[Tropical endomyocardial fibrosis: Perspectives]. / Fibrose endomyocardique tropicale : perspectives.

Tropical endomyocardial fibrosis (FET) is a leading cause of heart failure and the most common restrictive cardiomyopathy worldwide. Extensive fibrosis of the ventricular endocardium causing architectural distortion, impaired filling and valvular insufficiency define the disease. Confined to peculiar and limited geographical areas, the aetiology remains blurred and it carries a grim prognosis. The changing burden reported recently in some endemic areas and the refinement of diagnostic tools have emphasized alternative routes for understanding and treatment of the disease.

Coronary myocardial bridging in Noonan syndrome: definitive diagnosis with high-resolution CT.

Noonan syndrome is an autosomal dominant disorder reported at an incidence of 1 in 1000-2500 live-born patients. Pulmonary stenosis with a dysplastic pulmonary valve and hypertrophic cardiomyopathy are often associated with this syndrome. We report on a 9-year-old patient affected by Noonan syndrome with left ventricle hypertrophy, in whom a sudden clinical presentation of chest pain after effort led to the clinical suspicion of associated coronary myocardial bridging, which was confirmed by high-resolution CT. We also review the literature on this topic.

[Utilisation of gastric ring systems for pulmonary artery banding: an animal study]. / Utilisation d'un système de cerclage gastrique comme cerclage réglable de l'artère pulmonaire: une étude chez l'animal.

OBJECTIVES: Pulmonary artery banding is often required as a first palliative procedure in infants with congenital heart disease and high pulmonary blood flow or to retrain the left ventricle. The purpose of the study was to demonstrate the safety of a gastric banding system as an adjustable pulmonary artery banding in chronic implantation. METHODS: Five ewes underwent implantation of the banding system around the main pulmonary artery through a left thoracotomy. All had functional evaluation with progressive occlusion and opening of the device every two weeks for a total period of three months. Invasive pressure measurements in the right ventricle and aorta were carried out each time. RESULTS: Devices could be implanted easily. Progressive occlusion and re-opening were possible in all animals during each time point. All animals survived throughout the protocol. Retrieval of the device was achieved in all animals. In one, it was challenging because of the presence of a fibrotic reaction around the device. It died because of pulmonary artery perforation before the sacrifice. At autopsy, microscopic examination showed no signs of myocardial fibrosis. CONCLUSIONS: In animals, gastric banding system is a safe and effective implantable device to adjust pulmonary artery diameter over a prolonged period of time. This new device may be a valuable alternative to the repeated conventional pulmonary artery banding needed for ventricular retraining in humans.

[Anomalous left main coronary artery with an inter-aorto-pulmonary trajectory: diagnostic value of 64-slice CT scan with cardiac synchronisation]. / Anomalie de naissance du tronc coronaire gauche avec un trajet interaortopulmonaire: apport du scanner 64 coupes avec synchronisation cardiaque.

Anomalous left coronary artery arising from the right sinus with an inter-aorto-pulmonary trajectory is a classical cause of sudden death and myocardial ischaemia in young adults. The mechanism is compression of the coronary during physiological dilatation of the great arteries on exercise. The authors report the case of a 12 year old child who had syncope on effort preceded by angina due to this malformation. The CT scan with 3-D reconstruction confirmed the diagnosis and the interarterial trajectory of the left coronary artery and the anatomical relationships with the vessels at the base of the heart. Cardiac CT scan is the investigation of choice for diagnosis and preoperative work-up of congenital coronary anomalies. In the authors' experience, the investigation is performed systematically when an anomalous coronary artery is suspected, in addition to echocardiography and instead of conventional coronary angiography.

[Percutaneous closure of veno-venous fistulae after total cavopulmonary derivation: a case report]. / Fermeture percutanée de fistules veino-veineuses après dérivation cavo-pulmonaire totale: à propos d'un cas.

