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Biomedical subjects

L Iserin

Publications and source records attributed to L Iserin.

At least 37 records · Page 2Linked to original sources

[Compromise of the cardiac autonomic nervous system in patients with Fontan-type circulation: preliminary data].

BACKGROUND: Rhythm disturbances are frequent after Fontan operations. Arrhythmias related to reduced heart rate variability (HRV) have been described in various cardiovascular diseases. METHODS: We attempted to investigate HRV in 12 patients who underwent Fontan operation (age 11.4 +/- 3 years). Results were compared to a control group of 13 children matched for age, sex and heart rate (10.4 +/- 3 years). All patients underwent 24-hour Holter monitoring. The following time domain indexes were calculated: mean duration of RR intervals, standard deviation of all RR intervals (SD), square root of the mean squared differences of successive RR intervals (r-MSSD), percentage of differences between adjacent RR intervals > 50 msec (pNN50). The following frequency domain indexes were calculated: total power (TP), low frequency (LF), high frequency (HF), LF/HF ratio. RESULTS: The following indexes were significantly reduced in Fontan patients: SD (p < 0.0001), r-MSSD (p < 0.0001), pNN50 (p = 0.0002), TP (p < 0.0001), LF (p < 0.0001), HF (p = 0.0001). LF/HF increased significantly (p = 0.04). No differences were detected according to the type of operation (cavopulmonary connection vs atriopulmonary connection) or clinical status. CONCLUSIONS: Patients with Fontan circulation had a significantly reduced HRV and particularly abnormal sympatho-vagal balance. Surgery on the caval veins and the atria alters the intracardiac ganglia that are abundant at the cavo-atrial junction and in the myocardium of the right atrium. Abnormalities of HRV in Fontan patients may act as a co-factor in the initiation of arrhythmia in these patients.

Adolescent↗

[A new anatomical approach to congenital valve diseases by three-dimensional echocardiography].

Congenital valvular heart disease in childhood is often complex. Conventional echocardiography provides two-dimensional views which require mental reconstruction for three-dimensional assessment. This problem may be solved by the use of three-dimensional (3D) echocardiography which obtains images of valves comparable to those seen at surgery. This was confirmed by 4 cases of congenital valvular heart disease studied by 3D echocardiography: stenotic bicuspid aortic valve disease, parachute mitral valve and two cases of mitral regurgitation in patients with atrioventricular canal. The 3D views of the aortic valve showed the commissural opening after percutaneous balloon valvuloplasty of the bicuspid valve. The surface of the aortic orifice and the surface of the two mitral leaflets were measured from 3D reconstructions. The longitudinal 3D view analysed the extension of the single obstructive mitral papillary muscle of the parachute malformation. The 3D ventricular views allowed assessment of the extension of the cleft and the surface of the three mitral leaflets of the 2 cases of atrioventricular canal. In one of these cases, the results of surgical valvuloplasty were evaluated after 3D reconstruction of the valve. 3D echocardiography is not only a diagnostic tool for congenital heart disease but also a very useful complementary investigation for accurate evaluation of congenital valvular lesions to optimise possible valve repair.

Adolescent↗

[Treatment of obstruction of prosthetic conduct by percutaneous implantation of stents].

Prosthetic conduits, with or without biological valves, are often inserted in surgical procedures to correct or palliate cardiac malformations. The principal problem is degeneration which causes variable degrees of obstruction requiring reoperation for their replacement. The aims of this study were to assess the feasibility, safety and efficacy of a non-surgical method of treating these obstructive prostheses by dilatation-implantation of a metallic vascular endoprosthesis (stenting). Thirteen patients were treated (age range 7.7 to 36 years; mean: 15 years). Eight had pulmonary atresia with a ventricular septal defect corrected by a valved conduit from the right ventricle to the pulmonary artery which became obstructive nearly 10 years later: the implantation of the stent reduced the transconduit pressure gradient in all cases except one who had not undergone closure of the septal defect in which the cyanosis was improved. There are two cases of obstruction of a modified Blalock anastomosis in which the stent revascularised the shunt with improvement in cyanosis. In the final 3 cases, the whole Fontan procedure was compromised by obstruction of a conduit incorporated in the system, and which dilatation with stenting considerably improved. The efficacy of the procedure was constant with no complications other than rupture of the balloon in 3 cases. The good results were maintained for an average of 7.3 months (range 1 to 25 months), but it was necessary to redilate one restenosed stent after 8 months. Dilatation followed by stenting in obstructive cardiovascular prostheses is a simple, safe and effective alternative to surgical reoperation.

