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)

In a heart with hypertrophic cardiomyopathy without asymmetrical septal hypertrophy the number of transmural myocytes, the mean size of myocytes, and the percentage area of interstitial space were similar in the ventricular septum and left ventricular posterior wall, whereas in a reported series of 14 hearts with hypertrophic cardiomyopathy with asymmetrical septal hypertrophy the number of transmural myocytes was greater in the ventricular septum than in the left ventricular posterior wall. In hearts with hypertrophic cardiomyopathy without asymmetrical septal hypertrophy the mean size of myocytes was significantly greater than that of normal hearts, but the number of transmural myocytes was not increased. The extent and distribution pattern of myocardial fibre disarray and fibrosis in the left ventricle were similar in hearts with hypertrophic myopathy whether or not asymmetrical septal hypertrophy was present.
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PMID:Pathological features of hypertrophic cardiomyopathy without asymmetrical septal hypertrophy. 375 46

This is a report of a patient with mitral valve prolapse (MVP) and myocardial abnormalities on endomyocardial biopsy in whose relatives hypertrophic cardiomyopathy (HCM) was identified. A 19-year-old woman was admitted to our hospital for evaluation of a heart murmur. A systolic ejection murmur was audible in the third intercostal space at the left sternal border, and a standard 12-lead electrocardiogram showed ST-T wave changes in leads II, III and aVF. Echocardiography revealed prolapse of the anterior leaflet of the mitral valve, but no left ventricular hypertrophy. Endomyocardial biopsy disclosed mild hypertrophy and disarrangement of the myocardium. The family study revealed asymmetrical septal hypertrophy in her mother, who had no history of hypertension. Her younger sister had mild hypertrophy of the interventricular septum on echocardiography, and her histopathological findings suggested a diagnosis of HCM. This case was clinically regarded as MVP, but development of left ventricular hypertrophy as noted in her mother may occur in the future.
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PMID:[Mitral valve prolapse with myocardial disarrangement and familial hypertrophic cardiomyopathy: a case report]. 378 85

The authors report the clinical, ECG, PCG and echocardiography data obtained in mountaineers suffering from associated essential hypertension and high-altitude pulmonary hypertension. Demonstrate the advisability of distinguishing the high-altitude hypertrophic cardiomyopathy syndrome (HHCS) in part of mountaineers with essential hypertension living permanently at an altitude of 3600-4200 m over the sea level. The HHCS is marked by a lot of the clinical and echocardiographic signs which are regarded as characteristic of hypertrophic cardiomyopathy, particularly by appreciable asymmetrical hypertrophy of the interventricular septum. Criteria for the differential diagnosis between the HHCS and idiopathic hypertrophic subaortic stenosis are suggested. The possible mechanisms by which the HHCS develops in part of mountaineers are discussed.
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PMID:[Syndrome of high-altitude hypertrophic cardiomyopathy]. 404 Feb 72

To clarify the pathogenesis of apical hypertrophy with asymmetrical septal hypertrophy (ASH), left ventriculography in the right anterior oblique projection (LVG), biventriculography (BVG), and endomyocardial biopsy of the right ventricle were performed for patients with ASH. The patients were categorized in four groups according to ECG, LVG and BVG. Patients with hypertrophic cardiomyopathy (HCM) were divided into two subsets; (A) Apical hypertrophy group (AH: nine patients), with ECG showing left ventricular hypertrophy (LVH) and giant negative T waves (GNT), and with LV configurations showing the S or SR form at end-diastole on LVG. (B) Non-apical hypertrophy group (non-AH: 12 patients), with ECG showing LVH without GNT and LV configuration showing R form at end-diastole on LVG (cf: Fig. 1). Patients with ASH and hypertension (ASH-HT) were also divided into two subsets; (A) AH: seven patients. (B) non-AH: nine patients. Analysis of LVG and BVG: In HCM, the septal configuration showed the TS X S form in both two subset groups. The septal configuration in ASH-HT was divided into the NH form, which was clearly distinguishable from the septal configuration in HCM, and the TS X S form as in cases with HCM. In both HCM and ASH-HT, the diastolic thickness of the anterior apical wall was significantly thicker in all patients with AH than that in non AH. In HCM, the diastolic thickness of the septum and the percent systolic thickening did not significantly differ between AH and non-AH groups. In ASH-HT, the NH form showed similar diastolic thickness of the septum and % systolic thickening in AH and non-AH groups. On the other hand, the TS X S form in non-AH group showed greater thickness and lower % systolic thickening similar to those of HCM. Histological analysis of endomyocardial biopsy; In HCM, the transverse diameters of the myocytes and the biopsy scores did not differ significantly between AH and non-AH groups. In ASH-HT, the TS X S form in non-AH group had longer diameters and higher biopsy scores similar to those of HCM compared to the NH form in AH group. In conclusion, both HCM and ASH-HT may have apical hypertrophy manifested by giant negative T waves in the EKG and spade like form of left ventriculogram. In addition, apical hypertrophy in ASH-HT with the NH form of septal configuration seemed to be caused by hypertension.
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PMID:[Biventriculographic and clinicopathologic evaluation of apical hypertrophy: with reference to asymmetrical septal hypertrophy with hypertension]. 409 18

