Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P50583 (asymmetrical)
 12,197 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Four patients with Fabry's disease diagnosed by right ventricular endomyocardial biopsy had cardiac manifestations simulating hypertrophic cardiomyopathy (HCM). Case 1: A 51-year-old woman, whose elder sister had congestive heart failure, was hospitalized for exertional dyspnea and cardiomegaly. Her electrocardiogram (ECG) showed a short PQ interval (0.10 sec) and left ventricular hypertrophy. Her echocardiogram (Echo) showed moderate symmetrical hypertrophy of the left ventricle (IVST/PWT = 18 mm/17 mm). Case 2: A 32-year-old woman, whose elder sister had an abnormal ECG, was hospitalized for the ECG abnormalities consisting of a short PQ interval (0.10 sec) and ST-T changes in the left precordial leads. The Echo revealed mild symmetrical hypertrophy of the left ventricle (IVST = 13 mm, PWT = 13 mm). Case 3: A 44-year-old man was hospitalized for his ECG suggestive of left ventricular hypertrophy, and his Echo showed asymmetrical septal hypertrophy (ASH; IVST = 22 mm). Case 4: A 51-year-old man was hospitalized for his ECG showing high voltage in the left precordial leads, and his Echo showed ASH (IVST = 20 mm). The cardiac histopathological findings of these cases included cytoplasmic vacuolization by light microscopy, and electron-dense deposits consisting of parallel or concentric lamellae with periodic spacing, suggesting Fabry's disease. The urinary glycolipids of Case 1 were increased biochemically; then the diagnosis of Fabry's disease was confirmed. Cardiac hypertrophy in Fabry's disease has many aspects, because the histopathological changes and clinical manifestations are determined by genetic factors. It was concluded that Fabry's disease may be concealed in some patients with the clinical diagnosis of HCM.
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PMID:[Four cases of Fabry's disease mimicking hypertrophic cardiomyopathy]. 297 98

The value of echo-cardiography, CT and magnetic resonance tomography was evaluated in 10 patients with hypertrophic cardiomyopathy. Echocardiography made the diagnosis in most cases. The diagnostic criteria depended on functional and morphological changes. CT and magnetic resonance can show thickening in the myocardium, either symmetrical or asymmetrical. Magnetic resonance tomography provides excellent demonstration of the myocardium and accurate delineation of the degree of hypertrophy. It is well suited for measuring cardiac wall thickness.
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PMID:[Computed tomography, magnetic resonance tomography and echocardiography in hypertrophic cardiomyopathy]. 298 61

Gated magnetic resonance tomography (MRT) was conducted in 40 patients (13 normal volunteers, 9 hypertensives and 18 patients with hypertrophic cardiomyopathy) using a 0.35 Tesla superconducting magnet. Multisectional spin echo imaging (35/400 msec) was obtained in coronal, transversal and sagittal planes. Myocardial wall thickness was measured in different segments and the three groups were compared to each other. 15/18 patients with hypertrophic cardiomyopathy (HCM) had asymmetrical regional thickening involving the septum and the anterior wall, in 8/15 the lateral wall was also hypertrophic. The distribution pattern in 3/15 patients with HCM was symmetric. Involvement of the right ventricle was found in 14/18 patients with HCM. There were significant differences (p less than 0.001) in wall thickness for the septal segment in all three groups and for the ratio septal to posterior wall between the HCM and the hypertensives and the normal volunteers. We conclude that MRT can differentiate HCM from hypertensives and normals, and is superior to echocardiographic imaging in the evaluation of the distribution of left ventricular hypertrophy in hypertrophic cardiomyopathy.
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PMID:[Detection of regional hypertrophy in hypertrophic cardiomyopathy with ECG triggered nuclear magnetic resonance tomography--comparison with hypertensive patients and persons with healthy hearts]. 315 27

