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1/12. amiodarone treatment in pregnancy for dilatative cardiomyopathy with ventricular malignant extrasystole and normal maternal and neonatal outcome.

    amiodarone treatment in pregnancy might be difficult to handle because of the long half-life of the drug (14-28 days up to 2 months) and because it reduces maternal and neonatal thyroid activity. Although short-term use in pregnancy has been described in cases of fetal supraventricular tachycardia, there are few reports on the chronic use of the drug. In this paper we describe our experience with amiodarone treatment in two pregnant sisters with familial dilatative cardiomyopathy and ventricular malignant extrasystole. Prolonged administration of amiodarone (400-200 mg/die) since the beginning of pregnancy did not have any adverse effects; maternal and neonatal thyroid function was normal, as was the neurological and motor development of the neonates.
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ranking = 1
keywords = cardiomyopathy
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2/12. Atrial parasystolic trigeminy with sinus node echoes during wandering sinus node pacemaker in a patient with malignant lymphoma and hypertrophic cardiomyopathy.

    A case of atrial parasystolic trigeminy associated with shifting or wandering pacemaker in a patient with malignant lymphoma and hypertrophic cardiomyopathy is presented, in which concomitant unsustained sinus node echoes during flattening of sinus P wave were observed. This is the first report to prove that wandering sinus pacemaker was one of the causes of sinus echoes.
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ranking = 1
keywords = cardiomyopathy
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3/12. Radiofrequency ablation of symptomatic but benign ventricular arrhythmias.

    Two cases are presented where ablation of severely symptomatic ventricular arrhythmias not responding to medical therapy was accomplished with radiofrequency current application. After a routine programmed stimulation protocol, a quadripolar ablation catheter with a 4-mm tip was advanced percutaneously into the left ventricle in one case and into the right ventricle in the second case; and after precise pace mapping, the arrhythmogenic focus was successfully ablated using radiofrequency current. The postablation ambulatory recording revealed virtual eradication of ventricular ectopy in both cases. In conclusion, in severely symptomatic cases of "benign" ventricular arrhythmias, radiofrequency ablation offers an effective therapeutic alternative.
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ranking = 54.244811612332
keywords = arrhythmogenic
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4/12. Improvement of ventricular arrhythmia by octreotide treatment in acromegalic cardiomyopathy.

    We report a case of acromegalic cardiomyopathy in a 46-year-old Japanese man with pituitary adenoma. Increased secretion of growth hormone and insulin-like growth factor I were detected. He had left ventricular hypertrophy, impaired cardiac function, and frequent ventricular premature complexes. After 2-month treatment with octreotide, a long-acting somatostatin analogue, levels of both hormones were decreased. At the same time, left ventricular hypertrophy (intraventricular septal thickness: 22.5 to 17.8 mm), cardiac function (ejection fraction: 38 to 50%), and frequency of ventricular premature complexes (17,249 to 2,882 beats a day) were improved. Transsphenoidal surgery was then safely performed. Treatment with octreotide is thought to have some effect on improvement of ventricular arrhythmia in acromegalic heart.
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ranking = 1
keywords = cardiomyopathy
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5/12. Two cases of bi-ventricular dysplasia associated with ventricular tachycardia and familial occurrence of sudden death.

    Two strikingly similar patients with arrhythmogenic right ventricular dysplasia which severely impaired not only the right ventricle but also the left ventricle are described in association with familial occurrence of sudden death. A 49-year-old man experienced syncope which was due to ventricular tachycardia. electrocardiography revealed a first degree atrioventricular block, incomplete right bundle-branch block, T wave inversions in leads II, III, a VF and V1 to V5, and multiformal ventricular extrasystoles. echocardiography and ventricular cineangiography showed not only the right ventricular dilatation with an aneurysm in the right ventricular apex, inflow and outflow tracts, but also mild dilatation of the left ventricle with left ventricular apical and posterior aneurysms. radionuclide angiography also disclosed dysfunction of both ventricles, especially during exercise. His family history revealed that 3 members of his family died of sudden deaths. A 56-year-old woman experienced syncope secondary to ventricular tachycardia, with left bundle-branch block. electrocardiography showed complete right bundle-branch block, left axis deviation, and T wave inversions in leads V1 to V4. echocardiography and ventricular cineangiography revealed not only marked right ventricular dilatation with the "triangle of dysplasia", but also a left ventricular aneurysm in the apex and posterior portion. Her elder brother died of a sudden death, and electrocardiograms of 2 members of her family showed ventricular extrasystoles and T wave inversions. These 2 cases may well be termed "familial bi-ventricular dysplasia".
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ranking = 1596.505666585
keywords = ventricular dysplasia, right ventricular dysplasia, arrhythmogenic right ventricular dysplasia, arrhythmogenic, arrhythmogenic right, dysplasia
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6/12. Evaluation of left ventricular residual function using postextrasystolic potentiation. Relation between systolic time intervals and angiographic study.

