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I read with interest the article by Haddad et al concerning the bench testing of BeSmooth peripheral tests, and I would like to share some thoughts and highlight few points.BeSmooth stent is behaving like any pre-mounted stent available on the market. None can achieve the Holy Grail of being dilatable to adult size without losing their integrity. Until dedicated stents are available, pre-mounted stents are preferred to unmounted stents because of their better profile and deliverability. Technically, proper post-dilatation should be performed in order to avoid and extensive foreshortening and the formation of a ring that would limit further expansion of the stent. BeSmooth stent like other pre-mounted stents can also be used without restriction in situations where overexpansion is not required.BeSmooth stent platform with the knowledge of its intrinsic properties is, in our opinion, a good add-on to the armamentarium of pre-mounted stents used in paediatric cardiology.

A combination of aortic valve atresia and an interrupted aortic arch is a unique disease in which perfusion to the brain and myocardium depends on coexisting lesions or type of interruption. We report a case of aortic valve atresia with type B interrupted arch, bilateral arterial ductus in a neonate who was successfully palliated using a hybrid approach by placing stents in both arterial ductus and banding of branch pulmonary arteries.

Objectives: The aim of the study was to report the outcome of cardiac catheterisation in low-weight patients., Background: Data regarding cardiac catheterisation in infants weighing <2500 g are scarce., Methods: We reviewed all cardiac catheterisations performed in infants weighing <2500 g between January 2000 and May 2016. An analysis with respect to the type of procedure, the complexity of procedure (procedure type risk), and haemodynamic vulnerability index was finally carried out. We report the occurrence of deaths and complications using the adverse event severity score., Results: A total of 218 procedures were performed on 211 patients. The mean age and weight were, respectively, 15 ± 26 days (range, 0-152) and 2111 ± 338 g (range, 1000-2500). Procedures were interventional and diagnostic, respectively, in 174 (80%) and 44 (20%) patients. Out of 218, 205 (94%) were successful. Eleven complications (5%) occurred - six with an adverse event severity score of 4 and five with an adverse event severity score of 3. Ten patients (91%) showed a favourable outcome, and one died (stent thrombosis few hours after patent ductus arteriosus stenting). No correlation was found between lower weight and occurrence of death (p = 0.68) or complications (p = 0.23). The gravity scores (procedure type risk and haemodynamic vulnerability index) were not predictive of complications., Conclusions: Cardiac catheterisation in infants weighing <2500 g appears feasible and effective with low risk. The weight should not discourage from performing cardiac catheterisation in this population.

Objectives: The aim of this study was to evaluate the reduction of frame rate from 7.5 to 4 frames per second on radiation exposure and to provide new standards of radiation exposure., Background: Frame rate is a large contributor to radiation exposure. The use of 4 frames per second for closure of atrial septal defects has been reported not to affect the level of radiation exposure., Methods: We retrospectively reviewed radiation data from all patients referred to our catheterisation laboratory for closure of an atrial septal defect between January, 2015 and June, 2017. Fluoroscopic time, dose area product (μGy.m2), and total air kerma (mGy) were collected. These values were compared according to the frame rate used for closure of atrial septal defects., Results: A total of 49 atrial septal defects were closed using 7.5 frames per second and 85 using 4 frames per second. Baseline characteristics were similar in both groups. Procedural success was similar in both groups (100 versus 98.8%). Median total air kerma and dose area product were statistically lower in the 4 frames per second group (4 versus 1.3 mGy [p=0.00012]), 43.7 versus 13.1 μGy.m2 [p0.05)., Conclusion: Reduction of frame rate allows reducing significantly the radiation exposure while maintaining excellent clinical results in transcatheter closure of atrial septal defects. We recommend implementing this little change in every laboratory in order to achieve drastic reduction of radiation exposure to the patients and laboratory personnel.

