[5-year results of arterial correction in transposition of great vessels]. / Résultats à 5 ans de la détransposition artérielle de la transposition des gros vaisseaux.

A prospective study was performed on in-hospital patients between June 1985 and July 1992 to assess the 5 year results of surgical detransposition of the great arteries. Clinical examination, electrocardiography, echocardiography, right and left heart catheterisation with selective coronary angiography, isotopic right and left ventricular ejection fractions at rest and with infusion of dobutamine and SestaMibi myocardial perfusion scintigraphy at rest and with dipyridamole, were performed during the 5th year after surgery. Twenty-six children underwent this protocol: eight others did not come for examination because they had moved from the region, one of whom had suffered regressive postoperative myocardial infarction. All patients were asymptomatic and had only minor electrocardiographic changes. Stenosis of the pulmonary tract was observed in 38.5% but only one case of stenosis at the origin of the right pulmonary artery required percutaneous angioplasty, which was successful. Pulmonary regurgitation was a common echocardiographic finding (65.4% of cases) but rarely severe (1/26: 3.9%). Aortic regurgitation was also observed commonly (53.8%), nearly always mild, grade I (13/14 cases). No significant stenosis of the aortic anastomosis was observed. The right and left ventricular ejection fractions were normal at rest except in one case and all values improved with dobutamine. Myocardial scintigraphy did not show any perfusion defect and there was no stenosis or occlusion of the coronary arteries at coronary angiography. The authors conclude that the results of arterial detransposition at 5 years are satisfactory in this series, with no cases of major obstructive lesions, major ventriculo-arterial regurgitation, ventricular dysfunction or coronary lesions. However, longer term trials with larger numbers of patients are required to determine the real incidence of coronary lesions and the long-term outcome of the pulmonary valve in the systemic position.

[Tilt-table test. Value in children and adolescents]. / Test d'inclinaison ou tilt test. Intérêt chez l'enfant et l'adolescent.

The tilt test is a non-invasive investigation which reproduces malaises or vagal syncope. This is a common condition in children and adolescents and the value of this investigation in this population is therefore self-evident. The tilt test was performed from 4 years of age in 105 children aged 4 to 18 years (mean 10 +/- 4 years) who had suffered one or several malaises with syncope. This test, using a resting period of 20 minutes, then a tilt of 70-80 degrees for 40 minutes, was considered to be positive if the symptoms could be reproduced or if the heart rate slowed to less than 50/min or if the blood pressure fell by more than 30 mmHg. The test was positive in 46 children (44%). Ten remained asymptomatic but had significant reductions in blood pressure: 36 children reproduced their symptoms. The positivity was related to a cardio-inhibitory response in 4 cases, vasodepressive in 20 cases, mixed in 20 cases and "psychiatric" in 2 cases: sinus tachycardia preceded positivity in 43 of the 46 cases. Only 21 children were treated either by disopyramide (12 cases) or betablockers (9 cases). All but one of the children became asymptomatic but the tilt test remained positive in 7 of the 9 patients treated with betablockers and in 3 children treated with disopyramide. The essential value of this investigation was to stop investigations in positive cases, to reassure parents and the children and to allow the pursuit of sporting activities. The authors conclude that the tilt test is an investigation easy to carry out in the young child and which spares complementary investigation in cases of positivity and guides medical treatment.

[Familial supravalvular aortic stenosis. Investigation in a family and review of the literature]. / Sténose aortique supravalvulaire familiale. Observation d'une famille et revue de la littérature.

Familial supravalvular aortic stenosis is a rare autosomal dominant condition. It may be distinguished from the Williams-Beuren syndrome by the absence of the characteristic dysmorphic appearances and of mental retardation. The case of a 9-year-old girl with a severe surgical stenosis led to the diagnosis of the same malformation in the mother and two brothers. This family adds to the 121 cases reported in the literature describing the main features of SVAS. Molecular biological advances have shown that familial SVAS and the Williams syndrome are due to mutation of the elastin gene located at 7q11-23. In the Williams syndrome the allele of this gene is completely absent and there is also probably deletion of contiguous genes, which explains involvement of cognitive function. In SVAS, the genetic lesion, mutation or microdeletion is more limited, explaining the usually isolated aortic malformation. Other studies are necessary to confirm these results.

[Diagnosis using x-ray computed angiotomography of an iliac artery aneurysm in an infant]. / Diagnostic par angiotomodensitométrie d'un anévrysme de l'artère iliaque chez un nourrisson.

