[Ventricular septal defect and aortic regurgitation-pathophysiological aspects and therapeutic consequences].

The development of aortic regurgitation (AI) is a rare but serious complication of subaortic ventricular septal defects (VSD). Over a period of 5 years we observed VSD-related AI in 24 patients, a frequency of 4.5% of all isolated VSD's encountered during that time frame.

Effective doses to patients from paediatric cardiac catheterization.

The Council Directive of the European Communities 97/43/Euratom requires dose assessment, especially for X-ray examinations of children and if high doses to the patient are involved. Both these aspects apply in cardiac catheterization and angiocardiography of children. Effective doses are a good indicator of radiation risk, particularly for leukaemia.

[The incidental heart sound. Study of clinical diagnostic certainty and possible causes].

The diagnosis "innocent murmur" is made when there is no evidence for a heart disease either by history nor by examination, auscultation, electrocardiogram or echocardiography. The purpose of the present study was to determine the usefulness of echocardiography for diagnosing of an innocent heart murmur and further to compare the findings of ultrasound with those of the clinical examination. Children were accepted for the study after clinical examination and evaluation of the electrocardiogram (but before echocardiography) only when two independent pediatricians did not realize any symptoms of a heart disease.

Pharmacological characterization of the imidazoline receptor which mediates inhibition of noradrenaline release in the rabbit pulmonary artery.

Imidazoline receptors involved in modulation of noradrenaline release were characterized in the rabbit pulmonary artery preincubated with [3H]noradrenaline and superfused with physiological salt solution containing cocaine, corticosterone and propranolol. Tritium overflow was evoked by transmural electrical stimulation. The alpha 2-adrenoceptor blocking imidazolines tolazoline, BDF 6100 [2-(2-imidazoline-2-ylamino)-isoindoline] and BDF 7572 (4,7-dichloro-derivative of BDF 6100) increased the electrically evoked 3H overflow; the concentration-response curves were bell-shaped.

[ECG changes following balloon dilatation of pulmonary valve stenoses].

To evaluate the role of electrocardiography in the long-term follow up of children after balloon valvuloplasty of pulmonary stenosis the ECG findings of ten children (4 girls and 6 boys) with a mean age of 7.8 +/- 3.3 years who underwent valvuloplasty of pulmonary stenosis were studied.

Balloon valvuloplasty of pulmonary stenosis in infants and children--co-operative study of the German Society of Pediatric Cardiology.

Balloon valvuloplasty (BVP) of pulmonary stenosis (PST) was performed in 305 infants and children in 20 heart centres of the Federal Republic of Germany. 273 patients suffered from isolated PST. Their age ranged from 3 days to 18 years (mean = 5.

[The QT syndrome in newborn infants].

The QT-syndrome-the hereditary prolongation of the QT-interval with or without concomitant deafness--may cause ventricular tachycardia or fibrillation and is therefore a life-threatening disease. The prognosis, however, can be considerably improved by a therapy with beta-blockers. For this reason, neonates of affected families should undergo cardiologic examination.

[Diagnosis and differential diagnosis of heart tumors in infancy and childhood].

The clinical features of 10 children, in whom the diagnosis of a cardiac tumor has been made, are reviewed. There were nonspecific systolic murmurs in 9 patients, depression of the S-T segment in the ECG of 7 children, and premature extrasystoles or supraventricular tachycardia in 4 children. X-ray always showed mild to moderate enlargement of the heart, but an abnormal contour could be detected only in 2 of the patients.

Facilitatory presynaptic angiotensin receptors on the sympathetic nerves of the human saphenous vein and pulmonary artery. Potential involvement in beta-adrenoceptor-mediated facilitation of noradrenaline release.

Spirally cut strips of human saphenous vein and pulmonary artery preincubated with 3H-noradrenaline were superfused in the presence of corticosterone and desipramine or cocaine. In the saphenous vein angiotensin I, angiotensin II and angiotensin III concentration-dependently increased the electrically (2 Hz) evoked tritium overflow (relative order of potency: angiotensin II greater than angiotensin I greater than angiotensin III). The angiotensin receptor antagonist saralasin displaced the concentration-response curve of angiotensin II to the right, and also blocked the facilitatory effect of angiotensin III.

Further evidence for the involvement of cyclic AMP in Ca2+-dependent, but not Ca2+-independent, noradrenaline release in the rabbit pulmonary artery.

Strips of the rabbit pulmonary artery were incubated with 3H-noradrenaline and subsequently superfused. In strips superfused with Ca2+-free solution containing K+ 64.7 mmol/l, tritium overflow was stimulated by introduction of Ca2+ 1.

[Changes in blood pressure in children with valvular aortic stenosis in bicycle ergometer stress].

Children with aortic stenosis (n = 25) show a lower systolic blood pressure elevation during exercise than normal subjects (n = 47). The post-exercise systolic blood pressure minus the pre-exercise systolic pressure was calculated in both groups (23.60 +/- 10.

Noradrenaline release in the human pulmonary artery is modulated by presynaptic alpha 2-adrenoceptors.

We used strips of human pulmonary arteries from patients undergoing surgery for lung tumor to investigate whether or not this human tissue (like that of the rabbit) is endowed with inhibitory presynaptic alpha 2-adrenoceptors. The strips were incubated with [3H]noradrenaline and subsequently superfused with physiological salt solution containing cocaine, corticosterone, and propranolol. The electrically (2 Hz) evoked overflow of tritium consisted of 89% unmetabolized [3H]noradrenaline and was abolished by tetrodotoxin or omission of Ca2+ from the superfusion fluid.

