Biomedical subjects
Publications and source records attributed to L Fermont.
Two cases of a very uncommon congenital association (cor triatriatum and total anomalous pulmonary venous connection) are presented. This association should be suspected in any infant with signs of pulmonary venous obstruction.
From May 1977 to August 1982 50 patients who were 1.5 to 44 months old underwent anatomic correction of transposition of the great arteries (TGA) and closure of ventricular septal defect (VSD) at our institution. Thirty-nine patients underwent preliminary pulmonary arterial banding. Hospital mortality was 32%: four patients died as a result of technical problems, seven as a result of associated lesions, three of pulmonary hypertension, and two of left ventricular failure. Three other patients died after the first postoperative month (one of mediastinitis, one at reoperation for a residual VSD, and one of pulmonary hypertension). All 31 survivors are in excellent clinical condition and are in sinus rhythm after a mean follow-up period of 31 +/- 14 months. Twenty-five patients were reinvestigated by echocardiography (M mode and two-dimensional) and/or catheterization. Parameters of left ventricular contractility were within normal limits, but systolic aortic diameter was larger than normal (p less than .01). Seven patients had stenosis of the right ventricular outflow tract and five of these required reoperation. The two persistent problems with the anatomic correction of TGA associated with VSD are a relatively high operative mortality and secondary right outflow tract stenosis. However, use of this procedure results in better left ventricular function and fewer arrhythmias than does use of atrial repair techniques and also results in the use of the anatomically left ventricle as the systemic ventricle.
Eight cases of intracardiac thrombi in infants and children were compiled in a cooperative study involving five paediatric cardiological centres. Two babies were hospitalised for cardiac failure due to a severe supraventricular arrhythmia. Two-dimensional echocardiography (2D echo) showed a left atrial thrombus which disappeared after anticoagulant therapy. The third case was unusual: 2D echo performed 4 months after a Senning operation for complete transposition of the great arteries showed stenosis of the pulmonary venous canal and a thrombus above the stenosis: the mass was echogenic, rounded, of variable density and in contact with the pulmonary veins. These findings were confirmed at autopsy. The fourth case was a 34 month old child with Fallot's triad in whom 2D echo showed a right ventricular thrombus, confirmed at surgery. The four remaining cases were thrombi detected in patients with congestive cardiomyopathy. The thrombus was adherent to the left ventricular lateral wall or apex. Two of these thrombi disappeared after anticoagulant therapy, one of which after hemiplegia. 2D echo is a useful tool for the diagnosis and surveillance of intraatrial or intraventricular thrombi. Intraatrial thrombi may be due to supraventricular arrhythmias in children; intraventricular thrombi are usually seen in association with poor left ventricular contractility. The diagnosis of thrombosis should lead to institution of anticoagulant or even fibrinolytic therapy in order to avoid systemic embolism.
Between 1970 and 1981, 40 patients (6%) were reoperated after surgical correction of Fallot's tetralogy. The average age of these patients was 7,5 years (range 2 months to 37 years). The usual anatomical form was present in 30 cases and severe forms accounted for the other 10 cases (pulmonary atresia with septal defect were excluded). The 40 patients were divided into 3 groups according to the anatomical lesions corrected at reoperation: Group I: 16 patients with a residual isolated VSD; Group II: 14 patients with one or two residual right heart anomalies (RRHA) but without a septal defect; Group III: 10 patients with a residual VSD and RRHA. The only clinical difference between the patients of these 3 groups was the delay of onset of symptoms: the patients with residual VSD (Groups I and III) often developed cardiac failure immediately, whilst in those without residual VSD (Group II) cardiac failure was usually observed secondarily. Four patients (10%) died early after reoperation (less than 1 month). Three others died later, two during a third operation. The total mortality was similar in the three groups. The surgical result was assessed clinically after an average follow-up of 4,5 +/- 3 years: patients with a residual isolated VSD (Group I) had the best long-term results. In 12 patients, M mode and 2D echocardiography showed normal left ventricular function but the ratio of end diastolic right ventricular and left ventricular dimensions was increased to an average of 0,72 +/- 0,2.(ABSTRACT TRUNCATED AT 250 WORDS)
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Surgical correction of simple transposition of the great arteries by arterial detransposition can only be considered if the left ventricle is capable of functioning under systemic conditions. This is possible at birth but the operation is too big for it to succeed. After a few weeks the left ventricle may lose this capacity but it can be induced to redevelop if it is given a systolic stress by pulmonary banding. This was attempted initially in 30 children aged from 5 weeks to 3,5 years old (20 aged less than 2 months) but had to be abandoned for the following reasons: 8 immediate failures due to severe hypoxia and acidemia, 7 left ventricular failures, mainly in 3 children operated after 6 months of age; 7 severe hypoxemias, 5 pulmonary arterial lesions due to banding and/or the systemo-pulmonary anastomosis associated in some cases; in all, there were 8 failures out of the 17 anatomical corrections performed to date in 22 survivors of banding. The criteria of left ventricular readaptation after banding were difficult to determine. The final result did not appear to be related to either the duration of banding, the degree of aortic desaturation, the ratio of ventricular pressures, or to the various echocardiographic indices. Only the initial hypoxemia (less than 50 p. 100 saturation) with respect to the risk of banding, and the age at which banding was performed (over 6 months) with respect to the risk of anatomical correction, appeared to be obvious risk factors. This is the reason for a new protocol at present under study to try to maintain left ventricular growth by banding in the first days of life, so to ensure a more harmonious and effective, and also less dangerous preparation for anatomical correction. Six new born children have been "prepared" in this way: three had adequate loose banding and underwent atrial correction. Very good results were obtained in the other three patients who have already undergone detransposition at 2,5 and 3 months with excellent immediate results. We believe that neonatal pulmonary banding (before 5 days) when aortic saturation after atrioseptostomy is over 50 p. 100, provides a real alternative to the classical methods of treating simple transposition of the great arteries (Mustard or Senning). Anatomical correction can then be carried out a few weeks later under good conditions.
One 12 day newborn, a 2 year old infant and two children aged 10 and 14, operated for isthmic coarctation of the aorta by three different surgical techniques (resection-anastomosis, angioplasty with an autograft, prosthetic graft) suffered infection of the operative field, resulting in septicemia (3 staphylococcal and 1 yeast infections). The aorta ruptured after 4 days, before any collection of pus in the newborn but a mycotic false aneurysm developed in the three other patients: this was the cause of death by aortic rupture during the 5th postoperative week in one case but was diagnosed and operated successfully in the other two, 13 and 54 days after their first operation. These complications are well known. Therefore, these patients should be closely followed up for at least one month. The occurrence of pyrexia, even slight or late, should be treated with suspicion. Characteristic dilatation of the part of the aorta operated on should be actively looked for by simple radiography, 2D echocardiography and, when in doubt, right heart angiocardiography. The diagnosis is a surgical emergency: the safest technique consists in initially establishing an aorto-aortic deviation by a right sided approach followed by exclusion of the lesion. Surgery is the only means of preventing catastrophic rupture of the aorta.
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Myocardial ischemia is very rare in childhood but, as in the adult, it can result in very severe heart failure. The discovery of specific signs on the electrocardiogram provides a diagnostic orientation which can be confirmed by biologic data and radionuclide scans. The causes of the affections are related to the child's age group : severe fetal and neonatal anoxia in the neonate (transient myocardial ischemia syndrome) ; anomalous origin of the left coronary artery in the young infant ; acquired coronary anomalies of Kawasaki's disease in the older infant or the young child.