The authors describe an infant with an aneurysm of the right iliac artery, associated with three sites involving the inferior mesenteric artery and its first two branches, most likely related to an episode of chicken pox. Helical angiography with computed tomography, performed with a continuous-rotation scanner, allowed precise assessment of the lesions, in particular those affecting the inferior mesenteric artery.

[French experience in the closure of atrial septal defects of the ostium secundum type with the Sideris button occluder]. / Expérience française de la fermeture des communications interauriculaires de type ostium secundum par la prothèse boutonnée de Sideris.

From February 1992 to November 1995, four French teams used the Sideris button occluder to close 122 ostium secundum, foramen ovale or surgical fenestration atrial septal defects in 121 patients aged 2 to 79 years with body weights of 10 to 96 kg. a left-to-right shunt in 110 cases (average QP/QS = 2.09) or right-to-left shunt in 12 cases. The usual type of prosthesis was used in 115 cases, 8 centered on a guide wire, reverse type in 5 cases and the "centering-device" type in 2 patients. Nineteen implantation attempts were abandoned before releasing the prosthesis. The immediate results were: closure of the atrial septal defect in 116 patients: 59 were completely occluded, 43 had minimal residual shunts. Five patients were operated for non-buttoning or malposition of the prosthesis. In one other case, the device was removed by catheterisation. During follow-up ranging from 1 month to 3 years, 20 patients were operated for varying complications, the commonest of which was malposition of the prosthesis (17 cases) with a shunt of variable volume. In one other case, a second device was inserted. Seventy-seven patients were reviewed at 1 year, 28 a 2 years and 6 at 3 years. The residual shunts decreased with time but only completely disappeared in half the cases. Secondary fractures not requiring surgery were observed in 5 patients. Failures and complications were the result of various causes which are discussed. Successive technological improvements and the experience of the medical teams should reduce this incidence, but caution is required especially in the treatment of young children.

[Percutaneous valvotomy of aortic valve stenoses in children]. / Valvulotomie percutanée des sténoses valvulaires aortiques de l'enfant.

A retrospective cooperative study of percutaneous aortic valvuloplasty was undertaken in 12 French centres from 1985 and included 90 children over 3 months of age (average 8.5 +/- 5.2 years) treated for isolated aortic valve stenosis with peak transvalvular pressure gradients of 80.7 +/- 23 mmHg. over 50 mmHg in 92% of cases. The majority of cases were performed by a retrograde femoral arterial approach with inflation of a balloon with a diameter approximating that of the aortic annulus. There was one serious complication (lethal collapse occurring before dilatation) and 12% of local complications due to arterial trauma: the latter were temporary or accessible to simple therapeutic measures. Overall, the pressure gradient decreased by an average of 39.2 +/- 25.4 mmHg. There were 15 immediate failures (17%) and 21 partial results (23%) requiring a further procedure at varying intervals after the valvuloplasty. There were 54 primary successes (60%) with annulation of the pressure gradient and this result was sustained in 45 of the 51 cases followed up for an average of 34 +/- 21 months. Aortic regurgitation was observed or aggravated in 29 children; at the end of the study, this remained a serious problem in 15 cases (17%). The authors conclude that interventional catheterisation is an elegant. simple and relatively economical alternative to conventional surgery. It is as safe, but less immediately effective overall; aortic continence may be compromised in the long-term. In the absence of technical innovations, a parallel development of the two therapeutic procedures is to be expected.

[Anomalous origin of the left main coronary artery from the right coronary sinus and trajectory to the great vessels. Value of echocardiography in a child]. / Naissance anormale du tronc coronaire gauche à partir du sinus coronaire droit et trajet par rapport aux gros vaisseaux. Apport de l'échocardiographie chez un enfant.

During a routine medical check-up, a pathological electrocardiogram was recorded in an asymptomatic 6 year old girl. The demonstration of coronary insufficiency by exercise stress testing and sestamib myocardio-scintigraphy led to the diagnosis of an anomalous origin of the left coronary artery. This vessel arose from the right coronary sinus and continued anteriorly to the main coronary artery. The data of the literature suggest that the risk of sudden death and of coronary insufficiency is associated with the passage of the left coronary artery between the aorta and pulmonary artery. The authors describe the anomalous origin of the left coronary artery and its trajectory with respect to the great vessels by echocardiography, using a high frequency transducer. The anterior direction of the left coronary artery in front of the main pulmonary artery may lead to ischaemia on effort even without stenosis at coronary angiography. Echocardiography may therefore be useful in children with positive exercise stress tests for diagnosing anomalous coronary vessels and their relationship with respect to the great vessels.

