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Biomedical subjects

M Schlemmer

Publications and source records attributed to M Schlemmer.

At least 37 records · Page 2Linked to original sources

[L-carnitine therapy and myocardial function in children treated with chronic hemodialysis].

Six out of 14 chronically hemodialysed children with significantly decreased serum carnitine levels were substituted with L-carnitine (15-30 mg/kg/day-Biocarn) up to normal and above normal carnitine levels. None of these patients were digitalised. During the time of investigation plasma carnitine levels were investigated monthly and, simultaneously, three echocardiographic parameters in M-mode were quantitatively und qualitatively determined: shortening fraction (SF-%), ratio of left ventricular pre-ejection/ejection time (LVPT/LVET) and velocity of circumferential fibre shortening (Vcf). Carnitine substitution produced measurable changes in echocardiographic parameters, and a significant quantitative improvement in left ventricular function and performance: after 6 months of carnitine substitution a 24% improvement was seen, after 18 months a 44% mean improvement. No side effects of carnitine were observed; the compatibility was good. Beside these positive effects all patients reported decreased dialysis-associated spasms and polyneuropathic symptoms and increased somatic ability.

Administration, Oral↗

[Value of myocardial scintigraphy of coronary anomaly in the differential diagnosis of pediatric cardiomegaly].

The differentiation between anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) and other causes of cardiomegaly and thereby the decision to operate, as opposed to undertaking conservative therapy is often extremely difficult in clinical practice. Electrocardiographically, signs of myocardial infarction can be missed in ALCAPA, other forms of dilative cardiomyopathy (DCMP) often showing similar clinical features. Up to now aortography alone can make a clear-cut distinction between these two entities. In order to determine the diagnostic value of thallium-201 myocardial scintigraphy in ALCAPA, we investigated 18 children, 7 of them with angiographically or autoptically established ALCAPA and 11 patients with DCMP. The coronary artery anomaly was diagnosed on the basis of the typical myocardial perfusion defects in only 3 of the relevant 7 patients. In 9 of the 11 patients with DCMP ALCAPA was excluded by the homogeneous perfusion pattern or small focal reduction in thallium-201 activity in the left ventricular muscle mass. In view of the overall disappointing results of this method in achieving a specific diagnosis in ALCAPA, all patients with DCMP with or without suspected fibroelastosis should undergo invasive diagnosis with aortography.

Adolescent↗

[Immunosuppressive treatment of chronic myocarditis].

6 children aged 9 months to 13 8/12 years developed reduced physical capacity, failure to thrive, dyspnoea on effort and heart failure after viral infection. Bed rest and anticongestive therapy did not reduce the severe heart failure. Immunosuppressive therapy with prednisolone 2 mg/kg/d and azathioprine 2 mg/kg/d was added on the assumption of chronic myocarditis (n = 4) or endocardial fibrosis (n = 2). 3 of the 6 children showed a considerable improvement in heart size, ECG and systolic heart function. Interruption of immunosuppressive therapy due to side effects in one child led to cardiac deterioration, which was reversed by renewed prednisolone therapy. Two patients died of progressive congestive heart failure. Heart transplantation was successfully performed in the now 14 year-old girl who was suffering from terminal dilated cardiomyopathy. Since there is no specific test for selecting patients who are likely to benefit from immunosuppressive therapy we feel this regimen would be justified in hopeless cases of congestive cardiomyopathy who have not responded to conventional therapy with anti-congestive drugs.

Adolescent↗

Hemodynamic effects of nifedipine and oxygen in children with pulmonary hypertension.