The mode of right ventricular hypertrophy was assessed by two-dimensional echocardiography (2DE) for 24 patients with hypertrophic cardiomyopathy (HCM), and the results were compared with those of 51 patients with hypertension (HT). The patients with HT were categorized in four groups depending on the thickness of the interventricular septum (IVST) and left ventricular posterior wall (PWT): HT-ASH with both left ventricular hypertrophy (LVH) (IVST greater than or equal to 13 mm) and asymmetric septal hypertrophy (ASH) (IVST/PWT greater than or equal to 1.3), severe HT with LVH and without ASH, and mild HT without LVH and ASH. Anterior wall thickness (AWT), posterior wall thickness (PWT), and diaphragmatic wall thickness (DWT) of the right ventricle were obtained from 2DE in the parasternal long-axis view, the short-axis view and subxiphoid view, respectively. These were recorded on video tape, and the measurements were made on the stop frames. Right ventricular hypertrophy (RVH) was estimated by the maximal right ventricular wall thickness (max RVWT), and the ratio of the maximal and minimal thickness (max RVWT/min RVWT) was calculated to evaluate asymmetrical hypertrophy (AH) of the right ventricle (RV). The incidence of RVH (Max RVWT greater than or equal to 5 mm) and asymmetrical hypertrophy (AH) (max RVWT/min RVWT greater than or equal to 1.3) of the RV in HCM, HT-ASH and mild HT were 67% and 41%, 57% and 45%, and 15% and 11%, respectively. The incidence of RVH with AH was more frequent in patients with HCM as well as HT with ASH than in patients with HT without ASH.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Regional right ventricular hypertrophy in hypertrophic cardiomyopathy and hypertension]. 409 19

This study aims to evaluate and compare the current pathological criteria for the diagnosis of hypertrophic cardiomyopathy (HOCM). The following criteria were applied to 39 autopsy patients whose hearts showed myofibre disarray: (i) disarray involving more than 5% of ventricular septal myofibres, (ii) asymmetrical septal hypertrophy (ASH), (iii) mirror-image subaortic plaque, and (iv) a positive histologic HOCM index (HHI). Group 1 (27 patients) with greater than 5% ventricular septal myofibre disarray were diagnosed as HCM, whereas group 2 (12 patients) had less than 5% disarray. The mean disarray value in group 1 was 52% compared to 1.2% in group 2. The two groups showed significant differences regarding ASH and in the amounts of myofibre disarray in the free walls of both ventricles. A new finding was that the histologic HOCM index was significantly higher in patients who died suddenly. The HHI was the commonest positive criterion in group 1, followed by ASH and a mirror-image endocardial plaque. None of the current imperfect pathological criteria for the diagnosis of HCM can be used as the 'gold standard'.
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PMID:Evaluation of pathological criteria for diagnosis of hypertrophic cardiomyopathy. 653 46

To clarify clinically the pathogenesis of septal hypertrophy in terms of its morphology and wall dynamics, simultaneous biventricular cineangiography (BVG) and endomyocardial biopsy of the left ventricle were performed for patients with left ventricular hypertrophy. The patients were categorized in four groups according to their clinical and BVG findings; 1) 24 normal control subjects (C), 2) 39 patients with hypertension and symmetrical septal hypertrophy (SH-HT), 3) 17 patients with hypertension and asymmetrical septal hypertrophy characterized by the ratio (IVS/PW) greater than or equal to 1.3 by BVG, and 4) 25 patients with hypertrophic cardiomyopathy (HCM). The BVG's configuration of the septum in SH-HT showed a normal form of septal hypertrophy (NH form) essentially similar to that of the control subjects (N form), except for septal thickness increases of more than 10 mm. In HCM, a triangle or shell (TS) form or spindle (S) form of the septum was demonstrated by BVG. The TS form was characterized by straight or convex thickening of the septum from its center to its base toward the left ventricle. The S form was characterized by convex thickening localized to the central part and a relatively thin base. However, the configuration in ASH-HT was either the NH form (7 cases) or the TS or S form (10 cases). The configuration in ASH-HT with the NH form was not distinguishable from that of SH-HT, nor was the configuration in ASH-HT with the TS or S form distinguishable from that of HCM. Systolic thickening (%) of the septum and systolic shortening (%) of the septal length decreased significantly in this order: C, SH-HT, ASH-HT with the NH form, and ASH-HT with the TS or S form. Those of HCM were similar to those of ASH-HT with the TS or S form. In the cases of the N or NH form, there was a negative correlation between systolic thickening (%) and thickness of the septum in diastole. In cases with the TS or S form, systolic thickening (%) was not affected by the thickness of the septum in diastole, and a lower value was observed in all cases. Histological studies of endomyocardial biopsies revealed a positive correlation between the transverse diameters of the myocytes and the total systemic peripheral resistance (TSPR) in 25 patients with SH-HT. In 12 patients with HCM, there was no correlation, and severe hypertrophy of the myocytes was observed despite a lower TSPR.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:[Evaluation of septal hypertrophy and wall dynamics by biventricular cineangiography and endomyocardial biopsy]. 654 88