[Effects of age on the response to Tilt test in patients with malaise or syncopes]. / Effets de l'âge sur la réponse au test d'inclinaison chez les patients ayant des malaises ou syncopes.

The diagnosis of vasovagal malaise or syncope, suspected from the clinical history, may be confirmed by the tilt test. The aim of this study was to assess the effects of age on the results of this test in 346 patients who had unexplained malaise or syncope. Thirty-one patients were 7 to 19 years of age (group I), 59 were 20 to 40 (group II), 72 were 41 to 60 (group III) and 184 were 61 to 85 years old (group IV). The patients were maintained in the dorsal decubitus position for 20 minutes and then raised to 70 degrees until a malaise was observed or for a maximum of 40 minutes. The malaise or syncope was reproduced by the tilt test in 135 cases (39%). The number of positive responses was comparable in group I, II, III and IV (45, 42, 32 and 40%, respectively). The time before the malaise occurred was also similar in the four groups (17, 19, 15 and 20 minutes, respectively). Two responses to the tilt test characterised the different age groups: the greater number of malaises occurring independently of a drop in blood pressure or change in heart rate ("psychiatric" syncope) in group II compared with groups I, III and IV (40% versus 7, 9 and 9.5%); the higher frequency of pure vasodepressive forms in group IV compared with groups I, II and III (66% versus 28.5, 32 and 39%). In conclusion, the probability of a positive tilt test does not change with age. The mechanisms of the symptoms produced is the only difference observed with age.

Occlusion of large atrial septal defects with a centering buttoned device: early clinical experience.

A feasibility clinical study was conducted for the transcatheter occlusion of large ostium secundum atrial septal defects with the centering buttoned device. The centering buttoned device is a modification of the regular buttoned device in which a centering counter-occluder is sutured at the central 40% portion of the occluder. During centering it is stretched, forming a parachute-shaped structure and pulling the occluder over the center of the defect. During buttoning, the counter-occluder forms a double figure eight, opposing the right atrial side of the atrial septum. Occlusion was performed in 12 patients aged 6 to 56 years. All had been rejected for transcatheter occlusion by the regular buttoned device, because of either their defect size or the lack of adequate septal rim. The defect size varied between 23 and 31 mm, and the device size varied between 45 and 60 mm. Nine had immediate effective occlusions of their defects and three residual shunts. One patient with unbuttoning had hemolysis at 2 weeks and underwent surgery. Early results of the transcatheter occlusion of large atrial septal defects are promising, and larger clinical trials are justified.

[Long-term efficacy and tolerability of amiodarone in children]. / Efficacité et tolérance au long cours de l'amiodarone chez l'enfant.

The authors reviewed the files of 37 patients under 15 years of age (22 boys and 15 girls) in order to study the long-term efficacy and tolerance of amiodarone therapy. The mean age of the patients at the time of initiation of amiodarone was 6.2 +/- 4.7 years. Amiodarone was prescribed at a loading dose of 500 mg/m2 and at a maintenance dose of 250 mg/m2. This drug was prescribed in second intention in 29/37 patients and as monotherapy in 15/37 patients. The treated arrhythmias were supraventricular in 25 patients (atrial: 10; junctional: 15) and ventricular in 12 patients. Underlying cardiac disease was present in 21/37 patients (57%) and the arrhythmias were postoperative in 14/37 cases (38%). Efficacy and tolerance of amiodarone were estimated on clinical and biological data, the results of Holter monitoring, ophthalmological slit-lamp examination and thyroid function tests. The average duration of therapy was 4 +/- 3 years. The efficacy of amiodarone was judged to be good in 59% and satisfactory in 38% of cases. Secondary effects and complications included: corneal deposits: 14 cases (38%); skin pigmentation: 1 case; photosensitivity: 10 cases (27%). There was a high incidence of thyroid disorders: 7 cases (19%) with 4 cases of biological or clinical hyperthyroidism and 3 cases of clinical or biological hypothyroidism.(ABSTRACT TRUNCATED AT 250 WORDS)

[Repeated thrombolysis and difficulties in treatment with antivitamin K in an infant with mitral valve prosthesis]. / Thrombolyse itérative et difficultés du traitement par antivitamine K chez un nourrisson porteur d'une prothèse valvulaire mitrale.

