Biomedical subjects
Publications and source records attributed to C Dupuis.
Atrioventricular block which caused death in a 6-month-old baby is reported. Necropsy examination revealed the only abnormality in the heart to be an aneurysm of the membranous septum. Histological examination of the conduction system showed fibrosis and attenuation of the bundle of His at the lower border of the aneurysm.
Study on the LSIV has several points of value: It enters into the normal mediastinal outline, forming the aortic "nipple". It may be the site of dilatation, either congenitally or secondary to haemodynamics changes. This venous dilatation should not be confused with a mediastinal tumour. It forms, in the majority of cases, an anastomosis between the superior vena cava and the azgos vein and therefore plays a part in the collateral circulation of caval obstruction syndromes. Its transverse course explains its involvement in the anterior and posterior mediastinal syndromes. Opacification of the LSIV should be part of the study of mediastinal tumours. By virtue of its relations with the left upper lobe, its study should be part of the pre-therapeutic assessment of such lesions.
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Numerous causes exist for disturbances in pulmonary ventilation in the newborn and infant. Amongst the exobronchic causes, those of cardiovascular origin are not rare. The difficulty in radiological diagnosis varies in relation with the anatomo-radiological appearance of the lesions and their aetiology. Ventilation disturbances are not always obvious. Simple techniques (high penetration films, oblique views, films in inspiration and expiration) make possible the topographical localisation of the lesions and the detection of any obstructive factor. These films should also be adequate as far as the aetiological diagnosis is concerned: severe left-right shunt, pulmonary valve agenesis, fibro-elastosis, retrotracheal left pulmonary artery. Obviously, angiocardiography is often necessary to confirm and accurately determine the nature of the "cardiovascular" lesion responsible. Such disturbances of pulmonary ventilation may either reveal or complicate a cardiac or vascular disorder in the newborn or infant. They often present marked therapeutic problems.
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Three cases of aneurysm of the membranous septum were diagnosed in vivo. The fourth case was discovered at post-mortem examination in a patient who had a conductive disorder. These four cases give us reason to review the anatomical, clinical, radiological and haemodynamic features of this condition.
The authors present a clinico-pathological study of three cases of complete congenital atrio-ventricular block in the infant. In one case the disorder was familial, and the heart was macroscopically normal. In the other two cases, the associated abnormalities (complete discontinuity of the aortic arch in one, and situs inversus with a patent ductus and high VSD in the other) were not responsible for the conductive defect. This was explained after study of serial sections of the inter-atrio-ventricular septum; these showed an interuption or absence of the penetrating portion of the bundle of His associated, in one case, with agenesis of the atrio-ventricular node.
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