Adolescent↗

Prevalence of the microdeletion 22q11 in newborn infants with congenital conotruncal cardiac anomalies.

UNLABELLED: Conotruncal malformations account for about 50% of congenital heart defects diagnosed in newborns. We studied prospectively 104 patients admitted in our neonatal intensive care unit for conotruncal defects by fluorescence in situ hybridization to estimate the prevalence of the interstitial deletion in this category of congenital heart disease. Cardiac phenotypes were: truncus arteriosus (17), interrupted aortic arch (18), tetralogy of Fallot with or without pulmonary valve atresia (55), tetralogy of Fallot with absent pulmonary valves (5), ventricular septal defect with malalignment of the conal septum (9). We discovered a microdeletion 22q11 at loci D22S39 or D22S398 in 50 newborns (48%). The prevalence of this microdeletion in different groups of conotruncal defects was: truncus arteriosus 7/17, interrupted aortic arch 16/18, tetralogy of Fallot 19/55, absent pulmonary valves 2/5, and ventricular septal defect 6/9 respectively. Only two patients without any clinical or biological feature of the so called CATCH22 syndrome exhibited the deletion. Parental studies confirmed that the deletion occurred de novo in 47/50 cases (three parental microdeletions). On the other hand, recurrence of conotruncal heart defects in families of "undeleted probands" was higher than expected (13%). CONCLUSION: In 50/104 newborns with conotruncal defects, an interstitial deletion 22q11 was found. Fluorescence in Situ Hybridization should be performed in newborn infants with conotruncal defect and at least one additional manifestation of the CATCH22 phenotype.

Chromosome Deletion↗

Relation of genotype 22q11 deletion to phenotype of pulmonary vessels in tetralogy of Fallot and pulmonary atresia-ventricular septal defect.

OBJECTIVE: To compare the morphology of the pulmonary vessels in tetralogy of Fallot or pulmonary atresia-ventricular septal defect (PA-VSD) with (del22q) and without 22Q11 deletion (non-del22q). PATIENTS: 94 consecutive infants (54 with tetralogy of Fallot, 40 with PA-VSD) were studied using ultrasound and catheterisation. MOLECULAR INVESTIGATIONS: Identification of the 22q deletion was performed either by fluorescent in situ hybridisation or polymerisation chain reaction genotyping. RESULTS: 25 patients were del22q (16/40 (40%) PA-VSD v 9/54 (17%) tetralogy of Fallot; p < 0.02). Major aortopulmonary collateral arteries was more common in patients with PA-VSD-del22q (p < 0.03). Such collaterals were identified in 13 patients: 10 del22q and three non-del22q (p < 0.001). The size of the right and left pulmonary arteries expressed as a standard deviation (SD) difference of the normal range was -4.2 (quartiles -5.3 and -2.9) for PA-VSD del22q, and -2.6 (-3.1 and -1.8) for PA-VSD non-del22q (p = 0.02). The mean (SD) difference between the measured and theoretical Nakata index was -373 (94) for PA-VSD del22q v -245 (93) in PA-VSD non-del22q (p = 0.0002). In tetralogy of Fallot patients with and without del22q, the size of the pulmonary arteries was similar (p = 0.6). CONCLUSIONS: A "specific" phenotype could be defined in patients with deletion: PA-VSD, major aortopulmonary collateral arteries with complex loop morphology, and small central pulmonary arteries. Differences in the morphology of the pulmonary vessels may indicate a different timing of the faulty developmental pathway in patients with and without 22q11 deletion.

