Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Echocardiography has been used for cardiovascular evaluation of individuals and families with Ullrich-Noonan syndrome. Previously undiagnosed left ventricular disease has been found as a discrete lesion or in association with other cardiac abnormalities. This raises the estimated frequency of heart disease in the Ullrich-Noonan syndrome to about 50%. Since left ventricular disease in this syndrome may not be entirely typical of asymmetric septal hypertrophy, caution should be exercised in the echocardiographic diagnosis. To date, one notable difference between the echocardiograms in these patients and other patients with asymmetric septal hypertrophy is the absence of systolic anterior motion of the mitral valve. Since the most common cardiac lesion the the Ullrich-Noonan syndrome is pulmonary stenosis, the potential for septal thickening produced by severe pulmonary stenosis must also be taken into account.
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PMID:Echocardiographic studies of left ventricular disease in Ullrich-Noonan syndrome. 12 88

In a consecutive series of 1,225 routine echocardiograms, a pericardial effusion (PE) was present in 15% and was considered substantial in 10%. Sixty-one percent (68/111) of the latter were clinically unsuspected. A retrospective analysis of these patients showed that 60% (41/68) had underlying heart disease such as congestive heart failure, left ventricular hypertrophy, or asymmetric septal hypertrophy without other discernible causes for PE. In patients referred specifically for suspected pericardial effusion, 67% (29/43) had diseases recognized as causes of pericardial effusion. Unsuspected substantial PE are commonly detected by echocardiography, especially in patients with underlying heart disease.
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PMID:Unsuspected substantial pericardial effusions detected by echocardiography. 13 24

Dextroversion of the heart is an uncommon congenital anomaly characterized by situs solitus (normal position) of thoracic and abdominal viscera with right cardiac apex. Isolated dextroversion, i.e., without associated congenital heart disease, is rare, but its occurrence permits adult survival, setting the stage for late development of acquired heart disease. The patient herein reported was known since childhood to have a right thoracic heart that represented isolated uncomplicated dextroversion. He presented in the sixth decade with a new murmur that proved to be due to asymmetric septal hypertrophy (ASH) with obstruction. This combination of anomalies is possible only if dextroversion exists without ventricular inversion, since aortic-anterior mitral leaflet continuity is obligatory for obstructive ASH. This paper presented clinical, phonocardiographic, vectorcardiographic, echocardiographic, hemodynamic, angiocardiographic, and intracardiac electrophysiologic information on the unique combination of isolated dextroversion of the heart with obstructive asymmetric septal hypertrophy.
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PMID:Isolated dextroversion of the heart with asymmetric septal hypertrophy. 13 88

This study was aimed to evaluate the noninvasive method of radioisotopic angiocardiography for detecting a disproportionate thickning of the interventricular septum relative to the left ventricular free wall in the patient with idiopathic hypertrophic cardiomyopathy. The experimental study was performed to compare the imaged size of myocardial wall obtained my filling up with technetium 99 m labeled sponge in biventricular cavities, with the actually measured thickness of the heart specimen. The resultant correlation between actual and imaged size of the left ventricular wall was excellent (r = 0.95). In the clinical application of this method, a gated cardiac blood pool scan was performed after the intravenous administration of technetium 99 m labeled human serum albumin in a dose of 10 mCi. The cardiac images were obtained in anterior, 30, 35, 40 and 45 degree left anterior oblique projection, and evaluated by visual inspection and semi-quantitatively by tracing the myocardial borders. As a result, the imaged asymmetric septal hypertrophy was evident in all four patients with PMD, with a ratio of septum to left ventricular free wall of 1.35 compared to 0.76 in five patients with left ventricular hypertrophy of known heart disease, and 0.88 in twenty-one miscellaneous cardiac patients without left ventricular hypertrophy. Thus, RI angiocardiography provides a safe, repeatable method for detection and evaluation of Primary Myocardial Disease.
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PMID:[RI angiocardiography of idiopathic cardiomyopathy (author's transl)]. 13 76

Human cardiac myosin isolated from operatively obtained samples of ventricular septum and left ventricular free wall of subjects with asymmetric septal hypertrophy (ASH) was compared, with respect to structural and enzymatic properties, to myosin isolated from hearts of subjects without heart disease. The following parameters were studied: (1) activation of myosin ATPase activity by K+-EDTA and Ca2+, (2) molecular weight of the heavy and light chains of myosin as determined by electrophoretic migration in polyacrylamide-sodium dodecyl sulfate (SDS) gels and (3) ability to form bipolar aggregates at low ionic strength, as examined by electron microscopy. No difference was present in any of these parameters between human cardiac myosin from subjects with ASH and from subjects without heart disease. Thus, the genetic defect present in subjects with ASH is not expressed in the particular structural and functional characteristics of myosin evaluated in this study.
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PMID:Isolation and characterization of myosin from subjects with asymmetric septal hypertrophy. 14 25