Fourteen patients, 2 to 20 years old were investigated. Two had primary pulmonary hypertension, 11 had congenital heart disease and post-tricuspid shunts, and 1, a 20-year-old patient, was investigated after he had undergone surgical correction of truncus arteriosus I. Pulmonary arterial pressure, pulmonary flow index, peripheral systolic blood pressure and heart rate were measured before, and several times after intrapulmonary injection into the pulmonary artery of 0.5 microgram nifedipine/kg. Six patients were given an additional dose of 1 microgram nifedipine per kilogram into the pulmonary artery and hemodynamic measurements were repeated. In eight children, receiving 100% oxygen via a breathing mask, nifedipine effects were compared with oxygen effects. After 10 minutes under oxygen, the same hemodynamics were determined as after nifedipine. In addition, in four of these children aortic pressure and arterial oxygen saturation were also measured. Maximal effects occurred within 4 minutes. 0.5 micrograms nifedipine per kilogram caused a slight reduction in mean pulmonary arterial pressure (p less than 0.05), as well as increase in pulmonary flow index (p less than 0.005). However, no significant change in heart rate or in systolic blood pressure was observed. 1 microgram nifedipine per kilogram IP had almost the same effects. No adverse side effects occurred, besides mild headaches in one child. A comparison of nifedipine injected into the pulmonary artery with oxygen breathing in congenital heart disease combined with pulmonary hypertension, is reported for the first time. Nifedipine had a more pronounced and beneficial effect with a selective action on the pulmonary vascular bed.

Adolescent↗

Dysrhythmias following the Mustard and Senning operation for transposition of the great arteries.

The prevalence and nature of late dysrhythmias following operative repair of transposition of the great arteries were evaluated in 32 children using pre- and postoperative standard electrocardiograms and postoperative Holter monitoring. The Mustard procedure was performed in 17 patients, the Senning procedure in 15 patients. The mean age at operation was 40 +/- 35 months in the Mustard and 9 +/- 6 months in the Senning group. The mean interval between operation and Holter monitoring 7 +/- 2 years in the Mustard and 1.6 +/- 1 years in the Senning group. All patients had sinus rhythm preoperatively. Following operation, the incidence of sinus rhythm increased from 59% in the Mustard and 87% in the Senning group in the first postoperative week to 76% in the Mustard and 100% in the Senning group at the end of follow-up (mean 4.6 +/- 3.5 years). Holter monitoring revealed dysrhythmias or conduction abnormalities in 12 patients (9 Mustard, 3 Senning). A permanent pacemaker had to be inserted in 2 Mustard patients because of complete atrioventricular block and sinus bradycardia with sinus arrest, respectively. Results indicate a prevalence of late postoperative dysrhythmias of 20% in the Senning and of 59% in the Mustard group (p less than 0.05). However, the significantly longer mean follow-up period of the Mustard group precludes a direct comparison between the 2 types of repair.

Arrhythmias, Cardiac↗

[Hemodynamic studies of nifedipine (Adalat) in pulmonary hypertension in childhood].

Under long-term oral treatment with nifedipine (N) 2 of 3 patients (2 children with VSD and Eisenmenger, 1 patient with Truncus I after corrective surgery) showed a remarkable improvement in exercise tolerance and reduction in the total pulmonary resistance. Therefore a single-dose testing with N was performed in 9 patients aged 2 to 20 years with primary pulmonary hypertension (n = 2), secondary pulmonary hypertension because of congenital heart disease with intracardiac shunts (n = 6) and after correction of Truncus Type I (n = 1). Before and after the injection of 0.5 mcg/kg of N into the main pulmonary artery, pulmonary artery pressure, cardiac output and systolic blood pressure were measured at 1 minute intervals during right heart catheterization. The maximal reaction occurred 3 to 4 minutes after the injection. The total pulmonary vascular resistance decreased significantly from 2684.2 +/- 1829.2 to 1300.2 +/- 1117.0 dyn.sec.cm-5.m2 (p less than 0.025), the cardiac index increased significantly from 3.1 +/- 1.65 to 5.73 +/- 1.95 l/min.m2 (p less than 0.01) concomitantly with a mild reduction in the mean pulmonary artery pressure. No significant change in heart rate and systolic blood pressure was seen; no side effects were observed. 4 of the 5 patients with the highest pulmonary vascular resistance showed the best positive reactions. Therefore these 9 patients may benefit from a long-term oral treatment with nifedipine.

Adolescent↗

[Doppler echocardiographic evaluation of pressure in the pulmonary artery in children with congenital heart defects].

