[Case of restrictive cardiomyopathy with eosinophilia not associated with tropical countries].
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Adult↗
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Results for “Cardiomyopathy, Restrictive”
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[Restrictive cardiomyopathy after endomyocardial fibrosis].
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Adolescent↗
The angiographic appearance of the coronary arteries in constrictive pericarditis.
Coronary artery motion was evaluated in normal subjects and patients with constrictive pericarditis (CP), congestive cardiomyopathy, restrictive cardiomyopathy, and coronary artery disease. Seven of the 8 patients with CP had lack of motion of the major coronary arteries. Nine of the 10 patients with congestive cardiomyopathy and a markedly reduced ejection fraction showed normal motion, as did all 4 with restrictive cardiomyopathy and 9 of the 10 with segmental dysfunction due to coronary disease. Pericardial stripping in 5 patients with CP revealed epicardial involvement corresponding to regions of absent motion; one CP patient with normal motion had no epicardial involvement. Lack of motion may suggest CP when the diagnosis is unsuspected, right heart pressures are not obtained, or hemodynamics are atypical. This sign may also help to distinguish CP from restrictive cardiomyopathy, which produces similar hemodynamics.
Adult↗
[Idiopathic cardiomyopathy].
Cardiomyopathies are defined as diseases of the myocardium associated with cardiac dysfunction. They are classified as dilated cardiomyopathy, hypertrophic cardiomyopathy, restrictive cardiomyopathy. Dilated cardiomyopathy is characterized by dilatation and impaired contraction of the ventricles. Hypertrophic cardiomyopathy is characterized by ventricular hypertrophy, which is usually asymmetric. Restrictive cardiomyopathy is characterized by restrictive filling and reduced diastolic function, with normal systolic function and wall thickness. Except for cardiac failure, sudden death from arrhythmia is the leading cause of death among cardiomyopathy patients. ACE inhibitors and beta blockers are effective medicine for cardiac failure patients. However sudden death rate of cardiomyopathy patients is still high. Implantable cardioverter-defibrillator could save many cardiomyopathy patients from sudden death according to a lot of trials.
Cardiomyopathies↗
The challenge of cardiomyopathy.
The combined clinical and pathophysiologic characteristics and diagnostic features as well as current concepts of pathogenesis, therapy and prevention of the principal forms of cardiomyopathy are reviewed. These include hypertrophic cardiomyopathy, dilated cardiomyopathy, restrictive cardiomyopathy and specific cardiac muscle disease. Emphasis is placed on recent developments and unresolved questions requiring application of newer techniques of molecular biology and genetics and adult myocyte culturing.
Cardiomyopathy, Dilated↗
[Epidemiology-etiology of dilated cardiomyopathy].
Among the cardiomyopathies,--dilated cardiomyopathy (dcm), hypertrophic cardiomyopathy, restrictive cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy--, dcm is the most frequent entity. Its prevalence in the United States amounts to 36 cases per 100,000 inhabitants, men being almost 3-fold more involved than women. The etiology of dcm is very heterogenous; 50% of the cases are due to idiopathic dcm whereas the other half comprises a broad spectrum of various etiologies such as myocarditis, ischemic heart disease, peripartal cardiomyopathy, hypertension, HIV infection, toxic cardiomyopathy and others. In 20 to 30% of the cases of idiopathic dcm a genetic transmission of the disease has been found. Another 20 to 30% of idiopathic dcm are associated with inflammatory and immunological phenomena. Infectious myocarditis with enteroviruses, especially with coxsackie-virus type B has been suggested to be an important trigger for an immune-mediated dcm. In both, familiar dcm and infection with coxsackie-virus B, an impairment of constituents of the myocardial cytoskeleton has been shown. This is regarded as a possible pathogenetic mechanism in the development of dcm.
Adult↗
The emerging role of MRI in the diagnosis and management of cardiomyopathies.
Cardiac magnetic resonance (CMR) has emerged as an important tool for the evaluation of cardiomyopathies, providing highly accurate information on the macroscopic changes of cardiac morphology, function, and tissue composition. For myocardial tissue characterization, the technique of myocardial delayed enhancement is a potentially promising tool for diagnosis, management, and prognosis. Several CMR approaches are now available to better diagnose and prognosticate dilated cardiomyopathy, hypertrophic cardiomyopathy, restrictive cardiomyopathy, arrhythmogenic right ventricular disease, myocarditis, and other cardiomyopathies.
Arrhythmogenic Right Ventricular Dysplasia↗
Cardiac features of Emery-Dreifuss muscular dystrophy caused by lamin A/C gene mutations.
