[Retro-tracheal left pulmonary artery. Report of 2 cases]. / Artère pulmonaire gauche rétro-trachéale. A propos de 2 cas.

The authors report the cases of two neonates with retro-tracheal left pulmonary arteries (pulmonary artery sling). In the first case, there was rapid onset of cardiac failure with signs of airway compression. Echocardiography showed the vascular anomaly associated with a large ventricular septal defect (VSD). After standard radiological investigation and bronchoscopy to exclude an associated tracheo-bronchial malformation, the VSD was repaired surgically with reimplantation of the left pulmonary artery. Unfortunately, the patient died of major airways obstruction in the postoperative period. Autopsy showed tracheo-bronchial anomalies which had not been diagnosed preoperatively. The second patient presented with hypoventilation of the right lung. After echocardiographic diagnosis of the anomaly, a thorough investigation (thoracic CT, helicoidal scan, bronchoscopy) was carried out and no associated bronchial malformations were observed. Reimplantation of the left pulmonary artery was successful and the postoperative course was uneventful. Retro-tracheal left pulmonary artery is a rare malformation Which is difficult to diagnose. It requires extensive pulmonary investigations and a multi-disciplinary approach. The prognosis is poor when there are associated tracheo-bronchial malformations.

Tetralogy of Fallot: transannular and right ventricular patching equally affect late functional status.

BACKGROUND: In tetralogy of Fallot, transannular patching is suspected to be responsible for late right ventricular dilatation. METHODS AND RESULTS: In our institution, 191 patients survived a tetralogy of Fallot repair between 1964 and 1984. Transannular patching was used in 99 patients (52%), patch closure of a right ventriculotomy in 35, and direct closure of a right ventriculotomy in 55. Two had a transatrial-transpulmonary approach. To identify predictive factors of adverse long-term outcome related to right ventricular dilatation, the following events were investigated: cardiac death, reoperation for symptomatic right ventricular dilatation, and NYHA class II or III by Cox regression analysis. Mean follow-up reached 22+/-5 years. The 30-year survival was 86+/-5%. Right ventricular patching, whether transannular or not, was the most significant independent predictor of late adverse event (improvement chi(2)=16.6, P:<0.001). In patients who had direct closure, the ratio between end-diastolic right and left ventricular dimensions on echocardiography was smaller (0.61+/-0.017 versus 0. 75+/-0.23, P:=0.007), with a smaller proportion presenting severe pulmonary insufficiency (9% versus 40%, P:=0.005). There was no difference between right ventricular and transannular patching concerning late outcome (log rank P: value=0.6), right ventricular size (0.70+/-0.28 versus 0.76+/-0.26, P:=0.4), or incidence of severe pulmonary insufficiency (30% versus 43%, P:=0.3). CONCLUSIONS: In tetralogy of Fallot, transannular patching does not result in a worse late functional outcome than patching of an incision limited to the right ventricle. Both are responsible for a similar degree of long-term pulmonary insufficiency and right ventricular dilatation.

[Interventional catheterization in infantile congenital aortic valve diseases]. / Cathétérisme interventionnel dans les valvulopathies aortiques congénitales en pédiatrie.

The authors present their experience of percutaneous aortic valvuloplasty in 10 neonates and 12 children with critical aortic stenosis. In the neonate group the left ventricular aortic gradient dropped from 49 +/- 20 mmHg to 15 +/- 16 mmHg. This improvement persisted in 57 p. cent of the cases after 2 years. In the older group (mean age 6 years) the gradient was reduced from 73 +/- 22 mmHg to 30 +/- 17 mmHg with maintained results in 78 p. cent after 2 years. Aortic incompetence occurred in the majority of cases but did not seem to be more important than after surgical commissurotomy. In critical stenosis, balloon aortic valvuloplasty seems to be a valuable alternative to surgery as a palliative measure before prosthetic valve replacement.

[Dilation of the aortic root in ventricular septal defects. Risk factor of aortic insufficiency]. / La dilatation du bulbe aortique dans la communication interventriculaire. Facteur de risque d'insuffisance aortique?

The diameter of the aortic root was measured in 151 patients with small ventricular septal defect (Qp/Qs less than 2 and pulmonary vascular resistance less than 1.5 U.m-2) and correlated with the presence of absence of aortic regurgitation. In 26 cases the aortic juxtavalvular region (bulbus) was dilated above 2 SD compared to a control group. All patients with aortic regurgitation of with cusp prolapse belonged to this group of 26 cases. Aortic root dilatation seems to have a specificity of 89 p. 100, a sensitivity of 75 p. 100 and a negative predictive value of 98 p. 100 in relation to the occurrence of aortic insufficiency. The finding of an aortic root dilatation associated with a ventricular septal defect should incite to a closer supervision of non-operated patients should be used as an argument when discussing the surgical closure of the ventricular septal defect.