The authors report the case of a boy with a complex congenital heart malformation with a dextro-isomerism or Ivemark's syndrome. After several palliative procedures, a total cavopulmonary derivation was performed at the age of 11 years with implantation of a fenestrated tube between the inferior vena cava and the right pulmonary artery. The fenestration was closed two years later. At about 15 years of age, progressive systemic desaturation was observed; cardiac catheterisation with angiography showed a large number of intra-hepatic veno-venous collaterals draining into a large left suprahepatic vein which drained into the right atrium connected to the systemic ventricle. The largest fistula was embolised with a 20 mm Amplatzer Duct Occluder and two others with 14 x 12 and 10 x 8 Amplatzer Duct Occluders. The saturation rose from 75% to 94%. After 6 months' follow-up, the child is asymptomatic and the systemic saturation is 90%.

CT angiography of the artery of Adamkiewicz with 64-section technology: first experience in children.

BACKGROUND AND PURPOSE: Preoperative localization of the artery of Adamkiewicz (AKA) may be useful in selected children in prevention of ischemic spinal complications of spinal or thoracoabdominal aortic surgery. The aim of our study was to investigate the ability of 64-section CT for assessing the AKA in children. MATERIALS AND METHODS: Forty children (mean age, 7.5 +/- 5 years) underwent thoracic imaging with a 64-section CT scanner after intravenous injection of 1.5 mL/kg of contrast agent. Volumetric reconstructions were obtained for every patient. Identification of the AKA was performed on the basis of continuity from the aorta to the anterior spinal artery with a characteristic hairpin turn. Identification of the AKA and determination of its origin and course were analyzed by 2 independent radiologists. RESULTS: The AKA was successfully visualized in 38 patients (95%). Twenty-seven (71.1%) AKAs originated on the left side, whereas 11 (28.9%) originated on the right side. It was seen to originate at the level of the left 5th intercostal artery in 1 patient, the left 8th in 4, the left 9th in 15, the left 10th in 5, the left 11th in 2, the right 8th in 2, the right 9th in 6, the right 10th in 2, and the right 12th in 1. CONCLUSION: Sixty-four-section CT angiography depicted the AKA in a very high percentage (95%) of children. The results of this study suggest that 64-section CT angiography is a viable noninvasive technique that could be an alternate to selective angiography for delineating the AKA.

[Multislice thoracic CT scanner]. / Scanner thoracique multibarette.

The scanner, or computed tomography, is the reference imaging test for most thoracic diseases in both adults and children. It has gone through tremendously rapid technological progress over the last few years with the appearance of multirow (or multidetector or multislice) scanners. This technology currently provides volume acquisition of a child's thorax in 2-5 s. These technological advances are particularly useful for exploring airway diseases, largely because of the range of reconstruction possibilities (maximum and minimum intensity projection, volume rendering, virtual endoscopy), which offer advantageous perspectives in pediatrics. Multiplanar imaging and above all three-dimensional imaging provide a totally original analysis in space of the sometimes complex relations between vascular and airway structures, making it possible to understand certain extrinsic bronchial compression mechanisms more precisely. The present study briefly reviews the technical aspects of the thoracic scanner in children and illustrates its role today in volume CT in childhood thoracic diseases using clinical examples taken from our daily practice.

[Development and evaluation of a prosthetic valved conduit dilated by percutaneous approach: experimental study in the lamb]. / Développement et évaluation d'un conduit prothétique muni d'une valve dilatable par voie percutanée: étude chez l'agneau.

This study was undertaken to develop a dilated valved conduit for reconstruction of the right ventricular outflow tract in the animal. The conduits were made by sewing a valved tube (Medtronic Inc) inside a vascular stent (Numed Inc). After preparation, they were inserted surgically in five lambs. The conduits were then dilated 6 weeks and 3 months after their implantation. Before sacrificing the animals at 3 months, a 22 mm valved stent was implanted percutaneously inside the surgical conduits. One animal died suddenly due to kinking of the conduit. Balloon dilatation was performed in the surviving animals. The first dilatation only had a modest impact on valvular function but it was much aggravated after the second dilatation. A valved stent was successfully inserted percutaneously. At sacrifice, all the conduits were completely engulfed in an intense fibrosis. In conclusion, a valved biological conduit for reconstruction of the right ventricular ejection tract has been developed and can be dilated sequentially to follow growth. The new product could have an important role to play in the management of congenital malformations involving the right ventricular outflow tract.