AIMS: Retrospective studies have identified a mutation in the lamin A/C (LMNA) gene in patients selected on the basis of a phenotype characterized by dilated cardiomyopathy, atrioventricular conduction disturbances and sudden death. However, the features of cardiac abnormalities in patients with an initial diagnosis of Emery-Dreifuss muscular dystrophy (EDMD) are poorly known. Aim of the present study was to investigate the spectrum of cardiac disease in patients with an initial diagnosis of EDMD caused by a mutation in the LMNA gene. METHODS AND RESULTS: Ten consecutive patients with EDMD and a LMNA gene mutation were evaluated with structured medical interview, physical examination, ECG, echocardiogram and 24-h Holter monitoring. Electrophysiological testing and cardiac catheterization were performed if a class 1 or 2 American Heart Association guidelines indication was present. Cardiac disease was found in eight of 10 patients and consisted in the variable combination of supraventricular arrhythmias, disorders of atrioventricular conduction, ventricular arrhythmias, dilated cardiomyopathy, non-dilated cardiomyopathy, restrictive cardiomyopathy and sudden death despite pacemaker implant. CONCLUSIONS: Cardiac disease is common in patients with an initial diagnosis of EDMD caused by a mutation in the LMNA gene and consists of arrhythmias, disorders of atrioventricular conduction, cardiomyopathies and sudden death despite pacemaker implant.
Adolescent↗
Congenital Heart Surgery Nomenclature and Database Project: pediatric cardiomyopathies and end-stage congenital heart disease.
The extant nomenclature for cardiomyopathy is reviewed for the purpose of establishing a unified reporting system. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Database Committee and representatives from the European Association for Cardiothoracic Surgery. All efforts were made to include all relevant nomenclature categories using synonyms where appropriate. A functional classification based on pathophysiology is proposed. Cardiomyopathy is subdivided into: dilated cardiomyopathy, hypertrophic cardiomyopathy, restrictive cardiomyopathy, right ventricular cardiomyopathy, and end-stage congenital heart disease. A comprehensive database set is presented that is based on a hierarchical scheme. Data are entered at various levels of complexity and detail that can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analyses. A minimum database set is also presented that will allow for data sharing and would lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and various risk factors are presented.
Cardiomyopathies↗
Rate modulated pacing based on right ventricular dP/dt: quantitative analysis of chronotropic response.
Right ventricular contractility increases in response to catecholamine stimulation and greater ventricular preload, factors that increase with exercise workload. Thus, the maximum systolic dP/dt may be a potentially useful sensor to control the pacing rate of a permanent pacing system. The present study was designed to test the long-term performance of a permanent pacemaker that modulates pacing rate based on right ventricular dP/dt and to quantitatively analyze the chronotropic response characteristics of this sensor in a group of patients with widely varying structural heart diseases and degrees of hemodynamic impairment. A permanent pacing system incorporating a high fidelity pressure sensor in the lead for measurement of right ventricular dP/dt was implanted in 13 patients with atrial arrhythmias and AV block, including individuals with coronary artery disease, hypertension, severe obstructive pulmonary disease with prior pneumonectomy, atrial septal defect, dilated cardiomyopathy, restrictive cardiomyopathy, and mitral stenosis. Patients underwent paired treadmill exercise testing in the VVI and VVIR pacing modes with measurement of expired gas exchange and quantitative analysis of chronotropic response using the concept of metabolic reserve. The peak right ventricular dP/dt ranged from 238-891 mmHg/sec with a pulse pressure that ranged from 19-41 mmHg. There was a positive correlation between the right ventricular dP/dt and pulse pressure (r = 0.70, P = 0.012). The maximum pacing rate and VO2max were 72 +/- 6 beats/min and 12.61 +/- 4.0 cc O2/kg per minute during VVI pacing and increased to 124 +/- 18 beats/min and 15.89 +/- 5.9 cc O2/kg per minute in the VVIR pacing mode (P < 0.0003 and P < 0.002, respectively). The integrated area under the normalized rate response curve was 96.7 +/- 45.7% of expected during exercise and 100.1 +/- 43.4% of expected during recovery. One patient demonstrated an anomalous increase in pacing rate in response to a change in posture to the left lateral decubitus position. Thus, the peak positive right ventricular dP/dt is an effective rate control parameter for permanent pacing systems. The chronotropic response was proportional to metabolic workload during treadmill exercise in this study population with widely varying forms of structural heart disease.
Adult↗
[Definition and classification of cardiomyopathies and specific cardiomyopathies].
