[Abnormal origin of the left coronary artery from the pulmonary artery: a retrospective study of 36 cases]. / Anomalie de naissance de la coronaire gauche à partir de l'artère pulmonaire: étude rétrospective de 36 patients.

The aim of this study was to show the evolution of diagnostic techniques revealing an abnormal origin of the left coronary artery and present the follow-up results of patients operated for this malformation at different ages. This retrospective study includes 36 children or adults, with a mean age at the moment of of 28 months old, ranging from 7 days to 39 years. In 9% of cases, patients were asymptomatic. Before 1990, 81% of children had a cardiac catheterization, versus 25% after. Indeed, echocardiography with color Doppler enabled the diagnosis of abnormal origin of the left coronary artery in 70% of cases. The pre-operative mortality is at 21% (12.5% after 1990). Twenty-three patients had surgery: left coronary artery reimplantation (n=16), bypass (n=5), at mean of 44 months old for the entire series, but 21 months old after 1990. The follow-up was clinical, ECG and radiographic and echocardiographic after 1975; when possible an exercise test, cardiac nuclear imaging and more recently coronary CT scan and MRI have been performed. In conclusion, children are operated earlier and the follow-up should focus on the detection of occlusion of the re-implanted left coronary artery by echocardiography, with or without stress, exercise test, cardiac nuclear imaging, MRI and sometimes, coronary angiography.

[Percutaneous angioplasty of native coarctation of the aorta. Results in children and young adults]. / Angioplastie percutanée de la coarctation native de l'aorte. Expérience d'un centre chez l'enfant et l'adulte jeune.

The authors report the experience of one centre in the treatment of native coarctation of the aorta by percutaneous angioplasty. Between 1999 and August 2005, 22 patients (7 girls, 15 boys) underwent dilatation of their coarctation by a balloon catheter at an average age of 11.8 +/- 5 years and body weight of 44 +/- 21 kg. In 20 patients, balloon angioplasty alone was performed and, in the other two, the angioplasty was associated with the insertion of a covered CP stent. The dilatation was performed with a balloon/coarctation ratio of 2.18 +/- 0.6 (1.7 to 3.5) and a balloon/isthmus ratio of 1.0 +/- 0.23 (0.7 to 1.87). After the procedure, ascending aortic pressure decreased from 134.7 +/- 23.4 mmHg to 125 +/- 22.7 mmHg (p= 0.0003); descending aortic pressure increased from 93.4 +/- 14.9 mmHg to 104.8 +/- 21.7 mmHg (p= 0.003); transisthmic pressure gradient decreased from 41.7 +/- 14.1 mmHg to 19.8 +/- 9.5 mmHg (p< 0.0001) and the diameter of the coarctation increased from 5.9 +/- 2.6 mm to 9.3 +/- 2.6mm (p= 0.0015). The two patients treated by covered CP stents had excellent immediate results without significant residual gradients. After dilatation (sometimes repeated, N= 13), a gradient > 20 mmHg persisted in ten patients (36% of cases) but no predictive factor was found on statistical analysis. There were no cases of acute aneurysm. In one patient, the femoral pulse decreased but returned to normal with heparin therapy. No patient required emergency surgical treatment after the angioplasty. During the clinical follow-up, echocardiography and MRI showed no signs of aneurysm and one patient had mild irregularity of the isthmic region. Three patients underwent surgery by resection suture for persistent coarctation (one combined with a Ross procedure); another patient was treated by a CP stent. In conclusion, percutaneous angioplasty of native coarctation of the aorta gives satisfactory results with few complications in bigger children and young adults. The results can be improved by using a slightly higher balloon/coarctation ratio.

[The effect of an endothelin receptor antagonist in Eisenmenger syndrome: a single-center experience of 11 patients]. / Effet d'un antagoniste des récepteurs de l'endothéline dans le syndrome d'Eisenmenger: expérience d'un centre à propos de 11 patients.

