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

A case of acute idiopathic myopericarditis with transient severe swelling and akinesis of the interventricular septum on echocardiograms was reported. A 35-year-old man was admitted to our CCU because of cardiogenic shock five days after the onset of "common cold". Electrocardiograms revealed low voltage and ST-segment elevation in all leads except for a VR and a VL. Serum CPK, GOT and LDH were slightly elevated. Echocardiograms showed severe asymmetrical septal hypertrophy (ASH, 20 mm), akinesis of the interventricular septum and moderate pericardial effusion. With improvement of the clinical course and of the hemodynamic data, the thickness and wall motion of the interventricular septum became normal. Serum antiviral antibodies were not elevated throughout the course. Transvenous percutaneous right ventricular endomyocardial biopsy revealed degeneration and fragmentation of the myofibrils with interstitial cellular infiltration and edema in the subacute phase (8th hospital day), but showed only focal fibrosis in the chronic phase. Coronary arteriograms and left ventriculograms in the chronic phase (50th hospital day) were normal. From these findings we can conclude that the severe transient ASH is due to myocardial inflammatory swelling.
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PMID:[Acute idiopathic myocarditis having myocardial inflammatory swelling demonstrated by echocardiography: a case report]. 383 77

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