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.
J Cardiol 1988 Sep
PMID:[Four cases of Fabry's disease mimicking hypertrophic cardiomyopathy]. 297 98

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.
J Cardiol 1988 Dec
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.
J Cardiol 1987 Dec
PMID:[Familial cardiomyopathy with different clinical features in individual members]. 350 15

25 patients affected by Primary Hypothyroidism and a control group of 25 subjects were studied with M-mode and Two-dimensional echocardiography. In hypothyroid patients mean left ventricular and aortic root dimensions were normal compared to control subjects. Pericardial effusion was found in 22 out of 25 patients (88%). Septal hypertrophy was found in 12 hypothyroid patients (48%), only in 3 out of these 12 patients left ventricular posterior wall hypertrophy was found. A moderate left atrial enlargement was found in 7 out of 25 hypothyroid patients. Two-dimensional echocardiography revealed an uniform degree of parietal hypertrophy from the basal segments to the cardiac apex. Therefore the main echocardiographic findings in hypothyroidism are: presence of pericardial effusion and asymmetrical septal hypertrophy. No correlations between these echocardiographic findings and age of patients, severity of "biochemical" alterations and duration of the thyroid disease was observed.
G Ital Cardiol 1986 Jun
PMID:[Echocardiography in primary hypothyroidism. Study of 25 patients]. 375 84

Echographic abnormalities of cardiac amylosis are now well known and quite useful to the diagnosis. Forms that are morphologically atypical are rare. From three observations (2 hypertrophic, asymmetrical and obstructive forms, and 1 hypertrophic and dilated form) and a review from the literature, we study the nosological, diagnostic and therapeutic problems presented by these atypical echographic of cardiac amylosis.
Ann Cardiol Angeiol (Paris)
PMID:[Atypical echocardiographic aspects of cardiac amylosis]. 380 Feb 80

Four cases of "absent pulmonary valve" (APV) are described. This congenital heart disease consists of aplasia or extreme hypoplasia of pulmonary semilunar cusps and is always combined with aneurysmatic dilatation of pulmonary artery. In two of them the diagnosis was confirmed at autopsy. The remaining two are clinical reports surgically confirmed. One of them was studied by single cristal and two-dimensional echocardiography. In three cases APV was associated with tetralogy of Fallot, while in one case there was an intact ventricular septum. Review of literature allowed us to select 149 cases of APV anatomically confirmed, besides our ownes. It is stressed on that APV is usually associated with dextroposition of the aorta and ventricular septal defect by conoventricular malallignment. It is suggested that pathogenesis of this malformation is a consequence of an anomalous development of mesenchimal tissue of pulmonary cusps rather than an asymmetrical truncal sepimentation. We favour the hypothesis that aneurysmatic dilatation of pulmonary artery is caused by altered hemodynamics acting both in foetal and extrauterine life, even if differently expressed. Pathophysiologic and diagnostic value of cyanosis, dyspnea, and systo-diastolic murmur are discussed. Some outlines of the most important diagnostic procedures are reviewed and particularly echocardiography, which shows aortic overriding and dilatation of right ventricular outflow tract and pulmonary artery separated by a restricted pulmonary annulus. Prognosis and therapy are also mentioned.
G Ital Cardiol 1980
PMID:[Absent pulmonary valve. Report of 4 cases, echocardiographic assessment and review of the literature (author's transl)]. 700 35

We report on a patient suffering from asymmetrical hypertrophic cardiomyopathy, with alterations occurring in the signal-averaged electrocardiogram (SAQRS). Recordings of 3-lead orthogonal ECG were obtained and analyzed by a Del Mar Avionics 750A Innovator device. The time domain analysis showed late potentials and a slow inscription of the initial portion of the SAQRS, that we called "early potentials". On the frequency domain there was a high degree of spectral turbulence at the beginning and at the end of the SAQRS. We discuss the possibility that the slow and fragmented conduction of the initial portion of the SAQRS could be related to the electrical instability of the disease.
G Ital Cardiol 1994 Feb
PMID:[Initial and final changes in the signal-averaged QRS in the time and frequency domain in a case of hypertrophic myocardiopathy]. 801 67