The Eisenmenger syndrome is a complication of congenital heart disease with significant left-to-right shunts, such as large ventricular septal defects, and corresponds to fixed pulmonary hypertension with shunt reversal. Bosentan, an inhibitor of endothelin A and B receptors, is a new molecule previously validated in the treatment of primary pulmonary hypertension. The authors report their monocentric experience of bosentan in 11 consecutive patients with the Eisenmenger syndrome treated for at least one year. This retrospective study comprised 7 females and 4 males with an average age of 34 years (range 17 to 51 years). The underlying lesion was ventricular septal defect (n = 4), atrial septal defect (n = 3), pulmonary atresia with septal defect (n = 4 of which 2 were treated palliatively). Before treatment, the patients were classified according to the NHYA functional class (I, II, IIIa and IIIb, IV or, respectively from 1 to 5) with a distribution in this series between Classes IIIa and IV (average 3.81 +/- 0.75) and from 3 to 10 on Borg's dyspnoea scale (average 6.54 +/- 2.29). The ambient oxygen saturation (SaO2) at rest was, on average 77 +/- 9%, the haemoglobin concentration 16.6 +/- 2.4 g/dl; hepatic transaminase levels were normal. The 6 minute walk test before treatment was 216 +/- 111 m with marked desaturation on exercise (49 +/- 18%). With Bosentan, patients were globally much better clinically with a decrease in dyspnoea, improvement in NHYA class and increased 6 minute walking perimeter. Improvement in NYHA class was observed from 3 months' treatment (3.0 +/- 0.8, p = 0.0002) and was sustained to one year (2.54 +/- 0.7, p< 0.001). An improvement of dyspnoea on Borg's scale was observed from the second month's treatment (5.56 +/- 1.65, p = 0.0201) and persisted throughout follow up to one year (3.81 +/- 1.32, p < 0.0001). Similarly, the 6 minute walking perimeter increased from the first control at 6 months (323 +/- 82 m, p < 0.0001) and at one year (322 +/- 62 m, p <0.0004). Finally, although a significant increase in SaO2 was observed at 6 months (p = 0.0032), this was hardly significant at one year (82 +/- 10 %, p = 0.0512). Transaminase levels did not rise significantly at the follow up visits (p = ns) and the haemoglobin concentration was unchanged. No patient died during the study period. This study showed a clear functional improvement in patients with Eisenmenger's syndrome treated with bosentan. The drug was well tolerated clinically with few adverse effects and a good safety margin of usage.

[Pulmonary atresia with an intact interventricular septum]. / Atrésie pulmonaire à septum interventriculaire intact. Expérience d'un centre à propos de 35 patients.

We report this centre's experience of the treatment and follow up of pulmonary atresia with an intact septum. 35 infants were seen during the neonatal period. Opening via catheterisation was attempted in 21 patients with initial success in 10, and 11 failures leading to urgent surgery (surgical opening, n=9) and isolated Blalock-Taussig anastomosis (n=2). Fourteen other patients underwent immediate surgery: surgical opening, n=3, and Blalock alone, n=11. Four patients died in the neonatal period: 1 after successful opening via catheterisation, 3 others after Blalock anastomosis. Five others with a Blalock anastomosis died suddenly later. By the end of follow up, 16 patients had undergone biventricular type repair, of which 7 required additional procedures. 10 others had undergone cavo-pulmonary type repair, including 4 infants in whom the initial strategy of biventricular repair had failed. The only predictive factors at birth for subsequent progression to biventricular type repair were: larger tricuspid diameter at echography (10.9 +/- 2.25 mm versus 6.34 +/- 1.74 mm, p = 0.0007) or at angiography (10.07 +/- 2.09 mm versus 8.04 +/- 2.42 mm, p = 0.039), and the right ventricular morphology (p = 0.0011) with more tripartite ventricles, and less bipartite or even unipartite ones in the biventricular group.

"Infantile" form of the scimitar syndrome with pulmonary hypertension.

Twenty-five newborns and infants aged < 1 year with the scimitar syndrome and pulmonary hypertension from 12 European pediatric centers were examined. Cardiac failure and severe respiratory insufficiency were always present. In 23 cases, pulmonary hypertension was due to a large shunt between abnormal arteries originating from the abdominal aorta and supplying the lower part of the right lung (vascular sequestration). In the last 2 cases, pulmonary hypertension was secondary to stenosis of the common trunk of the right pulmonary veins. Three of 10 patients who received only medical treatment survived; 2 are doing well, with pulmonary arterial pressures that have returned to normal, and the other had severe residual pulmonary hypertension. Six of 15 patients who underwent surgery survived. There were 5 ligations of a patent ductus arteriosus with 5 deaths, 3 pulmonary resections with 2 deaths, 1 dilation of a tight stenosis of the common trunk of the right pulmonary veins with 1 death, and 6 ligations of the abnormal arterial vessels with 5 surviving patients who are in good condition. Ligation of the abnormal arterial vessels appears to be the best type of treatment.