To assess the validity of the quantitative 201Tl scintimetry in various diseases of the heart (coronary heart disease with and without myocardial infarction, non-coronary cardiomyopathy, scleroderma heart disease and asymmetric septal hypertrophy with IHSS), the 201Tl myocardial uptake values for five standardized projections (a) were correlated with the grade of LAD stenosis, (b) the pattern of myocardial wall motion and (c) were compared with the 201Tl uptake values derived from normal patients. Significant reduction (c) of 201Tl myocardial uptake could in individual cases be evaluated in acute myocardial infarction (95%), in dys- and akinesia (90%), in hypokinesia (71%), in scleroderma heart disease (50%), in non-coronary cardiomyopathy (50%) as well as in normokinesia (28%) when associated with LAD stenosis. The mean values (b) of 201Tl uptake in normo- and hypokinesia significantly differed between these two groups and from those evaluated in dys- and akinesia. The latter group showed the lowest 201Tl uptake values computed which in some cases were very close to the mean mediastinal 201Tl uptake. The correlation (a) of individual 201Tl values demonstrated that 201Tl distribution in the myocardium is not only equivalent to myocardial ""perfusion'' but is corresponding with the myocardial function. In non-coronary cardiomyopathy reduced 201Tl values sometimes could not be separated from values in coronary heart disease (and myocardial infarction). A regional increase of myocardial mass as in septal hypertrophy correlated well with an augmented 201Tl uptake when referred to the 201Tl storage in the mediastinum.
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PMID:Evaluation of myocardial function with the 201thallium scintimetry in various diseases of the heart. A correlative study based on 100 patients. 14 51

Human cardiac myosin isolated from operatively obtained samples of ventricular septum and left ventricular free wall of patients with asymmetric septal hypertrophy (ASH) was compared, with respect to structural and enzymatic properties, to myosin isolated from hearts of patients without heart disease. The following parameters were studied: 1) activation of myosin ATPase activity by K+-EDTA and Ca2+,2) molecular weight of the heavy and light chains of myosin as determined by electrophoretic migration in SDS-polyacrylamide gels, and 3) ability to form bipolar aggregates at low ionic strength, as examined by electron microscopy. No difference was present in any of these parameters between human cardiac myosin from patients with ASH and from patients without heart disease. Thus, the genetic defect present in patients with ASH is not expressed in the particular structural and functional characteristics of myosin evaluated in this study.
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PMID:Characterization of myosin from patients with asymmetric septal hypertrophy. 14 40

Necropsy studies were performed in 125 infants, 2 years old or less, with a variety of congenital heart malformations. Disproportionate ventricular septal thickening (septal-to-left ventricular free wall thickness ratio greater than or equal to 1.3) was present in 31 (25%) of the 125 patients. In the majority of patients, an abnormal septal-to-free wall ratio did not appear to be a manifestation of genetically transmitted hypertrophic cardiomyopathy, since: 1) marked disorganization of septal myocardium (involving greater than or equal to 5% of the tissue section) was present in only five of the 31 patients with disproportionate septal thickening; and 2) echocardiographic studies in first degree relatives of four other patients with disproportionate septal thickening and normal septal architecture did not show asymmetric septal hypertrophy. The relatively high prevalence of septal-to-free wall ratios of 1.3 or greater in this population of infants with congenital heart disease appeared to be due in part to minor differerences in ventricular wall thicknesses (in patients with relatively thin absolute wall thicknesses) which may produce particularly large deviations from unity in septal-to-free wall ratio. Hence, 1) nongenetically transmitted disproportionate septal thickening is relatively common in infants with congenital heart diseases studied at necropsy: and 2) a septal-to-free wall ratio of greater than or equal to 1.3 cannot be used as the sole criterion for identifying associated hypertrophic cardiomyopathy in infants with other congenital heart diseases, particularly if marked absolute septal thickening is absent.
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PMID:Prevalence and characteristics of disporportionate ventricular septal thickening in infants with congenital heart disease. 15 69

Echocardiography was used to compare the left ventricular function in 35 cases of congestive cardiomyopathy (COCM), 16 cases of obstructive and 28 cases of non-obstructive asymmetric septal hypertrophy, 43 cases of scleroderma heart disease, 21 cases of ischaemic cardiomyopathy (ICM), 2 cases of restrictive cardiomyopathy, and one case of cardiac amyloidosis. COCM was characterized by left ventricular dilatation, decreased contractility and signs of elevated end-diastolic pressure. In asymmetric septal hypertrophy there was a decreased distensibility of the obstructed left ventricle, both in early and end-diastole, but the pump function remained normal. In scleroderma and amyloid heart disease both the contractility and distensibility of the small, stiff heart were reduced. In restrictive cardiomyopathy the only abnormality was the impaired end-diastolic distensibility. Reduced contractility confined primarily to the interventricular septum and impaired early diastolic distensibility are the characteristic features distinguishing ischaemic cardiomyopathy from COCM. However, echocardiography is not suitable for individual differentiation of ICM and COCM. The COCM can be differentiated from left ventricular aneurysm by M-mode sector scanning technique.
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PMID:Functional differentiation of various types of cardiomyopathies by echocardiography. 15 92

The value of axial cineangiography in several forms of congenital heart disease serves as an illustrated supplement to Section I. These techniques visualize defects in the entire ventricular and atrial septum. In persistent atrioventricular (A-V) canal, it is possible to visualize all parts of both septa, status of the A-V valves (two valves versus a common A-V valve), and if a common A-V valve, its degree of override. In tetralogy of Fallot, the bifurcation of the pulmonary trunk, entire ventricular septum and coronary arteries are vividly shown. The presence of true and confluent pulmonary arteries versus systemic or bronchial arteries in pseudotruncus is clearcut. In double outlet right ventricle or in transpositions with or without double outlet right ventricle, the mitral valve-semilunar valve relationships, the left ventricular outflow tract, subpulmonary region and a straddling tricuspid valve are well demonstrated. In asymmetric septal hypertrophy, biventricular angiography may be circumvented.
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PMID:Axial cineangiography in congenital heart disease. Section II. Specific lesions. 56 26


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