[Indications for radical operation on tetralogy of Fallot].
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Biomedical subjects
Publications and source records attributed to P Vernant.
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Six cases of cor triatriatum documented and operated on at Henri-Mondor hospital between 1980 and 1984 are reported. Ages at the time of surgery ranged from 8 months to 57 years. Four of the 6 patients presented with pulmonary hypertension. Anatomic types consisted of 4 diaphragmatic types and 2 more complex malformations. Associated lesions were present in all but one patients. They consisted mostly of interatrial defects (depending on their position with regard to the intra-atrial membrane, they play a determinant role in the symptomatology) and of a left superior vena cava (which might play a role in the embryogenesis of the malformation). Except for one early postoperative death, results of surgery were excellent for all patients, with a mean follow-up of 5 years. This series, compared with the literature, allows precising the embryologic and anatomic aspects of the malformation and the surgical techniques in complex types.
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This report concerns a family in which several members presented an involvement of skeletal and velo-pharyngeal muscles, associated with hypertrophic cardiomyopathy, respiratory disturbances and lens opacities. The mode of transmission is autosomal dominant. The E.M.G. showed neither spontaneous activity nor myotonic discharges. In two patients, the muscle biopsies showed identical changes. By light microscopy it was seen that in numerous type I fibres, the intermyofibrillar network was "rubbed out" and this occurred along with splitting of such fibres. Electron microscopy revealed an intrasarcoplasmic accumulation of an electron-dense granulo-filamentous material: in some areas it formed a mesh of threads around the myofibrils and in others it was disposed regularly in small stacks facing the Z lines. Continuity or structural similarity of this material and the Z lines was not observed; its relationship with the dense strips of leptofibrils is suggested.
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Post-operative results in congenital heart diseases with septal defect are often darkened by the occurence of disorders in atrio-ventricular conduction. The incidence of these disorders is dependent upon the location of the septal defect, the anatomy of conduction pathways and the operative technique. Intracavitary recording of His bundle activity leads to a more precise localisation of the trouble. Such disorders are more frequent after cure of Fallot's tetralogy than after closure of an interventricular communication. They are rare after closure of an interatrial septal defect and more common after closure of the atrioventricular canal, where they are of atrial node type. The occurrence of an atrioventricular block, even transient, is of poor prognosis, and in case of proximal lesion the insertion of a pacemaker must be considered. The high incidence of conduction disorders justifies continuous per-operative recording and prolonged surveillance, if necessary by Holter's method. Locating the conductive tissue during surgery reduces the frequency.