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)

A 63-year-old male complained of palpitation and dyspnea. ECG showed paroxysmal supraventricular tachycardia. Echocardiogram revealed asymmetrical thickening of the cardiac septum, and by other clinico-pathological observations, nephrotic syndrome and colon adenoma were detected. For these diseases, steroid therapy and operation were performed, respectively. Ten months later, chest X-ray revealed diffuse abnormal shadow in the bilateral lung fields, and the patient expired due to respiratory distress syndrome. The patient's heart weighted 300g and showed extensive myofibril disarray in bilateral ventricles involving the septal region of the heart. Degeneration and atrophy of the atrioventricular node was seen. In the lungs, organizing pneumonia, old pleuritis, diffuse septal fibrosis and cytomegalovirus infection were demonstrated in the alveolar walls. Kidneys showed slightly sclelotic changes in glomeruli and edema-fibrosis in the interstitium. In this rare case, hypertrophic cardiomyopathy was combined with nephrotic syndrome due to glomerular change, and colon adenoma. The patient died of cytomegalovirus infection in the lungs. The histogenesis of myofibril disarray was discussed in these diseases.
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PMID:[An autopsy case of cardiomyopathy combined with nephrotic syndrome and pulmonary cytomegalic inclusion disease]. 164 80

Seven adult patients with old and severe arterial hypertension were found to have hypertrophic cardiomyopathy with left ventricular obstruction demonstrated by an isoproterenol test. Whenever feasible, confirmation that systolic obstruction of the left ventricular outflow tract was due to anterior systolic movement of the mitral valve was obtained. Echocardiography revealed a number of ultrasonic features (asymmetrical septal hypertrophy, small left ventricle and clear-cut reduction of the left ventricular outflow tract) which put these cases closer to the primary hypertrophic cardiopathy group than to the hypertensive cardiomyopathy group, with a similar history of hypertension. Detecting this group is facilitated by the use of vasoactive drugs in patients with these echocardiographic features. This is important since there is a risk of poor tolerance to vasodilators, notably nitrates, which may suddenly reveal the left ventricular dynamic obstruction syndrome. These patients are also exposed to paroxysmal atrial fibrillation.
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PMID:[Hypertrophic cardiomyopathy with left ventricular dynamic obstruction syndrome in hypertensive adult patients]. 183 84

Transient hypertrophic cardiomyopathy has been described in human infants of diabetic mothers. The purpose of this study was twofold: first, to document the features of cardiac hypertrophy in newborns of female rats with streptozotocin-induced diabetes and second, to investigate the natural history of this cardiomyopathy. Marked asymmetrical septal hypertrophy with hypertrophy of myocytes was observed in newborns of diabetic female rats, whether or not the mothers received daily administration of NPH insulin. The abnormal cardiac morphology was no longer apparent 4 weeks after birth. Thus, in this model, the asymmetrical septal hypertrophy was secondary and was not a manifestation of genetically transmitted hypertrophic cardiomyopathy.
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PMID:The cardiomyopathy in infants of streptozotocin-induced diabetic female rats. 207 51

In this study, we investigated correlations of left ventricular hypertrophy and its histopathology with diastolic dysfunction in patients with hypertrophic cardiomyopathy. Nine control subjects and 14 hypertrophic cardiomyopathy (HCM) patients with asymmetrical septal hypertrophy were evaluated. M-mode echocardiography was used to assess fractional shortening (FS), isovolumic relaxation time (IRT), and the left ventricular filling volume index during rapid and slow filling periods and atrial contraction period (RFVI, SFVI and ACVI). End-diastolic thickness of the interventricular septum and posterior wall was determined using biventriculography. Right ventricular endomyocardial biopsies were performed to calculate the diameters of myocytes, the percentage of fibrosis and the eccentricity e which indicates the degree of myocardial disarrangement including disorganization. The FS was normal in the two groups. The IRT of the HCM group was significantly greater and the RFVI significantly less than those of the controls. The left ventricular wall thickness, the diameters of myocytes and the percentage of fibrosis in the HCM group were significantly greater; and the eccentricity e was significantly less, suggesting that myocardial disarrangement was significantly more severe than that in the controls. Significant positive correlations were observed between the IRT and the wall thickness (r = 0.647), between the diameter of myocytes (r = 0.681) and the percentage of fibrosis (r = 0.628), and there was a significantly negative correlation between the IRT and the eccentricity e (r = -0.759). There was a significantly negative correlation between the RFVI and the wall thickness (r = -0.663); and a significantly positive correlation between the RFVI and the eccentricity e (r = 0.579). Multiple regression analyses showed that the diameter of myocytes, the percentage of fibrosis and the eccentricity e all correlated significantly with the IRT (R = 0.821) and the RFVI (R =0.604). The standard regression coefficients of the diameter of myocytes, the percentage of fibrosis and the eccentricity e were 0.253, 0.278 and -0.431 in respect to IRT, and those of the percentage of fibrosis and the eccentricity e were -0.204 and 0.469 in respect to RFVI, respectively. These results indicated that diastolic dysfunction in hypertrophic cardiomyopathy is related not only to the degree of left ventricular hypertrophy, but also to the degree of myocardial hypertrophy, increased interstitial fibrosis, and especially to myocardial disarrangement including disorganization.
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PMID:[Early diastolic dysfunction of the left ventricle affected by hypertrophy and abnormal histopathology in hypertrophic cardiomyopathy]. 215 Dec 36