The "adult" form of the scimitar syndrome.

One hundred twenty-two cases of the adult form of the scimitar syndrome were collected from different cardiologic centers. The clinical, radiographic and hemodynamic findings are described. The scimitar syndrome is defined as an anomalous right pulmonary venous drainage, partial or complete, to the inferior vena cava. Additional characteristics of this syndrome such as hypoplasia and abnormalities of the vascular supply to the right lung, dextrocardia and abnormalities of the bronchial segmentation are common; bronchiectases are rare. The left to right shunt was less than 50% in 100 of the 122 patients. The pulmonary arterial pressures were normal in 94 patients and slightly elevated in 28. A follow-up study of these patients showed that, without surgical correction, they lead a normal life. An awareness of this syndrome may avoid unnecessary invasive diagnostic procedures and surgical treatment for most patients.

Influence of clinical and hemodynamic characteristics on signal-averaged electrocardiogram in postoperative tetralogy of Fallot.

This study examines the relation between signal-averaged electrocardiographic measurements and the occurrence of spontaneous ventricular arrhythmias in 86 patients with a postoperative right bundle branch block after repair of tetralogy of Fallot; special attention was given to the influence of age, body surface area and right ventricular systolic pressure on signal-averaged electrocardiograms. Twenty-eight of the 86 patients had significant ventricular arrhythmias on 24-hour ambulatory monitoring. A positive linear correlation was found between filtered QRS duration and age at evaluation or body surface area (r = 0.45, p = 0.00001; r = 0.54, p < 0.00001, respectively) and between amplitude of the last 40 ms of the filtered QRS and right ventricular systolic pressure (r = 0.48, p < 0.001). A negative linear correlation was found between amplitude of the last 40 ms of the filtered QRS and age at evaluation or body surface area (r = -0.27, p = 0.01; r = -0.34, p = 0.002, respectively). When the age of the patients or the body surface area was considered with an analysis of covariance, the presence of ventricular arrhythmias was associated with a higher amplitude of the last 40 ms of the filtered QRS. In addition, an amplitude of the last 40 ms of the filtered QRS > 170 microV had an excellent sensitivity (100%) and a good specificity (88%) for identifying patients with both right ventricular systolic hypertension and spontaneous ventricular arrhythmia. Thus, adjustment of signal-averaged parameters for age at evaluation and body size is mandatory when studying postoperative tetralogy of Fallot.

The "horseshoe" lung: six new cases.

Six cases of horseshoe lung have been found in a group of 147 scimitar syndrome cases collected in a cooperative multicenter study. These cases were associated either with the severe infantile or with the benign adult form of the scimitar syndrome. The aim of this paper is to describe the clinical and imaging signs as well as the prognosis of 6 new cases of horseshoe lung in association with scimitar syndrome. The authors conclude (1) that the diagnosis of horseshoe lung may be strongly suspected on standard chest radiographs and confirmed by thoracic CT scan; (2) that the prognosis of scimitar syndrome does not seem to be worse when associated with horseshoe lung.

Use of Ethibloc in the treatment of cystic and venous angiomas in children. 19 cases.

The authors report the use of Ethibloc in the treatment of cystic lymphangiomas and venous angiomas in 19 children. This embolizing product is injected by puncture in situ. Ten cystic lymphangiomas were treated, including 7 of the cervico-facial region. Of these ten, 8 were embolized initially using Ethibloc, and 2 after surgery. The diagnosis was always confirmed by echography and in some cases also by scanner. Six were completely cured and 2 had partial but satisfactory results requiring further injections. The final 2 cases when Ethibloc was administered after surgery, gave very disappointing results as the residual lesion was microcystic and thus difficult to access for the Ethibloc injection. The 9 venous angiomas involved various regions, including 5 facial lesions. These angiomas required a more extensive blood-clotting and radiologic assessment: standard radiography to detect the clot, Doppler echography and M.R.I. precisely clarifying the exact extension in depth. For 6 patients the cure was complete, for 2 others the results were good. Finally, in 1 case Ethibloc embolization was carried out prior to surgery, allowing definitive cure. In all, this technique resulted in a complete cure in 2/3 of the cases and its simplicity makes it a seemingly interesting alternative to surgery.

