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)

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

A 41-year-old man was referred to our hospital for further examination because of abnormal electrocardiography findings at a health-check examination. Transthoracic echocardiography showed left ventricular hypertrophy confined to the most distal portion of the left ventricle, which is a typical feature of apical hypertrophic cardiomyopathy. Ten years later, he was again admitted for the evaluation of chest pain. Echocardiography showed asymmetrical septal hypertrophy in addition to apical hypertrophy. These findings demonstrate morphologic evolution in hypertrophic cardiomyopathy from apical hypertrophy to asymmetrical septal hypertrophy.
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PMID:Hypertrophic cardiomyopathy with progression from apical hypertrophy to asymmetrical septal hypertrophy: a case report. 1587 37

HOCM is a rare disorder of myocardium that may result in asymmetrical left ventricular septal hypertrophy and dynamic outflow obstruction. This may result in hemodynamic sequel that leads to deterioration of functional class in the majority of patients. Alcohol septal ablation may provide symptomatic relief in the majority of patients who fail medical therapy or who experience significantly high outflow gradients.
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PMID:Alcohol septal ablation for hypertrophic obstructive cardiomyopathy. 1596 18

A 27 year old female with Noonan syndrome and hypertrophic cardiomyopathy underwent cardiovascular magnetic resonance imaging. These images showed asymmetrical septal hypertrophy with maximal left ventricular end-diastolic wall thickness of 25 mm. Following administration of gadolinium, areas of hyperenhancement were seen in the anterior, anteroseptal and lateral walls. This is the first report of focal gadolinium hyperenhancement in hypertrophic cardiomyopathy due to Noonan syndrome and suggests that myocardial fibrosis can be imaged by MR hyperenhancement as seen previously in sarcomeric hypertrophic cardiomyopathy.
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PMID:Hypertrophic cardiomyopathy in Noonan Syndrome closely mimics familial hypertrophic cardiomyopathy due to sarcomeric mutations. 1626 21

Hypertrophic obstructive cardiomyopathy represents a genetic disorder characterized by hypertrophy, usually asymmetrical, of the ventricular musculature at the base of the septum in the left ventricular efflux tract. Patients suffering from this disorder can be extremely sensitive to small alterations in ventricular volumes, arterial pressure, cardiac frequency and rhythm. This disorder is found in pregnancy with an incidence of 0.1-0.5% and, because of its gravity, represents a contraindication which is often absolute to pregnancy. Hemodynamic variations such as those found in pregnancy, labor and delivery have complex influences on hypertrophic cardiomyopathy. Our clinical series includes 2 pregnant patients suffering from hypertrophic obstructive cardiomyopathy who both underwent caesarian section in general anesthesia, the first due to the gravity of cardiac obstruction and the second due to the emergent need to proceed after the beginning of labor. The small number of clinical cases in the literature, especially in the last few years, clearly underlines the difficulty of defining both the most correct method for delivery and the most appropriate anesthesiological techniques. In accordance with the literature and our clinical experience, we can conclude that a carefully managed pregnancy can proceed without complications in patients with moderate obstruction and that a regional anesthesiological approach is also possible with careful hemodynamic monitoring. General anesthesia, however, remains the safest method and has fewer risks for patients with serious obstruction or with worsening of their clinical condition during pregnancy.
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PMID:Hypertrophic obstructive cardiomyopathy and pregnancy: anesthesiological observations and clinical series. 1766 Jul 42

A 50-year-old man presented with chest pain and recurrent episodes of syncope. Physical examination revealed a loud systolic murmur at the left sternal border with accentuation in erect position. Transthoracic echocardiography demonstrated severe asymmetrical septal hypertrophy and systolic anterior motion of the anterior mitral valve leaflet which were diagnostic for hypertrophic cardiomyopathy. Interestingly, color Doppler examination of the left ventricle showed aliasing of the left anterior descending coronary artery and intra-myocardial coronaries.
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PMID:Myocardial bridge in hypertrophic cardiomyopathy: imaging with color Doppler echocardiography. 1826 19

The modern description of hypertrophic cardiomyopathy is credited to the London pathologist, Robert Donald Teare who likened the disease to "a tumour of the heart" and published his observations in the British Heart Journal 50 years ago. Teare recognised asymmetrical hypertrophy and myocyte disarray as a familial condition associated with premature and sudden death in young people. He rightly deserves the accolade for bringing a poorly understood, but well recognised phenomenon into the public domain. Thick and heavy hearts had been of interest and investigation to physicians and pathologists for many centuries. This article reviews the early history of hypertrophic cardiomyopathy and reflects on several centuries of progress in our understanding of the condition.
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PMID:Hypertrophic cardiomyopathy: lessons from history. 1865 79

In 1958, the British forensic pathologist, Donald Teare, reported a family in which eight young people had died suddenly from asymmetrical hypertrophy of the left ventricle. Five decades on, the prevention of premature death from ventricular tachyarrhythmia, heart failure and stroke remains a major aim of clinical management in what is now called hypertrophic cardiomyopathy. In this paper, we review the underlying mechanisms of death and discuss the strengths and weaknesses of current international guidelines for the identification and treatment of high-risk patients.
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PMID:Prevention of hypertrophic cardiomyopathy-related deaths: theory and practice. 1921 66

Only three facioscapulohumeral muscular dystrophy (FSHD) patients have been reported to have cardiomyopathy. An asymptomatic 38-year-old man was incidentally found to have electrocardiographic abnormalities. His echocardiogram demonstrated mild dilatation of the left ventricle and poor contractility. Cardiac histopathology indicated hypertrophic cardiomyopathy. Later he developed muscle weakness in the right arm. Scapular winging and asymmetrical facial weakness were evident. Muscle biopsy at the age of 44 years showed myopathic changes consistent with FSHD. His daughter had symptoms of infantile FSHD, which was genetically confirmed. This is the first report of an FSHD patient with biopsy-proven cardiomyopathy.
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PMID:Facioscapulohumeral muscular dystrophy presenting with hypertrophic cardiomyopathy: a case study. 1914 53

Our aim was to compare the frequency and distribution of late gadolinium enhancement (LGE) on contrast MRI between hypertrophic cardiomyopathy (HCM) patients with apical hypertrophic cardiomyopathy (APH) and those with asymmetrical septal hypertrophy (ASH). We studied 66 patients with HCM (50 men and 16 women; average age: 58.8 +/- 29.8 years) who had undergone MRI. All the MRI examinations were performed using a 1.5 T system. LGE images were acquired using the inversion recovery segmented spoiled-gradient echo and phase-sensitive inversion recovery methods. We evaluated 17 segments of the left ventricle as defined by the American Heart Association criteria for LGE determination. LGE was detected at the junction of the right ventricle and the interventricular septum in 25 (73.5%) of the 34 HCM patients with ASH and in the apex of the heart in 13 (40.6%) of the 32 patients with APH. LGE-positive areas were more widely distributed in the case of the ASH group than in the case of the APH group. The distribution of LGE was clearly different between the two groups (Fisher's exact probability test, P = 0.0068). The number of LGE-positive cases and LGE-positive segments were significantly higher in the ASH group than in the APH group and there was a significant difference in the distribution of the LGE-positive areas between the two groups. LGE was mainly detected in the hypertrophied areas of the myocardium.
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PMID:Frequency and distribution of late gadolinium enhancement in magnetic resonance imaging of patients with apical hypertrophic cardiomyopathy and patients with asymmetrical hypertrophic cardiomyopathy: a comparative study. 1916 22


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