[Congenital heart disease: from childhood to adulthood]. / Cardiopathie congénitale: de l'enfance à l'âge adulte.

With the improvement of congenital heart surgery, most children with congenital heart disease will survive into adulthood with a good quality of life. Regular cardiac follow-up is recommended for all patients. The adolescent period coincides often with medium and long term consequences and complications and repeat surgery or catheter interventions might be needed. It is therefore of prime importance to begin the transition process early and to pursue it well into adulthood. We have elaborated a formal transition program adapted to youngsters with congenital heart disease.

Novel approach to mitral valve repair in childhood dilated cardiomyopathy.

Evidence is mounting that mitral valve repair can improve symptoms in adults with dilated cardiomyopathy. Data is currently lacking for children with dilated cardiomyopathy and options for annuloplasty are limited in children. We report on the successful management of a 21 month-old child in heart failure from dilated cardiomyopathy and severe mitral regurgitation. The echocardiogram showed severely dilated left heart cavities, severe mitral regurgitation from a dilated annulus (23 mm, Z-score 1.74) with discrete anterior leaflet tethering, and moderate systolic dysfunction. The mitral valve was repaired using a 16 mm Bioring Kalangos biodegradable annuloplasty ring. The patient was extubated on the third postoperative hour and discharged on the fifth postoperative day with trivial mitral regurgitation and a 5 mmHg mean transvalvular gradient. At 12 months, the patient is in NYHA class I and presents trivial central mitral regurgitation without any transmitral gradient. This represents the first report in successfully managing a child with dilated cardiomyopathy with mitral regurgitation using a novel biodegradable annuloplasty ring, which has the potential to durably remodel the mitral annulus and grow with the patient.

Vascular ultrasound studies for the non-invasive assessment of vascular flow and patency in experimental surgery in the pig.

Vascular ultrasound is a reliable non-invasive tool used for the routine assessment of vascular flow and patency in human recipients. We describe the use at three different time points (immediately, 1 week and 4 weeks postsurgery) of ultrasound studies and its validation by angiographic studies in 37 swine undergoing carotid graft replacement. We calculated predictive values (>92%), sensitivity (>85%) and specificity (>92%) with high results at all time points. Ultrasound appeared as an accessible non-invasive technique, providing rapid, safe, repeatable and reliable results. It is an excellent alternative to angiography, avoiding risks inherent to invasive methods and therefore contributing to animal welfare.

[Does hypertension need treatment following correction of coarctation in childhood?]. / Faut-il traiter l'hypertension artérielle après cure de coarctation dans l'enfance?

Does hypertension need treatment following correction of coarctation in childhood? The results of the surgical repair of aortic coarctation (CoA) are excellent. Prenatal diagnosis of this defect is made in 40% of the cases and this allowed a reduction of preoperative mortality. Beside these successes, patients who underwent a CoA repair in infancy remain at high risk for resting hypertension (HT) later in life. Indeed, half of the adolescents are hypertensive and 2/3 of the patients around 30 years. This HT is responsible for an increased mortality mostly related to cardiovascular events. Screening for HT and its risk factors is the main objective of the follow-up. Some residual anomalies such as recoarctation or hypoplasia of the horizontal aorta may be treated either by surgery or by interventional catheterisation. Recently, new contributors to hypertension have been identified such as abnormal geometry of the aortic arch or alteration of the mechanical properties of the arterial wall. In a given patient, the co-existence of vascular dysfunction and abnormal geometry of the aortic arch confers a high risk for HT. The indications to treat exercise HT or the type of antihypertensive drug to be given remain unsolved questions. Prevention may rely on substantial modifications of the surgical techniques to optimise the aortic arch geometry. The indications to correct abnormal geometries at high risk without recoarctation are not yet defined. The long-term benefit of either preventive or curative strategies might be difficult to evidence and will probably need the analysis of intermediate markers such as vascular function and left ventricular hypertrophy.

[New approach to perimembranous ventricular septal defect by real-time 3D echocardiography]. / Nouvelle approche des communications interventriculaires périmembraneuses par échocardiographie 3D temps réel.