[Use of covered stents to occlude extracardiac Fontan fenestration]. / Utilisation de stents couverts pour la fermeture des fenestrations extracardiaques des dérivations cavopulmonaires totales.

INTRODUCTION: A fenestration is usually surgically created to improve the post-operative course of patients undergoing total cavopulmonary connection. It, however, has a potentially deleterious effect on the long-term period. Closure of these fenestrations is usually performed by interventional catheterization. No device has been specially designed and closure of extracardiac fenestration, in particular, can be challenging. We report our experience in occlusion of such fenestrations using covered stents (Numed Inc). METHODS: From July 2005 to October 2005, we attempted to occlude extracardiac Fontan fenestration using CP covered stents in 4 consecutive patients. RESULTS: All patients had a successful occlusion of the fenestration. The procedure was performed from femoral or jugular vein respectively in three and one patient. Mean central venous pressure did not increase significantly (from 11.25 to 12.75 mmHg) whereas mean oxygen saturation increased significantly from 92% to 99% (p=0.0047). Abolition of shunt was obtained immediately after insertion of the covered stent in all patients. No early complications were observed. All patients were ambulatory the day after the procedure. CONCLUSION: The insertion of a covered stent inside the extracardiac Fontan conduit allowed the exclusion of the fenestration in all patients without the need of crossing the fenestration and with no early mortality or morbidity. Long-term follow-up are needed before considering the use of such device as the device of choice in that application.

[Multislice CT scan in congenital heart disease]. / Le scanner volumique dans les cardiopathies congénitales.

The management of congenital cardiovascular malformations is a diagnostic challenge. It requires accurate assessment of the intra- and extra-cardiac malformations. Three-dimensional imaging by the multislice CT scanner is now a routine investigation for congenital heart disease, complementary to echocardiography, and, increasingly, an alternative to conventional angiography. Three dimensional imaging is particularly useful in the diagnosis of complex congenital heart disease, in the preparation of complex investigations of interventional cardiac catheterisation and the postoperative evaluation of surgical repair. This report describes the different acquisition protocols adapted for children and illustrates the present role of volumic CT scanner in congenital heart disease by clinical examples of everyday clinical practice.

[Transcatheter exclusion of a giant aneurysm of the left pulmonary artery using covered stents]. / Exclusion d'un anévrisme géant de l'artère pulmonaire gauche par cathétérisme interventionnel: utilisation de stents couverts.

An 11 year-old boy with an Alagille syndrome was referred for an urgent echocardiography because of new onset of febrile hemoptysis. Follow-up in our institution to that point had shown severe hypoplastic pulmonary arteries. The diagnosis of tricuspid endocarditis complicated by pulmonary embolisms and aneurysm of the left pulmonary artery was done. Despite effective antiobiotics, the evolution was marked by rapid progression in size of the left pulmonary aneurysm. Since the surgery was not an option because of high risk of death, this aneurysm was treated by a trans-catheter technique using covered stents (CP stents, Numed Inc). Its exclusion allowed to stop its progression in size and to avoid its rupture.

[Mycoplasma pneumoniae myopericarditis in children]. / Myopéricardite à Mycoplasma pneumoniae chez l'enfant.

Mycoplasma pneumoniae myocarditis is a rare condition, potentially very serious and seldom described in children. It is classically attributed to direct invasion or to an indirect immunological mechanism. The authors report the case of a 10 year old boy with myopericarditis, proved by authentic seroconversion, complicating congenital mitro-aortic valvular disease. In this case, the spectacular response to steroid therapy was in favour of an indirect immunological causal mechanism of the left ventricular dysfunction and pericardial involvement.

[Spontaneous idiopathic chylopericardium in childhood]. / Chylopericarde idiopathique spontané de l'enfant.

