[Ebstein's anomaly (author's transl)].
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Biomedical subjects
Publications and source records attributed to M Goenen.
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One hundred and twenty two cases of severe hyaline membrane disease are reported. 68 of them survived (57%). Adverse clinical, radiological and laboratory factors, and their effects on the early mortality rate, are analysed with particular reference to the referring centers, delay in admission, transport and the critical state of most infants on admission. The follow-up of 29 survivors treated before 1974 has been examined with reference to birthweight and assisted respiration. Four (30%) of the twelve infants with birthweights below 2000 g had major neurological sequelae. Only two out of the 17 babies with a birthweight over 2000 g had minor mental disturbances.
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The main haemostasis changes observed in a screening study performed in 40 patients who underwent an open heart surgery with extracorporeal circulation (ECC) are: a significant drop in platelet count from the onset of the ECC to the third postoperative day, a decrease of platelet retention and aggregation during ECC with an 8-day persistently increased heparin-neutralizing activity in plasma but not in serum, a moderate decrease of plasma factors I, II, VII-X, X and XIII and a more important drop in factor V which disappears 24 h after ECC, a transitory increase of fibrinolysis during ECC and the lack of FDP elevation in the serum. These disorders require a very good neutralization of the heparin used during ECC. The ratio protamine/heparin can be established by a titration clotting time test. Protamine chloride seems to be more efficacious and to act more quickly than protamine sulfate for the neutralization. An overload in protamine can enhance the hemostatic, biological and clinical disorders. The preventive administration of platelet concentrate immediately after the heparin neutralization contributes to reduce the bleeding disorders related to the quantitative and qualitative platelet defects.
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A case of congenital chliride diarrhoea with functional ileus, early diagnosed in a premature infant of North-Africa parentage, is described. A short review is given of the clinical and biological manifestations of the disease in the newborn. Especially, the influence of parenteral therapy on the course as well as on the development of potentially fatal complications is discussed.
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In order to study the haemodynamic effects of isoprenaline and glucagon in the immediate postoperative period, 16 patients who had single or multiple valve replacement or coronary artery bypass grafts were investigated. Measurements of cardiac index, stroke index, heart rate, arterial and right atrial mean pressures were made either before, during, or after the administration of both drugs. With the doses used, the inotropic effects of both drugs are similar, while the chronotropic action is statistically higher during isoprenaline infusion than after glucagon administration. This fact explains the greater increase in cardiac index after isoprenaline administration. Mean arterial pressure shows no significant changes after either drug, while right atrial mean pressure decreases significantly. Peripheral vascular resistance remained mostly constant. The activity of glucagon injected as a single bolus seems to be maximal 10 to 15 minutes after the injection and dissipates about 30 minutes later. The ideal dose of isoprenaline is between 0.8 and 2 mug/min. Within this range, the inotropic effect is maximal and the chronotropic and bathmotropic effects are limited. Above 2 mug/min, cardiac index and stroke index decrease, and arrhythmias become more frequent.
A detailed evaluation is presented about the frequency and the type of arrhythmias following the surgical correction of 147 cases of atrial septal defects not associated with other congenital heart malformations. A total of 131 arrhythmias occurred in 84 patients. The nodal and the coronary sinus rhythms are the most frequently encountered after surgery, attaining 53,8% of all arrhythmias, while atrial fibrillation or flutter represented about 22%. The follow-up of these arrhythmias concerned the 3 month period immediately after surgery. After that time 20% of the arrhythmias occurring in the early postoperative period persisted. The frequency of arrhythmias increased with age, was relatively independent of the importance of the left to right shunt, the level of the mean pulmonary artery pressure and the sex of the patients; but statistically increased in patients in whom the correction was made with a patch. The authors concluded that there must exist at least two mechanisms to explain the occurrence of arrhythmias. A first one must be in relation to the surgical manipulation; the second, which is only an hypothesis, stipulates an alteration of the conduction pathways due to the auricular distention or the existence of a congenital anomaly of the conduction pathways.
Hemodynamics of 12 patients with tetralogy of Fallot were monitored during the first 72 hours after surgical repair. Total immediate repair in 5 patients was followed after 24 hours by a greater decrease in cardiac index than that observed in the group of 4 patients with previous palliative shunt (minus 25 plus or minus 6 vs. minus 1 plus or minus 7 per cent, p smaller than 0.025). This difference disappeared after 48 hours, and the short-term follow-up periods of these two groups were equally smooth. Six patients with pulmonary stenosis requiring the placement of an outflow patch had higher right ventricular filling pressures (after 24 hours 13.8 vs. 10.8 mm. Hg, p smaller than 0.025; 2 to 4 weeks later 9.6 vs. 5.5 mm. Hg, p smaller than 0.05), suggestive of a persistent right ventricular depression. This ventricular depression must be attributed to the induced pulmonary insufficiency and to the presence of akinetic areas. Both these factors should therefore be carefully minimized during the surgical procedure.
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