Perimembranous ventricular septal defects (VSD) have a close anatomical relationship to the aortic and tricuspid valve which may make percutaneous closure difficult. The authors' objective was to study the anatomy of perimembranous VSD by real-time 3D echocardiography. Fourteen patients aged 1 day to 3 years (mean 3 months) underwent transthoracic 2D and 3D echocardiography. Two patients (> 10 kg) underwent closure of the perimembranous VSD with an Amplatzer occluder. The other 12 patients were operated (surgical closure in 11 and circling suture with resorbable thread in 1 patient). The 3D echocardiography showed variations in the geometry of the perimembranous VSD (eccentric, oval, cleft). The maximal diameter of the septal defect by 3D echocardiography was on average 2 mm greater than the diameter by 2D echocardiography. The 3D echo enabled visualisation of the close relationship of the septal tricuspid leaflet, the aortic valve and the perimembranous VSD, showing the mechanism of its closure and the associated aortic regurgitant lesions. The profile of the prosthesis implanted percutaneously was well shown in the different 3D views. The authors conclude that real-time 3D echocardiography is a technique which allows accurate anatomic description of perimembranous VSD. As a complement to classical 2D methods, it gives a better appreciation of the maximal diameter of the septal defect and of its relationship to the aortic and tricuspid valves.

[Percutaneous closure of three septal defects by two Amplatz occluders implanted during the same procedure]. / Fermeture percutanée de 3 communications interauriculaires par 2 dispositifs d'Amplatz implantés au cours d'un même cathétérisme.

Multiple atrial septal defects can be closed by interventional catheterisation. The procedure requires an accurate morphological evaluation: number of defects, distance from their edges to the main cardiac structures, resistance of the septum. The authors report the case of a 63 year old woman presenting with cardiac failure in whom 3 atrial septal defects were diagnosed. All 3 defects were successfully closed by the implantation of two Amplatz devices. Control echocardiography at 6 months showed the occluders in a normal position with no residual shunt and the patient was asymptomatic.

Transcatheter implantation of a bovine valve in pulmonary position: a lamb study.

BACKGROUND: Pulmonary regurgitation can lead to severe right ventricular dysfunction, which is a delicate postoperative problem in the long-term follow-up of patients who had surgery for congenital heart diseases. Clinical conditions of patients suffering from pulmonary valve incompetence are improved by valve replacement with a prosthetic valve. To date, the surgical approach is the only option to replace a pulmonary valve. We report the first experience of percutaneous pulmonary valve implantation. METHODS AND RESULTS: A fresh bovine jugular vein containing a native valve was sutured into a vascular stent and then cross-linked with a 0.6% glutaraldehyde solution for 36 hours. After being hand-crimped onto a balloon catheter, the device was inserted percutaneously according to standard stent-placing techniques. The valved stent was finally deployed in the position of the native pulmonary valve of the lamb. Hemodynamic evaluation was carried out before and 2 months after implantation. Anatomic evaluation was finally performed. Percutaneous pulmonary valve replacement was successful in 5 lambs. No complications were noted. Early and late angiographic and hemodynamic studies confirmed a good position of the stents with a competent valve at the end of the protocol. One stent was slightly stenotic, with macroscopically visible calcifications. CONCLUSIONS: Nonsurgical implantation of pulmonary valves is possible in the lamb. This new technique is similar to standard stent implantation. Thus, it should be feasible in humans, in whom it will lead to a significant reduction of reoperations in patients in need of pulmonary valve replacement.

Coronary artery obstruction after the arterial switch operation for transposition of the great arteries in newborns.

OBJECTIVES: We sought to describe a large series of coronary artery obstructions after the arterial switch operation for transposition of the great arteries and to discuss their clinical implications. BACKGROUND: Aortic root angiography and myocardial perfusion imaging yield ambiguous results regarding the fate of the coronary artery anastomoses after the arterial switch operation. Late death related to coronary artery obstruction and growth of the translocated coronary arteries are of major concern in these patients. METHODS: Selective coronary artery angiography was performed prospectively in a total of 165 children. RESULTS: A total of 12 coronary occlusions, 8 major stenoses, 6 minor stenoses of the left ostium and 4 stretchings of one coronary artery were identified. Obstructions were more frequent in types D and E (p < 0.001) of the Yacoub and Radley-Smith classification. Coronary obstruction was documented in all patients with electrocardiographic and ultrasound evidence of myocardial ischemia at time of study. Early postoperative ischemia did not predict coronary artery lesion if the patient had fully recovered. Persistent or delayed myocardial ischemia was highly predictive of coronary artery lesions. The incidence of coronary artery obstruction was very high (11 of 35) in patients operated on by a rapidly abandoned technique of single-orifice reimplantation of both coronary artery ostia. CONCLUSIONS: Selective coronary angiography is the most accurate means to assess coronary artery obstruction after the arterial switch operation. Precise diagnosis of coronary artery lesions after this operation will help to elucidate the pathogenesis, develop adequate therapeutic strategies and might indicate how to prevent coronary complications after operation.