[Arrhythmogenic dysplasia of the right ventricle in a 16-month-old child]. / Dysplasie arythmogène du ventricule droit chez un enfant de 16 mois.

The clinical history of a 16-month old child with an arrhythmogenic right ventricular dysplasia is reported. This is one of the youngest patients described in the literature. The pitfalls of the diagnosis and the particularities of the treatment of recurrent ventricular tachycardias are underlined.

[Congenital heart diseases and obstructive pulmonary vascular diseases in Down's syndrome. Apropos of 142 children with trisomy 21]. / Cardiopathies congénitales et maladie vasculaire obstructive pulmonaire dans le syndrome de Down.

Trisomy 21 accounts for 3 p. 100 of reasons for admission to the Paediatric Cardiology unit of the St Luc University Clinics, Brussels. In a series of 142 cardiac children with trisomy 21 evaluated by catheterization between 1969 and 1987, 54 p. 100 of the cardiac malformations observed consisted of persistent common atrioventricular canal (complete in 45 p. 100 of the cases). The other heart diseases were ventricular septal defect (23 p. 100), atrial septal defect of the ostium secundum type (10 p. 100) and tetralogy of Fallot (9 p. 100). In 40 p. 100 of the patients other cardiovascular abnormalities were associated with these predominant intracardiac shunts. These findings were in agreement with those usually reported in the literature. At the time of investigation (mean age 24 months), pulmonary vascular resistance had already reached a pathological level in 88 p. 100 of the cases. Oxygen tests only slightly improved these results, which suggested that the conditions were favourable to the early development of a pulmonary obstructive vascular disease in Down's syndrome, thus darkening the prognosis of congenital heart disease in mongoloid children. In this series to overall mortality rate of corrective surgery was 23 p. 100. The risk was maximum in infants aged less than 3 months with severe and rapidly symptomatic lesions. The outcome in patients successfully operated upon was satisfactory, with benign residual lesions.(ABSTRACT TRUNCATED AT 250 WORDS)

[Results of the intracardiac repair of tetralogy of Fallot and interventricular communication before the age of 2 years]. / Résultats de la réparation intracardiaque des tétralogies de Fallot et des communications interventriculaires avant l'âge de 2 ans.

The post-operative review of 81 patients operated for a large interventricular communication before the age of two years reveals excellent results: the mean age at operation was 7 months, extracorporeal circulation was performed in 25 patients for a mean duration of 77 minutes and profound hypothermia to 18 degrees C was induced in 56 patients. The atrial approach was used in 30% of cases. The mean follow-up period is 26 months. There was one case of complete bundle branch block, which was subsequently fitted with a pacemaker, 7 cases of bifascicular branch block and a right bundle branch block in the majority of cases. The pulmonary vascular resistance was virtually normal (a mean of 3.2 U.m-2 prior to the operation and a mean of 1.9 U.m-2 after the operation). The left ventricular volume, which was markedly increased in all patients preoperatively, returned to normal with maintenance of a normal ejection fraction. The neuro-psychomotor behaviour was abnormal in 13 of the 63 patients tested. These abnormalities are not related to the correction procedure, but to pre-existing pathology (small birth weight). 52 patients from a series of 63 cases operated for tetralogy of Fallot before the age of 2 years (median age 12 months) were also reviewed. Extracorporeal circulation lasting a mean of 115 minutes was performed in 31 patients, cardiac arrest under profound hypothermia was induced in the other cases, for a mean duration of 61 minutes. The mean follow-up period was 29 months.(ABSTRACT TRUNCATED AT 250 WORDS)

[Severe hypertrophic myocardiopathy in newborn infants. Intra-erythrocyte calcium and the effect of lidoflazine, a calcium-channel inhibitor. Apropos of 2 cases]. / Myocardiopathie hypertrophique sévère du nouveau-né. Calcium intra-érythrocytaire et effet de la lidoflazine, inhibiteur d'entrée calcique. A propos de deux cas.

Two cases of severe hypertrophic obstructive cardiomyopathy in the neonate are reported. The first case was poorly tolerated because of predominant stenosis of the right ventricular outflow tract causing right to left shunting through a patent foramen ovale. The second case presented with severe syncope at 6 weeks of age. The first patient was treated with propranolol without any success. Regression of the clinical and electrocardiographic signs was obtained in both cases with lidoflazine, within a few weeks. Red blood cell calcium concentrations were abnormally high in both patients before treatment and returned to normal levels with clinical and echocardiographic improvement. Abnormal accumulation of intracellular calcium in the myocardium is a possible mechanism of this cardiomyopathy. The relation between this type of accumulation and the effects of catecholamine discharge are recalled. The possible initiating role of an enzymatic abnormality of calcium entry is discussed: the chaotic geometric alignment of the myocardial fibres, characteristic of this type of hypertrophy, would therefore be a secondary phenomenon. Once constituted, the hypertrophy would then become irreversible. However, it may be possible to reverse it in the neonate by calcium antagonists as suggested by these two cases.