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Biomedical subjects

E Steil

Publications and source records attributed to E Steil.

At least 19 recordsLinked to original sources

[Anomalous origin of the left coronary artery from the pulmonary artery. Variability of clinical aspects, echocardiography and angiography findings].

OBJECTIVES: Analysis of symptoms, diagnostical difficulties and follow-up in infants and children with anomalous origin of the left coronary artery from the pulmonary artery. METHODS: Retrospective study; 12 children between 3 weeks and 2 years old; time period: 1980-1991. RESULTS: Three infants were detected on routine examination because of a new cardiac murmur, the others presented with signs of cardiac failure. Cross sectional echocardiography and color Doppler flow mapping allowed to verify the suspected diagnosis. However, in one infant a false-negative cross sectional echocardiographic result was obtained. In this case nuclear magnetic resonance imaging was able to delineate the exact anatomy. Mean preoperative left ventricular ejection fraction: 33 +/- 4%; percentage of infants below the age of 6 months: 92%; surgery related mortality: 66%; mean follow-up of the remaining 4 patients being in good clinical condition: 2.9 +/- 1 years. CONCLUSIONS: 1. An anomalous origin of the left coronary artery should be included into the differential diagnosis when a new cardiac murmur is detected. 2. Possibility of false-negative echocardiographic results is emphasized. 3. With early symptoms and highly reduced left ventricular function, the mortality is still high.

Angiocardiography

Early and late results of the modified Waterston shunt with PTFE grafts for palliation of complex congenital cyanotic heart disease in neonates.

During a 12 year period from 1978 to 1989, 35 infants under 4 weeks of age underwent palliative surgery for complex congenital cyanotic heart disease with a short (1-1.5 cm) PTFE graft between the ascending aorta and the right pulmonary artery (modified Waterston shunt). Twenty-three infants had pulmonary atresia and 14 had severe pulmonary stenosis. Underlying cardiac lesions were tetralogy of Fallot (n = 11), single ventricle (n = 7), transposition complexes (n = 6), and intact ventricular septum and hypoplastic right heart syndrome (n = 13). There were 4 early deaths (10.7%) in the entire series, 2 of which were shunt related. Three of the 4 occurred during our initial experience with this shunt in 1978 and 1979. They led to the modified Waterston shunt being abandoned for 3 years in favor of other shunt procedures. Since 1983 one early death occurred in 28 infants (3.5% mortality) with no death in the latest 26 patients. All patients were followed up between 6 and 108 months. There were 4 late deaths, one of which was shunt related. We observed a significant difference in the shunt patency rate between 4 and 5 mm grafts: palliation was adequate after 2 years in 52% of the patients when a 4 mm graft was used and in 89% of the 5 mm graft group (p less than 0.005). Reshunting was necessary in 7 infants between 5 and 60 months after primary surgery. Recatheterization was performed in 17 infants for suspected shunt failure (n = 6) or diagnostic reasons (n = 11).(ABSTRACT TRUNCATED AT 250 WORDS)

Actuarial Analysis

Prenatal diagnosis of a left ventricular aneurysm.

In a fetus with ventricular extrasystoles a congenital aneurysm of the left ventricle was diagnosed prenatally. At 32 weeks of gestation, echocardiography showed a large apical left ventricular aneurysm with a thin, hypokinetic wall. Congestive heart failure did not occur. Prenatal and postnatal examinations did not detect the aetiology of the aneurysm, but excluded the majority of possible causes. The 2-year-old child is now asymptomatic and normally developed. Neither medication nor surgical treatment have been necessary, except for antithrombotic prophylaxis with low-dose aspirin.

Adult

Pulmonary hypertensive vasculopathy--no indication for corrective operation of isolated ventricular septum defect in babyhood.

The frequency of surgical closure of a ventricular septum defect (VSD) in the first year of life has risen from 10% to 30% in the last seven years in West Germany. Whereas there was a decrease of mortality in older children, mortality has stagnated at 8% for correction at age 12 months or less (early correction). Development of an irreversible pulmonary hypertensive vasculopathy (PHVP) and recurrent heart failure with growth disorders are given as reasons for operation in the first year of life. In the last six years, we have operated on 31 infants for isolated VSD with pulmonary hypertension (PH) and closed the VSD. In approximately equal preoperative pressure in the two ventricles with a pressure ratio of 0.88 +/- 0.16, there was an immediate fall in pressure in the right ventricle with pressure ratios of 0.43 +/- 0.24. Three patients (9.7%) died perioperatively. In the same period, a primary VSD closure was carried out in 120 children even after over 12 months with three deaths (2.5%). Pulmonary tissue removed in this operation did not show any PHVP in four patients (13%), grades 0 to I and I to II in 10 patients each (total 33%) and a PHVP grade II to III in six patients (20%). This PHVP is capable of full regression. An irreversible PHVP does not develop up to the end of the first year of life in isolated VSD with PH, so that the correction can be safely postponed to the beginning of the second year of life provided that recurrent heart failure with growth disorders does not compel earlier correction.(ABSTRACT TRUNCATED AT 250 WORDS)

Aging

[Aneurysm of the vein of Galen as a possible cause of congestive heart failure: a report of 5 new cases].