Arterial mechanical changes in children with familial hypercholesterolemia.

Atherosclerosis is preceded by a phase of changes in the arterial wall that could have functional consequences even before the appearance of atheromatous changes. We hypothesized that early alterations of the mechanical properties of the arterial wall could precede clinical and echographic modifications. We used an automatic, computerized, ultrasonic procedure to evaluate geometric and mechanical characteristics of the common carotid artery (CCA) in normotensive children with primary familial class IIA hypercholesterolemia (FH; n=30; mean+/-SD age, 11+/-2 years old; mean+/-SD systolic/diastolic blood pressure, 109+/-9/55+/-7 mm Hg). These subjects were compared with age-matched, nonobese control subjects (n=27; 11+/-3 years old; 112+/-10/55+/-7 mm Hg). Noninvasive ultrasonic measurements were performed by the same investigator to measure the CCA luminal systolic and diastolic diameters and intima-media thickness (IMT). The cross-sectional compliance, cross-sectional distensibility, and the incremental elastic modulus of the CCA wall were then calculated. Finally, we assessed the degree of reactive hyperemia in the brachial artery produced after distal cuff occlusion and release. The changes in brachial arterial diameter in response to reactive hyperemia (endothelium-dependent dilation) and to glyceryltrinitrate (endothelium-independent dilation) were then measured. In patients with FH, we observed a significant reduction of systodiastolic variations in diameter (by 20%, P:<0.001) without a significant difference in IMT. Cross-sectional compliance and cross-sectional distensibility were significantly reduced in FH subjects (by 15%, P:<0.05 and 19%, P:<0.01, respectively). In parallel, the incremental elastic modulus was significantly increased (by 27%, P:<0.01) in children with FH. No correlation was evident between the carotid incremental modulus and either IMT or plasma low density lipoprotein cholesterol level. There was no difference in diameter of the brachial artery at rest in control and FH subjects (3.0+/-0.5 versus 3.0+/-0.4 mm). The reactive hyperemia and glyceryltrinitrate dilation were also similar in the 2 groups. However, the flow-mediated dilation of the brachial artery was smaller in the FH subjects (4.2+/-2.9%) than in controls (9.0+/-3.1%, P:<0.001). In FH, endothelium-dependent dilation was negatively correlated with the plasma low density lipoprotein cholesterol level (P:<0.04). These results indicate that increased stiffness of the CCA wall in children with FH is independent of blood pressure and could be related to endothelial dysfunction. Thus, alterations in CCA wall mechanics could be early and easily measurable markers of atheromatous changes in the arterial wall.

Skin elastic fibers in Williams syndrome.

The elastin gene is consistently deleted in Williams syndrome and as this protein represents the major component of the elastic fibers of the dermis, we sought to investigate skin elastic fibers in Williams syndrome as a key to unraveling extracellular matrix disorganization in this condition. Both morphometric parameters analyzed by using automated image analysis and immunofluorescence labeling with monoclonal antibodies against elastin and fibrillin 1 showed a disorganized pre-elastic (oxytalan and elaunin) and mature elastic fibers in the dermis of 10 Williams syndrome patients compared with five healthy children and one patient with isolated supravalvular aortic stenosis. Skin biopsies in Williams syndrome patients provide a simple mean to elucidate extracellular matrix anomalies. Hopefully, this method could give clues to the understanding of the elastic network anomalies in this condition and even to the consequences of these latter on elasticity and resilience of other tissues such as the arterial tree.

The multi-track angiography catheter: a new tool for complex catheterisation in congenital heart disease.

