Follow-up of children exposed to ionising radiation from cardiac catheterisation: the Coccinelle study.

Cardiac catheterisation has become an essential tool in the diagnosis and treatment of children with a wide variety of congenital and acquired forms of cardiovascular disease. Despite the clear clinical benefit to the patient, radiation exposure from paediatric cardiac catheterisation procedures (CCPs) may be substantial. Given children's greater sensitivity to radiation and the longer life span during which radiation health effects can develop, an epidemiological cohort study, named Coccinelle or 'Ladybird' (French acronym for 'Cohorte sur le risque de cancer après cardiologie interventionnelle pédiatrique'), is carried out in France to evaluate the risks of leukaemia and solid cancers in this population. A total number of 8000 included children are expected. Individual CCP-related doses will be assessed for each child included in the cohort. For each CCP performed, dosimetric parameters (dose-area product, fluoroscopy time and total number of cine frames) are retrieved retrospectively. Organ doses, especially to the lung, the oesophagus and the thyroid, are calculated with PCXMC software. The cohort will be followed up through linkage with French paediatric cancer registries.

Local reference levels and organ doses from pediatric cardiac interventional procedures.

Interventional cardiology (IC) procedures can be responsible for relatively high radiation doses compared to conventional radiology especially for young patients. The aim of this study was to assess current exposure levels in a French reference centre of pediatric IC. Dosimetric data including dose area product (DAP), fluoroscopy time (FT) and number of cine frame (NF) were analysed taking into account patient weight. Doses to the lungs, esophagus, breast and thyroid were evaluated using anthropomorphic phantoms and thermoluminescent dosimeters. Finally, effective doses (E) were calculated using DAP and conversion factors calculated with PCXMC 2.0 software. 801 IC procedures performed between 2010 and 2011 were analysed. Large variations were observed for DAP, FT and NF values for a given procedure and a given weight group. The assessment of organ doses showed high levels of dose to the lungs and esophagus especially in new-born babies. For diagnostic procedures, E varied from 0.3 to 23 mSv with a mean value of 4.8 mSv and for therapeutic procedures, values ranged from 0.1 to 48.4 mSv with a mean value of 7.3 mSv. The highest values were recorded for angioplasty procedures (mean 13 mSv, range 0.6-48.4 mSv). The increasing use of IC in pediatric population stresses the need of setting up reference levels and keeping doses to children as low as possible.

Prenatal diagnosis of isolated total anomalous pulmonary venous connection: a series of 10 cases.

OBJECTIVES: To report on a series of 10 fetuses with prenatally diagnosed isolated total anomalous pulmonary venous connection (TAPVC), focusing on echocardiographic features leading to diagnosis, assess accuracy of prenatal diagnosis and describe postnatal outcome. METHODS: In this review of our experience of prenatal diagnosis of isolated TAPVC, we analyzed retrospectively medical records and fetal echocardiography findings in all cases with prenatal diagnosis of isolated TAPVC delivered between 1 January 2001 and 1 October 2011 at a tertiary referral center, paying special attention to echocardiographic signs that led to referral. RESULTS: During the study period, 95 infants with isolated TAPVC were seen at the center. Initially, expert fetal echocardiography identified 14 fetuses with isolated TAPVC. Prenatal diagnosis was made at a mean gestational age of 31 (range, 25-37) weeks. Ten true-positive cases of TAPVC were confirmed after birth. The remaining four were considered false-positive cases: two had normal heart with left superior vena cava to coronary sinus, one had partial anomalous venous connection and one was lost to follow-up. Of the 85 diagnosed postnatally with TAPVC, only one had been seen prenatally by an expert cardiac sonographer. Echocardiographic signs leading to referral were related to pulmonary venous connection in half of the cases. Other suspected defects which led to referral were ostium prium atrial defect (n = 3), left-right asymmetry (n = 1), abnormal mitral valve (n = 1) and hepatic vascular malformation (n = 1). All infants with TAPVC underwent surgery. There was one postoperative death and nine survivors, with a mean follow-up of 31 (range, 2-104) months. CONCLUSION: Fetal diagnosis of isolated TAPVC is challenging even for experts. Echocardiographic anomalies may appear late in gestation. New tools should be proposed to identify abnormal venous drainage at the screening level.

[Interventional cardiac catheterization in children]. / Cathétérisme interventionnel et chirurgie cardiaque.

With the technological progress, the role of the cardiac catheterization has dramatically changed, moving from diagnostic to therapeutic and becoming adjuvant to surgical procedures. In various congenital heart defects, it allows to postpone the need for surgery or even cancel the surgical indication being less invasive and as powerful as surgery. It is thanks to many technological advances, in particular with the development of devices with memory alloy, that the catheterization makes such great strides today, and the miniaturization of the prosthetic material makes it possible to push back more and more the limits of feasibility which remain related to the smallness of the vascular accesses at the newborn age. The future of this discipline lies in the hybrid procedures, where a true teamwork between the surgeons and the pediatric cardiologists makes it possible to bring the best therapeutic strategy for patients with congenital heart defects.

A statistical model for quantification and prediction of cardiac remodelling: application to tetralogy of Fallot.