The morphological features, mode of presentation and physiopathology of hypertrophic cardiomyopathy (HCM) are variable. Autosomal dominant seems to be the usual mode of transmission but with variable presentation. From the anatomical point of view, the hypertrophy is asymmetrical with septal predominance. The main histological features are myocytic architectural disorganisation, fibrosis and abnormal coronary arteries of small diameter. Ventricular hyperkinesis is usually present and sometimes associated with outflow obstruction, the physiological role and mechanisms of which are still not fully understood. On the other hand, abnormal diastolic function is frequently observed, and, quite independently of disease of the epicardial coronary arteries, ischaemic phenomena may occur. Although the biological substrate of HCM is unknown, abnormalities of the adrenergic system and transmembrane calcium flux probably play a part in the expression of the disease.
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PMID:[Hypertrophic and/or obstructive primary cardiomyopathies: genetic, etiologic, physiopathologic aspects]. 225 45

Seven elderly patients with hypertrophic obstructive cardiomyopathy (HOCM), who had the three following characteristics on echocardiograms 1) extremely thickened septum, 2) systolic anterior motion of the mitral valve, 3) mid systolic semi-closure of the aortic valve, were clinically evaluated. Ages ranged from 73 to 86 years old (average 78.9% yr.) and all were women. None had not a family history of hypertrophic cardiomyopathy but they had mild hypertension. Six patients showed a significant high voltage on the ST-segment and T-wave abnormalities ("strain" pattern). The left ventricular posterior wall as well as the septum was thickened in 5 and the remaining 2 showed asymmetrical septal hypertrophy (ASH) on echocardiograms. The left ventricular cavity was narrowed due to left ventricular hypertrophy and the shape of the left ventricular cavity was ovoid in all patients. The aorto-septal angles in these 7 patients were 80 degrees to 120 degrees. In addition, proximal septal bulge in all and anterior displacement of the mitral posterior leaflet due to the mitral ring calcification (MRC) in some patients contributed to the narrowing of the left ventricular outflow tract, and the mitral valve was pulled up toward the septum because of the good left ventricular systolic function (ejection fraction: 70 to 94% by echocardiography) and blood was ejected at a high velocity through a narrowed outflow tract (Venturi effect). Pressure gradients in the left ventricular outflow tract was 38 to 146 mmHg in 5 examined by cardiac catheterization. Biopsy specimens were obtained from 2 patients, showing hypertrophic myocytes (diameter: 20 to 30 micron) in 2 and mild disarray in 1.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A clinical study of hypertrophic obstructive cardiomyopathy in the elderly]. 226 18

In order to develop the tentative criteria of the differential diagnosis, 18 patients with obstructive hypertrophic cardiomyopathy (OHCMP), 3 with nonobstructive hypertrophic cardiomyopathy (NOHCMP), 8 with essential hypertension (EH) with inadequate left ventricular hypertrophy (LVH) and 10 normal persons were subjected to clinical examination and to ultracardiosonography. The patients with OHCMP mainly complained of dizziness and syncopes. Since childhood they had systolic murmur and ECG abnormalities. Ultracardiosonography showed asymmetrical LVH, a considerable decrease of the ventricular cavity as well as abnormalities of the localization and function of the papillary muscles. NOHCMP was marked by the combination of the good well-being of the patients with gross abnormalities on the ECG. Ultracardiosonography demonstrated moderately pronounced and asymmetrical LVH. The group suffering from EH with inadequate LVH was characterized by the early development of severe circulatory failure with arterial hypertension of moderate intensity. The changes in the architectonics of the left ventricle and its subvalvular structures turned out to be similar to those in OHCMP but were less remarkable.
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PMID:[The differential diagnosis of various forms of hypertrophic cardiomyopathy and hypertension with inadequate left ventricular hypertrophy]. 253 35

