[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.

Successful use of a new Amplatzer Vascular plug for percutaneous closure of a large aortopulmonary collateral artery in a pulmonary atresia with ventricular septal defect prior to complete repair.

We report the case of a 14 year-old girl with a pulmonary atresia with VSD and multiple aortopulmonary collaterals who underwent a successful complementary occlusion of a large collateral vessel using an Amplatzer vascular plug after a previously failed attempt of occlusion with a coil. The percutaneous procedure, performed from the femoral artery before the complete surgical repair, provided an immediate closure of the vessel. This new device is safe and effective for the occlusion of aortopulmonary collaterals, specifically if of large dimensions.

[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.

[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.

[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.

[Multicentre study of percutaneous closure of interventricular muscular defects with the aid of an Amplatzer duct occluder prosthesis]. / Etude multicentrique de la fermeture percutanée des communications interventriculaires musculaires à l'aide d'une prothèse Amplatzer duct occluder.

Percutaneous closure of interventricular defects is an alternative or adjunct to surgery with the disadvantage of a sometimes prolonged and difficult catheterisation only during which the choice of prosthesis can be determined. Despite the existence of an occlusion prosthesis, the Amplatzer Septal Defect Occluder, specifically conceived for this purpose, an arterial canal occlusion prosthesis such as the Amplatzer Duct Occluder is sometimes better suited for the occlusion of certain interventricular defects. Since 1999, 11 Amplatzer Duct Occluders were used for the closure of interventricular muscular defects, during 10 catheterisations in 9 patients with a median age of 2.5 (0.1 to 43.9) years. In 5 cases there were residual septal defects after failure of initial surgery. 10 were successful with prostheses of 6/4 mm to 12/10 mm while in one patient the interventricular communication had to be closed surgically because the 14/12 mm prosthesis could not be positioned due to a tortuous introduction route. The only complication was a pericardial effusion requiring surgical drainage in one infant. After a median follow up of 2.1 (0.3 to 4.2) years, all of the patients had a satisfactory functional status, with minimal residual shunt on echocardiography in 2 cases. The Amplatzer Duct Occluder therefore seems better adapted for the closure of post-operative residual interventricular communications, with a thickened interventricular septum or even when the tricuspid valve is very close to the right ventricular side of the orifice.

[Experience of implantation of prefitted Palmaz Corinthian stents]. / Expérience de l'implantation de stents Palmaz Corinthian prémontés chez l'enfant.

We report our recent experience with the Palmaz Corinthian stent in children. Since 1/10/01 to 1/11/02 we implanted 18 Palmaz Corinthian stents in 12 children. Median age and weight of patients were 5.6 years (range 0.25-13) and 17.9 kg (range 3-36), respectively. Primary diagnosis were: pulmonary atresia with ventricular septal defect (n = 7), complex transposition of the great arteries (n = 2) and others (n = 3). Indications to stent implantation were: pulmonary branches stenosis (n = 9), stenosis of the ductus arteriosus (n = 1), stenosis of systemic veins (n = 2). All stents were pre-mounted and co-axially advanced over a standard guidewire across the stenotic site, using a 6F short introducer. Stent deployment was performed by hand inflation of the balloon. Final angiography confirmed correct positioning and ruled out complications. Median fluoroscopy time was 18 minutes (range 12-56). All stents reached their nominal diameter; 2 were post-dilated to 125 and 150%. No procedural complications occurred. Despite the impossibility of distal injection, stent positioning was always correct. In conclusion, due to the availability of pre-mounted stents, and the need of short 6F sheaths, the use of the Palmaz Corinthian stent in children is relatively simple. Primary results are generally good in terms of adequate delivery and absence of complications. However, the impossibility of distal injection makes more difficult the choice of the deployment site. Due to the limited possibility of over-dilation, the use of this stent has to be limited to the stenosis of peripheral pulmonary branches or to lesions that can be surgically treated during further surgical procedures.

[Early complications of stenting in children and young adults with congenital heart disease]. / Complications précoces de l'implantation de stents chez l'enfant et le jeune adulte ayant une cardiopathie congénitale.

We report the early complications of stent implantation. Since 1/1/97 to 1/12/02, we implanted 91 stents in 76 patients. Median age and weight were 15 years (range 0.3-43) and 49 kg (range 3-96). Primary diagnosis were: pulmonary atresia with ventricular septal defect (n = 17), tetralogy of Fallot (n = 13), aortic coarctation (n = 15), transposition of the great arteries (n = 3), truncus arteriosus (n = 6), univentricular heart (n = 7), other anomalies (n = 28). Indications to stent implantation were: stenosis of the pulmonary outflow and/or pulmonary branches (n = 44), aortic recoarctation (n = 14), native aortic coarctation (n = 1), systemic venous channels obstruction (n = 14), other (n = 3). The stents were: Palmaz (n = 57), non premounted CP (n = 8), premounted CP (n = 3), Palmaz Corinthian (n = 19), self-expanding (n = 4). Procedural complications occurred in 24 patients (31%) and included: 11 balloon bursting, 8 stent slipping, 1 stent migration, 4 wall dissections. Five patients (21%) needed urgent surgery. Sixty three percent of complications occurred during pulmonary outflow or pulmonary branches stenting, 25% during stenting of the aortic arch, 12% during other procedures. All but 2 complications were observed with non premounted stents. In conclusion, the use of not premounted stents, although widely diffuse, has a not negligible incidence of complications, leading to longer procedures, to the use of a large variety of materials and needing more than occasionally a surgical solution. A larger use of premounted stents, when possible, is recommended.