Objectives: The aims of this study were to describe radiation level at our institution during transcatheter patent ductus arteriosus occlusion and to evaluate the components contributing to radiation exposure., Background: Transcatheter occlusion relying on X-ray imaging has become the treatment of choice for patients with patent ductus arteriosus. Interventionists now work hard to minimise radiation exposure in order to reduce risk of induced cancers., Methods: We retrospectively reviewed all consecutive children who underwent transcatheter closure of patent ductus arteriosus from January 2012 to January 2016. Clinical data, anatomical characteristics, and catheterisation procedure parameters were reported. Radiation doses were analysed for the following variables: total air kerma, mGy; dose area product, Gy.cm2; dose area product per body weight, Gy.cm2/kg; and total fluoroscopic time., Results: A total of 324 patients were included (median age=1.51 [Q1-Q3: 0.62-4.23] years; weight=10.3 [6.7-17.0] kg). In all, 322/324 (99.4%) procedures were successful. The median radiation doses were as follows: total air kerma: 26 (14.5-49.3) mGy; dose area product: 1.01 (0.56-2.24) Gy.cm2; dose area product/kg: 0.106 (0.061-0.185) Gy.cm2/kg; and fluoroscopic time: 2.8 (2-4) min. In multivariate analysis, a weight >10 kg, a ductus arteriosus width <2 mm, complications during the procedure, and a high frame rate (15 frames/second) were risk factors for an increased exposure., Conclusion: Lower doses of radiation can be achieved with subsequent recommendations: technical improvement, frame rate reduction, avoidance of biplane cineangiograms, use of stored fluoroscopy as much as possible, and limitation of fluoroscopic time. A greater use of echocardiography might even lessen the exposure.

Objectives: The aim of this study was to determine the probability of intervention at birth after prenatal diagnosis of CHD., Methods: A 10-year retrospective study including all foetuses with a prenatally diagnosed CHD and those delivered in a tertiary-care cardiac centre between January, 2002 and December, 2011 was carried out. Patients were classified into eight groups according to the anticipated risk of neonatal intervention., Results: The need for urgent intervention and/or PGE1 infusion within the first 48 hours of life was 47% (n=507/1080): 72% (n=248) for CHD at risk for a Rashkind procedure, 77% (n=72) for CHD with ductal-dependent pulmonary flow, 13% (n=22) for CHD with potentially ductal-dependent pulmonary flow, 94% (n=62) for CHD with ductal-dependent systemic flow, 29% (n=88) for CHD with potentially ductal-dependant systemic flow, 50% (n=4) for total anomalous pulmonary venous connection, and 17% (n=1) for CHD with atrio-ventricular block. In all, 34% of the patients received PGE1 infusion and 21.4% underwent urgent catheter-based or surgical interventions; 10% of patients without anticipated risk (n=10) underwent an early intervention; 6.7% (n=73) of the patients died; and 55% (n=589) had an intervention before discharge from hospital., Conclusion: Half of the neonates with foetal CHD benefited from an urgent intervention or PGE1 infusion at birth. We recommend scheduled delivery and in utero transfer for transposition of the great arteries, double-outlet right ventricle with sub-pulmonary ventricular septal defect, total anomalous pulmonary venous connection, CHD with atrio-ventricular block with heart rate <50, all ductal-dependant lesions, and CHD with potentially ductal-dependant systemic flow.

Background: Discordant atrioventricular with concordant ventriculo-arterial connections is a rare cardiac defect. When isolated, the haemodynamics resemble transposition of the great arteries. In complex heart defects such as heterotaxy, haemodynamics guide the surgical approach., Objective: To report a series of eight patients with discordant atrioventricular and concordant ventriculo-arterial connections focussing on anatomical and diagnostic difficulties, surgical management, and follow-up., Methods: A retrospective review was carried out from 1983 to 2013. Anatomical description was based on segmental analysis. Emphasis was placed on the venoatrial connections., Results: Segmental arrangement was {I, D, S} in six patients, all with spiralling great vessels. There were two patients with parallel great vessels of whom one had {S, L, D} and the other had {S, L, A} arrangement. Of eight patients, five had heterotaxy syndrome. Median age at repair surgery was 1.4 years (with a range from 1.1 months to 8.1 years). The repair surgery finally performed was the atrial switch procedure in seven out of eight patients. The main post-operative complications were two cases of baffle obstruction and one sick sinus syndrome needing pacemaker implantation. There were two early post-operative deaths and six late survivors. Median follow-up was 4.2 years (with a range from 3.9 to 26.7 years) with good functional status in all survivors. Discussion Diagnosing discordant atrioventricular with concordant ventriculo-arterial connections remains challenging. There are ongoing controversies about the definition of atrial morphology and heterotaxy syndrome animating the anatomic discussion of these complex heart defects. Haemodynamically, the atrial switch procedure is the surgical method of choice with an encouraging long-term follow-up despite rhythm disturbances and baffle obstruction.