Here we report a case of a primary idiopathic chylopericardium in a 13 years old child. Pericardial effusion was diagnosed because the child suffered chest pain and fatigue. Pericardial drainage was performed and 800mL of chylous fluid was evacuated. Extensive investigations were performed but no cause could be found. Thoracic CT scan, lymphoscintigraphy and MRI did not evidence any communication between the thoracic duct and pericardium. After 2 recurrences of pericardial effusion while the child was on a medium chain triglycerides regimen, it was decided to ligate the thoracic duct and to do a partial pericardectomy. The result was excellent with complete resolution of the pericardial effusion and no recurrence since 3 years.

Right to left shunt through interatrial septal defects in patients with congenital heart disease: results of interventional closure.

OBJECTIVE: To study the effects of closure of interatrial communications associated with a right to left shunt in patients with congenital heart disease (CHD) who had a biventricular repair. DESIGN: Retrospective study. SETTING: Tertiary referral centre. PATIENTS: 15 patients with CHD with right to left shunt through an interatrial communication: three had repaired tetralogy of Fallot, five had repaired pulmonary atresia with intact ventricular septum, four had Ebstein's disease, and three had other CHDs. Two patients had had a stroke before closure of the interatrial communication. INTERVENTIONS: Percutaneous atrial septal defect (n = 6) or persistent foramen ovale (n = 9) closure. All patients underwent an exercise test before and after interatrial communication closure. RESULTS: Five patients were cyanotic at rest. During exercise, mean (SD) oxygen saturation diminished from 93.9 (3.8)% to 84.3 (4.8)% (p < 0.05). Interatrial communication closure led to an immediate increase of oxygen saturation from 93.9 (3.8)% to 98.6 (1.6)% (p < 0.05). At a median follow up of three years (range 0.5-5) all but one patient with a residual atrial septal defect had normal oxygen saturation at rest and during exercise. Maximum workload increased from 7.2 (1.9) to 9.0 (2.2) metabolic equivalents (p < 0.001). CONCLUSIONS: Percutaneous closure of interatrial communications associated with a right to left shunt allows restoration of normal oxygen saturation at rest, avoidance of desaturation during exercise, and improvement of exercise performance in patients with CHD.

[Aortic arch shape and flow dynamics]. / Architecture de la crosse aortique et circulation des fluides.

Post-operative deformation of the aortic arch architecture is associated with an increased risk of hypertension following correction of coarctation. In addition to morphological analysis, MRI allows a functional analysis of the thoracic aorta. We report three examples which illustrate the direct relationship between aortic arch morphology and blood flow in the thoracic aorta.

[Relationship between aortic arch shape and blood pressure response after coarctation repair]. / Relation entre l'architecture de la crosse aortique et la pression artérielle après cure de coarctation.

The mechanisms of secondary hypertension after repair of coarctation of the aorta are not well understood. Abnormalities of the architecture of the aortic arch and their consequences on blood pressure have not been studied. In order to study the relationship between abnormalities or aortic arch architecture and resting blood pressure ninety-four patients without re-coarctation were followed up prospectively from 1997 to 2004 (mean age 16.9 +/- 8.1 years; mean weight 57.5 +/- 18.3 Kg; interval since surgery 16.3 +/- 5.4 years). All underwent MRI angiography of the thoracic aorta which enabled the abnormalities to be classified in 3 groups: gothic arch, crenellated arch and roman arch. Twenty-four patients (25.5%) were hypertensive and 70 (74.4%) normotensive. There were 40 gothic arches (42.5%). 14 crenellated arches (15%) and 40 roman arches (42.5%). Gothic arches were more commonly observed in the hypertensive patients (18/40, [45%, 95% CI 31-62]) than the crenellated arches (4/14, [28.5%, 95% CI 7-48]) or the roman arches (2/40, [5%, 95% CI 2-12]). Only the gothic arch was independently correlated with hypertension on multivariate analysis. The authors conclude that gothic deformation of the aortic arch is an independent predictive factor of hypertension in patients operated for coarctation with an excellent result on the isthmic region. Patients with a gothic appearance of their aortic arch should be followed up closely.