Involvement of cAMP in modulation of noradrenaline release in the human pulmonary artery.

After incubation with 3H-noradrenaline, strips of human pulmonary arteries from patients undergoing surgery for lung tumour were superfused with physiological salt solution containing cocaine and corticosterone. Forskolin, AH 21-132 (a cAMP phosphodiesterase inhibitor), 8-Br-cAMP and isoprenaline did not affect the basal tritium efflux from the strips, but produced a concentration-dependent facilitation of the tritium overflow evoked by transmural electrical stimulation (2 Hz). The facilitatory effect of isoprenaline was potentiated by forskolin which produced a shift to the left of the concentration-response curve of isoprenaline.

Identification of presynaptic beta 2-adrenoceptors on the sympathetic nerve fibres of the human pulmonary artery.

Strips of human pulmonary arteries from patients undergoing surgery for lung tumour were incubated with [3H]-noradrenaline. Subsequently, they were superfused with physiological salt solution containing cocaine and corticosterone. Tritium overflow from the strips was stimulated by transmural electrical impulses (2 Hz).

[Echocardiographic studies in children with chronic kidney insufficiency and intermittent hemodialysis treatment].

Cardiac function was measured in 10 children (mean age 12 years) undergoing chronic intermittent hemodialysis. Blood pressure, heart rate and body weight were checked three times before and after hemodialysis. Echocardiographic studies were performed according to the references of the American society of echocardiography.

Partial replacement of the left ventricular wall for a large intramural fibroma.

A case of successful operative treatment of an intramural fibroma of the left ventricular posterior wall is presented. Symptomatology, diagnosis, and a surgical procedure based on ventricular replacement using a doubled Dacron patch, fixed in "sandwich" technique, are discussed. It is possible to resect and replace large parts of the ventricular wall because of the well-developed compensatory capacity of the remaining unaffected myocardium in children.

Role of cAMP for regulation of impulse-evoked noradrenaline release from the rabbit pulmonary artery and its possible relationship to presynaptic ACTH receptors.

Strips of the rabbit pulmonary artery preincubated with 3H-noradrenaline were superfused with physiological salt solution containing cocaine, corticosterone and propranolol. Basal tritium efflux and electrically evoked tritium overflow were determined. The basal efflux of tritium was not affected by forskolin 0.

[Unilateral proximal aplasia of the pulmonary artery--studies on the clinical significance and embryologic interpretation].

Description and discussion of the findings in a five month old infant and a six year old boy, in whom unilateral absence of the pulmonary artery contralateral to the aortic arch could be demonstrated by means of angiocardiography. The infant, having absence of the right pulmonary artery associated with patent ductus arteriosus and obstructive pulmonary vascular disease of the left lung died after recurrent pulmonary infections in acute heart failure. The boy with absence of the left pulmonary artery could be shown to have moderate-severe peripheral stenosis at the origin of the right pulmonary artery from the main pulmonary artery.

[Dilated cardiomyopathy in childhood: intravital differentiation of endocardial fibroelastosis using transvascular endomyocardial biopsy].

Possibly due to the lack of clinical reports concerned with dilated cardiomyopathy in childhood, pediatric cardiologists may inadvertently designate the angiographic finding of a dilated left ventricle as endocardial fibroelastosis. We report historical and noninvasively-obtained data from twelve children, aged seven months to 17 years, with poorly-functioning dilated left ventricles, in whom no differentiation between the two diseases was enabled. Only by means of transvascular endomyocardial biopsies, performed during cardiac catheterization with a 6-F guide-catheter and a Machida bioptome for light and electron microscopal examination, was differentiation achieved among six patients with endocardial fibroelastosis and six with the nonspecific histological findings of muscular hypertrophy, interstitial fibrosis and marked degenerative changes.

Modulation of noradrenaline release in human saphenous vein via presynaptic alpha 2-adrenoceptors.

Strips of human saphenous veins were incubated with [3H]noradrenaline and subsequently superfused with physiological salt solution containing cocaine, corticosterone and propranolol. The electrically (6 Hz) evoked overflow of tritium (78% of which was accounted for by unmetabolized [3H]noradrenaline) was abolished by tetrodotoxin or omission of Ca2+ from the superfusion fluid. Unlabelled noradrenaline, alpha-methylnoradrenaline, B-HT 920 and clonidine inhibited the evoked overflow (maximum effect of clonidine lower than that of the other compounds) whereas methoxamine was ineffective.

[Risks in heart catheterization and angiocardiography in infancy and childhood].

The results of cardiac catheterization with or without angiocardiography in 689 infants and 1311 children and adolescents were reviewed. In the first week of life 14,81% of the patients died within or after 24 hours of the procedure, 7,41% in the first month of life, 2,78% in the first year. And on the whole the mortality was 0,95%.

[Syndrome of supravalvular aortic stenosis (Williams-Beuren syndrome) in association with changes in the kidney and efferent urinary tract].

We investigated the urinary tract in 19 out of 30 children having the Williams-Beuren-Syndrome. 14 of these children showed all signs of the syndrome, whereas 8 children had only the typical cardiological findings without the pathognomonic facies and without major mental retardation. These two different types will be designed as type I and type II respectively.

[Recent viewpoints on the examination and therapy of infants and children with congenital cardiovascular defects].

Basic methods for the examination including echocardiography are described. Indications for cardiac catheterization, angiography, and a description of the risk of these invasive methods are given. Cardiac catheterisation is necessary as early as possible in all cyanotic infants who are suspected to have heart defects - even in newborns.