Aorta↗

[A pulmonary vascularization study in pulmonary atresia with an interventricular defect in relation to the presence of a chromosome 22 deletion].

A normal lung is supplied by a pulmonary artery branching from the pulmonary trunk. Major aorto-pulmonary collateral arteries (MAPCAs) are found in combination with various congenital heart malformations such as pulmonary atresia with ventricular septal defect (PA-VSD). Now that MAPCAs are used for unifocalization in patients with PA-VSD, the question arises as to whether the morphologic criteria of these collateral arteries could help to provide better results. We compared the morphology of the pulmonary vascular bed, the origin, course and connections of the MAPCAs in 40 consecutive infants with PA-VSD with or without 22q deletion (del22q11.2.). All underwent echocardiographic evaluation and catheterization. Identification of del22q11.2. was performed by FISH study. Del22q11.2. was identified in 16 pts (40%); the presence of MAPCAs was significantly higher in patients with del22q11.2. (9/16 vs 3/24, p = 0.01). While complex morphology of MAPCAs, anastomoses with the central pulmonary artery outside the lung and absent ductus arteriosus were associated with del22q11.2, confluence of the pulmonary arteries was not a relevant phenotypic difference. The size of the right and left pulmonary arteries expressed as a standard deviation difference of the normal range for body surface area was -4.2 (quartiles -3.1/-1.8) for PA-VSD with del22q11.2. and -2.6 (quartiles -5.3/-2.9) for PA-VSD without del22q11.2. (p = 0.02). The difference between measured and theoretical Nakata index was -373 +/- 94 for PA-VSD with del22q11.2. vs. -245 +/- 93 for PA-VSD without del22q11.2. (p = 0.0002). A specific pulmonary vascular bed phenotype could be defined in patients with PA-VSD with del22q11.2. deletion: MAPCAs with complex loop morphology and small but confluent central pulmonary arteries. These findings indicate a different timing of the faulty development pathway of the pulmonary vascular bed in patients with and without del22q11.2. This phenotype difference may help our understanding of maldevelopment and facilitate decisions concerning the suitability of these arteries for unifocalization procedures.

Angiography↗

[Anatomic evaluation of ostium secundum atrial septal defects by tridimensional echocardiography].

The decision to close an ostium secundum atrial septal defect by interventional catheterisation implies knowing its size, form and the relationship of its borders to neighbouring structures as accurately as possible. Three-dimensional echocardiography provides unique views of the interatrial septum and the authors set out to assess its performance. Ten patients, aged 8 to 20 years, included in a multicenter European clinical trial of closure of atrial septal defects with the CardioSEAL prostheses, were examined by transoesophageal echocardiography with three-dimensional reconstruction of the interatrial septum viewed from the left or right atrium. The septal defect had a very variable morphology, round, oval raquet-shaped and occasionally multiple. The surface area of these defects varied by about 70% during the cardiac cycle, maximal during ventricular systole and minimal during atrial systole. The maximal diameter measured by two-dimensional transoesophageal echocardiography underestimated that measured by three-dimensional echocardiography by about 30%. Two patients had a juxta-aortic caudal border or a juxta-superior vena caval cephalic border making the defect unsuitable for catheter insertion of a CardioSEAL occluder. On the other hand, another patient had an adequate juxta-aortic border although it seemed too narrow with conventional imaging techniques. The authors conclude that three-dimensional reconstruction of transoesophageal echocardiography is the best method of selecting candidates for closure of ostium septum atrial septal defect by intervantional catheterisation.

Adolescent↗

Iopentol (Imagopaque 300) compared with iopromide (Ultravist 300) in pediatric angiocardiography. A clinical trial assessing adverse events, ECG and diagnostic information.

