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)

An echocardiographic Study of 322 adults (age: 40 +/- 16 years), belonging to 20 families with hypertrophic cardiomyopathy (HCM), was undertaken. Affected subjects had a LV diastolic wall thickness > 13 mm. The patients were classified according to the distribution of left ventricular hypertrophy (LVH) and by Maron's classification: 189 subjects were normal, 127 were affected and 6 could not been classified. By Maron's classification: 3% were type I, 33% were type II, 58% were type III and 6% were type IV. LVH was asymmetrical in 95% of cases (septum/posterior wall ratio > 1.3). The familial distribution of LVH of the 4 families in which HCM was genetically related to different loci (chromosome 11, 14 exon 13, 14 exon 8, fifth locus); the LVH was analysed from two short axis LV parasternal views and each plane was divided into 5 segments. The distribution of LVH was said to be identical between two first degree relations when all the same segments were affected, similar when they differed by only 1 or 2 segments and different when they differed by 3 or more segments. In the 26 pairs studied, LVH was identical in 2/26 (8%), similar in 11/26 (42%) and different in 13/26 (50%). Familial HCM usually gives rise to asymmetrical LVH affecting the septum and free wall. An identical distribution in 50% of affected first degree relatives.
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PMID:[Clinico-pathological polymorphism of hypertrophic cardiomyopathy in echocardiography]. 748

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.
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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.
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PMID:[Study of hypertrophic cardiomyopathies with Doppler echocardiography]. 817 76

A French multicentre study of hypertrophic cardiomyopathy has recruited 260 subjects belonging to 18 families. At least 3 persons from each family included had a hypertrophic cardiomyopathy. A Doppler echocardiographic examination was performed in all members of these 18 families. The diagnosis of hypertrophic cardiomyopathy was based on M mode and/or 2D observations of parietal hypertrophy: wall thickness over 13 mm. Asymmetrical forms were distinguished from symmetrical forms by a septum/posterior wall ratio of over 1.3 in M mode or 2D study. The patients had an average age of 40 +/- 18 years, 127 were men and 133 women. One hundred and sixty eight were considered to be normal, 87 had hypertrophic cardiomyopathy and 5 were border line. The maximal diastolic wall thickness on M mode recording of pathological cases was 19.2 +/- 4.8 mm compared with 9.5 +/- 1.9 mm in healthy subjects (p < 0.001). The septum/posterior wall ratio was 1.8 +/- 0.62 in pathological cases and 1.1 +/- 0.8 in normal ones (p < 0.001). Eighty five per cent of the cardiomyopathic cases were asymmetrical (74/87) and only 15% were considered to be symmetrical (13/87) on M mode study whereas 5% were concentric on 2D echocardiography. By Maron's classification, type I hypertrophy was observed in 6% of cases (4/64), type II in 30% (19/64), type III in 58% (37/64), and type IV in 6% (4/64). Signs of obstruction were looked for and systolic anterior motion of the mitral valve was observed in 52% of pathological cases (45/86) and mid-systolic aortic valve closure in 30% (25/83).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[French familial multicenter survey of hypertrophic cardiomyopathy. Initial Doppler echocardiographic results]. 821 92

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.
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PMID:Asymmetrical septal hypertrophy in patients with hypertension: a type of hypertensive left ventricular hypertrophy or hypertrophic cardiomyopathy combined with hypertension? 841 59

123I-labelled 15-(p-iodophenyl)-3-(R,S)-methylpentadecanoic acid (BMIPP) myocardial single photon emission computed tomography (SPECT) was performed in 17 patients with hypertrophic cardiomyopathy, and was compared with 201Tl exercise stress myocardial perfusion SPECT images. Fourteen patients showed asymmetrical hypertrophy, and three demonstrated apical hypertrophy. SPECT was performed 20 min and 3 h after injection of 111 MBq 123I-BMIPP at rest. Exercise stress 201Tl SPECT was performed at 10 min and 3 h after injection and was compared with BMIPP imaging. In 13 patients BMIPP accumulation in the hypertrophied area in the 20 min image was lower than that of 3 h 201Tl uptake. Interestingly, six patients demonstrated 201Tl redistribution in the region where the uncoupling of BMIPP uptake at 20 min and 201Tl accumulation at 3 h after exercise was observed. These findings suggest that impaired fatty acid metabolism or utilization in hypertrophic myocardium and ischaemia or impaired coronary flow reserve may be one of the causes of the abnormality of fatty acid accumulation.
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PMID:123I-labelled BMIPP fatty acid myocardial scintigraphy in patients with hypertropic cardiomyopathy: SPECT comparison with stress 201Tl. 845 8