[Obstructions to pulmonary ejection and restrictive interventricular septal defects]. / Obstacles à l'éjection pulmonaire et communications interventriculaires restrictives.

The association of a restrictive ventricular septal defect with a pulmonary tract obstruction is rare and may cause diagnostic and therapeutic problems. The authors report their experience of such an association in 10 children. 3 had a tetralogy of Fallot (1 death), 4 a double-outlet right ventricle (2 deaths), and 3 a coarctation of the aorta with ventricular septal defect and pulmonary artery banding (no death). Prognosis depends on the type of malformation. It has improved with years because of sooner recognition of these malformations and better surgical technics and post-operative care.

[Surgical treatment of the scimitar syndrome in children, adolescents and adults. A cooperative study of 37 cases]. / Le traitement chirurgical du syndrome du cimeterre chez l'enfant, l'adolescent et l'adulte. Etude coopérative de 37 cas.

The authors report the results of a cooperative study of 37 operated cases of the scimitar syndrome. The diagnostic procedures, the operative indications, the surgical indications, the postoperative complications and the long-term outcome were reviewed. The results were disappointing irrespective of the surgical technique that was used (reimplantation of the right pulmonary veins in the left atrium, lobectomy, pneumonectomy). Only 12 patients had a satisfactory postoperative outcome and good long-term results; 21 patients had long-term chronic respiratory failure with a reduced exercise capacity and 4 patients died after surgery. Of the 21 patients with long-term sequellae, 17 had a thrombosis of the anastomosis between the right pulmonary veins and the left atrium; this occurred immediately after surgery.

[Pulmonary valvulotomy with the Nykanen radiofrequency guide in pulmonary atresia with intact interventricular septum]. / Valvulotomie pulmonaire avec le guide à radiofréquence Nykanen dans l'atrésie pulmonaire à septum interventriculaire intact.

We report 2 cases of pulmonary atresia with intact interventricular septum where perforation of the pulmonary valve was performed using a new Nykanen radiofrequency guide from Bayliss. The valvulotomy was successful in the two neonates, but one required secondary surgery to enlarge the pulmonary infundibulum. Moreover, in one of the two cases, this anomaly was associated with a right aortic arch without systemico-pulmonary vessels, which is a rare combination in this pathology. This technique is effective and safe, and represents a good alternative to open surgery in the forms with tripartite right ventricle and without coronaro-cardiac fistulae.

[Percutaneous closure of patent ductus arteriosus with the Amplatzer duct occluder. Results of 29 patients]. / Fermeture percutanée du canal artériel avec le dispositif Amplatzer duct occluder. Série de 29 patients.

The authors report their experience of percutaneous closure of large patent ductus arteriosus with the Amplatzer duct occluder. The age of the patients ranged from 3 months to 72 years (average 24 months) with body weights of 3.9 to 74 Kg (average 16 Kg). The smallest angiographic diameter of the ductus was 4.0 +/- 1.3 mm at angiography (range 1.8 to 6 mm). The occluder was inserted under local anaesthetic with control aortography in all but one case who required general anaesthesia. In two small babies, the implantation was performed after venous puncture alone with transthoracic echocardiographic monitoring. The implantation was successful in all but one case in which there was a distensible duct. Early angiographic control showed suppression of the shunt in 61% of patients. Secondary haemolysis was observed in one case and was corrected by a second catheterisation 4 days later, with occlusion of the duct with a balloon catheter. The patients were followed up clinically, radiologically and echocardiographically. Doppler echocardiography showed absence of a residual shunt in 24 patients (86%) one month after the procedure, in 25 patients (89%) 3 months after the procedure and in 26 patients (93%) one year after the procedure. Two patients were left with mild or moderate shunts 12 and 16 months after the attempted occlusion. No cases of aortic or pulmonary obstruction were observed. The authors conclude that the Amplatzer duct occluder is a reliable device for closing large patent ductus arteriosus. It may be proposed in young symptomatic children over 4 Kg in body weight. A high rate of occlusion is obtained with a minimal risk of complications.

[Treatment with urokinase of systemic arterial thrombosis in the newborn infant]. / Traitement par l'urokinase des thromboses artérielles systémique du nouveau-né.