Iopentol (Imagopaque, Nycomed Imaging AS, Oslo, Norway) 300 mg.l/ml was compared with iopromide (Ultravist, Schering AG. Berlin, Germany) 300 mg I/ml in pediatric angiocardiography in 97 children (48 and 49 patients, mean age 2.8 and 4.1 years in the respective contrast medium groups). The volume injected was usually 4-6 ml/kg b.w. ECG, blood pressure, heart rate, adverse events and efficacy were evaluated. Five patients in the iopentol group and four in the iopromide group reported adverse events. This difference was not statistically significant. One adverse event in each of the two groups was considered as possibly related to the contrast medium. Only small and transient changes in heart rate, blood pressure and ECG parameters were observed. No difference between the two contrast media was found for efficacy. It can be concluded that iopentol is well suited for angiocardiographic examinations in children.

Angiocardiography↗

Effects of chronic hypoxemia on chemosensitivity in patients with univentricular heart.

OBJECTIVES: We sought to compare the arterial blood gas chemosensitivity in relation to exercise ventilatory response in patients with univentricular heart and cyanosis and in patients with univentricular heart and Fontan-type circulation without cyanosis. BACKGROUND: Patients with univentricular heart demonstrate excessive ventilation during exercise. Chronic hypoxemia may alter chemoreceptor function, affecting ventilation. METHODS: Cardiopulmonary exercise testing was performed in 10 patients with rest or stress-induced cyanosis (cyanotic group: mean age +/- SE 30.5 +/- 2.3 years; 5 men), 8 patients without cyanosis with Fontan-type circulation (Fontan group: mean age 29.4 +/- 1.5 years; 4 men) and 10 healthy control subjects (normal group: mean age 30.7 +/- 1.9 years; 5 men). Hypoxic and hypercapnic chemosensitivity were assessed by using transient inhalations of pure nitrogen and the rebreathing of 7% CO2 in 93% O2, respectively. RESULTS: Peak O2 consumption was comparable in both patient groups (21.7 +/- 2.5 [cyanotic group] vs. 21.0 +/- 1.9 ml/kg per min [Fontan group]) but was lower than that in the normal group (34.7 +/- 1.9 ml/kg per min). The ventilatory response to exercise, characterized by the regression slope relating minute ventilation to CO2 output, was higher in the cyanotic group (43.4 +/- 4.0) than in the Fontan group (31.4 +/- 3.0, p = 0.02) and the normal group (23.1 +/- 1.1). Hypoxic chemosensitivity was blunted in the cyanotic group compared with that in the Fontan and normal groups (0.148 vs. 0.448 [p = 0.02] vs. 0.311 liter/min per percent arterial O2 saturation, respectively) and did not correlate with the ventilatory response to exercise (r = -0.36, p = 0.29). In contrast, hypercapnic chemosensitivity represented by the slope of the hypercapnic-ventilatory response line was similar in the cyanotic, Fontan and normal groups (1.71 vs. 1.76 vs. 1.70 liter/min per mm Hg, respectively), but the response line had shifted to the left in the cyanotic group (x intercept = 31.9 vs. 39.9 mm Hg [p = 0.026]), compared with 45.2 mm Hg in normal subjects. These findings suggest that in the cyanotic group, ventilation is greater for a given level of arterial CO2 tension and thus may partly explain the increased exercise ventilatory response in this group. CONCLUSIONS: Hypoxic chemosensitivity is blunted in patients with univentricular heart and cyanosis and does not determine the exercise ventilatory response. CO2 elimination appears more important. The blunting of hypoxic chemosensitivity is reversible once chronic hypoxemia is relieved, as evident in the Fontan group.

Adult↗

Dyspnoea and exercise intolerance during cardiopulmonary exercise testing in patients with univentricular heart. The effects of chronic hypoxaemia and Fontan procedure.

