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1/732. Epidural analgesia for labour and delivery in a parturient with congenital hypertrophic obstructive cardiomyopathy.

    We report a parturient delivering vaginally at term with symptomatic congenital hypertrophic obstructive cardiomyopathy. Epidural analgesia was used during labour and delivery and is likely to have made a useful contribution to the successful outcome. Although controversial, reported use of epidural analgesia during labour for hypertrophic obstructive cardiomyopathy parturients has been generally positive. A multi-disciplinary team approach, early anaesthetic assessment and a carefully managed epidural catheter inserted in early labour can optimize analgesia and minimize the stresses of labour and vaginal delivery provided the risks of reduced preload and afterload are minimized.
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2/732. Rapid progression of cardiomyopathy in mitochondrial diabetes.

    Cardiac involvement and its clinical course in a diabetic patient with a mitochondrial tRNA(Leu)(UUR) mutation at position 3243 is reported in a 54-year-old man with no history of hypertension. At age 46, an electrocardiogram showed just T wave abnormalities. At age 49, it fulfilled SV1 RV5 or 6>35 mm with strain pattern. At age 52, echocardiography revealed definite left ventricular (LV) hypertrophy, and abnormally increased mitochondria were shown in biopsied endomyocardial specimens. He was diagnosed as having developed hypertrophic cardiomyopathy associated with the mutation. However, at age 54, SV1 and RV5,6 voltages were decreased, and echocardiography showed diffuse decreased LV wall motion and LV dilatation. Because he had mitochondrial diabetes, the patient's heart rapidly developed hypertrophic cardiomyopathy, and then it seemed to be changing to a dilated LV with systolic dysfunction. Rapid progression of cardiomyopathy can occur in mitochondrial diabetes.
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keywords = cardiomyopathy
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3/732. A patient with hypertrophic cardiomyopathy accompanied by right ventricular dilation of unknown cause.

    Hypertrophic cardiomyopathy (HCM) is a disease characterized by an unknown cause of hypertrophy in the left or right ventricle. The dilated phase of HCM shows disease conditions resembling dilated cardiomyopathy, such as ventricular dilation, thin ventricular wall, and reduction of the ejection fraction. A patient presented with left ventricular concentric hypertrophy accompanied by right ventricular dilatation of unknown cause. Right ventricular endomyocardial biopsy specimens showed characteristic myocardial disarray. Therefore, there is the possibility that the patient had right and left ventricular HCM in the process toward the dilated phase, in which dilatation first occurred in the right ventricle.
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keywords = cardiomyopathy
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4/732. Resolution of neonatal hypertrophic cardiomyopathy in an infant with an affected mother.

    Hypertrophic cardiomyopathy (HCM) in infancy has been described in association with many medical disorders. The genetic location and mode of transmission of HCM in families also has been reported extensively. We present an infant with nonobstructive hypertrophic cardiomyopathy whose mother also had HCM. Regression of septal hypertrophy was documented in this patient by 1 year of age despite a positive family history.
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keywords = cardiomyopathy
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5/732. Pacemaker therapy in a pediatric patient with hypertrophic obstructive cardiomyopathy and rapid intrinsic atrioventricular conduction.

    A 13-year-old boy with hypertrophic obstructive cardiomyopathy was treated with dual-chamber pacing after severe progression of left ventricular outflow tract obstruction and of clinical symptoms despite drug therapy. Rapid intrinsic atrioventricular conduction was overcome and complete preexcitation of the septum achieved by omitting atrial sensing and programming constant atrial pacing with a short atrioventricular delay of 70 msec. After 8 weeks of therapy, a reduction of the left ventricular outflow tract gradient from 125 to 16 mmHg and remodeling of the left ventricle were demonstrated.
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ranking = 0.83333333333333
keywords = cardiomyopathy
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6/732. Diagnostic utility of metabolic exercise testing in a patient with cardiovascular disease.

    Disproportionate exercise limitation in patients with cardiovascular disease is a common problem faced by clinical cardiologists and other physicians. Symptoms may be attributed to psychological factors or hypothetical pathophysiological mechanisms that are difficult to confirm clinically. This case report describes how the use of metabolic exercise testing in a 28 year old woman with morphologically and haemodynamically mild hypertrophic cardiomyopathy and severe exercise limitation led to the diagnosis of an alternative cause for the patient's symptoms, namely a primary disturbance of the mitochondrial respiratory chain probably caused by a nuclear encoded gene defect.
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ranking = 0.16666666666667
keywords = cardiomyopathy
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7/732. Sudden death associated with group A streptococcal infection in an 8-year-old girl with undiagnosed hypertrophic cardiomyopathy.

    An 8-year-old girl died suddenly without prior symptoms. Post-mortem examination identified both systemic group A streptococcal infection and hypertrophic cardiomyopathy. She had no history of cardiac symptoms and was not in a high-risk group for sudden death due to hypertrophic cardiomyopathy. We believe the disseminated but asymptomatic group A streptococcal infection precipitated her early death from hypertrophic cardiomyopathy. Sudden unexpected death during systemic infection should be followed by post-mortem examination to look for evidence of hypertrophic cardiomyopathy, as this diagnosis has genetic implications for other family members.
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ranking = 1.3333333333333
keywords = cardiomyopathy
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8/732. Effect of disopyramide on left ventricular pressure gradient in hypertrophic obstructive cardiomyopathy in comparison with propranolol--a case report.

    The effect of intravenous administration of disopyramide (total dose 100 mg, bolus 20 mg every 5 minutes) was compared with that of propranolol (total dose 10 mg, bolus 2 mg every 5 minutes) in a patient with hypertrophic obstructive cardiomyopathy. Left ventricular pressure gradient (LVPG) was assessed by continuous wave Doppler flowmetry. LVPG markedly decreased (97 to 16 mmHg), and preejection period (PEP) increased with an increase in heart rate (HR) during disopyramide injection. No changes were observed in LVPG and PEP, and a decrease occurred in HR during propranolol administration. These results indicate that disopyramide produced greater effects on the reduction of LVPG than propranolol, a negative inotropic agent, did.
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ranking = 0.83333333333333
keywords = cardiomyopathy
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9/732. Fatal neonatal hypertrophic cardiomyopathy with severe stenosis of the right ventricular outflow tract: echocardiographic diagnosis and potential therapy.

    We discuss the clinical course of aneonate with idiopathic hypertrophic cardiomyopathy who showed rapid progression of stenosis of the right ventricular outflow tract and reduction in size of the right ventricular cavity due to thicken ing of the ventricular septum. Medical treatment proved unsuccessful. We suggest that balloon atrial septostomy combined with construction of a Blalock-Taussig shunt may be effective in view of the hemodynamics, which mimic pulmonary atresia with intact interventricular septum.
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ranking = 0.83333333333333
keywords = cardiomyopathy
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10/732. Nemaline myopathy and cardiomyopathy.

    A case report is presented in which a 4-year-old male is diagnosed with hypertrophic cardiomyopathy, respiratory distress, muscle hypotonia, and psychomotor retardation. Electron microscopic study of skeletal muscle biopsy revealed pathologic changes typical of congenital nemaline myopathy, and biochemical analysis revealed a disorder of mitochondrial fatty acid oxidation. Therefore a previously undescribed combination of a structural and metabolic myopathy is reported.
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ranking = 0.83333333333333
keywords = cardiomyopathy
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