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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

24 endomyocardial biopsies were performed in children aged 4-18 (x = 12.9) without any complications. The endomyocardial biopsy was performed in 12 patients with the heart failure of unknown origin (6 - restrictive heart disease, 4 - dilated cardiomyopathy with arrhythmias, 2-post-inflammatory dilated cardiomyopathy), in 5 patients with a clinical diagnosis of myocarditis, in 1 with the hypertrophic cardiomyopathy, in 2 cases of the dilated cardiomyopathy without heart failure and in 1 girl with the recurrent exudative pericarditis. Active myocarditis with fibrosis was stated in 2 cases of a restrictive heart disease, mild inflammatory state in 2 patients with a clinical diagnosis of the myocarditis and in 3 others with the dilated cardiomyopathy. All of 7 patients with the myocarditis underwent the immunosuppressive therapy. In the patient with endomyocardial fibrosis the result of left ventricular biopsy corresponded with angiocardiographic diagnosis. Non-specific changes in biopsies were stated in 2 children with the restrictive cardiomyopathy and in 5 with the dilated cardiomyopathy. Bioptic, morphologic lesions in patients with the dilated cardiomyopathy did not correlate with hemodynamic parameters of contractility. Biopsies were normal in 6 subjects. Endomyocardial biopsy influenced on making a decision of the therapy in 9 of 21 patients (42.8%). Diagnosis was verified in 7 patients (38.3%) basing on endomyocardial biopsy. In 18 of 24 subjects (75%) endomyocardial biopsy contributed to the interpretation of the disease pathology.
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PMID:[Endomyocardial biopsy in children and adolescents]. 261 40

The cardiomyopathies are defined and classified into hypertrophic, dilated and restrictive types. In hypertrophic cardiomyopathy the major abnormalities of structure (massive ventricular hypertrophy, myofibrillar disarray, and narrow intramural coronary arteries) and of function (excessive ventricular contraction, systolic pressure gradients, increased ventricular stiffness with impaired relaxation and a tendency for sudden death) are used as the basis for selective and rational treatment with beta-blocking, calcium blocking, or antiarrhythmic agents, or a combination. Treatment is aimed at relieving symptoms, improving prognosis and slowing the progress of disease. Additional methods of treatment involving pacemakers and defibrillators are covered, and the place of septal resection, mitral valve replacement and cardiac transplantation discussed. General management and advice to the patient, and the treatment of complications such as atrial fibrillation, congestive heart failure and infective endocarditis, are also covered. In dilated cardiomyopathy measures to improve ventricular function by vasodilator therapy and the place of antiarrhythmic and anticoagulant drugs are discussed. The controversial treatment with beta-adrenergic blocking agents is reviewed, and the place of immunosuppressive therapy assessed. The possible use of antiviral agents in the future is commented upon, and cardiac transplantation is emphasised as the most effective, although radical, means of improving prognosis in intractable cases. In restrictive cardiomyopathy due to endomyocardial fibrosis, treatment of the initial inflammatory stage with steroids or sulphonylurea, and of the later fibrotic and thrombotic stage with anticoagulants and endocardectomy, is surveyed. The possible place of cardiac transplantation both for endomyocardial fibrosis and amyloid heart disease is mentioned, caution being urged when either of these conditions involves organs other than the heart.
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PMID:Clinical decisions in the management of the cardiomyopathies. 269 49

To assess left ventricular diastolic filling in amyloid heart disease, 17 patients with familial amyloid polyneuropathy and 20 normal subjects were examined by radionuclide angiography. None of the patients showed clinical evidence of restrictive cardiomyopathy. All but two patients had normal left ventricular ejection fraction. Peak filling rate was significantly lower and time to peak filling rate was significantly greater in patients than in normal subjects (2.60 +/- 0.52 versus 3.10 +/- 0.44 EDV/s, p less than 0.001, and 215 +/- 53 versus 147 +/- 18 ms, p less than 0.001, respectively). The mean left ventricular filling volume during rapid diastolic filling and atrial systole in patients was 54.5 +/- 19.5% and 44.2 +/- 21.6% of the stroke volume, respectively, compared with 83.8 +/- 6.6% (p less than 0.001) and 20.0 +/- 6.0% (p less than 0.001), respectively, in normal subjects. Although 10 of the 14 patients without clinical evidence of overt heart disease had normal ventricular wall thickness as well as normal ejection fraction, 8 of the 10 showed abnormal diastolic filling. In patients with familial amyloid polyneuropathy, indexes of diastolic filling were significantly related to ventricular wall thickness alone. The incidence and magnitude of abnormalities in time to peak filling rate and contribution of rapid filling as well as atrial systole to ventricular filling increased with age and duration of illness. Thus, abnormal diastolic filling can be seen even in the early stage of familial amyloid polyneuropathy and may be related to myocardial amyloid deposition as well as to fibrosis. Careful consideration should be given to age and duration of illness when diastolic filling is assessed in this disorder.
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PMID:Radionuclide angiographic assessment of left ventricular diastolic filling in amyloid heart disease: a study of patients with familial amyloid polyneuropathy. 290 80

