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)

The auscultatory signs in 90 subjects with hypertrophic obstructive cardiomyopathy are described. The late-onset ejection systolic murmur and its responses to vaso-active manoeuvres reflect a volume-dependent outflow tract obstruction. Late vibrations of the systolic murmur, not uncommonly recorded at the apex, are due to associated mitral incompetence. Non-ejection systolic clicks may occur, and the likely explanation is inequality of the functional length of the mitral chordae tendineae secondary to asymmetrical myocardial hypertrophy. The second heart sound is often abnormal, usually with delay in the aortic component. Some correlation was demonstrated between the relative degrees of left and right ventricular outflow obstruction and the pattern of splitting of the second heart sound. Reversed or partially reversed splitting is usually associated with a more severe left ventricular outflow obstruction. Ejection systolic clicks and early diastolic murmurs occur infrequently, but are not incompatible with the diagnosis of hypertrophic obstructive cardiomyopathy.
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PMID:Auscultatory features of hypertrophic obstructive cardiomyopathy. A study of 90 patients. 116 71

We encountered a 65-year-old female with hypertrophic obstructive cardiomyopathy and mitral valve prolapse who had infective endocarditis and hemolytic anemia. The infecting organism of endocarditis was group A streptococci. With regard to the etiology of the hemolytic anemia, fragmentation hemolysis was considered because fragmented red cells and elevated lactic dehydrogenase were observed. Haptoglobin was markedly decreased. Coombs' test, Ham's test and abnormal hemoglobin were negative. She had not had a hemolytic attack in the past. Ultrasonic cardiography showed asymmetrical septal hypertrophy, mitral valve prolapse and 285 mmHg of calculated pressure gradient in the left ventricle. Cardiac catheterization showed 115 mmHg of left intraventricular pressure gradient and mitral regurgitation (grade 2). Hemolysis was slightly improved after treatment with propranolol. Thus, fragmentation of the normal red cells seemed to be due to shear stress.
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PMID:Fragmentation hemolysis in a patient with hypertrophic obstructive cardiomyopathy and mitral valve prolapse. 140 52

One hundred and two patients from South India with primary ankylosing spondylitis (AS) were analysed clinically and radiologically. The mean age of onset was 26 years, with a male to female ratio of 16:1. Eleven patients presented as juvenile ankylosing spondylitis. The mode of presentation of AS included axial involvement in 59, peripheral arthritis in 38, heel pain in 18 and acute anterior uveitis (AAU) in 11. The overall incidence of extra axial features was high (90 patients). These included subjects with peripheral arthritis (49), heel pain (35), AAU (14), rib pain (11), aortic regurgitation (8), apical pulmonary fibrosis (5), mitral regurgitation (2) and conduction defects (2). Peripheral arthritis was characteristically asymmetrical and oligo articular, and involved lower limb joints. No renal involvement was noticed. Radiologically, bilateral sacroilitis was seen in 80% of cases.
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PMID:Pattern of rheumatic diseases in south India. V. Ankylosing spondylitis. A clinical and radiological study. 208 81

Idiopathic restrictive cardiomyopathy is a rare myocardial disease characterized by restrictive physiology without a specific histologic basis. To assess its clinical, hemodynamic, morphologic and prognostic details we retrospectively evaluated all the patients hospitalized in our Institute from 1974 to 1988. Nine patients, aged 42 +/- 16 years, M/F ratio = 0.29, who represent 64% of all the restrictive myocardial diseases biopsied were identified. Severe cardiac heart failure (3-4 NYHA) and arrhythmias (ventricular and supraventricular) were extremely common. The electrocardiogram showed several non specific signs: low voltage of QRS in peripheral leads (4/7), pseudo-infarctional aspects (3/7), mono or biventricular hypertrophy (3/7) disturbance of ventricular conduction (3/7), aspecific abnormalities of ventricular repolarization (3/7). All patients showed a prolonged QTc. M-mode and 2-dimensional echocardiography demonstrated in 6 cases biatrial enlargement, normal or slightly enlarged ventricles, normal or moderately depressed fractional shortening; biventricular concentric hypertrophy was detected in 3 cases, asymmetrical septal hypertrophy in 1. Five patients showed pericardial effusion. Cardiac catheterization disclosed an increase of left and right ventricular end-diastolic pressures (8/8) with a dip-plateau pattern and/or characteristic W waveform in the atrial pressure tracing (9/9). Passive pulmonary hypertension was detected in 6/9 cases. The cardiac index was decreased in 4/8 cases. Left ventricular angiography showed mitral regurgitation in 5/8 patients, tricuspidal in 5/8. Ejection fraction was decreased in 3/8 cases. Endomyocardial biopsy showed interstitial fibrosis (8/9), cellular hypertrophy and/or nuclear alterations (7/9), slight endocardial thickening (2/9). At a mean follow-up of 22 +/- 15 months 3 patients died and 2 underwent heart transplantation. In conclusion idiopathic restrictive cardiomyopathy is one of the most frequent forms of restrictive myocardial diseases in our geographic area. Severe congestive heart failure and arrhythmias are extremely common. The disease can be suspected by clinical, electrocardiographic and echocardiographic features, but the final diagnosis requires cardiac catheterization and endomyocardial biopsy. Prognosis is severe and heart transplantation must be considered in the cases with severe heart failure.
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PMID:[Idiopathic restrictive cardiomyopathy: clinical, hemodynamic, histologic and prognostic profile]. 260 84

