilatation and hypertrophy of the heart in the chronic stage in DBA/2 mice with myocarditis caused by encephalomyocarditis virus.

To investigate whether lesions that develop in the chronic stage of viral myocarditis are similar to those seen in congestive (dilated) cardiomyopathy, we studied myocarditis in inbred strains of DBA/2 mice infected with encephalomyocarditis (EMC) virus. Myocardial necrosis with calcification appeared on day 4. Thereafter, myocardial necrosis became more extensive and mononuclear cell infiltration was evident and was most marked on day 14. On day 90, cellular infiltration had decreased and myocardial fibrosis was prominent. At this stage, the heart weight was significantly greater in the infected mice than in the controls (0.190 +/- 0.028 g vs 0.122 +/- 0.013 g, mean +/- SD) (p less than 0.001) and the cavity dimensions of the left ventricle were larger (1.67 +/- 0.29 mm vs 1.11 +/- 0.20 mm) (p less than 0.001). The diameters of myocardial fibers of the right ventricle, the interventricular septum and the left ventricle were significantly larger than those of the controls (right ventricle, 16.6 +/- 1.8 vs 13.4 +/- 1.5 micrometer; interventricular septum, 17.8 +/- 1.5 vs 13.8 +/- 1.5 micrometer; left ventricle, 19.4 +/- 1.7 vs 14.8 +/- 1.1 micrometer) (p less than 0.001). This study demonstrates that in viral myocarditis in the chronic stage, lesions develop that resemble those seen in congestive cardiomyopathy.

Animals↗
PubMed2005

[Cardiomyopathies. I: classification of cardiomyopathies--dilated cardiomyopathy].

Cardiomyopathies are common causes of heart failure and sudden cardiac death. According to the WHO classification, "specific" cardiomyopathies are differentiated from "idiopathic" cardiomyopathies. Thus, this classification is primarily based on pathophysiological characteristics. The diagnostic spectrum in cardiomyopathies comprises the entire spectrum of non-invasive and invasive cardiological examination techniques. The exact verification of certain cardiomyopathies necessitates additionally investigations. For example, immunohistological and molecular biological investigations of endomyocardial biopsies may confirm inflammatory cardiomyopathy, which is often induced by viruses. Several studies have shown that specific immunomodulatory treatment options can halt the progressive course of the disease. Several gene mutations have been identified in genetic/familial dilated cardiomyopathy. First-degree relatives should be screened for early stages. Primary prevention of sudden cardiac death shows increasing superiority of the implantable defibrillator compared with pharmacological approaches (i.e. amiodarone).

Cardiomyopathies↗
PubMed1998

Comparison of clinical and morphologic cardiac findings in patients having cardiac transplantation for ischemic cardiomyopathy, idiopathic dilated cardiomyopathy, and dilated hypertrophic cardiomyopathy.

This article compares intergroup and intragroup clinical and morphologic findings in patients with ischemic cardiomyopathy (IC), idiopathic dilated cardiomyopathy (IDC), and dilated hypertrophic cardiomyopathy (HC) undergoing cardiac transplantation (CT). Few previous publications have described findings in native hearts explanted at the time of CT. The explanted heart in 92 patients having CT was examined in uniform manner with particular attention to the sizes of the ventricular cavities and the presence of and extent of ventricular scarring. Of the 92 hearts examined, 47 had IC, 35 had IDC, and 10 had dilated HC. Although considerable degrees of intragroup variation occurred, the mean degree of left ventricular dilatation was similar among the patients with IC, IDC, and dilated HC. All patients with IC had left ventricular free wall scarring more extensive than that involving the ventricular septum, but the intragroup variation in the amounts of scarring was considerable. Nine of the 10 patients with dilated HC also had ventricular wall scarring, but it was more extensive in the ventricular septum than in the left ventricular free wall and involvement of the right ventricular wall also was present. Eight (23%) of the 35 IDC patients also had grossly visible ventricular scars but they were small and only 1 of the 8 had coronary narrowing and that was not in the distribution of the scarring. Narrowing of 1 or more epicardial coronary arteries >75% in cross-sectional area by plaque was present in all 47 IC patients, in 8 of the 35 IDC patients (7 had no ventricular scars), and in none of the 10 dilated HC patients. Coronary angiography was the major clinical tool allowing separation of the IC, IDC, and HC patients. Coronary angiography did not detect narrowing in any of the 8 patients with IDC who were found to have coronary narrowing on anatomic study. Thus, among patients with IC, IDC, and dilated HC having CT, distinctive anatomic features allow separation of patients with IC, IDC, and dilated HC, but within each group considerable variation in left ventricular cavity size and extent of ventricular scarring occurs.