Two brothers, 25 and 19 years old, were affected by asymmetrical hypertrophic cardiomyopathy. The older brother had waddling gait and weakness of the proximal girdle muscles, while the younger had a broad-based gait and weakness of selected limb girdle muscles. EMG exam was myopathic. Serum enzyme, CPK and aldolase were elevated. Histochemical reactions in muscle revealed "core-like" areas, subsarcolemmal rims of mitochondria and lipid accumulation. Succinate-dehydrogenase stain showed a lack of activity in both biopsies, with the exception of intrafusal fibers. Microphotometric quantitative measurements confirmed the defect in both biopsies. Biochemical measurements of several mitochondrial enzymes in muscle showed a reduced activity of succinate-dehydrogenase (33%) and succinate-cytochrome C reductase (36-47%) which are both components of complex II. On myocardial biopsy lipid and mitochondrial abnormalities were found. This mitochondriopathy represents a new phenotype of partial complex II defect.
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PMID:Hypertrophic cardiomyopathy with mitochondrial myopathy. A new phenotype of complex II defect. 851 73

There are three types of cardiomyopathy: hypertrophic, dilated and restrictive. The diagnosis and prognosis of these three types may be assessed from Doppler echocardiographic data. In hypertrophic cardiomyopathy, the diagnostic criterion is parietal hypertrophy. This hypertrophy is asymmetrical and usually affects the interventricular septum. Ventricular outflow obstruction is not necessarily present. The left ventricle is small and analysis of mitral inflow usually shows abnormal relaxation (E/A ratio < 1). The prognosis of this type of cardiomyopathy seems to be related to ventricular arrhythmias and the relationship between the hypertrophy and the presence of arrhythmias remains controversial. Dilated cardiomyopathy is characterized by ventricular walls of normal or decreased thickness, an increase in left ventricular dimensions and a reduction in the ejection fraction. An end-diastolic left ventricular dimension > 70 mm and an ejection fraction < 25% are poor prognostic factors. Left ventricular filling is abnormal and severe cases show a restrictive type of profile; in this case, an E/A ratio > 2 carries a poor prognostic. A mitral deceleration time of E wave < 150 msec usually indicates a bad outcome. Restrictive types of cardiomyopathy are more rare, amyloidosis being the commonest cause. Symmetrical wall thickening and a small ventricular chamber are observed. In advanced stages with abnormal systolic function, an E/A mitral ratio > 2 and deceleration time < 150 msec, the outcome is rapidly fatal.
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PMID:[Doppler echocardiographic investigation of cardiomyopathies]. 888 5

While hypertension is known to cause left ventricular and vascular hypertrophy, the relationship between alterations of vascular and cardiac structures in patients with hypertrophic cardiomyopathy has not been fully clarified. We measured intima-media thickness of carotid arteries by ultrasonography in patients with hypertrophic cardiomyopathy (n = 16), normotensive subjects (n = 358), and hypertensive subjects (n = 386) in a cohort of 7940 male employees of a bus company. Our object was to determine whether vascular alteration occurs in hypertrophic cardiomyopathy similarly as in hypertension. Hypertrophic cardiomyopathy (wall thickness > or = 15 mm; asymmetrical hypertrophy without hypertension) was screened with family history and electrocardiography followed by echocardiography. The intima-media thickness in patients with hypertrophic cardiomyopathy (mean, 0.61 mm) did not differ from that of normotensive subjects (0.60 mm) but was significantly less than that of hypertensive subjects with left ventricular hypertrophy (wall thickness > or = 14 mm; n = 22; 0.73 mm). In a scatterplot of intima-media thickness versus interventricular septal thickness, these two parameters were significantly correlated in normotensives and hypertensives. The patients with hypertrophic cardiomyopathy distributed outside the 95% confidence range of the normotensive and hypertensive subjects. In summary, the increase in intima-media thickness of the carotid artery paralleled left ventricular hypertrophy in normotensive and hypertensive subjects. Patients with hypertrophic cardiomyopathy had a normal intima-media thickness regardless of the hypertrophied left ventricle. Thus, information on intima-media thickness may be useful in differentiating hypertensive left ventricular hypertrophy from hypertrophic cardiomyopathy.
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PMID:Intima-media thickness of the carotid artery in hypertensive subjects and hypertrophic cardiomyopathy patients. 903 28

To investigate the left ventricular hypertrophic patterns and wall motion dynamics in hypertrophic cardiomyopathy, 51 patients were studied using electron beam computed tomography. The subject consisted of 26 asymmetrical hypertrophy, 9 diffuse hypertrophy, 14 apical hypertrophy and 2 papillary muscle hypertrophy. Concerning the wall motion dynamics in hypertrophic wall, 7 demonstrated homogeneous wall thickening involving the non-hypertrophic wall, 36 showed decrease wall thickening, 6 showed normal wall thickening, and 2 cases of papillary muscle hypertrophy had increased wall thickening in the apical wall. The percent wall thickening in hypertrophic wall was significantly reduced in relation to the increase of wall thickness; 14 +/- 8% in the wall over 20 mm, 23 +/- 12% in 16-9 mm and 56 +/- 36% in 13-15 mm. The reduced wall motion dynamics in hypertrophic wall were clearly observed by electron beam computed tomography.
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PMID:Left ventricular hypertrophic patterns and wall motion dynamics in hypertrophic cardiomyopathy: an electron beam computed tomographic study. 918 64


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