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Biomedical subjects

B Schwartzkopff

Publications and source records attributed to B Schwartzkopff.

79 records · Page 5Linked to original sources

Cardiac amyloid deposits in endomyocardial biopsies. Light microscopic, ultrastructural, and immunohistochemical studies.

In four patients with unexplained, abnormal thickening of the interventricular septum as demonstrated by echocardiography, right ventricular endomyocardial biopsy revealed unexpected cardiac amyloid deposits that resulted in increased myocardial thickness and rapidly progressive heart failure. Light microscopically, amyloid was observed in the subendocardial layer, interstitium, and walls of the intramural arterioles. Electron-microscopically, the amyloid fibrils were adjacent to the basement membranes of the heart muscle cells and the vascular smooth muscle cells. Immunohistochemical typing with specific antibodies against different amyloid fibril proteins on glutaraldehyde-fixed paraffin sections revealed different amyloid types. In two patients with generalized idiopathic amyloidosis and in two others with amyloidosis in multiple myeloma, the A-lambda form was diagnosed. In a fifth patient, AA-amyloidosis was found in familial Mediterranean fever with cardiac manifestation without thickening of the interventricular septum. The amyloid deposits were located almost exclusively within the walls of the myocardial arterioles. The amount of amyloid as observed in the myocardial biopsies correlates with the rapidly progressive cardiac failure. It is suggested that in patients with abnormal thickening of the interventricular septum of unknown origin the diagnosis should be clarified by endomyocardial biopsy.

Adult↗

[Heart involvement in progressive external ophthalmoplegia (Kearns-Sayre syndrome): electrophysiologic, hemodynamic and morphologic findings].

The Kearns-Sayre syndrome is combined with a progressive external ophthalmoplegia (PEO), retinal pigmentary degeneration and heart block. In some patients, progression of incomplete forms has been described and potentially fatal conduction disturbances may occur. The disease is considered as a systemic mitochondrial disorder. As part of an ongoing prospective study 6 patients (3 female, 3 male; age 32 +/- 9 years) with PEO and typical ultrastructural changes of a mitochondrial myopathy in their skeletal muscle were examined. The ECG disclosed atrio/intraventricular conduction defects in 5 patients: 1 patient had a third degree AV block which was treated by a pacemaker. Another patient had left anterior fascicular block with complete right bundle branch block. In 3 other patients an incomplete right bundle branch block was registered. In 1 patient, His-bundle electrography disclosed a block distal to His by atrial high rate pacing before and after i.v. injection of ajmaline; prophylactically a pacemaker was implanted. The mean HV-interval increased significantly under ajmaline by 44% in patients with sinus rhythm. Hemodynamic studies were normal in 5 patients at rest; only 1 patient was abnormal during exercise. Myocardial biopsy demonstrated mitochondrial abnormalities such as variability in shape and size. In 4 patients concentric cristae mitochondriales were seen. Our results suggest that atrioventricular conduction defects are common in patients with PEO. By means of endomyocardial catheterbiopsy a mitochondrial cardiomyopathy could be detected.

Adolescent↗

Comparison of tocainide and prajmalium bitartrate for the treatment of ventricular arrhythmias.

The antiarrhythmic efficacy of tocainide, a new antiarrhythmic substance, has been compared with that of prajmalium bitartrate, a drug in clinical use for many years in the German speaking countries. The investigation was performed as a double-blind cross-over study in 20 patients with ventricular arrhythmias (VA) of various origin. The efficacy was assessed by serial Holter monitoring. Plasma levels were measured to study the dose-effect relationship. Applying to the criterion of a reduction of VA of more than 75% or an improvement according to the Lown grading 8 pts. under tocainide and 7 under prajmalium bitartrate were responders. Side effects were few and under tocainide only 2 pts. had to discontinue the therapy. From the present findings tocainide and prajmalium bitartrate have the same efficient antiarrhythmic effects in the majority of patients.