We report on five newborns with an arterio-venous malformation of the vein of Galen. All newborns were cyanotic and in congestive heart failure without any evidence of congenital heart disease. Congestive heart failure in these cases was mainly due to an almost two-fold increase in cardiac output of approximately 8 l/min/m2 (normal: 4.5 l/min/m2). According to previous reports, mortality is very high in patients with this malformation when becoming symptomatic during infancy, and therapy by surgery or embolization is only successful in 10-30%. While three of our patients died shortly after diagnosis because of untreatable heart failure, the other two were operated on either by subtotal ligation of the draining vein or by ligation of 4 arterial feeders. In the first case secondary thrombosis of the aneurysm occurred and cardiac failure subsided. In the second case a large shunt remained and a balloon-embolization was performed successfully. However, in both patients severe neurologic defects occurred, the severity of which remains to be assessed later since the post-operative observation period is only 2 and 5 months, respectively.

Cerebral Angiography

[Continuous-wave Doppler velocimeters and blood pressure difference in aortic coarctation. A simultaneous comparative study of 52 children].

Sixty examinations in 52 children with coarctation of the aorta (eight pre- and postoperative studies) were performed to assess the relation of the Doppler derived gradient using the simplified Bernoulli equation (delta p = V2 X 4) with the blood pressure difference simultaneously measured with an automated oscillometer. There was a close correlation (r = 0.89, y = 16.1 + 0.73x, syx = 7.38 mmHg) with significant overestimation of the blood pressure difference in mild stenoses (p less than 0.0001). If the patients are divided in two groups, an operative (group 1: symptomatic patients, n = 24) and a nonoperative group (group 2: asymptomatic patients, in the majority recoarctations, n = 36) it becomes clear that the overestimation is due to the latter. The velocity superior to the coarctation was higher in the second than in the first group (152.1 +/- 31.5 vs. 114.5 +/- 43 cm/s, p less than 0.004) (mean +/- SD). If the prestenotic velocities are taken into account, the correlation is nearly the same (n = 37, r = 0.9, y = 9.4 + 0.73x, syx = 7.3 mmHg) with slight underestimation in high-grade and overestimation in mild coarctations. A pandiastolic increased velocity was found in 79% of the patients in the operative group, but in none of the nonoperative group. The reasons for the overproportional increased jet velocity in group 2 as discussed are that: the peak pressure gradient may exceed the peak-to-peak pressure gradient; the prestenotic velocity must be taken into account, if the modified Bernoulli equation is used; and the collateral vascularization may be substantial.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent

[Echocardiographic findings and function analysis of the left ventricle in infants with endocardial fibroelastosis (author's transl)].

7 infants with the clinical picture and the typical hemodynamic and angiocardiographic findings of endocardial fibroelastosis were studied echocardiographically. The echocardiograms were digitized and analyzed by the method of Gibson and Brown and compared with those of 8 normal infants. The echoes of EFE-patients showed a nearly twice as large LV-diameter. The mitral valve was displaced posteriorly, the septal motion in 4 of them abnormal. The shortening fraction was significantly reduced, the LPEP/LVET quotient increased. The comparison of LPEP with the Q to mitral valve closing interval revealed a considerable prolongation of isovolumetric contraction time. The result of computer analysis was a decrease of peak Vcf and maximum lengthening rate. The echocardiographic pattern is typical but not pathognomonic, it gives no prognostic information.

Echocardiography

Cardiac hypertrophy due to physical exercise--an example of hypertrophy without decrease of contractility: unreliability of conventional estimation of contractility by simple parameters.

In 100 young, male Sprague-Dawley rats, a long term swimming training (2 hr/day for 8-12 weeks) produced an increase in heart weight of 10 percent, and an increase of about 15 percent in the relation of heart weight to body weight compared with control rats of the same age and initial weight. In examinations of the mechanical properties of the whole ventricle as well as of trabecular preparations, there was no evidence of impaired myocardial contractile ability because of the swimming training. Some parameters for the estimation of "contractility" increased, whereas others decreased. At a muscle length near lmax, the developed force and the maximal rate of force development were slightly augmented. The results reveal the limited value of some indices of contractility. Alterations in the shape of the contraction curve must to be considered adequately in order to avoid misinterpretations.

Animals

Mechanics of the isolated ventricular myocardium of rats conditioned by physical training.

Force-velocity relations from after-loaded contractions, from isometric and isotonic QR experiments, resting-tension curves and biochemical analyses were conducted on sixteen trabecular muscles (SH) from hearts of rats conditioned by eight weeks of swimming training (increase in heart weight 8%), and compared to a control (CH) of eighteen trabecular muscles. (SH) showed increased tension development (p less than 0.01), whereas the diastolic properties remained almost unchanged. Analysis of the amount of hydroxyproline did not prove any variation. Vmax of (SH) was only slightly increase when there was a singificnat rise in actomyosin and myosin ATPase activity, while PO of the force-velocity relations of (SH) on the x axis (tension) shifted clearly to the right (p less than 0.01). Consequently, the maximum instantaneous power of (SH), expressed by the maximum rectangular plane under the force-velocity curve, increased considerably (p less than 0.01) in comparison with (CH). The experiments show that haemodynamic load induced by training does not alter the passive properties of the myocardium, but does bring about an increase in the contractile capabiltiy.