Adult↗
PubMed1995

The autoimmune basis of dilated cardiomyopathy.

Dilated cardiomyopathy is a form of heart disease characterized by ventricular dilatation and reduced systolic function. In most patients, dilated cardiomyopathy is a sporadic disease. However, 20% of dilated cardiomyopathy patients may have a familial form of the disease. The aetiologies of both the sporadic and familial forms of dilated cardiomyopathy are unknown in most cases. Dilated cardiomyopathy has a spectrum of clinical and subclinical presentations. During the last 10 years, there have been many investigations concerning the possible aetiologic role of immune factors in dilated cardiomyopathy. It is plausible that an antecedent viral infection initiates an immunological cascade which in turn leads to production of autoimmune antibodies resulting in dilated cardiomyopathy. However, in most dilated cardiomyopathy patients, an antecedent viral infection cannot be identified. Similarly, the trail of immunological research has diverged as different groups have identified distinct autoantibodies or other immune factors in heterogeneous subsets of dilated cardiomyopathy and control patients. In this manuscript, we review the studies which have contributed supportive and confounding evidence to the theoretical autoimmune basis of dilated cardiomyopathy.

Autoantibodies↗
PubMed2005

[Review: genetics of familial dilated cardiomyopathy].

Dilated cardiomyopathy is the most frequent cardiomyopathy. Twenty to 35% of dilated cardiomyopathies are familial. The transmission of the disease is most frequently dominant autosomic. Dilated cardiomyopathy is genetically heterogeneous. Hence, mutations have been identified on 14 genes, and 9 loci have been associated to familial dilated cardiomyopathy. The incriminated mechanisms in the pathogeny of dilated cardiomyopathy include mutations on proteins of the sarcomere, the cytosqueletton, the nuclear membrane or involved in calcium signaling. This review indicates the genes and proteins implicated in the pathogeny of familial dilated cardiomyopathy, and their potential clinical effects.

Calcium↗
PubMed2001

[Genetics of dilated cardiomyopathy].

Dilated cardiomyopathy is one of the leading causes of heart failure and a primary cause for heart transplantation in patients below the age of 40 years. Despite major advances in diagnostic procedures such as examination of myocardial biopsies, the etiology remains unknown in many patients. Chronic inflammation or myocarditis and chronic alcohol abuse are considered two main etiologic factors in dilated cardiomyopathy. A third causal factor, namely genetic transmission of the disease, is at least as common as myocardial inflammation or toxic damage. Several prospective studies of relatives of patients with dilated cardiomyopathy proved that about 25-30% of all cases are of familial etiology. The most common mode of inheritance is autosomal dominant. Less frequently is the disease inherited as an X-chromosomal trait. Autosomal recessive and mitochondrial transmission is rare. The penetrance is highly variable and age dependent. Many relatives of patients with DCM show only minor cardiac abnormalities and it is unknown whether they progress to full cardiomyopathy in later life. Examination of families has identified so far eight disease genes, namely the dystrophin, tafazzin, cardiac actin, desmin, lamin A/C, delta- sarcoglycan, cardiac beta-myosin heavy chain, and cardiac troponin T gene. Certain mutations in lamin A/C cause conduction system disease and dilated cardiomyopathy, whereas other mutations cause in addition skeletal muscle myopathy. Dystrophin mutations are the cause of the rare X-linked dilated cardiomyopathy without skeletal muscle involvement and a progressive course in young men. Other mutations in the dystrophin gene, mainly deletions, are the cause of the muscular dystrophy Becker and Duchenne which also present with dilated cardiomyopathy. Mutations of the desmin, delta-sarcoglycan, the cardiac actin and beta-myosin heavy chain as well as the troponin T gene are known to cause autosomal dominant-dilated cardiomyopathy without other abnormalities. The infantile X-linked DCM is caused by mutations of the tafazzin gene. The onset of the disease is typically within the first year of life and death occurs usually in childhood. Most patients may in addition be characterized by skeletal myopathy, short stature, neutropenia and abnormal mitochondria, also referred to as Barth syndrome. Knowledge of the DCM disease genes led to the new hypothesis that dilated cardiomyopathy is a disease of the myocardial force generation or force transmission. Many more disease loci are known but the responsible disease genes are not yet identified. Better understanding of the expression and function of disease genes may eventually result in new diagnostic and therapeutic tools in order to improve the prognosis of this severe disorder.

