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.
In the young adult, the symptomatology of partial abnormal infradiaphragmatic pulmonary venous return is often very nonspecific. However, this condition may sometimes be diagnosed when the classic scimitar image is found.' Occasionally, this abnormal type of venous return can be a component of a larger malformation syndrome: the scimitar which includes dextroposition of the heart, hypoplasia of the right lung (associated with various degrees of bronchial and vascular anomalies), and diaphragmatic malformation. Angiography remains the method of choice for confirmation of the diagnosis and for preoperative assessment, especially for the associated cardiac anomalies. We report a case of a young adult with infradiaphragmatic venous return diagnosed by ultrasonography. Real-time ultrasonography can easily and precisely demonstrate the transdiaphragmatic trajectory of the aberrant pulmonary vein and its confluence with the inferior vena cava. CASE REPORTA 16-year-old male presented with a history of cardiac murmur since birth and slight dyspnea on exertion. Auscultation revealed a systolic murmur (2/6) at the second left intercostal space with persistent splitting of the second heart sound. The electrocardiogram showed signs of right ventricular hypertrophy. The chest X-ray ( Fig. 1) revealed discrete dilatation of the right side of the heart and, at fluoroscopy, bulging and pulsatile shadows of the hilum. A curvilinear scimitar image could be suspected behind the right heart cavities. M-mode sonography revealed moderate dilatation of the right ventricle, normal movement of the interventricular septum, and normal dimensions of the left atrium. There was no echofree space behind the left atrium. Heart catheterization showed abnormal oxygen saturation in the blood found in the lower part of the right atrium (pulmonaryhystemic output ratio: 1.5). An angiogram confirmed partial abnormal pulmonary venous return; all the veins of the right lung coalesced into a "collecting vein" that drained into the inferior vena cava at the level of the diaphragm (Fig. 2).To better evaluate the site of the opening of the collecting vein into the inferior vena cava in relation to the diaphragm, a n abdominal ultrasound investigation was carried out (mechanical 3.5 MHz sector scan). This procedure readily demonstrated the lower thoracic and abdominal trajectory of the anomalous pulmonary vein; the opening of this vessel into the inferior vena cava was located just below the diaphragm and behind the ostia of the hepatic veins [Figs. 3 and 4(A)1. No diaphragmatic malformation was found nor was a systemic artery with a transdiaphragmatic trajectory visualized.At surgery, confirmation was made of the position of the collecting vein's entry into the inferior vena cava (just below the diaphragm); the collecting vein was reimplanted in the left atrium by means of a prosthetic enlargement of this cavity. 425
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.
This a report of myxomatous mitral valve degeneration in a child with Turner syndrome. The diagnosis was first suspected at 7 months of age. At 5 years of age, ultrasonic and angio-cardiographic confirmation prompted a successful mitral valve replacement with a Starr prosthesis. The child also had partial anomalous pulmonary venous return and a dysplastic right kidney. The child has remained well for 4 years after surgery.
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