OBJECTIVE: To develop a simple and versatile catheter system for complex cardiac catheterisation because angiography and pressure measurements during diagnostic and interventional cardiac catheterisation are often unsatisfactory. METHODS: The Multi-Track Angio catheter system is a single lumen side-hole catheter with a short distal extension containing a lumen for a standard guidewire. The catheter is introduced over a previously placed guidewire running through this distal extension. It can then be manipulated within the heart by sliding along the guidewire. The tip of the catheter is always stabilised by the guidewire. This stability enhances angiography and pressure recordings. RESULTS: The Multi-Track Angio catheter system was used in 84 patients (age 1 day-20 years). Thirty one procedures were diagnostic and 53 interventional. The decision to use the Multi-Track Angio catheter was based on three criteria: firstly, unsatisfactory angiography obtained with conventional equipment; secondly, difficult catheter course requiring use of a guidewire; and thirdly, requirement for angiography and pressure recordings during interventional procedures. No complications were encountered. High quality angiography could be performed in all cases without catheter recoil. CONCLUSIONS: The Multi-Track Angio catheter system allows for high quality angiography and pressure recordings during diagnostic and interventional cardiac catheterisation. The advantage of the system is that both angiography and pressure recordings can be performed repeatedly from stable catheter positions using a previously placed guidewire. This reduces the need for guidewire manipulations or catheter exchanges and decreases procedure time and the risk of complications.

Pulmonary balloon valvuloplasty in the palliation of complex cyanotic congenital heart disease.

OBJECTIVE: To assess the value of pulmonary balloon valvuloplasty in the interim palliation of complex congenital heart disease and pulmonary stenosis in children, who often require numerous palliative operations before definitive surgical repair. METHODS: Evaluation of pulmonary balloon valvuloplasty procedures performed over a five year period in 18 patients (age 8 days--29 years; mean 5.5 years) with complex cyanotic congenital heart disease. RESULTS: After pulmonary balloon valvuloplasty oxygen saturation increased from a mean (SD) of 69 (7.5)% to 83 (7.0)% (P < 0.001). Mean pulmonary artery pressure increased from a mean (SD) of 11.3 (3.8) mm Hg to 15.7 (3.9) mm Hg (P < 0.001). Transient complete atrioventricular block occurred in one patient. No other complications were encountered. In 5 patients (28%) there was an inadequate improvement in cyanosis compared with pre-procedure values (72 (4.7)% v 66 (8.1)%). Reasons for failure were increasing infundibular stenosis in three and inadequate mixing in one child. In 13 patients (72%) pulmonary balloon valvuloplasty gave adequate interim palliation over a mean follow up of 1.1 (1.3) years. Oxygen saturation was 81 (5.6)% at last follow up compared with 70 (7.3)% before pulmonary balloon valvuloplasty (P < 0.001). CONCLUSION: Pulmonary balloon valvuloplasty is a safe and effective technique in the palliation of patients with complex cyanotic congenital heart disease associated with pulmonary valve stenosis.

Long-term fate of the coronary arteries after the arterial switch operation in newborns with transposition of the great arteries.

OBJECTIVE: Concern continues to be expressed about the long-term impact of coronary artery translocation after the arterial switch operation for transposition of the great arteries. This study was conducted to determine the prevalence of obstructions of the translocated coronary arteries by the use of selective coronary artery angiography. METHODS AND RESULTS: 64 children (mean age 7.6 (SD) 1.5 years) who had survived an arterial switch operation underwent evaluation. They had been operated on by one surgeon and they were followed up by a single hospital. Selective coronary artery angiography was possible in 58 patients. Five patients showed occlusion or stenosis of a coronary artery: one occlusion and two stenoses of the left coronary trunk, two occlusions of the circumflex artery. The prevalence of late coronary artery complications was 7.8 (SD) 6.6% (95% CI 1.2 to -14.4%). The three patients with occlusion of one coronary artery had perioperative ischaemic complications, with associated electrocardiogram evidence of ischaemia and left ventricular dysfunction with mitral valve insufficiency. Both patients with stenosis of the left main coronary artery trunk did not have any evidence of an anomaly before catheterisation. CONCLUSIONS: The prevalence of the late coronary artery complications after an arterial switch operation was low in this series. This accords with the view that the arterial switch operation remains the preferred treatment for such patients. Screening for late coronary artery patency should be done by using selective coronary artery angiography, because even patients who remain symptom free can have coronary artery anomalies.

Maintaining tricuspid valve competence in double discordance: a challenge for the paediatric cardiologist.

