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

Cardiomyopathies in the elderly have certain characteristic features. The dilated form appears to be less common than in younger patients. Hypertrophic cardiomyopathy is more often associated with severe and concentric hypertrophy. The prognosis of hypertrophic cardiomyopathy seems to be better in the elderly, because they appear to have a lower incidence of sudden death. Restrictive cardiomyopathies are not common in the elderly, and senile amyloid heart disease rarely, if ever, results in congestive heart failure. A syndrome of clinical heart failure with reduced diastolic compliance and preserved systolic function is more common in elderly patients.
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PMID:Cardiomyopathies in the elderly. 329 76

Hypertrophic cardiomyopathy (HCM) presented in 10 children under 2 years of age (group 1) and in 5 between 3 and 8 years (group 2). The clinical, ECG, chest radiographic and echocardiographic features are reviewed and prognosis over a mean follow-up period of 3.5 years is reported. Patients in group 1 had more symptoms and 7 had evidence of heart failure at some stage; all had ECG abnormalities. Group 2 patients presented with murmurs and only 1 had heart failure. Medical management of these patients is discussed and the importance of accurate diagnosis stressed, since HCM may have a poor prognosis in childhood; 3 out of 15 patients have died.
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PMID:Hypertrophic cardiomyopathy in infancy and childhood. 356 11

Retrospective analysis was performed to assess the natural history in relation to clinical and haemodynamic features in 37 patients in whom hypertrophic cardiomyopathy had been diagnosed in childhood. At diagnosis they were aged 1 to 14 years (mean 9 years). Eighteen presented with chest pain and either dyspnoea or symptoms of impaired consciousness or both; 19 were asymptomatic and were referred for evaluation of abnormalities detected during physical examination. During a mean follow up of nine years, 18 patients died, a cumulative annual medical mortality of 4.8%. Five patients experienced severe dyspnoea or chest pain: two of these had progressive dyspnoea and died in cardiac failure, and three died after myectomy. The 19 survivors were compared with the 11 sudden deaths. Eleven of the survivors and five of the sudden deaths were asymptomatic. Of 18 clinical, electrocardiographic, and haemodynamic features only syncope and electrocardiographic evidence of right ventricular hypertrophy were associated with sudden death. In children with hypertrophic cardiomyopathy sudden death was common and was not well predicted by clinical, electrocardiographic, or haemodynamic findings. Hypertrophic cardiomyopathy indicates a poor prognosis even if symptoms are absent or mild.
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PMID:Hypertrophic cardiomyopathy: an important cause of sudden death. 654 94

The incidence of heart failure in octogenarians is high and its diagnosis not always easy. In many cases it is made by excess or by omission. Obtaining a history is often difficult. Signs may be masked, false or indicative of another disease process. Dyspnea, edema of the lower limbs and crepitations are relatively non-specific. Jugular distension, tender hepatomegaly and a diastolic gallop are much more valuable. Diagnosis of the underlying etiology also raises problems. While hypertension is commonplace and easy to identify, ischemic heart disease is common and often missed. Tight aortic stenosis must be identified since its treatment is surgical. Hypertrophic cardiomyopathy is often an echocardiographic discovery. Post-embolic chronic cor pulmonale, or secondary to chronic obstructive lung disease, must always be considered in the presence of right heart failure without hypertension or chest pain. Appropriate treatment is dependent upon accurate diagnosis.
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PMID:[Difficulties in the diagnosis of cardiac insufficiency in octogenarians]. 782 52

Hypertrophic cardiomyopathy is a primary myocardial disorder with an autosomal pattern of inheritance, characterized by asymmetric left ventricular hypertrophy with myocyte and myofibrillar disarray. Approximately 30% to 50% of all cases are accounted for by mutations in the beta-cardiac myosin heavy chain gene on chromosome 14q1. Recent linkage analysis led to the association of the disease with additional loci on chromosomes 1q3, 11p13-q13, and 15q2, but the underlying gene defects are as yet unidentified. To date, about 34 mutations of the beta-cardiac myosin heavy chain gene have been described and shown to have important prognostic implications. Definite genotype-phenotype correlations have been described; however, wide diversity in cardiac morphology, pathophysiologic features, and clinical manifestations is still evident, even within the same family. The disease has an annual mortality of approximately 3%, related to both progressive heart failure and sudden cardiac death. Not only diastolic but also progressive systolic dysfunction with cavity dilatation occurs in a minority of patients with severe left ventricular hypertrophy. These patients usually have a poor prognosis, especially when atrial fibrillation ensues. Sudden death often occurs in young, asymptomatic or mildly symptomatic patients. The degree of hypertrophy and the presence of a pressure gradient are of little prognostic significance. Nonsustained ventricular tachycardia is associated with a poor prognosis in the presence of a history of syncope.
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PMID:Risk stratification in hypertrophic cardiomyopathy. 804 90