[Closure of extrocardiac Fontan fenestration by using the Amplatzer duct occluder]. / Fermeture percutanée à l'aide de l'Amplatzer duct occluder des fenestrations extracardiaques des dérivations cavopulmonaires totales.

INTRODUCTION: A direct or tubular communication between the systemic venous system and the systemic atrium, generally called fenestration, is surgically created to improve the postoperative period of patients undergoing total cavopulmonary connection. However, a fenestration prompts a potentially deleterious right to left shunt, and is generally closed after the postoperative period. Direct fenestrations can be closed using coils, or devices designed for atrial septal defect closure. However, no devices have been designed for closure of extracardiac fenestrations. We report our experience concerning the closure of extracardiac Fontan fenestration by the Amplatzer duct occluder (ADO). METHODS: From January 2001 to December 2002, we closed extracardiac Fontan fenestrations using the ADO device in 10 consecutive patients. Indications to fenestration closure were: low velocity shunt through the fenestration, mild desaturation, and absence of effusions. RESULTS: All patients had a successful closure of the fenestration. The procedure was performed through the femoral vein in 7 cases and through the right jugular vein in 3. Mean central venous pressure increased not significantly from 12 to 13-mmHg. Mean oxygen saturation increased significantly from 90 to 97% (p<0.001). Immediate shut abolition was obtained in 9 cases. No complications were observed. At a median follow-up of 12 months (range 6-18 months), all patients are free of symptoms and have a normal oxygen saturation at rest as well as at exertion. CONCLUSION: The ADO device allowed closing the extracardiac Fontan fenestration in all patients with no mortality, no morbidity and a rate of 100% of complete closure at mid-term follow-up.

[Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries]. / Atrésie pulmonaire à septum ouvert avec collatérales aorto-pulmonaires majeures.

INTRODUCTION: In order to establish the best strategy of treatment and predictors of outcome in infants with pulmonary atresia with ventricular septal defect and major aorto-pulmonary collateral arteries, we reviewed our institutional experience concerning 47 infants. METHODS: Inclusion criteria included an angiographic diagnosis of pulmonary atresia with ventricular septal defect and major aorto-pulmonary collateral arteries with or without central pulmonary arteries and a repair by the same surgeon. Thirty-one patients had confluent (type III) and 16 absent (type IV) central pulmonary arteries. Pulmonary arteries were considered to be adequate when they measured > or = 4 mm. RESULTS: There were 8 deaths (17%). Complete repair was performed in 24 patients (18 in group III and 6 in group IV) with 79.3% had a right/left ventricular pressure <0.5, 16; 5%<1 and 4.2%>1. Eleven patients are waiting for complete repair and 4 will be operated on pulmonary arteries. One stage complete repair was performed in 3 patients, two and third stage repair (after unifocalisation or right ventricle to pulmonary arteries conduit) was attained in 21 patients. Among patients having had a complete repair and showing a right/left ventricular pressure <0.5, 88.9% had a pulmonary atresia with ventricular septal defect type III and 50% a pulmonary atresia with ventricular septal defect type IV. Only 56% of type III patients with a right/left ventricular pressure <0.5 had adequate central pulmonary arteries. CONCLUSION: In our study, the small size and the absence of central pulmonary arteries do not prevent a positive outcome.

[Successful recanulization of superior venous vessels: a new challenge for interventional pediatric cardiology]. / Reperméabilisation des gros troncs veineux supérieurs: un nouveau défi pour la cardiologie interventionnelle pédiatrique.

UNLABELLED: The use of long-term central venous catheters is a routine in chronic pediatric diseases. Thrombotic complications progressively reduce the central venous capital and hamper the long-term management of these patients. OBSERVATION: We report two cases of obstruction of the central upper venous system and discuss of the techniques used to repermeabilize venous axes before the placement of a new central line. CONCLUSION: The control of the permeability of the central veins should be performed before any withdrawal of central catheters, repermeabilization of the venous axes being simpler when the central catheter is kept in place in the occluded vessel.