Thirty three cases of hypertrophic cardiomyopathy (HCMP) were reviewed to estimate the relative frequencies of the subtypes of HCMP and to clarify whether there is any racial difference in clinical and morphological features of HCMP. The diagnosis was made by echocardiography, cardiac catheterization and left ventriculography. Twenty four patients underwent coronary angiogram. Numbers of cases by the types of HCMP were 20 (61%) with asymmetrical septal hypertrophy (ASH), 11 (33%) with apical hypertrophy (APH) and 2 (6%) with midventricular hypertrophy (MVH). Mean ages of the patients with APH, ASH and MVH were 54, 46 and 31 years respectively, and the differences were statistically significant (p less than 0.05). The giant negative T wave on electrocardiogram was seen in 4 patients (20%) of ASH and 5 patients (45%) of APH. On echocardiogram mean ratio of interventricular septal to left ventricular posterior wall thickness was 1.9 in ASH, 1.2 in APH and 1.6 in MVH, and the differences were statistically significant (p less than 0.05). All patients with APH showed "spade of ace" deformity in left ventriculography. Coronary angiograms were normal in all patients who had the procedure. Our study showed high frequency of APH of which characteristics were similar to those of the Japanese type APH.
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PMID:Clinical and morphological features of hypertrophic cardiomyopathy in Korean patients. 263 92

The evolution of 60 cases of hypertrophic cardiomyopathy over a mean period of 6.3 years is described. The cases were separated according to echo-cardiographic findings into 4 categories: obstructive cardiomyopathy (29 cases), asymmetrical septal hypertrophy (22 cases), diffuse cardiomyopathy (6 cases) and apical cardiomyopathy (3 cases). A number of complications occurred during the follow-up, consisting of cardiac failure (20 cases), embolic accident (9 cases), aortic endocarditis (1 case) and arrhythmia (32 cases including 12 cases of supraventricular arrhythmia and 20 cases of ventricular arrhythmia). Five patients died suddenly. These data are compared with those obtained from the literature in an attempt to determine the main prognostic factors in hypertrophic cardiomyopathy.
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PMID:[Hypertrophic myocardiopathy. Course and prognosis. 60 cases]. 293 68

A prospective echocardiographic investigation was undertaken to determine the prevalence and significance of localized subaortic hypertrophy in 1000 consecutive patients presenting for a routine echocardiographic examination. Localized septal hypertrophy was diagnosed when the subaortic septum was hypertrophied (greater than 1.4 cm) and was 50% thicker than the mid-point of the septum. Patients with hypertrophic cardiomyopathy and fixed subvalvular aortic stenosis were excluded. Eight cases of localized subaortic hypertrophy were identified. In 7 the appearances of the left side of the interventricular septum were similar with an apparently sigmoid shape (reversed S on its side) and in 1 with associated mitral stenosis the septum was a tapered wedge. All patients with localized subaortic hypertrophy had left ventricular hypertrophy (left ventricular mass or posterior wall thickness greater than 2 SD from normal) with a normal size cavity due to aortic valve disease (2 patients were also hypertensive). Of the 180 patients with aortic valve disease, localized subaortic hypertrophy was found in 10% of those with left ventricular hypertrophy and 33% of those with asymmetrical septal hypertrophy (septum to posterior wall ratio of greater than 1.5:1). There was no evidence of subaortic stenosis by pulsed and continuous wave doppler echocardiography (8 cases) and cardiac catheterization (6 cases). The aetiology of this discrete localized muscular bulge is unclear but is presumably due to change in shape of the septum with left ventricular hypertrophy. However, this finding has important implications as a cause of asymmetrical septal hypertrophy and because of the possible false diagnosis of subvalvular stenosis and its effect on ultrasound measurements.
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PMID:An echocardiographic study of localized subaortic hypertrophy. 293 53


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