Hypertrophic cardiomyopathy is a heterogeneous disease with autosomal dominant Mendelian inheritance. In 1989, the 1st locus for hypertrophic cardiomyopathy was mapped to cardiac myosin genes located on chromosome 14q1. Soon, several mutations that cosegregated with inheritance of the disease were identified in the beta-myosin heavy chain gene, or MHY7. More than 30 missense mutations and 1 deletion mutation in the beta-myosin heavy chain gene have since been described. Recently, expression of both the mutant beta-myosin heavy chain mRNA and the mutant protein has been shown in the cardiac and skeletal muscles of individuals with hypertrophic cardiomyopathy. Characterization of the clinical features of beta-myosin heavy chain mutations has shown that certain mutations, such as Arg403Gln and Arg719Trp mutations, are associated with high rate of sudden cardiac death. In addition to the beta-myosin heavy chain gene, 3 new loci for hypertrophic cardiomyopathy have recently been described, but the candidate genes have not yet been identified. Dilated cardiomyopathy can be inherited as an autosomal dominant, autosomal recessive, and X-linked disease. The familial form of dilated cardiomyopathy comprises approximately 20% of the cases of idiopathic cardiomyopathy. Echocardiographic abnormalities such as left ventricular enlargement are present in 10% of asymptomatic relatives. No gene for familial dilated cardiomyopathy has been identified, but linkage studies using polymorphic, short-tandem repeat markers are ongoing. Dilated cardiomyopathy is a common manifestation of Duchenne/Becker muscular dystrophy. Heart failure is a common cause of death in the affected individuals. The gene responsible for this disease is the dystrophin gene located on X chromosome. There have been reports in these patients of several dystrophin-gene deletion mutations, which result in a decrease in the expression of the dystrophin protein in the cardiac and skeletal tissues. X-linked cardiomyopathy, in which the disease is restricted to the heart, has also been linked to the dystrophin gene. Myotonic dystrophy is an autosomal dominant disease that commonly involves the myocardium and the conduction tissue, resulting in conduction defects and heart failure. Sudden cardiac death is the most common cause of mortality in patients with myotonic dystrophy. Recently, the myotonin protein kinase gene located on chromosome 19 was identified as the gene responsible for this disease. Expansion of the number of trinucleotide repeats in the myotonin protein kinase gene results in myotonic dystrophy. Mutations in mitochondrial DNA have been associated with hypertrophic and dilated cardiomyopathy. The inheritance of mitochondrial cardiomyopathy is maternal and the disease is associated with certain systemic disorders.
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PMID:Molecular basis of hypertrophic and dilated cardiomyopathy. 818 May 12

Hypertrophic cardiomyopathy is characterized by abnormalities of the myocardium, and the activation and conduction tissues, that may have separate manifestations, but often occur together in complex clinical pictures. The subaortic gradient, although not always present, is the most classical manifestation of the disease, with its typical dynamic behavior, changing with preload, afterload and contractility. In most cases it is due to systolic motion of the mitral valve against the septum in systole, but in a few it is caused by midventricular "constriction". Alteration of diastolic ventricular function is important, and probably the main cause of heart failure, that is usually accompanied by normal systolic function. Mitral insufficiency is common in the obstructive forms, due to the abnormal mitral valve motion, but in some cases it may be due to structural abnormalities of the valve. There may be systolic constriction, or nonatherosclerotic occlusion of the intramyocardial coronary arteries, causing myocardial infarction and ventricular aneurysms, that may lead to systolic dysfunction. The electrocardiogram is rarely normal. Hypertrophy patterns, deeply inverted T waves, deep Q waves, QRS slurring suggestive of WPW syndrome without true preexcitation are the most common manifestations. Rhythm disturbances are common and include sinus node dysfunction, superconductor atrioventricular node or heart block. Atrial fibrillation is frequent and may have catastrophic consequences, including systemic embolism. Non-sustained ventricular arrhythmias are often present, but its predictive value for sudden death is unclear. Monomorphic ventricular tachycardia is infrequent, and programmed stimulation is more likely to precipitate polymorphic ventricular tachycardia of difficult clinical interpretation. Sudden death may be due to multiple mechanisms, and it is difficult to predict and prevent.
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PMID:[Hemodynamic and electrophysiologic changes in hypertrophic cardiomyopathy]. 868 13

