[Cardiac heterotaxia and ciliary anomalies].
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Biomedical subjects
Publications and source records attributed to M Fuster Siebert.
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Four widely used bubble oxygenators-the Optiflo I, the Bentley Q 200 A, the Harvey 200, and the Shiley 100 A-were tested and compared in 182 patients undergoing cardiac valve surgery. Fifty-six cases were performed with normothermia and 126 cases incorporated mild hypothermia (28-30 degrees C). There was no significant difference in the average age of the patients (51 yrs) or the perfusion time (60 min). All components of the extracorporeal circuit were identical, and anesthetic regimens and surgical techniques were also similar. In this study, the Shiley 100 A oxygenator was found to be the most suitable for cases requiring mild hypothermia and was generally considered to be the oxygenator of choice.
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A case of a one month old girl admitted in hospital because of cardiac failure who died at three months of age is described. Her necropsy demonstrated a ventricular septal defect and showed a blood cyst in the tricuspid valve. It might be possible that the "precordial honk" or "whoop" heard during life, the cottony, shaggy pictures recorded in the echocardiogram behind the anterior tricuspid leaflet in diastole, the tricuspid regurgitation appreciated in the angiocardiography, and the failure of the pulmonary artery banding were related to the blood cyst 4 mm. in diameter attached to the atrial surface of the anterior tricuspid leaflet near the free margin. The microscopic findings of haemosiderin loaded macrophages and calcified deposits in the haemorrhagic cyst speak in favour of its relatively ancient nature, not associated with an asphyxial mode of death.
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A case of tricuspid atresia with a concordant ventriculoarterial connection associated with hypoplasia of the aortic isthmus and malformation of left atrioventricular valve is presented. Hyoplasia of aortic isthmus in absence of transposition of great vessels was explained by a posterior deviation of infundibular septum that reduced subaortic outflow tract and created an ample bulboventricular orifice. In the left atrioventricular valve an anterolateral papillary muscle that was prolonged in a thick chorda tendineaee fused to ventricular face of the cusp up to its insertion in the atrioventricular ring was observed. A systolic murmur of high tonality compatible with regurgitation of the left atrioventricular valve and a notch that was registered in postmortem angiography were attributed to this anomalous chorda tendinea. In the posterior wall of the heart there were two descending coronary arteries. One descended fro the crux cordis; the other, to its right, adjacent ot the acute margin, followed the insertion of the trabecular septum, delimited the outlet chamber, and was anastomosed to the anterior descending coronary artery.
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A check is made of the hematologic disorders in 20 children with neuroblastoma and its relation with the stage of the tumour. The 75% of the patients had metastasis when they are examined, 70% anemia, 15% leukopenia, 20% thrombopenia and 50% leukoerythroblastosis. The patients with leukoerythroblastosis all had remote metastasis and made up 66% of the children with IV or IV-S stage. In 50% of the patients there was metastasis in the bone marrow and 80% of these had leukoerythroblastosis.