[Late rhythm disorders occurring after surgical closure of interactrial communications].
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Biomedical subjects
Publications and source records attributed to P Vernant.
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Aortic arch hypoplasia is rare but carries a poor prognosis. It is usually associated with a left to right shunt at ventricular level. The association of an aorto-pulmonary fistula is exceptional. A case of a 5 1/2 month old infant treated successfully by simultaneous correction is reported with a review of the physiopathological changes. Pulmonary hypertension and aortic perfusion depend on the calibre of the ductus arteriosus and its evolution. The therapeutic problems which result are the suppression or palliation of pulmonary hypertension and resaturisation of distal aortic vascularisation. Surgery is the only hope of cure, and current techniques are reviewed: two stage procedures with revascularisation of the distal aorta and pulmonary artery banding, or simultaneous correction of aortic continuity with suppression of the left to right shunt under cardiopulmonary bypass. The technical conditions are analysed: perfusion of the different aortic segments, methods of reestablishing aortic continuity. Good results are as yet rare, and are obtained after complete correction under cardiopulmonary bypass.
120 cases of the Williams-Beuren syndrome were collected by 11 cardiological centres in France, to determine the incidence of the various clinical signs and to obtain information on its aetiology, outcome and treatment. The selection criteria for inclusion in the series was typical facies. No particular complications were reported during pregnancy. Boys were a little more affected than girls. The birth weight was low and problems, especially digestive, often occurred in the first months. Cardiac signs were usually detected from the first year, although the exact diagnosis was usually made later on. 3/4 patients had subaortic stenosis, which was severe in 1/3 cases. Involvement of the branches of the aorta was not looked for systematically: the incidence (1/5 cases) found was lower than the true figure. Half the patients also had stenoses on branches of the pulmonary artery, but only rarely were they severe. These vascular malformations often seem to be progressive and, over a 10 year period, half the patients deteriorated. Many extracardiac abnormalities were reported. The most frequently encountered were inguinal and/or umbilical hernia. Surgery on the subvalvular stenosis gave good results in over 80 p. 100 cases; operative mortality was about 10 p. 100. Surgery should be performed before irreversible coronary or myocardial lesions develop. The study of the previous history did not give any new information on the cause of the syndrome, whose association with idiopathic hypercalcaemia of infancy is emphasised once again.
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An aneurysm of the membranous part of the interventricular septum associated with a complex congenital malformation of the endocardial cushions was diagnosed in a 39 year old woman who presented with syncope. Diagnosis was made by echocardiography and confirmed by angiography. The operative findings were: a double aneurysm of the membranous septum, a left ventricular--right atrial fistula and two hemivalves attached to papillary muscles. The usfulness of echocardiography in the diagnosis of aneurysm of the membranous interventricular septum and in the follow up of ventricular septal defects from which they arise is emphasised. The pathogenesis of the arrhythmias observed (accelerated idioventricular rhythm, reciprocating tachycardias and syncope possibly related to transient heart block) is discussed.
Transverse sections of the heart and great vessels may be obtained by tomodensitometry using an exposure time short enough to carry out the investigation during apnoea, and an infusion of contrast medium. The definition is good enough for the investigation to be considered as a useful adjunct to the normal investigations in complex congenital cardiac abnormalities. All measurements are instantaneous, including the study of pulmonary density which allows assessment of the pulmonary vascularisation.
19 patients were studied after operation for coarctation of the aorta. The average age at operation was 13.7 years. Crafoord's operation was performed in 16 cases and in the other three an aortic patch was necessary. The average post-operative period was 4.7 years. 14 cases were classified as good results: normal femoral pulses and arterial blood pressure. The other 5 cases were considered as unsatisfactory: 2 mediocre results with normal arterial blood pressure but diminished femoral pulses and oscillometric indices; 3 poor results with hypertension at rest. On exercise with bicycle ergometry the following results were obtained: the systolic blood pressure rose in a comparable manner to that observed in normotensive individuals of the same age on exercise except in 2 cases. These two patients had an aortic patch and one had a residual intraaortic pressure gradient. The pronostic significance of these findings is not known. However the use of an aortic patch is often associated with abnormal blood pressures at rest or on exercise. The fact that the majority of patients operated for coarctation of the aorta have normal blood pressure profiles on exercise should be emphasised.