Cardiomyopathies, and more specifically hypertrophic cardiomyopathy, have opened the route to what is now called genetic cardiology. Hypertrophic cardiomyopathy (HCM) is characterized by unexplained left and/or right ventricular hypertrophy, and disorganisation of tissular architecture. Approximately 60% of HCM are transmitted as an autosomal dominant trait. The clinical aspects of HCM vary markedly, and several morphological variants were described, depending on the localization of hypertrophy. This pathology is often complicated by cardiac failure, but the major risk is sudden death, and the predictive factors are presently very unrefined. Several pathogenic hypotheses were forwarded in the past, and one surprising result of genetic analyses is that none of these hypotheses was confirmed. Four disease genes were identified, and they encode sarcomeric proteins, cardiac myosin heavy chain, troponin T, tropomyosin and cardiac myosin binding protein C. To this high intergenic heterogeneity is associated a high intragenic heterogeneity. A major fall out of these genetic findings is the recent discovery of adult healthy carriers, around 30% in our experience. Genetype/phenotype relationships are being performed, and this is the first approach to a prognostic evaluation based on genetic localisation. The work on hypertrophic cardiomyopathy is currently being used as a model to analyse dilated cardiomyopathies, characterized by dilatation and impaired contraction of the left or both ventricles. The mode of inheritance of these forms of cardiomyopathies is complex. Five families with an autosomal inheritance were analyzed since two years, the loci were found, but the disease genes are not identified yet. Identification of patients at high risk and early treatment or prevention are the current goals.
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PMID:[Cardiomyopathies]. 929 66

Hypertrophic cardiomyopathy is a disorder which primarily affects the myocardium. It is characterized by a hypertrophic left ventricle that shows normal systolic function but impaired diastolic relaxation. The most important complication of HCM, sudden cardiac death, is mainly responsible for a mortality of approximately 3.5% in childhood. While most patients remain asymptomatic for years, there are some reports about patients progressing from hypertrophic cardiomyopathy to a dilative form within years. We report on a boy who was at the age of twelve when hypertrophic cardiomyopathy was diagnosed first. He remained stable for the first years, but at the age of sixteen his cardiomyopathy progressed to a dilative form with reduced systolic function and severe cardiac failure. The possible pathogenesis is discussed and an overview of similar cases reported earlier is given.
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PMID:[Rapid progression of hypertrophic cardiomyopathy into a dilated form--unusual course in a young patient. A case report]. 932 73

Hypertrophic cardiomyopathy is a dominantly inherited disease of the heart. Heterogeneous sets of mutations responsible for this condition have been identified in seven genes coding for proteins involved in the contraction mechanism or in the control of contraction of the myocardium. Known mutations imply structural and functional changes in the following proteins: in ventricle specific beta-myosin heavy chain, in essential and regulatory myosin light chains, in troponin subunits T and I, in alpha-tropomyosin and in myosin binding protein-C. The gene of one additional genomic HCM-locus is not known. Since two thirds or more of all cases can be traced to one of the respective genes, HCM has been classified as a disease of the cardiac sarcomere. Heterogeneity does not only exist between genes, but also within genes. At least 84 different mutations have been identified to date. More than half of them have been detected in the beta-myosin heavy chain gene. Thus, mutations in this gene account for most of the cases of HCM. The extent of data about causes is in contrast to the lack of definite knowledge about pathogenic mechanisms. Since the disorder is in many cases mild with symptoms developing frequently not before the end of the second decade, myocardial dysfunctions can presumably not directly be traced to altered contractility, but rather to effects which accumulate with a long asymptomatic lag period and which gradually lead to hypertrophy, conduction problems and ultimately to cardiac failure. The disease may be considered as an indirect and secondary response to a mildly distorted contraction process. The rapid progress in the analysis of causes suggests that the study of genes will assume a role in the context of the clinical management of HCM, in particular regarding diagnosis, prognosis, counselling of patients and families and--possibly--therapy.
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PMID:[Genetic causes of hypertrophic cardiomyopathy]. 959 35


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