Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0205700 (ash)
 15,125 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Echocardiograms were performed in thirty-six patients (aged 4 to 36 years) with proven coarctation of the aorta. Nineteen patients (53%) were found to have marked diastolic eccentricities of their aortic valves (Eccentricity Index greater 1.5), indicating the presence of bicuspid aortic valves. One of these patients also had multilayered aortic root echoes in diastole. Five patients had angiographic proof of their aortic valve morphologies which corroborated the echo findings. Five patients with bicuspid aortic valves showed mitral valve diastolic flutter indicative of aortic regurgitation. Idiopathic hypertrophic subaortic stenosis (IHSS) was suspected in four patients (11%) with abnormal systolic anterior motion of the mitral valve; three of these patients also had asymmetric septal hypertrophy. There was catheterization proof of IHSS in one patient. Two patients (5.6%) demonstrated mitral valve proplapse.
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PMID:Echocardiographic studies of abnormalities associated with coarctation of the aorta. 13 58

Hypertrophic cardiomyopathy (HCM) or idiopathic hypertrophic subaortic stenosis (IHSS) has been defined as an autosomal dominant myocardial disease characterized by specific physical findings, echocardiographic features, asymmetric septal hypertrophy and disordered myocardial architecture. Echocardiographic and scintiphotographic studies failed to reveal evidence of asymmetric septal hypertrophy in four patients with systolic anterior movement of the mitral valve and the typical ausculatory and peripheral pulse abnormalities characteristic of HCM. Postmortem examimination in one patient demonstrated mild concentric left ventricular hypertrophy and a normal arrangement of myocardial muscle fibers. These observations in four patients demonstrate that both systolic anterior movement of the mitral valve and the physical findings characteristic of HCM are not specific for the autosomal dominant myocardial disease characterized by asymmetric septal hypertrophy and abnormal septal histology.
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PMID:Hypercontractile cardiac states simulating hypertrophic cardiomyopathy. 14 30

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

The specificity and significance of the asymmetric septal hypertrophy (ASH) and myocardial fiber disarray of idiopathic hypertrophic subaortic stenosis (IHSS) is uncertain. To examine this we studied 215 hearts, including normal embryos, fetuses, children, and adults; and hearts with congenital and acquired disease. Disproportionate septal thickening was present in all embryos and in some abnormal hearts, particularly those with severe right ventricular hypertrophy due to congenital malformations. Some myocardial fiber disarray was present in all hearts at the junctions of interventricular septum and ventricular free wall. In hearts with semilunar valve atresia with intact ventricular septums, and in the infundibulum of some with tetralogy of Fallot, however, extensive fiber disarray was present. Thus, ASH occurs in the normal developing heart and in some malformed hearts with RVH; marked muscle fiber disarray may occur in certain congenital lesions with abnormal systolic contraction. Neither morphologic finding independently or in combination is pathognomonic of idiopathic hypertrophic subaortic stenosis.
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PMID:Asymmetric septal hypertrophy and myocardial fiber disarray. Features of normal, developing, and malformed hearts. 14 48

Asymmetric septal hypertrophy (ASH) is considered the unifying link in the spectrum of hypertrophic cardiomyopathies. ASH and mitral valve systolic anterior motion (SAM) are the two most important echocardiographic criteria for the diagnosis of idiopathic hypertrophic subaortic stenosis (IHSS). Ten patients found have SAM without ASH were studied. Septal thickness, thickening, and excursion were normal. Seven patients had left ventricular ejection times (LVET) measured before and after amyl nitrite inhalation. In six of them, corrected LVET increased by more than 40 msec. Four patients underwent diagnostic cardiac catheterization. Resting or provocable left ventricular outflow tract (LVOT) gradients were demonstrable in all four patients. The echocardiographic features in patients with SAM alone, ASH and SAM, and ASH alone were compared. compared. LVOT measurements in patients with SAM alone (2.2 +/- .4 cm) and ASH and SAM (2.1 +/- .5 cm) were similar and narrower than in patients with ASH alone (2.8 +/- .5 cm, P less than 0.001). Ejection fractions in patients with SAM alone (79 +/- 10%) were greater than in patients with ASH and SAM (66 +/- 16%, P less than 0.02) or ASH alone (60 +/- 15%, P less than 0.001). Thus, dynamic left ventricular outflow obstruction can exist in the absence of echocardiographic ASH. LVOT width and abnormal LV ejection dynamics may contribute to the LVOT obstruction with or without the presence of ASH.
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PMID:Systolic anterior motion of the mitral valve in the absence of asymmetric septal hypertrophy. 14 28