[Percutaneous closure of an interatrial communication via the internal jugular route using an Amplatzer prosthesis]. / Fermeture percutanée d'une communication interauriculaire par voie jugulaire interne par une prothèse d'Amplatzer.

Closure of interatrial communications by interventional catheterisation is recommended and effective. The usual approach is via the femoral vein. However, difficulty in positioning the probe and the guide in a stable fashion across the septal defect occurred during closure of the interatrial communication by the percutaneous route in a 71 year old female with severe scoliosis. This deformity most certainly contributed to the failure of the femoral approach. The decision for closure by the internal jugular route was taken and allowed closure of the interatrial communication to be achieved. The internal jugular route can be an alternative for closure of interatrial communications with difficult access.

[Arterial rigidity and endothelial dysfunction in obese children]. / Rigidité artérielle et dysfonction endothéliale chez l'enfant obèse.

Obesity is a cardiovascular risk factor in adults. Poorly is known about effect of obesity on cardiovascular system in children. Mechanical properties of a great elastic trunk, the common carotid artery (CCA) and endothelium function of the brachial artery were studied in 130 obese children (age: 12 +/- 3 years, body mass index (BMI): 29 +/- 5.5 kg/m2, without hypertension (115 +/- 19/58 +/- 8 mmHg). These patients had a vascular high resolution echographical analysis. Cross sectional compliance (CSC), cross sectional distensibility (CSD) and incremental elastic modulus (Einc) were analysed at the CCA site. The brachial artery dilation was measured after hyperthemia (flow mediated dilation, FMD), an endothelium dependent function and after sublingually glyceryl trinitrate (GTNMD), an independent endothelium function. Fat mass composition and distribution were assessed by dual-energy X-ray absorptiometry in 70 patients. In 50 obese patients an oral glucose tolerance test was done to determine insulin resistance. The obese children had significantly lower CSC and CSD than the healthy controls (respectively 0.12 +/- 0.04 vs 0.14 +/- 0.05 mm2.mmHg-1; p < 0.05 and 0.5 +/- 0.2 vs 0.8 +/- 0.4 mmHg(-1).10(-2); p < 0.001). Obese children had higher value than the controls for Einc (2.4 +/- 0.4 vs 1 +/- 0.24 mmHg.10(3); p < 0.001) that correlated poorly with fasting insulin concentrations (r = 0.34; p < 0.06) and BMI (r = 0.34; p < 0.01). FMD was significantly lower in obese children than in controls (6 +/- 3 vs 8 +/- 4%, p < 0.01) without modification of GTNMD (17 +/- 6 vs 18 +/- 7%, NS). These two parameters were respectively correlated with the android fat distribution (r = 0.36; p < 0.01; r = 0.49; p < 0.001). The CCA stiffness of obese children is linked to the amount of the overweight and to insulin resistance. The android fat distribution is related to endothelium dysfunction.

[Atrial septal defect calibration: comparison of methods using balloons and 3D-transesophageal echocardiography]. / Calibrage des communications interauriculaires: comparaison des méthodes du ballon et de l'échocardiographie transoesophagienne 3D.

The atrial septal defects (ASDs) occlusion by cardiac catheterization is an alternative to the surgical technique. The use of stretching by balloon is considered as the gold-standard for the ASDs calibration. Three dimensional transesophageral echocardiography (3D-TEE) brings an imaging of the ASD surface and allows the measurement of its maximal diameter. With the goal of a percutaneous ASD occlusion, seventy patients had a simultaneous calibration of the ASDs by 3D-TEE and the balloon method. The mean maximal diameter measured by 3D-TEE was at 20 + 15 mm (range 10-28 mm) compared to the mean balloon-stretched diameter at 22 + 5 mm (range 9-31 mm). The correlation between both methods is good (y = 3.15 + 0.77x, r = 0.8, p < 0.0001). The mean difference between the diameters measured by both methods was 1.85 + 3.17 mm. The ASD visualized by 3D-TEE was single in 54 cases and multiple in 16 cases. The correlation between both methods was excellent in those with a single ASD (y = 1.74 + 0.84x; r = 0.85. p < 0.0001) but it was poor in those with multiple ASD (y = 12.4 + 0.4x; r = 0.45). The calibration by the balloon method which tests the septum resistance is an additive method to 3D-TEE which measures the anatomic diameter of ASD.