OBJECTIVES: To establish the prevalence of tricuspid valve abnormalities in children with a double discordant heart (or congenitally corrected transposition of the great arteries); to study the influence of the loading conditions induced by various surgical interventions on the right and left ventricle in patients with double discordance and an abnormal tricuspid valve; and to propose a rational surgical approach. METHODS: Case notes were reviewed of 141 consecutive patients admitted in the first year of life with various types of double discordance (intact ventricular septum (group 1), ventricular septal defect (group 2), ventricular septal defect and pulmonary obstruction (group 3)). A study group of 62 patients with an abnormal tricuspid valve was selected by cross sectional echocardiography. These were followed up through palliative and open heart procedures with grading of tricuspid regurgitation. RESULTS: Tricuspid valve abnormalities were more common in groups 1 and 2 (60% and 56%) than in group 3 (31%). Preoperative tricuspid regurgitation was more common in group 2 (90%) than in groups 1 and 3 (38% and 36%). Ten patients in groups 1 and 2 died in the neonatal period with severe tricuspid regurgitation, associated with coarctation of the aorta in 60%. Eight patients in group 1 had no surgery and are doing well, with a competent tricuspid valve. Palliative procedures were undertaken in 28 patients: 14 had pulmonary artery banding, which resulted in a decrease in tricuspid regurgitation, 12 in group 2 by reducing the pulmonary blood flow and two in group 1 by changing the septal geometry; 14 in group 3 had an aortopulmonary shunt, which induced tricuspid regurgitation in two. Twenty patients are still alive after palliation, with stable tricuspid valve function. Repair of the tricuspid valve was unsuccessful in the three patients who underwent conventional surgery, leaving the right ventricle facing the systemic circulation. In two patients with a competent but abnormal tricuspid valve, conventional surgery induced severe tricuspid regurgitation. Of the 15 patients who underwent conventional surgery, only 10 survived (mortality 33%): eight with a tricuspid valve prosthesis and two with severe residual tricuspid regurgitation. However, tricuspid regurgitation decreased after anatomical correction (nine patients), restoring a systemic left ventricle and a subpulmonary right ventricle, even when the tricuspid valve was not repaired (five patients). Eight patients are doing well after anatomical correction (mortality 11%). CONCLUSIONS: Tricuspid valve function in double discordance with an abnormal tricuspid valve depends on the loading conditions of both ventricles and on the septal geometry. Interventions that increase right ventricular volume or decrease left ventricular pressure are likely to induce tricuspid regurgitation, while those that decrease right ventricular volume or increase left ventricular pressure are likely to improve tricuspid valve function. Repair of the tricuspid valve always failed when the right ventricle was left in a systemic position and always succeeded when the right ventricle was placed in a subpulmonary position. These results should be taken in to account when dealing with patients with double discordance and an abnormal tricuspid valve.

[Arterial dysfunction after treatment of coarctation of the aorta]. / Dysfonction artérielle après traitement de la coarctation de l'aorte.

The long term follow-up after successful coarctation repair has a late cardiovascular morbidity, includes systemic hypertension at rest or/and after exercise. The pathophysiology mechanisms responsible have not been well determined. We studied 70 normotensive subjects at rest (age, 14 +/- 5 y; pressure, 116 +/- 13/56 +/- 9 mmHg), who had an isolated coarctation with a good repair defined by the absence of gradient between upper and lower right limb (0.-26 mmHg). After exercise testing we defined two groups: Coa HT: Hypertension at exercise equal or over 200 mmHg, n = 20 (228 +/- 23 mmHg) and Coa HT: Normotensive at exercise = 10 (163 +/- 24 mmHg). These subjects were mached sex-age and blood pressure to 70 controls (age, 13 +/- 3 y; pressure, 115 +/- 10/56 +/- 6 mmHg). Using a high-resolution echographic technique, we assessed the systolic, diastolic diameter and the intima media thickness (IMT) of the common carotid artery (CCA) to define mechanical indexes: Cross sectional compliance (CSC), distensibility (CSD) and incremental elastic modulus (Einc) in each group. CCA pressure waveform and the local pulse pressure were determined in 32 subjects to define augmentation index (AI). The changes of the brachial artery diameter in response to reactive hyperaemia (flow mediated dilation: FDM) and to glyceryltrinitrate (GTNMD) were measured. The IMT was significantly increased in the Coa group (by 8%, p < 0.001) and higher in the Coa HT group compared with the Coa NT group (0.57 +/- 0.04 mm vs 0.54 +/- 0.05 mm, p < 0.05). The CSD was lower and the Einc was higher in both groups. The carotid pulse pressure amplitude was significantly higher in the Coa HT Group (41 +/- 14 vs 33 +/- 7 mmHg; p < 0.05). The AI was higher in both Coa repair groups. Both flow-mediated dilation (FMD) and GTN-mediated dilation (GTNMD) of the brachial artery were lower in the Coa group (respectively 5 +/- 3 vs 7 +/- 3%; p < 0.01; 16 +/- 8 vs 23 +/- 9%; p < 0.01). GTNMD was inversely correlated with maximum systolic blood pressure on exercise (r = 0.31, p = 0.03). The IMT of the CCA was related to the local pulse pressure in both groups of coarctation repair. The combination of distensibility decrease in the proximal arterial bed with an impairment of distal artery reactivity would account for the elevation of exercise blood pressure in subjects who had coarctation repair. The increase of local pulse pressure influences the carotid wall hypertrophy.