The cardiomyopathies were previously defined as "heart muscle diseases of unknown cause" and were differentiated from specific heart muscle disease with known cause. With increasing understanding of etiology and pathogenesis, the difference between cardiomyopathy and specific heart muscle disease has become indistinct. Accordingly in 1995 WHO/ISFC task force, the definition and classification of cardiomyopathies were revised as cardiomyopathies are defined as disease of the myocardium associated with cardiac dysfunction. On the other hand, since the classification has become established clinical entities, the terminology is preserved as follows: dilated cardiomyopathy, hypertrophic cardiomyopathy, restrictive cardiomyopathy. While arrhythmogenic right ventricular cardiomyopathy is additionally included. The term specific cardiomyopathies is used to describe heart diseases that are associated with specific cardiac or systemic disorders. These were previously defined as specific heart muscle diseases. They included ischemic cardiomyopathy, valvular cardiomyopathy, hypertensive cardiomyopathy, inflammatory cardiomyopathy, metabolic cardiomyopathy, general system disease, muscular dystrophies, sensitivity and toxic reactions and peripartal cardiomyopathy. The cardiomyopathies are therefore classified by the dominant pathophysiology or, if possible, by etiological/pathogenetic factors.
Cardiomyopathies↗
The cardiomyopathies, a review for the primary physician.
The cardiomyopathies constitute a group of diseases with direct involvement of the heart muscle itself, and is a significant cause of morbidity and mortality. The World Health Organization (WHO) and the International Society and Federation of Cardiology (ISFC) have promulgated a classification taking into consideration the etiology and pathophysiology, which includes dilated cardiomyopathy, hypertrophic cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, restrictive cardiomyopathy, and unclassified cardiomyopathies. Over 25 causes are associated with the development of the cardiomyopathies. The classification of the diseases affecting the heart and causing the cardiomyopathies is presented including the highlights of the most important data for diagnosis and management of each one.
Cardiomyopathies↗
Epidemiology of idiopathic cardiomyopathy in Japan: results from a nationwide survey.
OBJECTIVE: To estimate the total number of patients with idiopathic cardiomyopathy in Japan and the prevalence of the disorder. DESIGN: A nationwide epidemiological survey. SETTING: Hospitals selected randomly from among all hospitals in Japan. PATIENTS: Patients presenting with any of the three types of idiopathic cardiomyopathy: dilated cardiomyopathy, hypertrophic cardiomyopathy, and restrictive cardiomyopathy. MAIN OUTCOME MEASURES: The total number of patients in Japan was estimated using the sampling and response rates in each stratum with respect to hospital size. The second survey was conducted for patients reported in the first survey in order to obtain detailed information, including age, sex, and specific clinical data. RESULTS: Estimated patient totals and 95% confidence intervals (CI) were 17 700 (95% CI 16 500 to 18 800) for dilated cardiomyopathy, 21 900 (95% CI 20 600 to 23 200) for hypertrophic cardiomyopathy, and 300 (95% CI 250 to 350) for restrictive cardiomyopathy. Crude prevalence per 100 000 population was estimated as 14.0 for dilated cardiomyopathy, 17.3 for hypertrophic cardiomyopathy, and 0.2 for restrictive cardiomyopathy; crude incidence per 100 000 person-years was estimated as 3.58, 4.14, and 0.06, respectively. CONCLUSIONS: The total number and prevalence of patients with idiopathic cardiomyopathy in Japan are estimated for the first time in a nationwide survey. The prevalence of dilated cardiomyopathy in Japan appears to be about half that of Western populations, while that of hypertrophic cardiomyopathy is about the same.
Adolescent↗
Magnetic resonance imaging of primary cardiomyopathies.
Cardiomyopathies are diseases of the myocardium of unknown etiology associated with cardiac dysfunction. On the grounds of their morphology and pathophysiology, primary or idiopathic cardiomyopathies may be classified into a number of disorders; namely, hypertrophic cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, dilated cardiomyopathy, and restrictive cardiomyopathy. The term "secondary cardiomyopathies" is reserved to specific heart muscle diseases clinically very similar to primary cardiomyopathies. Cardiac magnetic resonance imaging has long been used to study cardiac morphology and, more recently, to assess blood flow, perfusion, and contractile function. The emerging role of magnetic resonance imaging for the understanding and treatment of primary cardiomyopathies cannot be underestimated. From a clinical point of view, an examination based on a single, efficient, and noninvasive MR study focusing on the clinically relevant features of cardiomyopathies is an objective and reproducible means for diagnosing and monitoring hypertrophic, arrhythmogenic, dilated, and restrictive cardiomyopathies.
Adult↗
Cardiomyopathies with particular reference to the diagnostic relevance of endomyocardial biopsies.