A study of the extent and local distribution of the heart muscle hypertrophy affecting the left ventricle and the septum has been performed in 70 hypertrophic cardiomyopathy patients. According to these signs the patients have been distributed into 5 types. Type I, where the hypertrophy affected only the interventricular septum, appeared in 14.2% of the cases. In type IIa the anterior wall of the ventricle was simultaneously affected (37.1%), in type IIb it was also the lateral wall of the ventricle (30.0%). Type III manifested hypertrophy of the posterior wall (4.3%), type IV affected the apical portion of the left ventricle (8.5% of the patients). Under the heading of type V there figured patients who had a concentric hypertrophy of the whole left ventricle including the septum (5.7%). Among the obstructive forms we revealed especially the types Ia, IIa, IIb. Using the M-mode alone, detection of the hypertrophy was impossible in such patients who had isolated affection of the dorsal part of the septum, the anterolateral or the apical portion of the left ventricle (type Ib, IV and some of the type IIa and IIb patients--a total of 13%). Signs of asymmetrical septal hypertrophy were lacking in 23% of the patients.
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PMID:Ultrasound characteristics of myocardial hypertrophy in patients affected by hypertrophic cardiomyopathy. 315 98

A dipyridamole-induced Tl-201 perfusion abnormality was evaluated from its clinical features, echocardiography and myocardial histopathology in 39 patients with hypertrophic cardiomyopathy (HCM). From the findings of Tl-201 emission computed tomography (ECT), subjects were divided into three groups: group 1 (n = 16) which did not show a perfusion abnormality in the hypertrophic region; group 2 (n = 12) which showed a perfusion defect on the initial image with complete redistribution on the delayed image; and group 3 (n = 11) which showed a persistent perfusion defect--this group included most patients who revealed partial and/or incomplete redistribution. Echocardiography revealed that group 2 showed a marked asymmetrical septal hypertrophy and an incidental obstructive pattern, and that group 3 had a significantly dilated left ventricular diastolic dimension and a decreased percentage of fractional shortening. Group 3 also showed frequent ventricular tachycardia and a familial history of cardiomyopathy. As for the myocardial biopsy findings, group 3 had significantly advanced myocardial fibrosis, the percentage being 6.0 +/- 3.1% in group 1; 5.5 +/- 2.5% in group 2; and 11.9 +/- 3.4% in group 3. Thus, it was concluded that the persistent perfusion defect on dipyridamole stress Tl-201 ECT testing is an important finding corresponding to the advanced clinical and pathological aspects of HCM.
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PMID:Clinical significance of dipyridamole Tl-201 emission computed tomography perfusion abnormality for evaluating pathophysiological and pathological aspects in hypertrophic cardiomyopathy. 325 28

The effect of hypertension on asymmetrical septal hypertrophy was studied by echocardiography to differentiate idiopathic asymmetrical septal hypertrophy (ASH) from ASH with hypertension. One hundred eight patients with ASH proven by echocardiography were categorized in two groups; 53 patients with hypertension (greater than 160 systolic, greater than 95 diastolic) (hypertensive group: HT) and 55 patients with normal blood pressure (normotensive group: NT). Septal hypertrophy was classified as mid-portion (M-type), diffuse (D-type), and basal (B-type) hypertrophy by the long-axis view, and also diffuse (I-type), anterolateral (II-type), anteroseptal (III-type), and anterior septal (IV-type) by the short-axis view, respectively. Endomyocardial biopsy and left ventriculography were performed in 50 patients (18 hypertensives and 32 normotensives). In the hypertensive group, 45%, 30%, and 25% of cases had diffuse, basal and mid-portion hypertrophy, respectively. There was no case in the basal hypertrophy whose biopsy findings were compatible with hypertrophic cardiomyopathy. In the normotensive group, 78% and 22% of patients had midportion and diffuse hypertrophy, respectively, but none of them had the basal hypertrophy. Type IV was seen in only six patients in the normotensive group.
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PMID:[Effect of hypertension on asymmetrical septal hypertrophy: an echocardiographic study]. 326 13

The mother and three children of a family whose parents were consanguineous, each had cardiomyopathy with various patterns of hypertrophy and dilatation. All members had asymmetrical septal hypertrophy (ASH), and three of them were characterized as hypertrophic cardiomyopathy (HCM). Another one had ventricular dilatation mimicking dilated cardiomyopathy (DCM). Case 1: The 57-year-old mother had a typical ASH pattern; her septal/posterior wall thickness ratio (IVST/LVPWT) was 2.5. Case 2: The 37-year-old daughter had basal septal hypertrophy. Case 3: The 32-year-old elder son had typical concentric hypertrophy. Case 4: The 30-year-old younger son had an episode of congestive heart failure, and showed DCM-like features with considerable dilatation and impaired wall motion of the left ventricle. The hypertrophic pattern in cardiomyopathies is thought to depend partially on the ages of the onset, or its evolution with aging.
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PMID:[Familial cardiomyopathy with different clinical features in individual members]. 350 15