To assess left ventricular diastolic filling in patients with amyloid heart disease 12 patients with familial amyloid polyneuropathy and 15 normal subjects were studied by pulsed Doppler echocardiography. None of the patients had clinical evidence of overt heart disease or restrictive cardiomyopathy and only two of them showed ventricular wall thickening. The peak flow velocity of rapid diastolic filling and the acceleration rate of early diastolic inflow were significantly lower in patients with familial amyloid polyneuropathy than in controls. The pressure half time was significantly longer in patients than in controls. In addition, the peak flow velocity during atrial contraction and the ratio of atrial peak flow velocity to rapid diastolic peak flow velocity were significantly greater in patients than in controls. Although there were no significant correlations between measurements of diastolic filling and clinical findings in patients with familial amyloid polyneuropathy, the ratio of atrial peak flow velocity to rapid diastolic peak flow velocity was significantly related to left ventricular posterior wall thickness. These findings suggest that in patients with cardiac amyloidosis without restrictive cardiomyopathy, abnormal left ventricular diastolic filling, manifested by a reduction in the rate and volume of rapid diastolic filling with enhanced atrial contraction, can be seen even in the early stage of the disease.
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PMID:Impaired left ventricular diastolic filling in patients with familial amyloid polyneuropathy: a pulsed Doppler echocardiographic study. 292 60

Aim of this study is the analysis of clinical, morphologic and haemodynamic features of cardiac amyloidosis. Cardiac amyloidosis was demonstrated histologically in 7 of our patients: in 6 by endomyocardial biopsy during cardiac catheterization, in one at autopsy. The clinical picture was characterized in every patient by signs and symptoms of congestive heart failure. The electrocardiogram showed several non specific signs: low voltage of the QRS complexes, both in peripheral (4/7) and precordial leads (7/7 cases); marked leftward and upward deviation of the QRS axis (6/7 cases); first degree A-V block (5/7); abnormal Q waves (7/7). M-mode and two-dimensional echocardiography invariably demonstrated a typical pattern: a non dilated left ventricle with thickened and hyper-refractile walls, and usually a slight-moderate diffuse hypokinesia. Other common features were a thickening of right ventricular walls, interatrial septum, and atrioventricular and semilunar valves. Computerized analysis of the M-mode tracings disclosed a marked impairment of the indexes of both systolic and diastolic ventricular function in all patients. By correlating electrocardiographic and echocardiographic data, we found in every case a striking disproportion between the low QRS voltage and the high muscle cross-sectional area (an echocardiographic index of left ventricular mass): this pattern appears to be highly suggestive of infiltrative heart disease. Cardiac catheterization (performed in 6 cases) showed an increase of left ventricular (6/6) and right ventricular (5/6) end-diastolic pressure, with a dip plateau pattern in some cases (4/6 of the left, 2/6 in the right ventricle). The cardiac index was decreased in 3/6 cases. Left ventricular angiography confirmed the echocardiographic data of normal volumes and a slight-moderate decrease of the ejection fraction. We conclude that cardiac amyloidosis usually mimics a restrictive cardiomyopathy (severe congestive heart failure with increased ventricular filling pressures, in the absence of severe systolic ventricular dysfunction). This disease can be suspected clinically by the correlation of the clinical, electrocardiographic and echocardiographic data. The final diagnosis requires an endomyocardial biopsy.
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PMID:[Cardiac amyloidosis. Invasive and noninvasive diagnosis]. 350 97

In order to evaluate the etiology of so-called idiopathic ventricular tachycardia, endomyocardial biopsies were performed in four patients with electrocardiographically documented recurrent and sustained ventricular tachycardia. During the episodes of ventricular tachycardia, standard ECG showed a QRS pattern of right bundle branch block with left axis deviation in two patients and left bundle branch block in two patients. The episodes were associated with palpitation, dyspnea and hypotension in all cases. No organic heart disease was detected by physical examination, chest X-ray films, echocardiograms, left ventriculograms or coronary cineangiograms. His bundle electrograms showed blocks at various sites in the atrioventricular conduction system. The biopsy specimens revealed nonspecific myocardial degeneration in the right and left ventricles. These findings suggest mild but wide-spread myocardial damage in both the working myocardium and the conduction system. The clinical course of these patients appeared benign according to follow-up data of one to nine years' duration. None developed overt clinical signs of dilated, hypertrophic or restrictive cardiomyopathy.
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PMID:Histological findings of the right and left ventricular myocardium and clinical follow up in idiopathic ventricular tachycardia. 376 28