[Outcome of Wolff-Parkinson-White syndrome in children. Transesophageal study of anterograde permeability of the accessory pathway and of atrial vulnerability]. / Evolution du syndrome de Wolff-Parkinson-White chez l'enfant. Etude transoesophagienne de la perméabilité antérograde de la voie accessoire et de la vulnérabilité atriale.

Twenty-nine children with the Wolff-Parkinson-White syndrome (WPW) were evaluated by transoesophageal electrophysiological studies to determine the quality of anterograde-conduction in the accessory pathway and the atrial vulnerability. The study group included 15 neonates, 1 to 30 days old, and 14 children from 5 to 15 years of age; Anterograde conduction through the bundle of Kent was tested by incremental transoesophageal atrial pacing and by the determination of the shortest conducted cycle with preexcited RR waves; bursts of atrial pacing were then used to try to trigger an atrial arrhythmia. In the group of the 15 neonates, 11 had accessory pathways capable of conduction to the ventricules at frequencies > 300/min (stimulation cycle < or = 2.00 ms) but no atrial arrhythmias could be induced. The older children had slower conduction in the accessory pathways with the shortest conducted cycle length > 200 ms in 11/14 cases; on the other hand, atrial fibrillation was easily induced in 4 children, all over 12 years of age. The risk of syncope by rapid conduction of an atrial arrhythmia through the accessory pathway is negligeable in young children, including those on digoxin. This study suggests that this low risk is explained more by the absence of atrial vulnerability than by the electrophysiological properties of the accessory pathways.

[Cardiovascular impact of end-stage renal insufficiency in children undergoing hemodialysis]. / Retentissement cardiovasculaire de l'insuffisance rénale terminale chez l'enfant hémodialysé.

Cardiac hypertrophy and arterial dysfunction have been described in end-stage renal disease (ESDR) in adults. The incremental elastic modulus (Einc), is a marker of vascular wall material stiffness and an independant predictor of cardiovascular mortality in adults with ESRD on hemodialysis. The relationship between arterial changes and the heart is unknown in the children with ESRD in the same conditions. Using a high-resolution vascular ultrasound and a computerized system of measurement (Iotec), we assessed noninvasively 10 ESRD patients (mean +/- SD, age, 11.5 +/- 4 years; blood pressure [BP], 120 +/- 10/63 +/- 4 mmHg) and 10 age-, sex-, and BP-matched controls (mean +/- SD, age, 11 +/- 4 years; BP, 114 +/- 8/58 +/- 8 mmHg). The systolic and diastolic diameter of the common carotid artery (CCA), the thickness of the wall (intima-media thickness, IMT), the cross sectional compliance (CSC), the cross sectional distensibility (CSD) and the (Einc) were determined. CSC and CSD were evaluated at the same level of pressure. The CCA pressure waveform was obtained by applanation tonometry to assess the reflected wave by the augmentation index (AI). Further the left ventricular mass index was assessed. The flow mediated dilation (FMD) (endothelium-dependent function) and the vasodilation induced by glyceryl-trinitrate (GTNMD) (GTN, an endothelium-independent dilator) were evaluated at the brachial artery site. Compared to control subjects, ESRD patients have mechanical artery dysfunction with lower CSC and CSD (0.11 +/- 0.04 vs 0.18 +/- 0.05 mm2.mmHg-1; p < 0.01; 0.43 +/- 0.10 vs 0.82 +/- 0.20 mmHg-1.10(-2); p < 0.001) and higher Einc (2.60 +/- 1.00 vs 1.40 +/- 0.30 mmHg.10(3); p < 0.001). Furthermore an earlier return of the reflected pulse wave (AI -0.24 +/- 0.08 vs -0.58 +/- 0.06; p < 0.005) is correlated to LV mass index (r = 0.55, P < 0.01) that is significantly increased (134 +/- 63 vs 69 +/- 25 g/m2; p < 0.005). These patients have an impaired FMD (4 +/- 2 vs 7 +/- 1%; p = 0.02) with a normal GTNMD. This study shows that early arterial dysfunction can occur in children with ESRD.