Objective: The use of covered stents to close fenestration in total cavopulmonary connection is presented., Methods: We retrospectively reviewed data of all patients undergoing the procedure of a covered stent to close fenestration of total cavopulmonary connection between 2005 and 2012., Results: A total of 50 patients met the inclusion criteria. Median age and weight were 7.7 years and 20 kg, respectively. Median interval between Fontan completion and fenestration closure was 13 months. The femoral vein was used in 42 patients and the jugular vein in eight patients. Of the patients, seven received two stents. Covered stents were CP stents in 42 patients and Atrium Advanta V12 in eight patients. BIB balloons were used in 24 patients and simple balloons in 18 patients. Simultaneous occlusion of venous collaterals was observed in five patients. Median procedural and fluoroscopy times were 49 and 8 minutes, respectively. Mean central venous pressure rose from 10 to 12 mmHg. Mean oxygen saturation increased from 88% to 96%. Full occlusion was confirmed in 47 patients. The remaining had residual shunts: two patients had intracardiac Fontan, and one patient had a stent that could not be fully opened. Following the procedure, five patients had local bleeding, and three delayed discharge 48 hours after the procedure. There was no thromboembolic event after a mean follow-up of 49 months., Conclusion: Covered stent is a good option to close fenestration in extracardiac total cavopulmonary connection. It is safe, easily achievable with low fluoroscopy time, with very low risk of complication or failure. Good results are sustainable when excluding patients with none circular pathway.

Objective: The objective was to report two new patients with the diagnosis of alveolar capillary dysplasia and congenital heart disease, to describe the associated cardiac defects seen in these cases and in the literature, and to consider recent genetic advances concerning the FOX transcription factor gene cluster in chromosome 16q24.1q24.2., Methods: We retrospectively analysed the records of all patients with congenital heart disease and alveolar capillary dysplasia seen in the Pediatric Cardiology Department between 2005 and 2010. We reviewed all literature published in the English language relating to cases of alveolar capillary dysplasia and congenital heart disease., Results: Two infants with alveolar capillary dysplasia and cardiac malformation were identified: one had an atrioventricular septal defect and a de novo balanced reciprocal translocation t(1;16)(q32;q24), the second infant had a ventricular septal defect. Analysis of 31 cases of the literature including these new cases showed a predominant association of alveolar capillary dysplasia with obstructive left heart disease (35%), as well as an atrioventricular septal defect (29%). FOX gene cluster defects were identified in eight of these patients., Discussion: Genetic background of alveolar capillary dysplasia is discussed in the light of the balanced reciprocal translocation t(1;16)(q32;q24) identified in the first child of this report. Alveolar capillary dysplasia should be suspected in neonates with congenital heart disease and unexpectedly elevated pulmonary vascular resistances, especially in cases of obstructive left heart disease or atrioventricular septal defect. Detecting FOX gene cluster defects should be considered in infants with alveolar capillary dysplasia with or without congenital heart disease.

Background: Neonatal aortic coarctation presenting with multiorgan failure is a life threatening condition., Objective: Our aim was to investigate whether emergency balloon dilation improved the prognosis of neonates with aortic coarctation who present with multiorgan failure., Methods: We studied all neonates referred over a period of 6 years with aortic coarctation and left ventricular dysfunction, with or without multiorgan failure. During the first half of the period, from January, 2001 to December, 2003, patients were treated surgically after regression of the multiorgan failure. During the second half, from January, 2004, to January, 2007, patients not responding to medical management were referred for balloon dilation of the coarcted segment., Results: Among 113 and 181 neonates, respectively, treated during the 2 periods, 40 and 36 had left ventricular dysfunction (p less than 0.01), and 11 and 23 had multiorgan failure (p less than 0.01). Prior to 2004, 6 deaths occurred before surgery. After 2004, 3 out of 14 patients (21%) treated by balloon angioplasty died in consequence of the procedure.During the period of the study, the global mortality of patients with left ventricular dysfunction diminished from 17.5 to 13.8%, this difference not being statistically significant. Mortality due to multiorgan failure, however, diminished from 54 to 13% (p less than 0.01). Multiorgan failure was reversed under medical treatment in 45% of patients, but in 87% under the regime of medical combined with interventional treatment (p less than 0.01)., Conclusions: To the best of our knowledge, this is the first series of neonates with aortic coarctation and multiorgan failure receiving a palliative treatment by balloon dilation. Although the prognosis of this condition remains severe, emergency balloon dilation can diminish mortality, providing a bridge to surgery in severely ill patients.