A distinction is made between heart muscle diseases with a known cause, or occurring as part of a systemic disease, and those of unknown cause. The first category is termed "specific heart muscle disease". The term "cardiomyopathy" is then limited to the latter category. Cardiomyopathies are categorized as dilated cardiomyopathy, hypertrophic cardiomyopathy and restrictive cardiomyopathy. The pathology of dilated cardiomyopathy depends on the clinical stage of the disease. Basically, the histology shows hypertrophic myocytes, often with degenerative signs, and interstitial fibrosis. Lymphocytes may be observed, particularly in cases studied shortly after the onset of symptoms. Endocardial thickening may occur in time. The role of endomyocardial biopsies in the clinical setting is limited and relates mainly to excluding other diseases, such as myocarditis. The pathology of hypertrophic cardiomyopathy is characterized by myocardial wall thickening, either asymmetric or symmetric, and disorganization of the normal myocardial texture. The latter phenomenon should be distinguished from disarray, since it may occur as a natural phenomenon. The differentiation between these two may be extremely difficult on the basis of only small tissue samples, as with endomyocardial biopsies. This, therefore, poses serious limitations in the use of endomyocardial biopsies. The pathology of restrictive cardiomyopathy also depends on the stage of the disease.(ABSTRACT TRUNCATED AT 250 WORDS)
Biopsy↗
Human myocardial ATP content and in vivo contractile function.
The study was designed to characterize the relationship between the metabolite content of human cardiac muscle and in vivo cardiac function. ATP, total adenine nucleotides, and NAD were quantified in human myocardial biopsies using high performance liquid chromatography. Right ventricular endomyocardial biopsies were obtained from 43 patients with dilated cardiomyopathy, 6 with restrictive cardiomyopathy, 10 with normal systolic and diastolic function, and from 24 cold preserved human donor hearts. Transmural samples of failing right and left ventricular free walls were obtained during cardiac transplantation surgery in 8 patients. ATP, total adenine nucleotides, and NAD were similar in the cold-preserved donor hearts and in right ventricular endomyocardial biopsies from the 10 individuals with normal systolic and diastolic function. In contrast, these values were significantly depressed in tissue samples from patients with dilated or restrictive cardiomyopathy. There was a significant correlation between ATP and pulmonary capillary wedge pressures but not ejection fractions. Declines in the sizes of myocardial ATP, adenine nucleotide, and pyridine nucleotide pools in the human myocardium are associated primarily with diastolic but not systolic dysfunction.
Adenosine Triphosphate↗
Epidemiology, pathophysiology, and etiology of congestive heart failure in older adults.
OBJECTIVES: To review the epidemiology, pathophysiology, and etiology of congestive heart failure (CHF) in older adults. METHODS: Published reports relevant to the epidemiology, pathophysiology, and etiology of CHF were systematically reviewed. Studies involving older adults and more recent studies were emphasized. RESULTS: More than 75% of patients with CHF in the United States are older than 65 years of age, and CHF is the leading cause of hospitalization in older adults. CHF is also a major cause of chronic disability, and annual expenditures for CHF currently exceed $10 billion. In addition, both the incidence and prevalence of CHF are increasing, largely as a result of the aging of the population. Older adults are predisposed to developing CHF as a result of age-related changes in the cardiovascular system and the high prevalence of hypertension, coronary artery disease, and valvular heart disease in this age group. Although the fundamental pathophysiology of CHF is similar in younger and older patients, older individuals are more prone to develop CHF in the setting of preserved left ventricular systolic function. This syndrome, referred to as diastolic heart failure, accounts for up to 50% of all cases of CHF in adults more than 65 years of age. Coronary heart disease and hypertension are the most common etiologies of CHF in older adults, and they often coexist. Valvular heart disease, especially aortic stenosis and mitral regurgitation, are also common in older adults, whereas nonischemic dilated cardiomyopathy, hypertrophic cardiomyopathy, and restrictive cardiomyopathy occur less frequently. CONCLUSIONS: Congestive heart failure is a major public health problem in the United States today as a result of its high and increasing prevalence in the older population as well as its substantial impact on healthcare costs and quality of life. There is an urgent need to develop more effective strategies for the prevention and treatment of CHF in older individuals.
Age Factors↗
[Restrictive infiltrating cardiomyopathy in primary amyloidosis].
The authors describe a typical echocardiographic finding in restrictive infiltrative cardiomyopathy which after two years led to the diagnosis of primary amyloidosis in a 51-year-old woman. The predominating manifestations of the disease were renal damage with final failure, heart failure, monoclonal gammapathy and damage of other organs. Aimed treatment of amyloidosis and repeated haemodialysis did not prevent the patient's death from cardiac and renal failure. Primary amyloidosis was confirmed by histological examination and necropsy.
Amyloidosis↗