Among one hundred and five consecutive patients with pre-excitation (PE) syndrome studied during a 10-year period, eight had an associated hypertrophic cardiomyopathy (HC) (7.62 per cent), eight had a coronary heart disease (7.62 per cent) and nine had a hypertensive heart disease (8.57 per cent). Of the eight patients with HC, four had an asymmetrical form (three of them with an obstructive component), and four a symmetrical form. Seven of these patients had a Wolff-Parkinson-White (WPW) type of PE and the remainder a Lown-Ganong-Levine type of PE. The incidence of paroxysmal tachycardias in the total group was 56.2% (61/105) and in the patients with associated HC was 62.5% (5/8). One of these latter patients had a concomitant brady-tachy syndrome and a severe obstructive form of HC. He was surgically treated (septal myomectomy and section of accessory atrioventricular pathway). The ECGs and VCGs of the seven patients with the HC-WPW type of PE association showed the coexistence of incomplete left bundle branch block of left ventricular hypertrophy patterns. The eight patients with associated HC were closely followed up from two to seven years (total follow-up period 435 patient/months). One of them died suddenly during the 40th month of follow-up. This study suggests that: 1) HC-PE association is not infrequent; 2) the incidence of paroxysmal tachycardias in the subgroup is quite similar to that presented in isolated PE; and 3) the electrocardiographic and vectorcardiographic changes in the HC-WPW type of PE association are highly specific.
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PMID:Pre-excitation syndrome and hypertrophic cardiomyopathy. 668 36

The manifestations and workload in a district general hospital cardiac unit of 39 unselected cases of hypertrophic cardiomyopathy over a five year period are reported. The "typical" form with asymmetrical septal hypertrophy and a gradient was found in only one third of patients, serious ventricular arrhythmias were probably no more common than in the general population, and no deaths occurred during a relatively short follow up (mean 3.1 years). It is concluded that although hypertrophic cardiomyopathy occupies a not insignificant proportion of cardiac workload, unselected cases presenting to a district general hospital represent a relatively mild disease without a grave prognosis.
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PMID:Hypertrophic cardiomyopathy: a common disease with a good prognosis. Five year experience of a district general hospital. 668 58

The diastolic thickness of the interventricular septum was measured by echocardiography and by biventricular angiography in 48 patients (40 male, 8 female) with age ranging between 15 and 67 years (average 44.4 years). These patients were classified in four groups according to clinical and paraclinical parameters:--normal subjects (29 cases),--patients with concentric hypertrophy (5 cases),--patients with asymmetrical septal hypertrophy without obstruction (4 cases),--patients with hypertrophic cardiomyopathy with obstruction under basal conditions or under stress (10 cases). Biventricular cineangiography was performed in 70 degrees left anterior oblique projection after measuring the intracavitary pressures. Analysis was performed by projecting the film and reproducing one or several diastolic frames on paper. Septal thickness was measured at the two levels suggested by Redwood. The septum was then divided into 5 parts and the thickness measured at the corresponding 4 levels. The values obtained were then corrected for magnification artefact. Qualitative comparison of the results obtained by echocardiography and angiocardiography showed a good correlation in 35 cases (73%) and less significant correlations in the other 13 cases (27%). The poorest correlations were observed in the groups of patients with concentric or asymmetrical hypertrophy without obstruction. Of these 13 cases, angiography confirmed the clinical diagnosis in 6 cases. In the remaining 2 cases, echocardiography and angiocardiography gave divergent diagnoses which also differed from the clinical diagnosis. The quantitative correlations between the echocardiographical and angiocardiographic measurements of diastolic septal thickness were quite satisfactory in most patients. The correlation improved when the echocardiographical measurement was compared to an average of the 4 angiographical measurements; (R = 0,74; p < 0.001). The correlation was poorer in groups 2 and 3 in patients with concentric or asymmetrical hypertrophy without obstruction. The disadvantages and limitations of these two methods are discussed. Possible ambiguity in the identification of segments of the septum measured by the two methods may be a significant factor especially in cases of asymmetrical hypertrophy.
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PMID:[Interventricular septum. Angiographic and echocardiographic correlations]. 677 42


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