Actins↗
PubMed2003

Mutational analysis of the beta- and delta-sarcoglycan genes in a large number of patients with familial and sporadic dilated cardiomyopathy.

Dilated cardiomyopathy (DCM) is defined by ventricular dilatation associated with impaired contractile function. Approximately one-third of idiopathic dilated cardiomyopathy cases are due to inherited gene mutations. Mutations in the beta- and delta-sarcoglycan genes have been described in limb girdle muscular dystrophy and/or isolated DCM. In this study, the aim was to investigate the prevalence of these genes in isolated DCM. We screened these two genes for mutations in 99 unrelated patients with sporadic or familial DCM. The coding exon and intron-exon boundaries of each gene were amplified by polymerase chain reaction. Mutation analyses were performed by single-strand conformation polymorphism for the beta-sarcoglycan gene and by direct sequencing for the delta-sarcoglycan gene. New polymorphisms, as well as already described ones, were found in these two genes, but none appeared to be responsible for dilated cardiomyopathy. We, therefore, conclude that these genes are not responsible for idiopathic isolated dilated cardiomyopathy in our population. Furthermore, based on previously published and present data, we could estimate the prevalence of delta-sarcoglycan gene mutations to be less than 1% in idiopathic dilated cardiomyopathy, demonstrating that this gene is only marginally implicated in the disease.

Adult↗
PubMed1999

Left ventricular end-systolic wall stress is a potent prognostic variable in patients with dilated cardiomyopathy.

Dilated cardiomyopathy is an important cause of morbidity and mortality among patients with heart failure. Left ventricular dilation is viewed as a compensatory response to maintain stroke volume, and left ventricular dilation is directly related to the increase of wall stress. However, only a few studies have examined whether wall stress can be a prognostic variable in patients with dilated cardiomyopathy. This study was designed to elucidate whether left ventricular systolic wall stress was related to the prognosis in patients with dilated cardiomyopathy. Twenty-five normal control subjects and 68 patients with dilated cardiomyopathy participated in this study. Hemodynamic parameters and left ventricular systolic wall stress were determined using echocardiography. In addition, the extent score determined by thallium-201 myocardial scintigraphy was measured as an index of cumulative loss of myocardium. During the 53-month follow-up period, 13 patients died of cardiac events. In a stepwise multivariable analysis, end-systolic wall stress and fractional shortening were significant predictors of survival. The extent score was markedly greater in the patients who died than in alive patients. There was a significant correlation between end-systolic wall stress and extent score (r=0.501, p=0.0001). Left ventricular end-systolic wall stress is an important predictor of mortality in patients with dilated cardiomyopathy.

Adult↗
PubMed2002

Familial dilated cardiomyopathy.

Dilated cardiomyopathy is a disorder affecting heart muscle, characterized by ventricular dilation and reduced systolic function. It represents the most common cause of heart failure. Until recently, dilated cardiomyopathy was considered an exclusively sporadic and idiopathic disease. Now, as defined by the World Health Organization, cardiomyopathy includes not only the idiopathic form, but secondary ones such as ischemic or hypertensive. It is estimated that familial occurrence accounts for 30% of cases of idiopathic dilated cardiomyopathy. The most common mode of inheritance is the autosomal dominant type. The X-linked, autosomal recessive and mitochondrial forms are less common. Different genes or loci are responsible for the cardiac dilatation, and code for sarcomeric, cytoskeleton and nuclear lamina proteins. The molecular interactions of the mutated proteins with factors such as infectious agents or alcohol could explain the variety of presenting signs and symptoms of this type of cardiomyopathy. Recently the European Society of Cardiology published a definition and a protocol for the study of familial dilated cardiomyopathies. Genetic research in the field of dilated cardiomyopathy can increase our understanding of its pathogenesis and lead to new treatment modalities for the disease.