Cardiac remodelling plays a crucial role in heart diseases. Analyzing how the heart grows and remodels over time can provide precious insights into pathological mechanisms, eventually resulting in quantitative metrics for disease evaluation and therapy planning. This study aims to quantify the regional impacts of valve regurgitation and heart growth upon the end-diastolic right ventricle (RV) in patients with tetralogy of Fallot, a severe congenital heart defect. The ultimate goal is to determine, among clinical variables, predictors for the RV shape from which a statistical model that predicts RV remodelling is built. Our approach relies on a forward model based on currents and a diffeomorphic surface registration algorithm to estimate an unbiased template. Local effects of RV regurgitation upon the RV shape were assessed with Principal Component Analysis (PCA) and cross-sectional multivariate design. A generative 3-D model of RV growth was then estimated using partial least squares (PLS) and canonical correlation analysis (CCA). Applied on a retrospective population of 49 patients, cross-effects between growth and pathology could be identified. Qualitatively, the statistical findings were found realistic by cardiologists. 10-fold cross-validation demonstrated a promising generalization and stability of the growth model. Compared to PCA regression, PLS was more compact, more precise and provided better predictions.

[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.

[Transfer of cardiac catheterisation from a paediatric to an adult cardiological centre: results at 3 years]. / Transfert de l'activité de cathétérisme cardiaque d'un centre pédiatrique à un centre de cardiologie de l'adulte: trois ans d'activité

The advances of surgical and interventional treatment of congenital heart diseases have allowed a large number of patients with congenital heart disease to reach adult age. This population involves almost 0.3/1000 of total population in West Europe and North America and can be estimated around 200000 patients in France. Patients with operated Tetralogy of Fallot, benign forms of pulmonary atresia with ventricular septal defect, simple or complex transposition of the great arteries usually survive beyond childhood. These patients can need repeated interventions to treat lesions of native or reconstructed pulmonary arteries and/or aortic arch, to occlude residual shunts, to treat pulmonary incompetence. More complex heart diseases such as single ventricle, rarely allow survival until the adult age. The majority of these patients undergo heart transplant, often made difficult by multiple cardiac surgeries, anomalies of pulmonary arteries, chronic cyanosis, aorto-pulmonary shunts. Patients with relatively simple or complex congenital heart diseases need to be followed-up in specialized units, like those created more than twenty years ago in the United States, Canada, and United Kingdom. Interventional cardiac catheterisation play a major role in the management of this population. The results of 3 years of activity in a new centre treating GUCH patients are illustrated.

[Percutaneous occlusion of a left superior vena cava draining into the left atrium: two case reports]. / Occlusion percutanée d'une veine cave supérieure gauche s'abouchant à l'oreillette gauche. A propos de deux observations.

Persistent left superior caval vein draining into the left atrium is responsible for a right-to-left shunt which can be source of desaturation and systemic embolism. We present 2 cases of successful percutaneous treatment in 2 patients in whom no bridging vein existed and systemic desaturation was found after repair of the underlying congenital heart.

[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%.

Closure of extracardiac Fontan fenestration by using the covered Cheatham Platinum stent.

OBJECTIVE: The purpose of this study was to describe our experience concerning the use of covered Cheatham Platinum (CP) stent in patients with fenestrated total cavopulmonary connection (TCPC). BACKGROUND: Closure of TCPC fenestrations has been achieved by utilizing different devices designed to close intra-cardiac or aorto-pulmonary communications. METHODS: We used the covered CP stent in 6 patients with fenestrated TCPC. Median age and weight were 11 years and 38 Kg, respectively. Femoral approach was used in all but 1 patient having bilateral thrombosis of femoral veins. The CP stent was crimped on a BiB balloon in 5 patients and on a simple balloon in 1 patient. The balloon's diameter was the same size or 1-2 mm larger than the TCPT conduit, according to angiographic diameter. RESULTS: Mean procedural and fluoroscopy time were 41 +/- 8 and 8 +/- 2 min, respectively. Immediate full occlusion of fenestration was obtained in all patients. Mean central venous pressure was not significantly increased from 10.8 +/- 2.5 to 11.8 +/- 2.8 mm Hg and oxygen saturation significantly increased from (91.5 +/- 4.4)% to (98.5 +/- 1.9)% (P = 0.003). No procedural or intra-hospital complications occurred. In particular, no arrhythmias, systemic embolism, or acute venous thrombosis were observed. At a median follow-up of 2.8 months all patients have normal oxygen saturation and are symptom-free. CONCLUSIONS: The covered CP stent can be easily and effectively used for closure of TCPC fenestrations. This method has the advantage to avoid protrusion of prosthetic material into the left atrium and to prevent early or late embolism.

Stroke after surgical treatment of sinus venosus type atrial septal defect: percutaneous treatment.

BACKGROUND AND PURPOSE: Sinus venosus-type atrial septal defect can be associated with anomalous drainage of the upper right pulmonary vein into the superior vena cava. Surgical correction provides reconstruction of superior vena cava and rerouting of the pulmonary vein into the left atrium. SUMMARY OF CASE: We report the case of a young woman who experienced 2 strokes 11 years after surgical repair of sinus venosus-type atrial septal defect. CONCLUSIONS: Interventional treatment resolved the superior vena cava stenosis, rerouted the pulmonary vein into the left atrium and abolished the right to left shunt.

[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.

[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.

[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.

[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.