A case of severe asymmetrical hypertrophic cardiomyopathy occurring in a 11 month-old infant presenting with adrenocortical adenoma is reported. Cardiac involvement, as shown by echocardiography, recovered after complete excision of the tumor. Despite few published cases, the etiology of the associated cardiomyopathy is discussed.
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PMID:[Severe hypertrophic cardiomyopathy associated with an adrenocortical adenoma]. 354 24

The clinical and echocardiographic features of cardiac amyloidosis may closely resemble those of hypertrophic cardiomyopathy, and the disorders may thus be mixed up. The present study was undertaken in an attempt to identify features separating the two conditions by analysis of electro- and echocardiographic findings in patients with familial amyloid polyneuropathy and hypertrophic cardiomyopathy. Twenty-nine patients with familial amyloidosis and 22 with hypertrophic cardiomyopathy were studied. Particular attention was given to the sum of the S wave in V1 and R wave in V5 or V6, the echocardiographic left ventricular mass and cross-sectional area, the presence or absence of asymmetrical septal thickening, granular and sparkling myocardial appearance, thickened heart valves, systolic anterior motion of the mitral valve, and pericardial effusion. A granular and sparkling appearance of the myocardium and thickened heart valves were found to be the best predictors of cardiac amyloidosis, while low QRS amplitudes in relation to echocardiographic left ventricular mass and a pericardial effusion seemed less important. The presence of systolic anterior movement of the mitral valve, a large left ventricular mass and a sum of S in V1 and R in V5 or V6 greater than 35 mm indicated hypertrophic cardiomyopathy. When the four strongest predictors (left ventricular mass, thickened heart valves, a granular sparkling myocardial appearance, and systolic anterior movement of the mitral valve) were used to reclassify the present patients, 28 of 29 amyloidosis patients and 21 of 22 patients with hypertrophic cardiomyopathy were correctly categorized. Noninvasive methods may thus be useful for detecting the myocardial infiltrative process, and cardiac amyloidosis may be confidently diagnosed by typical noninvasive findings together with histopathological documentation of amyloid in an organ other than the heart.
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PMID:Differentiation of cardiac amyloidosis and hypertrophic cardiomyopathy. A comparison of familial amyloidosis with polyneuropathy and hypertrophic cardiomyopathy by electrocardiography and echocardiography. 356 84

To clarify the correlation between the configuration of the left ventriculogram and serial ECG changes, 16 patients with hypertrophic cardiomyopathy (HCM) associated with asymmetrical septal hypertrophy were examined. In the right oblique view at end-diastole, the configurations were classified by form as round (R, n = 7), round with inferior concavity (R-i, n = 2), spade (S, n = 4) and spade with inferior concavity (S-i, n = 3). These patients were divided into two groups according to serial T wave changes; nine with marked changes (A group) and seven without (B group). Furthermore, group A was separated into two subgroups; seven with increasing negativity or appearance of the negative T wave (A-1 group) and two with decreasing negativity or disappearance of the negative T wave (A-2 group). The results were as follows: Five (71%) of the seven cases with the S and S-i form belonged to the A group. Their apical walls showed marked hypertrophy and their ECGs showed deep negative T waves. The other two cases (29%) belonged to the B group, and did not show marked apical hypertrophy. Four (44%) of the nine cases with the R and R-i form belonged to the A group. They showed mild apical hypertrophy, and initially did not show deep negative T waves. A deep negative T wave appeared in three during observation. The initial depth of the maximum negativity of T wave correlated significantly with apical wall thickness, SV1 + RV5, and the total depth of the negative T wave in precordial leads. During the observation, the A-1 group showed a marked increase of SV1 + RV5. The A-2 group showed a decrease of SV1 + RV5. In conclusion, HCM with deep negative T waves has a tendency to present wide changes in the T wave during serial ECG observation and to show apical hypertrophy on left ventriculography. Cases of increasing negativity of the T wave showed marked increase in voltage of SV1 + RV5. However, cases of decreasing negativity of the T wave showed decreasing SV1 + RV5. These ECG changes, especially the negative T wave changes are reputed to be related to apical wall thickness.
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PMID:[Left ventriculography and serial ECG changes in hypertrophic cardiomyopathy with special reference to the negative T wave]. 358 66


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