The results of endomyocardial biopsy (EMB) are presented in 174 patients. Techniques and apparatus are described. EMB is safe and useful for diagnosing inflammatory myocarditis and as a guide to therapy. Unsuspected lymphocytic myocarditis was found in 17% of patients with dilated cardiomyopathy. The results confirm that the EMB findings in dilated cardiomyopathy are nonspecific and that restrictive cardiomyopathy is not confined to hypereosinophilic states and amyloid heart disease.
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PMID:Endomyocardial biopsy study of cardiomyopathy in Poland. 384 94

Twenty-five years ago clinical investigators began to appreciate that cardiomyopathy is an important and reasonably common form of heart disease. Since then, several functional classifications have been proposed, the specific myocardial diseases have been classified and chronic ischemic ventricular failure has been described. The boundary separating myocarditis from dilated cardiomyopathy remains hazy and, despite intensive research, the causes of dilated cardiomyopathy remain obscure. In particular, we still do not understand the role that may be played by viral infection and alcohol. Myocardial biopsy has proved useful in patients with specific myocardial disorders, heart transplant recipients and patients receiving Adriamycin, but is disappointing in patients with dilated cardiomyopathy. It has become increasingly evident that exercise capacity does not correlate with ventricular function, being highly dependent on peripheral factors. Measurements of oxygen consumption during exercise promise to be useful in assessing treatment of dilated cardiomyopathy. True restrictive cardiomyopathy is uncommon, and the term should be reserved for cardiomyopathies that meet strict criteria. A restrictive component to filling is common to many cardiac disorders, including some cases of cardiac amyloidosis. The concept of hypertrophic cardiomyopathy has evolved rapidly over the past 25 years, and continues to evolve. The importance of arrhythmia as a cause of sudden death is becoming increasingly clear. The place of calcium channel blocking agents in the treatment of hypertrophic cardiomyopathy will probably emerge soon. Amiodarone is finding an increasing role in the treatment of dilated and hypertrophic cardiomyopathy. Surgical treatment is still required for some patients despite unanswered questions on how it works.
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PMID:Cardiomyopathy: how far have we come in 25 years, how far yet to go? 668 23

Cardiac involvement in hemochromatosis typically results in congestive cardiomyopathy; a restrictive cardiomyopathy due to hemochromatosis is distinctly rare. A restrictive cardiomyopathy, which developed in the patient described in this report, was due to hemochromatosis which mimicked constrictive pericarditis clinically, echocardiographically and hemodynamically, and resulted in a thoracotomy for attempted surgical therapy. The fact that hemochromatosis represents the only cause of a restrictive cardiomyopathy that is potentially reversible by medical therapy makes early recognition of hemochromatosis heart disease important.
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PMID:Hemochromatosis heart disease: an unemphasized cause of potentially reversible restrictive cardiomyopathy. 744 57

MRI is an effective tool in the evaluation of cardiovascular diseases. With continued improvements in MR angiography, velocity mapping, myocardial tagging, imaging speed and display, it is anticipated that MRI will play an ever-increasing role in the morphological evaluation of many cardiovascular anomalies. The need for quantification in cardiology has led to the development of a variety of techniques to assess cardiac size and function. In attempting to answer these clinical questions, MRI provides major advantages several: extraordinary flexibility, powerful contrast mechanisms, sensitivity to flow and motion, and freedom from ionizing radiation, contrast agent and acoustic windows. Because of accurate initial results in quantifying cardiac chamber size, global and segmental function, it is likely that MRI methods will play an increasing role in the evaluation of cardiac structure and function. Recently, MRI has emerged as an important tool in the evaluation of great vessel disease, particularly in the evaluation of both congenital and acquired abnormalities of the aorta. Early evidence suggests that magnetic resonance may help in distinguishing constrictive pericarditis from restrictive cardiomyopathy (e.g., amyloid heart disease). Cardiac-MRI lends itself to assessment of intracardiac masses. Spin-echo imaging alone is often sufficient for diagnosis. Cine-MRI has important additive value, however, when a mass lesion shows dynamic motion, or when abnormal flow patterns in conjunction with a mass lesion require evaluation.
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PMID:[MRI in cardiology: clinical applications and perspectives]. 763 16


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