Cardiomyopathy, Dilated↗
PubMed2002

Electrophysiology studies in patients with dilated cardiomyopathies.

Dilated cardiomyopathy is a diverse group of heart diseases with variable arrhythmia substrates. The response to programmed stimulation is dependent on spontaneous arrhythmia presentation. In patients with dilated cardiomyopathy, the majority of sustained monomorphic VT is caused by a scar-related reentrant mechanism, similar to that of coronary artery disease. The arrhythmia is uniformly inducible and is often refractory to pharmacologic therapy. Sustained VT is associated with more extensive myocardial fibrosis and non-uniform anisotropy, involving both the endocardium and epicardium, compared to those without sustained reentry. The response to programmed stimulation is more variable in patients presenting with nonsustained arrhythmia, cardiac arrest or syncope. Inducibility of monomorphic VT is much lower compared to those with ischemic heart disease. Other non-reentrant mechanism, such as focal automaticity, can also be observed in patients with monomorphic VT, in the absence of myocardial scar or evidence of slow conduction. The utility of electrophysiology studies to determine prognosis and to guide therapy remains limited in this patient population. The clinical outcome does not correlate with arrhythmia inducibility, and suppression of induced arrhythmia does not predict a good prognosis. The diagnosis of sarcoidosis or Chagas' cardiomyopathy should be considered in patients with dilated cardiomyopathy of unknown etiology, particularly in those with marked regional wall motion abnormalities and inducible VT. Epicardial reentrant circuits may be more prevalent in these cardiomyopathies, especially in those with VT related to chronic Chagas' disease. Although bundle branch reentry VT is a common finding in patients with dilated cardiomyopathy, it can occur in cardiomyopathy of any type and may coexist with other myocardial reentrant VT. It often has a typical bundle branch block QRS pattern during VT and is associated with His-Purkinje conduction delay. Evidence of macroreentry involving the bundle branches can usually be demonstrated, and catheter ablation of the bundle branches provides an effective and specific treatment.

Bundle-Branch Block↗
PubMed1995

[Clinical aspects and follow-up in dilated cardiomyopathy].

Dilative cardiomyopathy is a heterogeneous myocardial disease characterized by a depressed contractile function and ventricular dilation. The exercise intolerance of patients with dilative cardiomyopathy is partly explained by changes of metabolism and composition of skeletal muscle, whereas the physical findings result from progressive heart failure and cardiac remodeling. The clinical course of the disease is highly variable and prognostic indicators of progressive heart failure or cardiac arrhythmias are of little value in the assessment of cardiac risk in an individual patient. During recent years the mortality of patients with dilative cardiomyopathy was 5-15%, which appears to be lower than previously reported. The differences in mortality today and in previous years may be explained by a more precise and earlier diagnosis of the disease and possibly better treatment strategies. Patients with a severely depressed left ventricular function but a stable clinical course, a good hemodynamic response to therapy, and an oxygen uptake of > 12 ml/kg min can be followed without heart transplantation. It can be expected that the definition of the molecular causes of dilative cardiomyopathy will lead to a better classification of the patients and the development of more efficient treatment strategies.

Cardiomyopathy, Dilated↗
PubMed1998

Pulsed Doppler echocardiographic study of left ventricular diastolic function in patients with idiopathic dilated cardiomyopathy.