The finding of cardiac failure in a neonate led to the diagnosis of congenital mitral regurgitation complicating dystrophic valves. After failed surgical valvuloplasty, the child underwent mitral valve replacement with a Saint-Jude medical prosthesis at the age of 4 months. The child developed four episodes of prosthetic valve thrombosis in the two years that followed. The first was treated surgically but the three others were treated by thrombolysis associating plasminogen tissue activator and urokinase. All but one of the thromboses occurred in a context of recent destabilisation of oral anticoagulant therapy despite the initiation of heparin. Repeat thrombolysis was successfully undertaken, thereby widening the indications of this type of treatment in the infant. This case also underlines the difficulties of oral anticoagulants in infants.

[Interventional catheterization in infants and neonates]. / Cathétérisme interventionnel chez le nourrisson et le nouveau-né.

Indications for interventional catheterism in new-born and very young infants have been widened through technical progress and clinical experience. Such procedures are still very difficult to perform at this age and many indications are still under debate. Currently interventional catheterism is used for curative treatment of tight pulmonary valve stenosis, the pulmonary stenosis in Fallot's tetrology, clinically threatening aortic valve stenosis in infants or new-borns. Other procedures include vessel dilatations, in particular for coarctation of the aorta, placing vascular stents, closing ductus arteriosus or vascular embolizations. The question of the respective role of surgery versus interventional catheterism remains open for many indications. As progress is made, both techniques remain valid and should be considered as complementary tools for many congenital heart diseases.

International experience with secundum atrial septal defect occlusion by the buttoned device.

Several devices are available for transcatheter occlusion of atrial septal defect. This report describes the international experience with the buttoned device. During a 4.5-year period ending in February 1993, 180 transcatheter atrial septal defect occlusions were performed with the buttoned device. Patient age varied between 0.6 and 76 years and stretched atrial defect diameter between 5 and 25 mm. The defects were closed with 25 to 50 mm devices delivered through 8F (148 patients) or 9F (32 patients) sheaths. Twelve patients were adults whose defects were closed to prevent recurrence of cerebrovascular accidents caused by presumed paradoxic embolism. In the remaining patients the atrial defect was closed to treat the left-to-right shunt. The atrial septal defects were effectively occluded as demonstrated by (1) decrease in pulmonary-to-systemic flow ratio from 2.1 +/- 0.6 (mean +/- SD) to 1.05 +/- 0.1 (p < 0.01) by oximetry; (2) normalized S2 and disappearance of the diastolic murmur by auscultation; and (3) improvement in right ventricular volume overloading by echocardiogram. However, trivial to small shunts could be detected by color Doppler studies in 76 (45%) of 168 patients in whom such data are available. Complications included unbuttoning in 13 and whole-device embolization in 1. All patients remained stable, and retrieval of the device and surgical closure of the atrial septal defect were accomplished in 10 patients. Transcatheter retrieval was used in the remaining 4 patients. The incidence of unbuttoning, a major complication of the procedure, appeared to decrease with the increasing experience of the investigators and with device modification (third-generation). The follow-up duration varied between 1 month and 4 years. Six patients required surgery during the follow-up period. In the remaining patients (n = 160), clinical examination did not reveal signs of atrial shunts. Color Doppler studies revealed either complete disappearance of the previously demonstrated shunts or further diminution of their size. The results indicate that transcatheter occlusion of the atrial septal defects with buttoned devices is feasible, relatively safe, and effective, and it appears to be a viable alternative to surgery for some patients with secundum atrial septal defect. Complications are infrequent and should improve with experience.

[Total cavopulmonary shunts. Short- and mid-term results apropos of 19 cases]. / Dérivations cavopulmonaires totales. Résultats à court et moyen terme, à propos de 19 cas.

Experimental and clinical studies show that total cavopulmonary shunt operations may prevent certain complications of the Fontan atriopulmonary procedure. The authors have used the total cavopulmonary shunt instead of the Fontan procedure since February 1990. Nineteen consecutive patients were operated by three different surgical teams; 18 patients had undergone prior palliative surgery. The shunt was carried out by Puga's modified Kreutzer technique or by the construction of an extracardiac autologous pediculated pericardial tube. There were two deaths (10.5%) attributed to severe stenosis of the left pulmonary artery. The postoperative course was complicated in most of the 17 survivors but after a follow-up of 3 to 57 months (average 24.9 +/- 13.6 months) all were in the NYHA functional classes I (N = 15) or II (N = 2) with an arterial saturation over 85% in 15/17 cases and a cardiothoracic index less than 53% in 14/17 cases. All were in sinus rhythm. Doppler echocardiography showed no obstruction of the bypass. Pulmonary flow in 11 cases had a venous morphology. The function of the single ventricle was slightly depressed in 3 cases (2 of which were operated late, at 16 years of age). Catheterisation and cavography in 13 children showed uniform pressures in the cavopulmonary connection with no pressure gradient, except in 1 child. A right atrial'a'wave transmitted to the pulmonary arteries and inferior vena cava accompanied by retrograde vena cava flow was observed in 4 of the 6 cases with a Puga or modified Kreutzer procedure. Fenestration of the connection was closed by a Sideris button device in one case; reoperation was required in another.(ABSTRACT TRUNCATED AT 250 WORDS)

[Mid-term results of balloon percutaneous valvuloplasty in the treatment of aortic valve stenosis in children and adolescents]. / Résultats à moyen terme de la valvuloplastie percutanée par ballonnet dans le traitement des sténoses valvulaires aortiques de l'enfant et de l'adolescent.