BACKGROUND: Patients with univentricular hearts have decreased exercise tolerance and may demonstrate exertional dyspnoea. It is not known if chronic hypoxaemia exacerbates exercise intolerance and contributes to symptomatic limitation. The extent to which surgical correction of a right-to-left shunt by a Fontan-type procedure can increase exercise tolerance by reducing arterial deoxygenation is not well documented. The cardiopulmonary exercise responses and the symptomatic status in two groups of univentricular patients, those who are cyanotic and those who are acyanotic with Fontan-type circulation, were compared. METHODS AND FINDINGS: Cardiopulmonary exercise testing was performed in 10 univentricular patients with rest or stress-induced cyanosis (age 30.5 +/- 2.3 [SE] years; 5 men) who had palliative or no surgery and eight patients (age 29.4 +/- 1.5 years; 4 men) with Fontan-type circulation. Peak oxygen consumption was comparable in both groups of univentricular patients (21.7 +/- 2.5 vs 21.0 +/- 1.9 ml.kg-1.min-1, P = 0.85) but was less than an age-matched group of 10 healthy subjects (34.7 +/- 1.9 ml.kg-1.min-1, P < 0.001 for both). Arterial oxygen saturation was 90.6% at rest in the cyanotic patients compared with 95.1% in the Fontan patients (P < 0.001) and at peak exercise, 66.2% compared with 90.5% (P < 0.001). Using a modified Borg scale (0-10), the symptoms of dyspnoea and fatigue were also assessed during exercise in the patient groups. The Borg scores for dyspnoea in the cyanotic and the corrected univentricular patients were, respectively, as follows: Stage 1: 0.5 vs 1.7; P= 0.04; Stage 2: 1.8 vs 2.3, P = 0.5; Stage 3: 3.0 vs 3.5, P = 0.7; Peak Exercise: 4.9 vs 4.8, P = 0.9. In addition, the Borg scores for fatigue were: Stage 1: 0.4 vs 1.6, P = 0.08; Stage 2: 2.0 vs 2.2, P = 0.9; Stage 3: 3.0 vs 4.3, P = 0.5; Peak Exercise: 4.9 vs 5.4, P = 0.5. The major limiting symptom at peak exercise was dyspnoea in four cyanotic patients compared with one in the Fontan group (Chi-square 0.982, P > 0.10). The arterial oxygen desaturation at peak exercise in the cyanotic patients limited by dyspnoea was not different from those limited by fatigue (67.5 +/- 10.1% vs 66.7 +/- 13.7%, P = 0.92). Exercise tolerance was also not related to the arterial oxygen saturation at peak exercise (r = 0.47, P = 0.17) in these patients. CONCLUSIONS: Despite correction with Fontan-type surgery, the exercise tolerance and symptoms of these univentricular patients remained similar to those who were cyanosed. Cyanotic patients have adjusted to chronic hypoxaemia and it does not appear to determine the exercise tolerance or the genesis of dyspnoea in these patients. Further randomized prospective studies are required to investigate the long-term benefits of Fontan-type procedures in these patients on exercise tolerance, symptoms and prognosis.

Adult↗

[Marfan's syndrome. Cardiovascular manifestations and therapeutic indications].

Marfan's syndrome is a disease of collagen microfibrils, the essential composition of which, fibrillin, is defective. The coding gene of this large protein has been localised on chromosome 15 and partially cloned. Its mutation is transmitted in the autosomal dominant mode. It is common, affects many organs, especially those rich in collagen. They include the skeleton, the eye and cardiovascular system. The prognosis of these patients depends on the degree of cardiovascular involvement; the aorta is the principal site of the lesions, particularly the aortic root which tends to develop aneurysmal dilatation, then dissection, the main cause of premature death of these patients. Mitral valve prolapse giving rise to mitral regurgitation may also be observed. Much progress has been made is recent years in the management of Marfan's syndrome, especially in ultrasound follow-up of the aortic root and the definition of risk factors for dissection. In parallel, advances have been made in the treatment, both medical with the use of betablockers to try to slow progression to aneurysm, and surgical, in replacement of the aorta and aortic valve in time and under optimal conditions. Life expectancy is thereby significantly increased. Similar progress has been made to allow these patients to have a pregnancy, classically thought to carry a high risk of maternal death.