[Palliative treatment of tetralogy of Fallot by percutaneous dilatation of the right ventricular outflow tract. 40 cases]. / Traitement palliatif de la tétralogie de Fallot par dilatation infundibulo-pulmonaire percutanée. Etude de 40 cas.

This study was undertaken to assess the value of percutaneous dilatation of the right ventricular outflow tract as a substitute for surgical systemic pulmonary anastomosis in varieties of tetralogy of Fallot with severe irregularity or major pulmonary arterial hypoplasia unsuitable for complete repair of first intention. Fifteen neonates aged 3 to 23 days with severe desaturation (SaO2 = 73 +/- 11%) and twenty five children aged 1.2 to 174 months with anoxic crises or severe desaturation (SaO2 = 67 +/- 15%) fulfilled these criteria. They underwent right heart catheterisation completed by an attempted pulmonary dilatation. This manoeuvre failed in 6 cases, all neonates, because it was not possible to cross the annulus or maintain the balloon in position, or because of infundibular perforation. There were complications in 5 cases, all neonates: 2 tamponades with 1 death, one dissecting aneurysms of the annulus, 1 gastroenteritis and 1 caval thrombosis. Of the 34 patients dilated, 8 were poor results, mainly in the older age group (7 cases) and 26 were successful as judged by a significant improvement in arterial saturation. However, 7 patients rapidly deteriorated due to anoxic crises (6 infants and children) or fatal secondary tamponade (1 neonate). Therefore, there remained 19 stable successful procedures, 49% of attempts, representing 56% of successful attempts and 73% of primary successes. In neonates, these proportions were respectively: 47%, 78% and 87%, and in the older children: 48%, 48% and 63%.(ABSTRACT TRUNCATED AT 250 WORDS)

[Cardiac rhabdomyomatosis and Bourneville's tuberous sclerosis in the fetus. Apropos of 2 cases]. / Rhabdomyomatose cardiaque et sclérose tubéreuse de Bourneville chez le foetus. A propos de 2 cas.

Two cases of cardiac rhabdomyoma discovered fortuitously at foetal ultrasonography gave rise to no obstructive cardiac signs or arrhythmias either in the antenatal or postnatal periods. These multiple tumours often observed in Bourneville's tuberous sclerosis orientated the clinical investigations to the diagnosis of this disease from the outset. MRI demonstrated cortical tubers and subependymal nodules in both cases. A retinal hamartoma was present in one case. No renal involvement could be detected by ultrasonic examination. No neurological or cardiovascular symptoms appeared during follow-up (20 and 4 months after birth). Investigations in the parents were negative, these two cases being sporadic forms of Bourneville's tuberous sclerosis. The functional prognosis is related to the neurological outcome. Doppler echocardiography would appear to be the best method of following up cardiac rhabdomyomas, and enabled the demonstration of partial regression of the largest tumour in one of these two cases. Cardiac MRI is also an excellent diagnostic tool. As it is usually performed at the same time as cerebral MRI, essential in the follow-up of Bourneville's tuberous sclerosis, it does not represent additional discomfort to the patient.

[Pulmonary atresia with ventricular septal defect: therapeutic strategy in newborn infants]. / Atrésies pulmonaires à septum interventriculaire ouvert: stratégie thérapeutique chez le nouveau-né.

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)