The long term results of mitral valve annuloplasty for pure mitral regurgitation are assessed. Thirty-three of the 100 patients (mean age 16 years) had acute rheumatic carditis at the time of operation. Three basic techniques were used: measured asymmetrical annuloplasty (Reed) in 37 patients, posterior plication annuloplasty in 56, Carpentier ring in seven. There were six early deaths, two of which were valve related. The mean follow up period for surviving patients was 4.8 (SD 1.2) years (range 3-9 years). Fourteen patients died late, all but one from valve related causes. Twenty-five had reoperation and all these had mitral valve replacement. The actuarial survival with initial repair was 56% at five years and 42% at eight years. Thirteen patients were lost to follow up. Of the remaining 42 patients, only 27 have an excellent or good clinical result. Eleven patients have only a satisfactory result, with a significant degree of residual or recurrent mitral regurgitation or disability or both. Possible causes of valve related failure were investigated; no significant difference was found between age groups or between acute and chronic cases. Poorer results, of statistical significance, were observed in patients in functional class IV and in those whose operation was performed on an emergency basis. No difference could be found between the results of the three techniques of mitral valve annuloplasty used. This retrospective study indicates that conservative procedures which are limited to annular repair produce inferior results. Valvuloplasty has many theoretical advantages which need practical confirmation.
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PMID:Mitral valve annuloplasty: results in an underdeveloped population. 664 51

Mitral valve repair in patients with mitral regurgitation requires a precise evaluation of the mechanism of valvular pathology before surgery. Transesophageal echocardiography has become the principal method for imaging valvular pathology, especially with the latest introduction of multiplanar transesophageal technology. Traditionally, echocardiographic analysis of regurgitant mitral valves has concentrated on the description of abnormalities of leaflet motion such as prolapse, while surgical correction aims at restoring the coaptation of the edges of the leaflets. To reconcile these different approaches, the echocardiographic features the findings on direct inspection, and the types of repair performed were analyzed in 37 incompetent mitral valves. The zone of coaptation of the mitral valve was studied in a series of left ventricular long-axis views obtained with a transesophageal multiplanar echo-transducer from the anterolateral via the central, to the posteromedial segment. Four patterns of leaflet closure were found: normal apart from a dilated annulus (24%), normal apposition but absent coaptation (8%); asymmetrical apposition but intact coaptation (16%); and abnormal apposition and absent coaptation (52%). In addition leaflet motion was described (prolapse, retraction, normal), and the anteroposterior dimension of the mitral annulus was measured. There was a direct relationship between these echocardiographic findings and the types of reconstructive techniques used. The echocardiographic analysis offers a logical approach to the preoperative diagnosis of regurgitant mitral valves, with the possibility to predict the feasibility and the type of mitral repair.
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PMID:[An echocardiographic analysis of the insufficient mitral valve: its intraoperative functional anatomy in relation to valvular reconstruction]. 785 31

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

Color Doppler echocardiography does not provide adequate information about the severity of mitral regurgitation in patients with eccentric mitral regurgitation. We have developed a new procedure for 3-dimensional (3D) color Doppler reconstruction and for segmentation of regurgitant jets. The volume of regurgitant jets was compared with jet area in 63 patients with mitral regurgitation. Mitral regurgitation was assessed by angiography, regurgitant fraction and volume by pulsed Doppler, JA by planimetry, and JV by 3-dimensional Doppler. Twenty-eight patients with central jets were compared with 35 patients with eccentric jets. In the patients with eccentric jets, JV showed significant correlations with regurgitant volume (r = 0.90; P <.01) and regurgitant fraction (r = 0.76; P < .01) and was able to separate groups with different degrees of mitral regurgitation (P <.01). Three-dimensional Doppler revealed origin, direction, and spatial spreading of complex jet geometry. JV, a new parameter of mitral regurgitation, was also capable of quantifying asymmetrical jets.
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PMID:Three-dimensional color Doppler: a new approach for quantitative assessment of mitral regurgitant jets. 1007 Jan 81

Eleven patients aged 8 to 15 years underwent measured asymmetrical annuloplasty for severe mitral regurgitation in the years 1961 through 1966. They had had a total of 20 attacks of acute rheumatic fever. The intervals between the last attack of acute rheumatic fever and operation ranged from 2 to 8 years. The criteria for surgery were congestive failure and progressive cardiac enlargement. Using the hydraulic formula of Gorlin, a mitral annuloplasty was tailored to the size of each patient so that insufficiency was eliminated without producing hemodynamically significant stenosis. In this group of 11 children there has been one death. The majority of our 11 patients reacquired murmurs of mitral regurgitation. Satisfactory results, however, are not dependent on complete hemodynamic correction. All patients have improved remarkably and have sustained this improvement up to 7 years. These results suggest that mitral annuloplasty should be the operation of choice in children with severe mitral regurgitation.
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PMID:Annuloplasty in children and young adolescents with severe rheumatic mitral insufficiency. 1171 78

Hypertrophic obstructive cardiomyopathy (HOCM), which shows left ventricular outflow pressure gradient (LVPG), is often complicated with mitral regurgitation (MR). We examined a 62-year-old Japanese female with HOCM and MR. Ultrasound echocardiography showed severe MR, asymmetrical septal hypertrophy, systolic anterior movement of the mitral valve anterior leaflet, and left ventricular outflow stenosis. Her LVPG, measured using continuous wave Doppler recording, was 118 mmHg. During heart catheterization, the aortic pressure and left ventricular pressure were simultaneously measured. An intravenous injection of 70 mg cibenzoline decreased the LVPG from 110 mmHg to 16 mmHg. Left ventriculography was performed immediately after the injection and did not show MR. This clearly demonstrates that cibenzoline decreases LVPG in patients with HOCM and also improves the MR that arises from LVPG.
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PMID:Mitral regurgitation disappearance after cibenzoline treatment in a patient with hypertrophic obstructive cardiomyopathy. 1496 71


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