[Klinefelter's syndrome. Trilogy of Fallot. Teratoma of the mediastinum and early puberty].
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Biomedical subjects
Publications and source records attributed to A M Worms.
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In relation with the report of two cases of branching of one of the pulmonary arteries from the aorta, it was recalled that this is a rare malformation, responsible for a severe cardiac failure with or without cyanosis in the first months of life, intensly suggestive of a left-to-right shunt. Demonstration of surprizing or unhabitual clinical or haemodynamic findings should indicate the performance of a right-sided angiography then an aortography, this being the only way to secure the diagnosis. Combination of such an anomaly with Fallot's tetralogy is exceptional. The clinical diagnosis is made more difficult by the presence of an anastomosis between the pulmonary and the systemic circulations. Only a careful analysis of right angiograms might make it possible to suspect the presence of an abnormal branching of the pulmonary artery, and to perform the aortography. The treatment is surgical in all cases, and should be undertaken early in life, as it is the only way to control the heart failure or to prevent irreversible pulmonary arteriolar lesions.
In relation with 8 cases, the features of Ebstein's malformation were studied in the neonatal form. In spite of its rarity, it should be recognized because of its peculiar prognosis, as it is liable to be permanently improved by medical treatment, after a difficult adaptation period. Besides, it is essential to distinguish it from other neonatal heart diseases which ressemble to it, but require an urgent surgical treatment.
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