Fourteen consecutive patients aged 10.2 +/- 4.2 years with congenital valvular aortic stenosis underwent percutaneous balloon aortic valvuloplasty (PBAV) which reduced the peak-to-peak LV-aortic pressure gradient from 81.2 +/- 16.7 mmHg to 27.5 +/- 12.5 mmHg and the maximal instantaneous Doppler gradient (Dopp G) from 70.0 +/- 13.4 mmHg to 31.4 +/- 12.3 mmHg. These 14 patients were followed up 3.6 +/- 1.1 years after PBAV. Control Doppler examination showed the Dopp G (34.8 +/- 15.4 mmHg) to be comparable to that measured just after PBAV (31.4 +/- 12.3 mmHg). One patient, the only one with a mediocre, immediate result, had to be operated 5.5 years after PBAV. Aortic regurgitation was observed on aortography before PBAV in 10 patients: it remained unchanged in 7 and was aggravated in 3 patients. Aortic regurgitation was observed for the first time after PBAV in 3 patients. At control Doppler examination, aortic regurgitation was present on color Doppler in all cases but was mild or minimal in 9 cases. In one patient, secondary aggravation of aortic regurgitation required aortic valve replacement (homograft) 3 years after PBAV. Four of the 16 PBAV (25%) were performed in these 14 patients were complicated by a femoral arterial thrombosis. This study shows that the good primary results of PBAV in the treatment of congenital valvular aortic stenosis in childhood and adolescence, are maintained at medium-term. The risk of creating severe aortic regurgitation is not completely negligible but does not seem to be out of proportion compared with surgical valvotomy.

[Percutaneous angioplasty of aortic recoarctation: short- and mid-term results in 18 cases]. / Angioplastie percutanée de recoarctation de l'aorte: résultats dans 18 cas à court et moyen terme.

Between January 1986 and July 1992, 18 percutaneous angioplasties were performed in 18 consecutive patients with recoarctation of the aorta, who were aged 2 months to 29 years. Fifteen had been operated for coarctation of the aorta and 3 for interruption of the aortic arch. The diameter of the dilating balloon was the same, to 1 mm, as that of the healthy aorta, usually measured just proximal to the stenosis. There were 3 femoral artery thromboses; one small aneurysm which did not increase in size, and 1 hypertensive crisis in a child. The diameter of the stenosis increased by 42.39 +/- 34.24%, the gradient decreased from 41.39 +/- 14.58 mmHg to 23.7 +/- 16.30 mmHg and the Doppler gradient from 57.89 +/- 15.29 mmHg to 36.55 +/- 16.50 mmHg. Eight angioplasties, including 5 of the last 7 procedures, were considered primary successes by 2 criteria: increase of the diameter of the stenosis by at least 40% and a residual catheter gradient of less than 20 mmHg. The best results were obtained in severe, localised central stenoses. Sixteen patients were followed up for 2 to 69 months. Two were operated after failed angioplasty, without complications. No late aneurysms or restenoses were observed in the cases successfully dilated followed up clinically (7), by MRI (5) or catheterisation (2). In one case, aortography showed remodelling with normalisation of the aortic arch after 24 months. These results indicate that percutaneous angioplasty is a simple method of treating recoarctation of the aorta, which is most effective in severe stenoses: the immediate risk is low. The late risks of recoarctation and aneurysm justify systematic follow-up.

The Noonan syndrome. The Nancy experience revisited.

67 patients with Noonan syndrome seen over the last 29 years were selected preferentially on cardiac involvement. The cardiac anomalies consisted in the association of dysplastic pulmonary stenosis with asymmetric cardiomyopathy. In one patient, a translocation (3;22) was found. The relationship with cardio-facio-cutaneous syndrome and with the group of phacomatoses is discussed. The familial occurrence (10 families) seems compatible with autosomal dominant inheritance. A gene location on chromosome 22 cannot be excluded.