[Tetralogy of Fallot with congenital absence of a pulmonary branch. Apropos of 4 cases].
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Biomedical subjects
Publications and source records attributed to F Laborde.
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Between 1972 and 1978, forty-six infants under six months of age underwent surgery for isolated coarctation of the aorta. These forty-six patients represent 32% of the total number of infants aged less than six months who had surgery for coarctation of the aorta during the same period. At the time of operation, 41% were aged less than one month and the youngest was five days old. The main cause for surgery was heart failure in infants under one month (14/19) and severe systemic hypertension (150 to 300 mm Hg) in the one to six month age group (18/27). Overall mortality rate in our series was 17%. Six of the eight infants who died were under one month of age. Recoarctation occurred in 31,5% of infants; in eight cases the first surgical procedure had been done before one month of age. A second procedure was necessary in four cases. Early surgical repair of severe coarctation diagnosed during the first six months of life leads to functional improvement and avoids residual hypertension. After repair, the main risk is recoarctation.
In spite of the availability of small size models, intra-abdominal implantation of epicardial pacemakers is difficult in infants. The alternative, which is implantation within the pleural cavity, entails a risk of compression of the lung. These problems can be overcome by using a totally extrapleural thoracic approach.
A series of 25 congenital malformations of the atrioventricular valves underwent valve replacement (10 mechanical and 15 bioprostheses). Seven children died during surgery. The operative mortality was higher before 2 years of age (4/7) than in older children (3/18). It was also higher when valve replacement was performed at second intention (5/14) than when decided on from the outset (2/11). With an average follow-up over 3 years, 9 of the 18 survivors are considered to be good surgical results. Of the other 9, 3 are considered to be moderate results (2 associated subaortic stenoses), 3 present indications of reoperation for calcification of the bioprosthesis, and 3 died in the late follow-up period. This series suggests that plastic surgery of congenital malformations of the atrioventricular valves should continue to be developed.
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142 infants under 6 months of age were operated on for coarctation of the aorta from 1972 to 1978. Coarctation was isolated in 46 (32.4%) and associated with another heart defect in 96. Surgery was decided upon because of congestive heart failure sometimes associated with arterial hypertension refractory to medical treatment: 9 patients died during surgery. 89 underwent Crafoord's operation and in 44 ligature of the subclavian artery or aortoplasty using the subclavia were carried out. Early postoperative death rate was 30.9% and 13 patients (13.2%) died later. Recoarctations occurred in 25 patients (27%); more than half of these patients were less than 1 month-old at the time of surgery. Recoarctation was more frequent (46.4%) in cases with than in cases without (18.7%) aortic hypoplasia and it was more severe (5 out of 7 deaths) when there was an associated cerclage resulting in a double obstacle to right and left ventricles ejection.
Iatrogenic arteriovenous fistulas in infants are usually due to an arterial puncture performed in the neonatal period. On the occasion of 5 cases who were operated on, the peculiar features of these fistulas are established. Surgery consists of endo-aneurysmorrhaphy.
Cor triatriatum is a rare heart defect that may be surgically repaired, with good results when preoperative diagnosis is correct. Ultrasonography, angiography and catheterism reduce preoperative diagnostic errors such as abnormal pulmonary venous return. The authors report their experience with 7 cases, and discuss diagnostic problems and causes of errors.
M mode echocardiography was performed one year after surgical correction of Fallot's tetralogy in 32 patients (average age: 2, 7 years). All patients were asymptomatic without treatment and the average cardiothoracic ratio was 0,56 +/- 0,03. Echocardiographic indices of left ventricular function distinguished 4 patients with myocardial dysfunction (2 cases of poor myocardial protection and 2 double Blalock-Taussig anastomoses). Right ventricular diastolic internal dimensions were increased in 75 % cases. The ratio of right to left ventricular internal dimension was used to classify patients into three groups: Group I (ratio Less Than 0,70) 17 patients; Group II (ratio 0,70 Greater Than 0,80) 4 patients, and Group III (ratio Greater Than 0,80) 11 patients. This ratio was not related to the age of the patient of surgery or to the type of patch used on the right ventricular outflow tract but was related to the pressures recorded at operation after correction. A ratio Greater Than 0,80 was strongly suggestive of a significant residual abnormality which was often curable. Echocardiography has become an essential investigation in the follow up of Fallot's tetralogy after surgical correction: a right: left ventricular ratio Less Than 0,70 obviates the need of control catheterisation. On the other hand, a ratio Greater Than 0,80 is a strong indication for cardiac catheterisation even in the absence of clinical abnormalities.
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From 1972 to September, 1979, 20 patients underwent transplantation of the anomalous left coronary artery to the aorta, either directly or via a graft. Correction of ischemia-induced mitral insufficiency was associated in eight patients and a postinfarction left ventricular scar was excised in 12. Operative mortality was high among patients under 1 year of age (4/5). Among older children it was 15%. There were not late deaths among patients surviving the operation (mean follow-up 3 years). All but one had marked clinical improvement and reduction of cardiomegaly. Eleven patients underwent angiographic control, with a patent graft or anastomosis demonstrated in every case. Operation is advocated for patients over 1 year of age. The best treatment of symptomatic infants remains controversial.