The patency of 24 aorto-coronary grafts was studied by tomodensitometry. --16 out of the 18 bypasses examined within the two weeks following surgery were seen. --4 older bypasses were not visible, either by scanner or by angiography and were therefore occluded: two more, performed more than a year previously, were patent on tomodensitometric examination. The interpretation of the results, discussed taking into account the small number of angiographic check ups does not permit any formal conclusion as to the validity of the procedure. The results of this technique seem satisfactory and quite comparable with other non invasive methods used in the study of aorto-coronary grafts.
Clinical findings and results of treatment in 17 patients with acute mitral incompetence are described. The lesions were secondary to a recent myocardial necrosis causing severe cardiac failure (shock, or acute left ventricular failure), and the presence of mitral incompetence was confirmed by right catherization (15 cases) and/or left ventricular angiography (13 cases). The anatomical lesions are described in 14 cases: rupture of the posterior mitral papillary muscle in 7 cases, and acute dysfunction of the muscle in 7 other cases. Eight patients were treated by an intra-aortic balloon to assist circulation, and 14 were given vasodilators (phentolamine, trinitrin) associated in 5 cases with positive inotropic drugs. Surgical treatment was given (mitral valve replacement) in 7 cases. The respective effects of vasodilators and circulatory assistance are discussed, as well as the indications for, and results of urgent surgical treatment (6 successes in 7 operations).
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A child of 10 was admitted to a cardiology unit with adiastole. The marked degree of dwarfism and anatomical features were characteristic of the so-called 'Mulibrey' dwarfism. Surgical intervention confirmed the constrictive pericarditis which is normally found in this condition. Unfortunately, the adiastole persisted which, taking into account the marked eosinophilia, made an associated endomyocardial fibrosis seem likely. The distinctive features of constrictive pericarditis and of the constrictive type of cardiomyopathy are recalled.
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Treatment by phentolamine was carried out in 30 patients presenting with an acute myocardial infarction complicated by left ventricular failure, 8 of which had cardiogenic shock. The response to treatment was closely related to the level of overload of the left ventricle. Significant improvement in the haemodynamic state was only observed in the most severe cases of cardiac failure, and in these cases was accompanied by only a minimal and insignificant change in mean arterial pressure and heart rate.
The case is reported of carcinoid heart disease in a lady of 70 with intractable congestive cardiac failure 5 years after the removal of a primary carcinoid tumour of the ovary. The special features of primary carcinoid tumours of the ovary are recalled, with emphasis on their rarity and of the absence of liver metastases. The various features of carcinoid syndrome are recalled in the light of current knowledge of the pathogenesis. A review of the literature on cardiac involvement in primary carcinoid tumours of the ovary, amounting to 10 cases, is included. The possibility of surgical cure of the heart lesions in carcinoid tumour by a prosthetic tricuspid valve are discussed, in the light of the 6 reported cases and the present one. Our report is the first one of replacement of the valve after removal of a primary ovarien carcinoid tumour, and the excellent result has been maintained after three years.
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The "small aortic ring" was until recently an unresolved problem for surgeons operating on the aortic valves. It used to contraindicate valve replacement in the child, and sometimes led to the insertion of too small a valve in the adult. Some hope of a solution has appeared in the form of three new techniques: enlargement of the aortic ring over the mitral valve, aorto-ventriculoplasty, and apico-aortic shunt. Two adults have had the ring enlarged above the mitral valve with good results in both cases. Five children have had an aorto-ventriculoplasty with good results in two cases, post-operative atrio-ventricular block in one, and two deaths, one early and the other late. These deaths appear to be more closely related to the severity of the initial lesion and to the degree of decompensation of the myocardium than to the operation itself. There are advantages and problems with this technique. An attempt has been made to systematise indications for treatment as a function of the lesions and the state of the patient.