Biomedical subjects
Publications and source records attributed to C Pernot.
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Poorly tolerated solitary ASD in the baby is rare. The main criteria for diagnosis have been drawn from a series of 9 cases. The symptoms are often atypical, especially at first: usually a marked pansystolic murmur, a tricuspid flow murmur, late appearance of a doubling of B2, and a non-specific cardiac outline, except for the cardiac enlargement and the increased pulmonary vascularity. The ECG is rather more helpful, with constant RVH. At catheterisation, there is a major left-to-right shunt, the pressures in the right and left atria are equal, and pulmonary hypertension is rare. It is particularly common to find associated extra-cardiac abnormalities. Under medical treatment the prognosis is good, and early surgical closure is not normally required.
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One case of localized stenosis of the extrapulmonary portion of pulmonary veins is reported in a 3 years old child. The chest X-Ray showed a rounded and pulsatile opacification over the right hilum and on angiocardiography the right superior pulmonary vein looked very dilated. 20 other cases of segmental stenoses of pulmonary veins were collected from the literature.
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Bicuspid aortic valves are much more common than monocuspid valves and diagnosed by A. P. aortography and, above all, lateral views. One may distinguish the true bicuspid valves which include two Valsalva sinuses, often asymmetrical, and bicuspid valves whith a supplementary raphe, the commonest type, in which on angiography, one may distinguish three Valsalva sinuses, one of which is larger than the two others. These cases of bicuspid aortic valve may be associated with other malformations of the aorta or may occur alone. In the latter case, the main risks are aortic valvular stenosis or aortic incompetence.
Left ventriculography is of great interest in the diagnosis and prognosis of congenitalaortic stenosis. It permits one to distinguish valvular stenosis, which is the commonest type, from sub-valvular stenosis, of which there exist 5 main type; type I and II are the commonest, they have a better prognosis than types III and IV. Sub-sigmoid aortography shows the appearance of the sinus of Valsalva, thus whether there exists a tricuspid or bicuspid aortic valve. In all cases, one should seek stenosis at various levels of the aortic outlet and possible myocardiography of the left ventricle, the frequency of which is well known, together with the poor prognosis.
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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.
Various congenital malformations of the pericardium are describe. The defects of the pericardium are considered, which are more often partial and on the left side than total. Diagnosis is based on angiography and diagnostic pneumothorax. The pleurocardial cysts are frequent and diagnosed on plain films with pneumoperitoneum. The intrapericardial teratoma are seldom. Diagnosis is made on angiocardiography with pneumopericardium. Radiology is very useful for the diagnosis of various types of congenital malformations of the pericardium.
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