Dilated cardiomyopathy is basically regarded as a disease of left ventricular systolic dysfunction. There are only a few studies evaluating diastolic function in patients with dilated cardiomyopathy. To assess the LV diastolic function, 25 patients with idiopathic dilated cardiomyopathy and 20 age and sex matched normal subjects were studied with transmitral spectral tracings derived from pulsed Doppler echocardiography. All cardiomyopathy patients were in New York Heart Association class III to IV with dilated left ventricles and reduced systolic function (mean ejection fraction of 36.6 +/- 6.7 Vs 65 +/- 6 in normal subjects, p < 0.001). Patients with cardiomyopathy demonstrated an increased ratio of early to late diastolic velocity (E/A) (1.89 +/- 0.59 Vs 1.50 +/- 0.27 m/sec, p < 0.05), short deceleration time (E-E/2) (57.05 +/- 13.36 Vs 70.20 +/- 16.56 msec, p < 0.01) and short isovolumic relaxation time (IVRT) (53.5 +/- 22.7 Vs 72 +/- 12 msec, p < 0.05) as compared to normal subjects. The early filling fraction (EFF) was higher (0.71 +/- 0.11 Vs 0.66 +/- 0.06, p < 0.05) and atrial filling fraction (AFF) was lower (0.28 +/- 0.11 Vs 0.33 +/- 0.06, p < 0.05) in cardiomyopathy patients than in normal subjects. Our observations in a select group of dilated cardiomyopathy patients with advanced disease demonstrate a restrictive pattern on pulsed Doppler echocardiography.

Adult↗
PubMed1994

[Epidural anesthesia with high dose fentanyl for a patient with dilated cardiomyopathy].

Dilated cardiomyopathy is defined as a syndrome of dilated ventricles with gross impairment of ventricular systolic function. Recently, there are several anesthetic reports regarding this syndrome. But any appropriate anesthetic method has not been established. A 59-year-old man with dilated cardiomyopathy underwent low anterior resection of rectum. We employed the technique of high dose epidural fentanyl anesthesia as the anesthetic method. During surgery, mild hypotension developed, but other cardiovascular abnormalities were not observed during and after the operation. Based on this result, the authors recommend that high dose epidural fentanyl anesthesia is an anesthetic method of choice for patients with dilated cardiomyopathy.

Anesthesia, Epidural↗
PubMed1993

[Virus and dilated cardiomyopathies].

Dilated cardiomyopathy is so called when an etiological investigation is negative and no cause can be found for ventricular dilatation-hypokinesia. Current research points to genetic, immunological and infectious factors, often associated, and the passage of subclinical viral myocarditis to chronic disease. There is a lot of evidence in favour of this hypothesis. In the experimental model, the relationship between viral myocarditis and dilated cardiomyopathy has been demonstrated with, as cofactors, a genetic predisposition and an immunitary deficiency leading to an auto-immune subacute myocarditis. In the clinical setting, the enterovirus with a high cardiac tropism seems to play an epidemiological role in the genesis of dilated cardiomyopathy. The concentrations of neutralising anti-coxsackie B virus antibodies is higher in subjects with dilated cardiomyopathy than in a control population. The frequency of lymphocytic infiltration, a marker of dysimmunitary myocarditis, is variable from study to study but the presence of sequences of enterovirus genome in the myocardium could explain slow replication of the virus progressively destroying the myocytes. Techniques of molecular hybridization with or without prior genic amplification by the "Polymerase Chain Reaction" have demonstrated such sequences of specific enterovirus genome but discordant results require further studies.

Cardiomyopathy, Hypertrophic↗
PubMed2003

[Genetics and dilated cardiomyopathy].

Dilated cardiomyopathy is characterised by dilatation associated with ventricular dysfunction and represents the leading cause of cardiac transplantation. The aetiology of dilated cardiomyopathy is either multifactorial (about 70% of cases) with genetic and environmental components or monogenic (about 30% of cases) by autosomal dominant transmission. One of the features of this cardiomyopathy is its very wide range of genetic abnormalities and of its phenotype expression. In the multifactorial forms, several polymorphisms have been associated with the predisposition or with the severity of the phenotype. However, these associations have only been observed in a small number of cases and require further confirmation. In the monogenic forms, many culprit genes and morbid loci have been identified. These genes code essentially the proteins involved in the architecture of the cardiomyocyte suggesting mechanisms of action which still remain largely hypothetical. To improve the management of patients and their relatives, other morbid genes remain to be identified in addition to understanding the precise molecular and physiological mechanisms which trigger the disease.

Cardiomyopathy, Dilated↗