The aim of this review is to demonstrate the usefulness of Doppler echocardiography in the study of hypertrophic cardiomyopathy. Two-dimensional imaging enables confirmation of hypertrophy and identification of its type (usually asymmetrical), site and extent. Intraventricular obstruction can be confirmed by echocardiography (mesosystolic aortic closure, systolic anterior mitral movement with prolonged septal contact) and Doppler (intraventricular obstruction flow). This obstruction may be obvious (present under baseline conditions), latent (appearing during provocative tests) or absent. The maximum velocity of obstruction flow can be used to calculate intraventricular gradient by application of Bernouilli's equation. This intraventricular obstruction flow must be distinguished from apical obliteration flow (with which it may be associated) and from left mid-ventricular stenosis flow (which may cause diastolic obstruction associated with the systolic obstruction). Mitral insufficiency is usually a consequence of intraventricular obstruction (loss of systolic coaptation of the mitral leaflets secondary to systolic anterior mitral movement). Ejection parameters are increased because of a fall in left ventricular afterload (hyperdynamic state). Left ventricular diastolic function is most often abnormal (relaxation anomaly). Doppler echocardiography can also be used to seek associated abnormalities, in the evaluation of family members and to monitor progress (treated or not treated). Doppler echocardiography thus enables complete anatomical and functional study of hypertrophic cardiomyopathy. Invasive hemodynamic investigations are justified only if a diagnostic problem persists, if surgical treatment is considered or if coronary arteriography is thought necessary.
Ann Cardiol Angeiol (Paris) 1994 Jan
PMID:[Study of hypertrophic cardiomyopathies with Doppler echocardiography]. 817 76

We present the case of a 55-year-old man with rapid progression from asymmetrical septal hypertrophy to hypertrophic obstructive cardiomyopathy over a 1-year period leading to persistent anginal symptoms despite adequate treatment of his concomitant coronary artery disease. The potential mutagenic side effects of XeCl excimer laser-radiation that was used to remove the arteriosclerotic plaque from the left anterior descending coronary artery may have contributed to the sudden increase in septal thickness.
Int J Cardiol 1993 Jun 15
PMID:Rapid progression of septal hypertrophy in an adult with hypertrophic cardiomyopathy and excimer laser-treated coronary artery disease. 834 70

To determine whether asymmetrical septal hypertrophy (ASH) in patients with essential hypertension (HT) is a type of hypertensive left ventricular (LV) hypertrophy or hypertrophic cardiomyopathy (HCM) combined with HT, we investigated a group of 7 hypertensive patients with ASH compared with 12 HCM patients and 10 healthy controls using radionuclide angiography and right ventricular endomyocardial biopsy. The LV time-volume curve and its first and second derivative curves were constructed from cardiac output and time-activity curves constructed by combined forward and reverse-gating from the R wave. The LV wall thickness and ejection fraction were significantly greater in both the HT and HCM groups than in the control group, whereas there were no differences in these indices between the HT and HCM groups. Rapid filling volume index and rapid filling fraction showed significantly lower values in the HCM group than in the control group (p < 0.005). In contrast to the HCM group, these indices in the HT group did not differ from those in the control group. The time to peak filling rate was prolonged in the control, hypertension, and HCM groups in increasing order. Histopathological study revealed a higher incidence of myocardial cell disarray in the HCM than in the HT group. The above results suggest that ASH in hypertensive patients is a type of hypertensive LV hypertrophy.
Clin Cardiol 1993 Jan
PMID:Asymmetrical septal hypertrophy in patients with hypertension: a type of hypertensive left ventricular hypertrophy or hypertrophic cardiomyopathy combined with hypertension? 841 59


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