The records of 5 neonates with systemic arterial thrombosis (aortic in one case, peripheral in four cases) were reviewed. Fibrinolysis was performed with urokinase administered by infusion (1,000 to 4,000 U/kg/h). This treatment was combined with heparin therapy in 4 cases. Thrombosis was due to various causes: umbilical arterial catheter (1 case), disorders of supraventricular rhythm in utero (1 case), aneurysm of the ductus arteriosus with dysplastic aortic arch vessels (2 cases); one of these patients also had myocardiopathy. No cause could be found in a premature child weighing 1,300 g. The presenting symptoms of systemic arterial thrombosis are ischaemia of the extremities and suppression of peripheral pulses; heart failure with arterial hypertension is frequent. In our series the diagnosis was confirmed by doppler-ultrasonography in one case and by angiography in three cases (angiography in the left ventricule with foramen ovale, or umbilical aortography). Treatment with urokinase lasted 1.5 to 7 days. In 2 children the initial dosage had to be increased as there was no clinical improvement. Four children were completely cured; the fifth child, who had left renal thrombosis, shows slight functional impairment of the left kidney. There were no haemorrhagic complications. The fibrinolytic treatment with urokinase of systemic arterial thrombosis in the newborn is effective and has few drawbacks.

[Aortic valve insufficiency: an unrecognized complication of the surgical repair of ostium primum atrial septal defect]. / L'insuffisance aortique: une complication méconnue de la fermeture chirurgicale de la communication interauriculaire ostium primum.

Between 1974 and 1989, eight children undergoing surgical repair of a partial atrioventricular canal comprising an ostium primum atrial septal defect, mitral regurgitation due to a cleft mitral valve (6 cases) and a small ventricular septal defect (1 case) developed aortic regurgitation. This complication was diagnosed on the finding of a diastolic murmur 1 day to 9 years after the repair of the atrial septal defect. The degree of regurgitation was Grade I in 4 cases but in the other patients it was severe from the outset requiring emergency surgery the next day (1 case), or moderate at the beginning but rapidly progressive (1 case) or slowly progressive (2 cases) leading to aortic valvuloplasty in these 3 patients. The cause of the regurgitation was iatrogenic: perforation of the non coronary cusp secondary to repair of the ostium primum atrial septal defect. None of the patients required aortic valve replacement. After surgical valvuloplasty, all children had Grade 1 aortic regurgitation. Color coded Doppler echocardiography confirmed the diagnosis and helped assess the clinical course of this lesion during follow-up.

[Agenesis of the pulmonary valves. Experience over 20 years]. / L'agénésie des valves pulmonaires. Expérience sur 20 ans.

Absent pulmonary valve is a rare cardiac malformation, usually associated with a tetralogy of Fallot. It features aneurysmal dilatation of the pulmonary arteries which may compress the bronchial tree and lead to respiratory distress. The experience of our group over a 20 year period (20 patients) is reported. The diagnosis was usually made in the neonatal period in the presence of cardiomegaly and a systolic and diastolic murmur at the left sternal border. The diagnosis was made at antenatal echocardiography in 2 cases. Ten patients were not operated and 4 of them died in the neonatal period of respiratory distress and 2 others of extracardiac causes. Three children are on the waiting list for surgical correction and the remaining child is asymptomatic. The other 10 cases were operated with a minimum closure of the ventricular septal defect and widening of the pulmonary tract. The latter procedure was performed with an infundibulo-pulmonary patch (6 cases) and insertion of a pulmonary valve (4 patients) (monocusp patch in 3 cases, pulmonary heterograft in one case). Eight patients underwent a reduction procedure of the pulmonary arteries to relieve branchial compression. Of those who were operated, two died during follow-up: the first of complete atrioventricular block and the second of infectious endocarditis of the stenosed pulmonary heterograft. These results show that reduction procedures of the pulmonary arteries are promising approaches which should improve the future prognosis of young patients with respiratory distress. The need for insertion of a pulmonary valve remains debatable.

[Acquired pulmonary atresia complicating tetralogy of Fallot]. / Atrésies pulmonaires acquises compliquant la tétralogie de Fallot.

Pf 51 cases of Fallot's tetralogy who underwent preoperative angiography before a palliative anastomosis and then a second angiography before complete correction, 10 had developed pulmonary atresia (infundibular in 8 cases, valvular in 2 cases). The type of anastomosis (Blalock-Taussig of Waterston), and the interval between the two operations do not seem to be determinant factors. The initial severity of the stenosis seems to favour the development of atresia but this is not invariable. The authors discuss the aggravating role of the anastomosis and the consequences of this type of complication.