Adult↗

[Left ventricular basal fibrous myocardial aneurysm in a 3-week-old girl].

A congenital bulging of the myocardial wall is a rare finding; little is known about its etiology and clinical course. The case of a 3-week-old girl in whom rapidly progressive cardiac insufficiency occurred after a completely normal postnatal development is described. Selective ventricular angiography revealed ventricular malfunction and an akinetic area occupying 57% of the basal free left ventricular wall. The child died in acute cardiac failure. Autopsy disclosed a basal fibrous aneurysm which extended to 34% of the free ventricular wall. There was no indication of a coronary or inflammatory etiology, so a focal disorder of myocardial architecture was presumed. Congenital bulging of the ventricular wall can be divided into broad-based aneurysms and diverticula, the latter being connected with the ventricle by a small orificium. A further distinction can be made on the basis of localization and histology. A reclassification of the congenital bulging of the left ventricular wall presented in the literature showed that apical muscular diverticula are the most frequent, whereas only four cases of fibrous aneurysms were reported. Differences were found, with regard to the distribution of age and race, the clinical course, and the possible etiology.

Diagnosis, Differential↗

Physiological aspects of the role of human albumin in the treatment of chronic and acute blood loss.

Human albumin is the most important oncotic-active protein (1 g albumin attaches 18 g water). It is essential for the water exchange between intra- and extracellular space and for homeostasis. The physiological distribution of albumin, its daily exchange and degradation are being discussed. At the example of normo- and hypovolaemic patients and acute blood-loss the stabilising effect on the blood-volume and the hemodynamic efficacy of human albumin are shown. Human albumin was infused into patients with hypoproteinemia and hypovolaemia as well as to surgical patients with normovolaemia. Volunteers received albumin after an acute blood-loss. Under and after the albumin-infusion the albumin disappeared partly from the blood-stream. The loss to the extravascular compartments was greatest among patients with hypoproteinemia. Among volunteers with experimental blood-loss the infused volume disappeared in an amount of 45 to 106 ml per hour. When human albumin is given over a longer period the synthesis of endogenous albumin and of globulins may be inhibited or at least depressed. Albumin has a positive effect on the hemodynamic. The cardiac-output and the stroke volume increased. The peripheral resistance fell in the same time. Renal filtration rate and the urine volume increased, in contrast renal resistance was lowered.

Adult↗

Successful thrombolysis of st. Jude medical aortic prosthesis with tissue-type plasminogen activator in a pregnant woman: a case report.

Mechanical valve thrombosis is a life-threatening event. Pregnancy is associated with a hypercoagulable state that further emphasizes the importance of adequate anticoagulation. This is associated with a therapeutic dilemma. Continued anticoagulation with warfarin throughout the first trimester can result in fetopathic effects, while replacement of warfarin by heparin between 6 and 12 weeks of gestation does not completely prevent the risk of valve thrombosis. There are a small number of reported cases of pregnant women with prosthetic heart valve thrombosis under low molecular weight heparin and consecutive lytic therapy. The authors report a 33-year-old pregnant woman with a St. Jude Medical aortic prosthesis, anticoagulated with a therapeutic dosage of low molecular weight heparin from 6 weeks of gestation, who developed prosthetic heart valve thrombosis at 17 weeks of gestation. A thrombolysis with recombinant tissue-type plasminogen activator (50 mg for 2 hours) was performed. Under thrombolysis, ST-segment elevation in leads II, III, aVF, V5, and V6 developed electrocardiographically with a maximal creatine kinase (CK) of 349 U/L (CK-MB isoenzyme of 48 U/L). Echocardiography revealed normal function of the St. Jude Medical aortic prosthesis 2 hours after thrombolysis and normal wall motions. Short-course thrombolytic therapy appears to be an effective alternative to surgical intervention for the treatment of thrombotic dysfunction of valve prostheses in pregnancy.

Adult↗