Typical SAM of the mitral valve was detected by echocardiography in a 71-year-old patient with acute myocardial infarction. The patient showed auscultation signs of IHSS and was digitalized and hypovolemic secondary to diarrhea. Following rehydration and digitalis being discontinued, SAM was no longer seen. There were no signs of IHSS at post-mortem examination. This case demonstrates that obstruction of left ventricular outflow tract together with echocardiographical findings of SAM and the typical clinical signs of IHSSS may be observed without the presence of asymmetric septal hypertrophy. This supports the hypothesis that ejection dynamics in a small and asynergically contracting left ventricle may contribute substantially to the appearance of SAM. On the other hand echocardiographic findings of SAM without asymmetric septal hypertrophy may not exclude symptoms of IHSS.
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PMID:[Transient systolic anterior movement of the mitral valve (SAM) without asymmetric septal hypertrophy (case report with post-mortem examination) (author's transl)]. 15 83

The echocardiogram of a patient presenting with a systolic ejection murmur and ECG evidence of left ventricular hypertrophy revealed asymmetric septal hypertrophy but no abnormal systolic anterior motion of the mitral valve as in IHSS. Her pulmonary echo disclosed small A waves, partial valve closure in early systole and coarse systolic fluttering. This motion pattern--which is similar to that of patients with infundibular pulmonary stenosis and which resembles the aortic valve motion pattern in discrete subaortic stenosis--led us to the diagnosis of hypertrophic cardiomyopathy with subpulmonic obstruction, which was later confirmed by cardiac catheterization.
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PMID:[Echocardiographic diagnosis of subpulmonic obstruction in hypertrophic cardiomyopathy (a case report) (author's transl)]. 15 93

Septal and left ventricular posterior wall (LVPW) thicknesses and their ratios were studied at the left ventricular outflow tract and left ventricular cavity in 66 patients with echocardiographically diagnosed left ventricular concentric hypertrophy, 20 with idiopathic hypertrophic subaortic stenosis (IHSS), and 34 normal subjects. Concentric hypertrophy was due to hypertension in 41 subjects and to valvular disease in 15 subjects. Septal thickness in normal subjects was related to body surface area (p less than 0.02). In 12% of normal subjects, 39% of patients with concentric hypertrophy and 95% with IHSS, the septal/LVPW ratio was greater than or equal to 1.3. Thirty-two percent of patients with hypertension, 78% with aortic stenosis, and 60% with aortic insufficiency had septal/LVPW ratios greater than or equal to 1.3 at left ventricular midcavity level. In conclusion, a septal/LVPW thickness ratio of greater than or equal to 1.3 is common in patients with concentric left ventricular hypertrophy and may also occur in normal subjects. A ratio greater than or equal to 1.5 may be more specific for genetically determined asymmetric septal hypertrophy.
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PMID:Interventricular septal thickness and left ventricular hypertrophy. An echocardiographic study. 15 45

45 children with echocardiographically proven mitral valve prolapse are the basis to give a review about this syndrome (MVPS). Symptoms in this syndrome, like palpitations and fatigue are rare. Only half of the patients show the typical auscultatory findings: the midsystolic click and the late systolic murmur. The ECG abnormalities are disturbances of rhythm, left ventricular hypertrophy and left ventricular T-wave inversion. The hemodynamic is mostly normal or depends upon the degree of the mitral insufficiency. The angiocardiograms show often abnormalities of the contraction of the left ventricle. Echocardiographically one ought to obtain the pattern of the mid-systolic backward motion of the leaflet. As additional lesions we found in 11% of the patients with MVPS an ASD, in 16% an ASH or IHSS of different degrees corresponding to the echocardiographical sign of the ASH. The pathogenetic ideas of the valvular and myocardial theory are discussed. The prognosis is good. Complications are disturbances of rhythm, bacterial endocarditis and in case of a chordal rupture a mitral regurgitation.
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PMID:[The mitral valve prolapse syndrome in childhood (author's transl)]. 55 79

Previous studies have shown that asymmetric septal hypertrophy (ASH) may occur in embryonic and malformed hearts and that muscle cell disarray is found in myocardium that contracts isometrically. To account for the characteristic ASH and septal muscle fiber disarray of idiopathic hypertrophic subaortic stenosis (IHSS), we postulated that a catenoid shape of the septum, i.e., net zero curvature, would have the mechanics required to produce IHSS. Accordingly, hearts from eight autopsied patients with IHSS were studied for curvature and thickness of free walls and septum, and they were compared to similar measurements in 80 other hearts. In all eight hearts with IHSS the septum was concave to the left in the transverse plane but convex to the left in the apex-to-base plane. Such a catenoid configuration of the septum was not observed in any of the other 80 hearts. The distinctive shape of the septum in IHSS would account for isometric contraction, since adjacent fiber tracts with opposite curvatures would develop maximum tension but would not have motion. Fiber disarray and local hypertrophy would result from such isometric contraction. Since ventricular configuration is acquired early in cardiogenesis, IHSS might therefore result from a genetic or embryonic determination of a catenoid septum.
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PMID:Catenoid shape of the interventricular septum: possible cause of idiopathic hypertrophic subaortic stenosis. 56 3


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