A congenital fistulous connection between the right pulmonary artery and the left atrium is a rare condition, resulting in early cyanosis and cardiac failure. These patients usually required urgent surgery. We present a neonate in whom we successfully closed such a large fistula via catheterization with an Amplatzer Duct Occluder, resulting in rapid clinical improvement, and obviating the need for surgical repair.

Objectives: To identify predictive factors of complications occurring during paediatric interventional catheterisation., Background: Interventional paediatric catheterisation is still burdened by a substantial risk. Risk factors, however, have rarely been investigated., Methods: We analysed prospectively 1,022 interventional procedures performed over a period of 8 years, excluding 260 procedures for atrial septostomy. We considered several patient-related variables, specifically age, weight, and gender, type of procedure, times required for fluoroscopy and the overall procedure, technical challenge, and the severity of the clinical condition. We also analysed variables linked to the environment, specifically the date of the examination, whether the operator remained in training, the novelty of the material, any breakdown in the installation, and errors made by the operator. We classified complications as those without clinical consequence, those which proved lethal, those requiring cardiopulmonary resuscitation, elective or emergency surgery, hospitalisation in the intensive care unit, and those leading to recatheterisation., Results: Our average incidence of complications was 4.1 per cent, which did not change significantly during the period of study. Of the patients, 4 died, 7 needed urgent surgery, 5 elective surgery, 3 hospitalisation in intensive care unit, and 8 recatheterisation. Independent risk factors for complications were technical challenge, critical clinical condition, operator in training, operator error, and breakdown of the installation. Young age was not associated with a higher risk of complications. Patients in whom no cause for complication could be found, either related to their own features or the environment, had a risk of complication of 1.4 per cent (95 per cent confidence intervals from 0.7 to 2.5 per cent)., Conclusions: Our data show that variables relating either to the patient or the environment of catheterisation are associated with an increased risk of procedural complications. Knowledge of the risk factors can improve the odds of paediatric interventional catheterisation.

We report our experience with an 8-year-old boy with complete atrioventricular block and syncopal bradycardia who required urgent pacing. Each attempt to cross the tricuspid valve with a femoral lead triggered ventricular standstill, followed by fibrillation, and pacing through the coronary sinus failed. Successful ventricular pacing was finally achieved through the oesophagus, allowing subsequent implantation of a transvenous pacemaker.

We report the interventional procedures performed on a 12-year-old child with obstruction of the right ventricular outflow tract, pulmonary valvar insufficiency, pulmonary arterial stenosis, and an atrial septal defect. A staged repair of all anomalies was performed successfully using transcatheter techniques.

A 53-year-old man with tetralogy of Fallot had been palliated with two classic Blalock-Taussig shunts. Cardiac catheterization in our center revealed acquired atresia of the pulmonary valve. We perforated and dilated the valve as a palliative procedure with a new system using radiofrequency energy.

Balloon angioplasty is now the elective technique for relief of aortic recoarctation, since it has low mortality, low morbidity, and good results at follow-up. Some concern exists concerning the possible increased risk in young children. To investigate such age-related aspects, we examined 58 children undergoing consecutive balloon angioplasty for postsurgical recoarctation. Of the children, 30 were younger and 28 older than 1 year. Recoarctation was more frequent with ventricular septal defect or other cardiac anomalies (p < 0.001). Systolic hypertension was present in 70% of children aged less than 1 year, but in only 32% of those older than 1 year (p < 0.001). The initial gradient was inversely related with the age at dilation (r = -0.28, p < 0.05), and correlated with systolic blood pressure (r = 0.81, p < 0.01). The procedure was successful in 87% of children older than, and 86% of those under 1 year. Age was not related with residual gradient, nor with the percentage increase of diameter of the site of stenosis. After balloon angioplasty, systolic hypertension was still present in 37% of children younger than 1 year, and in 25% of those older than 1 year (p < 0.05). Three complications occurred in children younger than 1 year, and 6 in those older (p < 0.05). Our results show, first, that recoarctation occurs earlier in the setting of complex disease, second that children suffering recoarctation at a younger age are more likely to be hypertensive, either before or after balloon angioplasty, third, that balloon angioplasty has the same rate of success in children below or above the age of one year, although the procedure still carries a not negligible risk. Finally, the procedure does not carry a higher risk for those children below the age of 1 year.