[Hemangioma and superficial arteriovenous malformations]. / Hémangiomes et malformations vasculaires superficielles.

Haemangiomas are different from true superficial vascular malformations. The haemangiomas, mainly affecting the newborn and small babies, will, after a phase of progression, sometimes regress completely. Therapeutic abstention is the rule except in high risk angiomas when steroid therapy may be effective. Visceral involvement poses problems. Superficial vascular malformations, on the other hand, arise at all ages and may affect any blood vessel. Each type has a specific clinical presentation, complementary investigations and appropriate treatment. Some are slowly progressive, for example capillary, venous and lymphatic malformations. Others are haemodynamically active, such as the arteriovenous malformations. Capillary malformations are flat angiomas with aesthetic consequences, apart from the Sturge-Weber-Krabbe syndrome. Cold, blue venous malformations confirmed by ultrasonography and magnetic resonance imaging, when necessary, require treatment adapted to their site and size: compression, embolisation, surgery or abstention. Lymphatic malformations may be cystic or tissular: the cystic lymphangioma, a soft swelling of often healthy skin, with compartments separated by septa on ultrasound scan, is usually treated by ethibloc embolisation. Arteriovenous malformations, warm and pulsatile, demonstrated at arteriography, may progress rapidly and treatment by surgery or embolisation, when necessary, has to be complete. Finally, there are complex vascular malformations which pose very difficult problems of management.

[Incidence and factors favouring ventricular arrhythmia after surgical repair of tetralogy of Fallot]. / Incidence et facteurs favorisant les arythmies ventriculaires après réparation de tétralogie de Fallot.

The prevalence and factors favorising postoperative ventricular arrhythmias in 62 patients undergoing surgical repair of tetralogy of Fallot between 1971 and 1982 were analysed. Nineteen patients (31 %) had significant ventricular arrhythmias (Lown greater than or equal to 2) on Holter monitoring or exercise stress testing. The arrhythmia could only be recorded after stress testing in 5 patients. The patient's age at operation, the duration of follow-up and age at evaluation were significantly greater in the group with ventricular arrhythmias. However, age at operation was closely related to the other two parameters and a discriminating analysis showed that the age at operation was not a favorising factor for the occurrence of ventricular arrhythmias (p = 0.23), in contrast to the duration of follow-up (p = 0.0015) and age at evaluation (p = 0.0007). No relationship was found between ventricular arrhythmias and the following factors: previous anastomosis, outflow patch, necessity of a ventriculotomy or reoperation, presence of residual ventricular septal defect, postoperative systolic right ventricular pressure, severity of intraventricular conduction defects, and effort tolerance. These results suggest that after surgical repair of tetralogy of Fallot, the incidence of ventricular arrhythmias increases with time. The main problem is to identify patients with a high risk of sudden death, i.e. those with high degree ventricular arrhythmias and poor haemodynamic results of repair.

[Congenital subclavian steal syndrome. Apropos of 14 cases. Review of the literature]. / Le vol sous-clavier congénital. A propos de 14 observations. Revue de la littérature.

Fourteen cases of the congenital subclavian steal syndrome are presented with a review of 99 cases in the literature. This abnormality is usually asymptomatic and a fortuitous finding (clinical: asymmetric blood pressure; radiological: investigation of an associated cardiovascular malformation). The possible malformations are numerous and may be diagnosed by angiography: however, they can be suspected from three simple investigations, clinical examination indicating the side of lower blood pressure, chest X-ray and barium swallow. Seventy one per cent congenital subclavian steal syndromes are associated with a right-sided aortic arch, the steal being then almost always left-sided via a left subclavian artery anomaly. In this group, a left subclavian artery isolated from the aorta represents about a half of the cases. A cardiac malformation is present in one out of two cases and a patent ductus arteriosus uniting the left subclavian artery and the left pulmonary artery is observed in one out of four cases. Twenty nine per cent of congenital subclavian steal syndromes are associated with a left-sided aortic arch. In these cases the abnormalities usually involve the aortic arch (57% of cases) or a right or left subclavian artery. Therefore, in this group, there is no preferential side for the subclavian steal which can be right, left or bilateral. These abnormalities usually only require medical surveillance. When they become symptomatic, vertebro-subclavian revascularisation is justified. The presence of a subclavian steal may have surgical implications: it is important to exclude a subclavian steal before performing a Blalock anastomosis.(ABSTRACT TRUNCATED AT 250 WORDS)