In children with congenital heart disease serial noninvasive assessment of the pulmonary vascular bed is desirable in order to determine the appropriate timing of cardiac catheterization and corrective surgery. To assess the value of pulmonary Doppler echocardiography for the estimation of pulmonary arterial pressure we correlated the rightsided systolic time intervals (preejection period, acceleration period, ejection period) derived from pulmonary artery Doppler traces with catheterization data (systolic, mean and diastolic pulmonary arterial pressure, total and vascular pulmonary resistances). 62 children aged from 1 month to 15 years suffering from congenital heart disease (n = 52), from rheumatic heart disease (n = 4), from cardiomyopathy (n = 5) or from primary pulmonary hypertension (n = 1) were investigated. The major finding was a highly significant correlation between the acceleration period and the systolic pulmonary arterial pressure (r = 0.79; p less than or equal to 0.0001). However the acceleration period could not be used for prediction of pulmonary arterial pressure in children with poor myocardial contractility.

Adolescent↗

[Rare cause of a left ventricular aneurysm in a 10-year-old boy].

A 3-year-old boy had an accident with a blunt chest trauma, 9 years later a left ventricular aneurysm was diagnosed by echocardiography and angiography. The 12-year-old-boy was operated successfully. The pathogenesis of cardiac lesions in association with blunt chest traumas is described, and frequent control examinations in the affected patients are recommended.

Adolescent↗

[Idiopathic enlargement of the right atrium].

In a 3 year old male patient with a very rare congenital enlargement of the right atrium the diagnosis was made only because of an occasional chest X-ray film. These dilated right atria rarely cause symptoms, but in rhythmic disorders or thrombosis they can be life threatening. The differential diagnoses of this entity are listed.

Arrhythmias, Cardiac↗

[Radiological examination of the thoracic organs for persistent foetal circulation (author's transl)].

The radiological changes seen on chest x-rays of five children with persistent foetal circulation are analysed. There is inconstant cardiac enlargement and a reticulo-nodular appearance of the lungs, indicating interstitial oedema with heart failure. The value of the chest x-ray consists mainly in excluding a pulmonary or a characteristic cardiac abnormality as a cause of dyspnoea; it does not provide direct diagnostic information.

Angiocardiography↗

[Mexiletine in the treatment of ventricular arrhythmias (author's transl)].

The indication and efficacy of mexiletine is described in five patients. In four children the first signs of arrhythmias were seen a short time after viral infection. Two girls had numerous ventricular extrasystoles, which showed very promptly a positive reaction to mexiletine. Two patients had ventricular tachycardias beside ventricular premature beats. Because of signs of cardial insufficiency a 13 year old boy was defibrillated before his cardiac rhythm could be established with mexiletine. Because of ventricular tachycardias the second patient was treated with a combination of mexiletine and propranolol. Seven years after corrective operation of tetralogy of Fallot a nine year old boy showed ventricular extrasystoles. Because of a very low plasma concentration of mexiletine the therapy was without any effect. In all children, a long term ECG over 24 hours was deduced. To control the efficacy of mexiletine in all patients the plasma-concentration was determined. The therapeutic effective plasma concentration of mexiletine lays between 0.5 and 2,5 mcg/ml. This level can be reached by a daily dose of 12.5--16.2 mg/kg body weight.

Arrhythmias, Cardiac↗

[Prostaglandin E2 in newborns with congenital heart disease].

Prostaglandins of the E-type are potent ductus dilatators not only in vitro but also in vivo, as could be shown in the former literature. We report on 20 newborn children, in whom we only used prostaglandin E2 to perform a better lung or kidney perfusion by opening the ductus arteriosus. The diagnoses of the children were: pulmonary atresia + ventricular septal defect +/- transposition of the aorta, tricuspid atresia, hypoplastic left heart syndrome, transposition of the great arteries +/- ventricular septal defect, coarctation of the aorta and tetralogy of Fallot. The dosis pattern of the prostaglandin E2 infusion was in 17 patients the same, starting with 0.1 microgram/kg body weight/min and consecutive reduction depending on the capillary pO2, in the last 3 patients we started with 0.05 microgram/kg body weight/min. In 7 patients we gave an oral preparation of prostaglandin E2 intermittently. The duration of treatment was between 15 min and 37 days. There was a significant increase of the capillary pO2 under prostaglandin E2 treatment (p less than 0.0001), the increase of capillary pO2 correlates negatively with the initial pO2 before treatment (p less than 0.05), but there is no dependence on the age of the children. The side effects of this therapy are discussed, especially the dangerous apnoic spells.

Administration, Oral↗