Adrenergic beta-Antagonists↗

[Cardiac MIBG imaging: a new marker for myocardial function in children?].

MIBG cardiac imaging is a non-invasive procedure of studying neuronal recapture of noradrenaline. In adult cardiomyopathy, abnormal results are observed earlier than the increase in circulating catecholamines and constitute a reliable prognostic indicator of the disease. The authors assessed this technique in children with severe cardiac dysfunction in whom the evaluation of ventricular pump function was of primordial importance for the therapeutic decision. Twenty-eight patients aged 3 months to 20 years (average 6.4 +/- 5.8 years) were included in the study. Twenty had hypokinetic dilated cardiomyopathies: with respect to the normal values in adults MIGBG uptake was reduced in all cases. This alteration was correlated with the severity of myocardial dysfunction assessed by echocardiography but is was without doubt the expression of another type of physiopathological process. No relationship was observed with the aetiology of the cardiomyopathy. Eight children had a single ventricle treated by cavopulmonary bypass in 7 cases and by pulmonary artery banding in the other case: MIBG imaging did not allow assessment of myocardial function in these patients probably because the cavopulmonary bypass denervated the heart and thereby changed MIBG uptake independently of the cardiac function. These preliminary results are encouraging and should lead to the adoption of MIBG imaging as one of the key investigations in the functional evaluation of childhood cardiomyopathy and perhaps as an important parameter in the decision of transplantation in this indication.

3-Iodobenzylguanidine↗

[Treatment and prognosis of tachyarrhythmia after atrial surgical repair of transposition of great vessels].

The Mustard and Senning procedures may be complicated by atrial tachyarrhythmias. In order to determine their prognosis, the authors undertook a review of the 288 patients operated and followed up in their department. Thirty-six (12.5%) had documented atrial tachycardias. In 13 cases, Holter monitoring showed bursts of atrial tachycardia. In the other 23 cases, the tachycardia was sustained and often caused severe symptoms (syncope). Reduction of the sustained forms was obtained by rapid atrial pacing, cardioversion or amiodarone therapy. After restoration of sinus rhythm, several therapeutic protocols were used, often in the same patient: abstention, leading to 5 recurrences in 6 cases; treatment with betablockers in 12 patients with well tolerated or exercise-induced atrial tachycardia with 11 successes; amiodarone, with 4 relapses out of 5 when the dosage was less than 200 mg/m2/day and 13 successes out of 18 when the dosage was 200-250 mg/m2/day. During a follow-up of 1 to 19 years, 6 severe complications (neurological sequellae, cardiac failure), and 6 deaths, were observed. All these patients were poorly controlled and continued to be have tachyarrhythmias. The authors conclude that tachyarrhythmias are neither rare nor always symptomatic, so justifying their systematic investigation. They should be reduced by oral amiodarone or rapid atrial pacing depending on the clinical urgency, never by intravenous antiarrhythmics which can be dangerous. The poor prognosis associated with tachyarrhythmias makes their control essential either with betablockers or good doses of amiodarone, eventually covered by permanent cardiac pacing.

Adolescent↗

Sternal cleft: case report and review of a series of nine patients.

Cleft sternum is a rare malformation due to partial or total failure of sternal fusion at an early stage of embryonic development. Sternal clefts can be classified as superior, inferior, or complete. Here we report on a 2-year-old boy with inferior sternal cleft and complex cardiac malformation. We review a series of 9 children with sternal clefts, referred to us over a 10-year period. Hypothetical mechanisms for this developmental anomaly are discussed.

Abnormalities, Multiple↗

Upper limb malformations in DiGeorge syndrome.