    The effects of postextrasystolic potentiation (PESP) on systolic time intervals and left ventricular wall motion were studied during diagnostic cardiac catheterization in 20 patients (4 normal individuals, 11 patients with coronary artery disease and 5 patients with idiopathic dilated cardiomyopathy). Postextrasystolic changes in the aortic pressure and systolic time intervals were measured from the electrocardiogram and aortic pressure tracing. After a micromanometer-tipped catheter was positioned in the ascending aorta just above the aortic valve, a single ventricular premature beat was introduced using an R-wave coupled stimulator. PESP was then studied during left ventriculography which was undertaken simultaneously in the right anterior oblique 30 degrees and left anterior oblique 60 degrees positions. Following two or three normal sinus beats, a right ventricular extrastimulus was delivered again under the same stimulating condition. PESP in all patients caused a decrease in the ratio of the preejection period to the left ventricular ejection time (PEP/ET). The average percent decrease was 21% (from 0.429 /- 0.162 to 0.339 /- 0.102, p less than 0.001). The left ventricular ejection fraction (EF) increased in all patients with PESP from 0.52 /- 0.20 to 0.61 /- 0.17 (p less than 0.001). The postextrasystolic changes in the PEP/ET ratio and EF were greater in patients with low cardiac performance. There was a good correlation (r = -0.85, p less than 0.001) between the changes in the EF and those in PEP/ET in PESP. Thus, it is possible to determine left ventricular residual function (the postextrasystolic change in the global EF) using the postextrasystolic change in PEP/ET in patients with coronary artery disease and dilated cardiomyopathy.
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ranking = 0.4
keywords = cardiomyopathy
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7/12. Ventricular arrhythmias in children and adolescents.

    The spectrum of ventricular arrhythmias, as observed in adolescents and children, is reviewed. The significance and prognosis of such rhythm disturbances depend to a great extent on the clinical setting in which they occur. Ventricular premature beats (VPBs) are found in a significant percentage of normal adolescents, though usually in very small numbers over 24 h. Frequent unifocal VPBs, even in bigeminy, may occasionally be observed. They are considered benign in the absence of heart disease, especially when they disappear during exercise. Ventricular tachycardia (VT) appears as a complication of some heart lesion in a majority of cases. This may be cardiomyopathy, mitral valve prolapse, sometimes congenital heart defects or occasionally rare anomalies such as long Q-T syndrome, tumors, or right ventricular dysplasia. Idiopathic (primary) VT also occurs in the young, its overall prognosis is more favorable compared to VT with heart disease. The problem of ventricular arrhythmias late after open heart surgery deserves special comment, as it seems to be connected with late sudden death. The investigation and treatment of ventricular arrhythmias in children and adolescents is discussed. The decision on who should be treated is easy in the presence of symptoms, but difficult and controversial in asymptomatic youngsters.
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ranking = 437.68016444154
keywords = ventricular dysplasia, right ventricular dysplasia, cardiomyopathy, dysplasia
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8/12. Two-dimensional echocardiographic diagnosis of arrhythmogenic right ventricular dysplasia presenting as frequent ventricular extrasystoles in a child.

    Two-dimensional echocardiographic investigation of a child with frequent isolated ventricular extrasystoles revealed an area of right ventricular dysplasia. This finding may signify a less favorable prognosis and justifies careful echocardiography in the evaluation of asymptomatic ventricular extrasystoles of left bundle branch block morphology in children.
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ranking = 2933.8058143288
keywords = ventricular dysplasia, right ventricular dysplasia, arrhythmogenic right ventricular dysplasia, arrhythmogenic, arrhythmogenic right, dysplasia
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9/12. arrhythmogenic right ventricular dysplasia: case report presentation and review of the literature.

    A man complaining of palpitations was found to have ventricular tachycardia (VT) with LBBB configuration. From the investigations which followed, he was diagnosed as having arrhythmogenic right ventricular dysplasia (ARVD). The patient has been treated with amiodarone and propafenon for 7 months without VT recurrence. ARVD and Uhl's anomaly, which is its most extreme form, may be familial and represent an important cause of sudden death among young people: Prophylactic antiarrhythmic therapy and sometimes surgical treatment are required in case of refractory VT.
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ranking = 2374.002070238
keywords = ventricular dysplasia, right ventricular dysplasia, arrhythmogenic right ventricular dysplasia, arrhythmogenic, arrhythmogenic right, dysplasia
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10/12. disopyramide-induced ventricular tachycardia.

    A patient with ischemic cardiomyopathy and congestive heart failure was treated with disopyramide phosphate for ventricular arrhythmias. The development of ventricular tachycardia was associated with elevated plasma disopyramide levels and prolongation of the QRS and QT intervals, suggesting a disopyramide-induced depression of ventricular conduction and a prolongation of recovery. Thus, in patients with congestive heart failure, as well as in patients with renal and/or hepatic dysfunction, special care must be exercised with respect to the use of disopyramide.
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ranking = 0.2
keywords = cardiomyopathy
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