[3D-transthoracic echocardiography: a selection method prior to percutaneous closure of atrial septal defects]. / Echocardiographie transthoracique 3D: une méthode de sélection avant fermeture percutanée des communications interauriculaires.

Prior to putting in place a percutaneous device, the assessment of the atrial septal defect anatomy is mandatory. The 3D transthoracic echocardiography is a non-invasive method bringing an imaging of the septal defect surface and its borders. Fifty-two patients ageing from 3 to 16 years old had a rotational 3D transthoracic echocardiography. Thirty-six (69%) were selected for a percutaneous closure (borders > 7 mm and a septal surface/atrial septal defect ratio > 2). Thirty-two of these selected patients (89%) benefited from the Amplatz prosthesis implantation with success. The maximal 3D diameter of the septal defect was 20 + 4 mm (14-30) compared to the mean size of prosthesis at 22 mm (18-30). Four of the 36 patients (aneurismal septum or a borderline septal surface/atrial septal defect ratio) were secondarily oriented to surgeons. Sixteen patients (31%) were selected directly to a surgical closure. The lack of borders or septal surface were confirmed by the surgical view. The 3D transthoracic echocardiography allows to define new criteria for the selection of patients prior to a percutaneous closure of atrial septal defects. This prospective study demonstrates that 9 out of 10 patients who had the 3D criteria had a percutaneous closure of the septal defect.

[Perforation of the aorta after percutaneous closure of an atrial septal defect with an Amplatz prosthesis, presenting with acute severe hemolysis]. / Perforation de l'aorte après fermeture percutanée d'une communication interauriculaire par prothèse d'Amplatz, révélée par une hémolyse aiguë sévère.

We report a case of aortic perforation three weeks after transcatheter occlusion of an atrial septal defect (ASD) by an Amplatzer device. Revealed by acute hemolysis, this complication needed an emergency surgical operation. The fistula between the no coronary Valsalva sinus of the aorta and the left atrium was repaired. The ASD was closed by patch. This serious accident should consider a short antero-superior rim as a risk factor for aortic perforation in transcatheter closure for ASD.

Idebenone and reduced cardiac hypertrophy in Friedreich's ataxia.

BACKGROUND: Friedreich's ataxia encodes a protein of unknown function, frataxin. The loss of frataxin is caused by a large GAA trinucleotide expansion in the first intron of the gene, resulting in deficiency of a Krebs cycle enzyme, aconitase, and of three mitochondrial respiratory chain complexes (I-III). This causes oxidative stress. Idebenone, a short chain quinone acting as an antioxidant, has been shown to protect heart muscle against oxidative stress in some patients. OBJECTIVE: To assess the efficiency of idebenone on cardiac hypertrophy in Friedreich's ataxia. DESIGN: Prospective, open trial. SETTING: Tertiary care centre. METHODS: Idebenone (5 mg/kg/day) was given orally to 38 patients with Friedreich's ataxia aged 4-22 years (20 males, 18 females). Cardiac ultrasound indices were recorded before and after idebenone treatment. RESULTS: After six months, cardiac ultrasound indicated a reduction in left ventricular mass of more than 20% in about half the patients (p < 0.001). The shortening fraction was initially reduced in six of the 38 patients (by between 11-26%) and it improved in five of these. In one patient, the shortening fraction only responded to 10 mg/kg/day of idebenone. No correlation was found between responsiveness to idebenone and age, sex, initial ultrasound indices, or the number of GAA repeats in the frataxin gene. CONCLUSIONS: Idebenone is effective at controlling cardiac hypertrophy in Friedreich's ataxia. As the drug has no serious side effects, there is a good case for giving it continuously in a dose of 5-10 mg/kg/day in patients with Friedreich's ataxia at the onset of hypertrophic cardiomyopathy.

Noninvasive assessment of arterial function in children: clinical applications.

Non invasive methods to assess arterial function are widely used in adults. The development and progression of arterial vascular disease is a multifactorial process that can start early in life, thus even in a pediatric population. Risk factors for cardiovascular disease mediate their effects by altering the structure, properties and function of wall and endothelial components of the arterial blood vessels. The ability to detect and monitor sub-clinical damage, representing the cumulative and integrated influence of risk factors in impairing arterial wall integrity, holds potential to further refine cardiovascular risk stratification and enable early intervention to prevent or attenuate disease progression. Measurements that provide more direct information in relation to changes in arterial wall integrity clearly hold predictive and therapeutic potential. The aim of this current review will be to describe the non-invasive procedure used in children to investigate the mechanical properties of a great elastic artery, the common carotid, and the endothelial function of the brachial artery. The accuracy of recording noninvasively the blood pressure wave contour along the arterial tree has been improved by the technique of applanation tonometry. The results obtained with these methods in previous studies are described.