Actomyosin

[Progress and change in nuclear magnetic resonance diagnosis of congenital and acquired heart defects].

Since 1987, the authors have examined 186 patients (76 girls, 110 boys) with a variety of congenital and acquired heart diseases by means of magnetic resonance imaging (MRI). The patients' ages ranged from 2 days to 20 years (mean age 3.97 years). During the study new techniques were developed, which extend the applicability of MRI. Especially two-dimensional and three-dimensional angiography are demonstrated and discussed. Using all diagnostic tools offered by MRI more than 90% of cardiovascular malformations are correctly diagnosed. MRI is of unquestionable value in the evaluation of the thoracic and abdominal aorta and in demonstrating the vascular status in pulmonary atresia and anomalous pulmonary venous return. It may be useful if question arise in patients with complex lesions, intra- and extracardiac tumours and cardiomyopathies. Although MRI is still under investigation, it may play a major role in diagnosing congenital heart diseases.

Adolescent

[Spiro-ergometric studies after corrective surgery of tetralogy of Fallot].

Treadmill exercise electrocardiography and spirometry was recorded in 61 patients (aged from 5 to 23 years) who underwent corrective surgery for tetralogy of Fallot. The Bruce-protocol and a modified interval-protocol were compared to find out their ability to induce arrhythmias. Supraventricular arrhythmias were predominantly provoked by the interval-protocol while maximal exhaustion is often necessary for inducing ventricular arrhythmias which we found in 23% of our patients. Most of them were seen in the periods of recovery between or after the exercise. We propose a combination of both protocols, which will be compared with established forms of exercise-testing in further studies. The results of rhythm analysis of consecutive exercise tests in one patient after surgical repair could possibly be compared by using an "arrhythmia score" which tries to indicate the severity of different forms of arrhythmias. The values of maximal heart rate, oxygen consumption and endurance time recorded at maximal exercise (Bruce-protocol) were about 15% lower than correspondent values of healthy persons found by other authors.

Adolescent

[Combination of scimitar syndrome and horseshoe lung. A rare but typical finding--case report and review of the literature].

The scimitar syndrome is a rare congenital cardiopulmonary malformation. In association with a horseshoe lung it is extremely uncommon. We describe a child with typical scimitar syndrome (anomalous right pulmonary venous return, hypoplasia of the right lung with dextro-position of the heart and aberrant systemic arterial supply of the right lung), associated with a horseshoe lung. No clinical symptoms are present, surgical management is not necessary. This is the 20th case in addition to the 19 reported cases with scimitar syndrome associated with horseshoe lung. Eight other additional cases of horseshoe lung without scimitar syndrome are mentioned here. In horseshoe lung the pulmonary parenchyma extends from the right lung base across the midline and fuses with the left lung. In only 3 cases continuous parenchymal tissue could be found histological. In 8 cases the lungs were separated by fissurelike structures, seen in CT or chest film, and in 5 cases fissures were demonstrated histological. From 6 patients no findings are available. The various symptoms, the diagnostic tools and the therapeutic procedure will be discussed.

Bronchopulmonary Sequestration

[Adrenocortical nodular hyperplasia as a cause of Cushing syndrome in the neonatal period].

The case report of a female child born preterm (30th wk of gestation) who developed symptoms of Cushing's syndrome beginning in the neonatal phase is presented. The disease was caused by a unilateral adreno-cortical nodular hyperplasia and was successfully treated by unilateral adrenalectomy. Preoperative treatment with ketoconazole and metyrapone proved to be effective. Symptoms of Cushing's disease including hypertrophic cardiomyopathy were completely reversible within one month after surgery.

Adrenal Hyperplasia, Congenital

[Pacemaker therapy in children].

Between 1975 and June 1987 55 children underwent first permanent pacemaker (PM) implantation. The patients' age at time of implantation ranged between 1 month and 17 1/2 years (mean 5 5/12 years). In all cases - except a 17 years old girl who received a transvenous endocardial lead - only epicardial screw-in electrodes were used. Only VVI-PM have been implanted. In 84% the indication for implantation was post-surgical brady-arrhythmia, in 16% it was an inborn or acquired disturbance of the conducting system. With our patients we have a survey of 2603 months of PM implantation. During this period 14 children underwent totally 20 revisions, in 50% the electrode was the cause of failure. The interval between two revisions calculated as a quotient from months of PM implantation and number of revisions has increased significantly during the last years and amounts now to 130 months. The relative low incidence of revisions is in our opinion mostly related to the nearly exclusive use of epicardial leads.

Adolescent