We report on upper limb anomalies in two children with a complete DiGeorge sequence: conotruncal defects, hypocalcemia, thymic aplasia, and facial anomalies. One child had preaxial polydactyly, and the other had club hands with hypoplastic first metacarpal. In both patients, molecular analysis documented a 22q11 deletion. To our knowledge, limb anomalies have rarely been reported in DiGeorge syndrome, and they illustrate the variable clinical expression of chromosome 22q11 deletions.

Chromosome Deletion↗

[Percutaneous occlusion of patent ductus arteriosus by the Rashkind double-umbrella device].

BACKGROUND: The transcatheter option consisting of implanting and releasing an occlusive device designed as a double-umbrella is an interesting alternative to surgery aimed to close persistently patent ductus arteriosus. POPULATION AND METHODS: Closure of a duct with the Rashkind device had been planned in 113 children. The procedure was abandoned in 12 with inadequately sized ducts (too large or too small). This study therefore included 101 attempts in patients aged 2.3 months to 18.5 years (m +/- 1 SD = 45.9 +/- 43.2 months) whose weights ranged from 3.3 to 87 kg (m +/- 1 SD = 15.7 +/- 11.7 kg). The narrowest dimension of the duct on the aortograms ranged from 1.2 to 6.2 mm (m +/- 1 SD = 2.9 +/- 0.9 mm). RESULTS: The procedure failed in seven patients because of a too large and/or tubular vessel, causing removal of the device prior to release in five patients, or surgical extraction after it had embolized into a pulmonary artery branch in two patients. An early acute hemolysis requiring again the surgical removal of an instable device in a tubular duct was seen in one case. Two patients had femoral artery occlusion successfully treated with thrombolytic agents. Complete occlusion was immediately proven in 32 (35%) of the 92 successful and stable implantations. These figures raised to 64% (59 cases) prior to discharge. At final follow-up (0.3-59 months, m +/- 1 SD = 13.8 +/- 14.4 months), another 16 total occlusions were observed and one patient was successfully managed by a second implantation. The final occlusion rate was 83% (76 cases). Of the 16 residual shunts, five were surgically suppressed and the remaining were minimal. CONCLUSION: Transcatheter occlusion of the patent ductus arteriosus is safe in children weighing more than 5 kg, having ducts with a narrowing ranging from 1 to 6 mm. It is efficient in five out of six cases and has less disadvantages than surgery.

Adolescent↗

[Pulmonary atresia with ventricular septal defect: therapeutic strategy in newborn infants].

Pulmonary atresias with ventricular septal defect, a right ventricular infundibulum and pulmonary artery separated by an imperforated membrane and a complete pulmonary tree with two branches in continuity are called "favourable" forms of this malformation. The authors studied 29 neonates, less than 1 month old, in whom the malformation was both ductus- and prostaglandin-dependent, prostaglandin infusion being essential for pulmonary flow and impossible to stop because of the resulting severe hypoxia. The choice of treatment depended on the anatomical form defined by angiocardiography. In the 19 regular forms with regular pulmonary arteries with little hypoplasia, percutaneous perforation-dilatation was successful in 3 out of 5 attempts with one secondary death and 2 good results leading to complete repair; anastomosis was performed in 9 cases with 3 deaths, 1 partial result and 5 good results which were followed by complete repair in 3 cases; primary complete repair attempted in 7 cases led to 1 death and 6 successes completed in 3 cases by reoperation for left pulmonary artery stenosis. In the 10 less favourable anatomical forms with stenosis or severe hypoplasia or the pulmonary branches, only palliative procedures were proposed: 2 perforations-dilatations which only gave partial results, 7 anastomoses with 1 death and 4 partial results; and 1 ventriculo-pulmonary connection without closure of the ventricular septal defect (good result). In view of the good results obtained over the 6 years of the study, the authors advise primary complete correction for the anatomically favourable forms of the malformation when weaning from prostaglandin infusion is impossible.(ABSTRACT TRUNCATED AT 250 WORDS)

Angiocardiography↗