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Background Over the past twenty years, progress of cardiac surgery has been such that actual life expectancy of a patient with congenital heart disease (CHD) approaches that of the average for the general population. The current challenge is no longer to decrease cardiac surgery's mortality rate, but rather to manage morbidity and a child's overall development regarding nutrition and neurodevelopment. Malnutrition is a common cause of morbidity in infants with CHD, especially before the age of one. It can easily be explained by an increased metabolic demand linked to cardiac failure, hypoxemia or pulmonary hypertension. Secondly, food intake is frequently not optimal in this population and the infant's growth is insufficiently examined by paediatric cardiologists. Another factor is that much needed therapeutics such as diuretic drugs for example, often lead to weight loss. Ultimately, malnutrition in infants with CHD increases surgical risk, morbidity (infections, …), hospital stay, and alters long-term cognitive development. This study reports prevalence and associated factors of malnutrition on short and long-term follow-up of a large cohort of patients with various CHD, and who underwent cardiac surgery before the age of one. Method We conducted a retrospective and multicentre study that included infants from the North of France with CHD who underwent cardiac surgery before the age of one, between 2013 and 2017. To determine malnutrition, anthropometric measures (weight and height) were collected on the day of the surgery and during follow-up until 3 years after surgery to calculate Z-score for weight, height and weight/height. To test the statistical significance of the association between nutritional status and risk variables, univariate analysis was done applying Chi-square test. All variables significant at 0.1 in univariate analysis were included in the multivariate logistic regression analysis. The study was approved by French national ethic committee Commission national de l’informatique et des libertes (CNIL) and by the GFHGNP ethic committee (Groupe francophone d’hepatologie–gastro-enterologie et nutrition pediatrique). The author declares that he has no competing of interest. Result In total, 331 patients with CHD who underwent their first cardiac surgery (palliation or repair) before the age of one were included. Table 1 lists the cardiac diagnosis of patients included in the study. Demographic and clinical characteristics are summarised in Table 2 . Caesarean section was performed in 23.5% of cases. Only 38% infants were breastfed after maternity stay. An important part of our population was treated with diuretic drugs before surgery for congestive heart failure (38.5%). 69 (21%) infants needed a gastric tube before surgery. Feeding difficulties (including need for dietary enrichment and/or presence of a gastric tube) represented 35% of our population at the day of surgery. Only 39 (12%) were specifically assessed by speech therapist for feeding problems. Concerning surgery, 246 (74.5%) infants had undergone a biventricular repair, 51 (15.5%) infants had undergone a palliative intervention before a biventricular repair and 33 (10%) had a palliative intervention before a univentricular repair. 84 (26%) were cyanotic after their first surgery, 104 (32%) needed at least a second intervention. 49 (15%) infants had postoperative complications and 84 (26%) infants were treated by diuretics for congestive heart failure one month after surgery. 19 (5.9%) infants needed intravenous (IV) nutrition for more than 7 days. There were 19 (6%) deaths. Regarding nutritional status, at the time of the surgery, 14% of the infants presented moderate to severe growth failure (with a Z score weight for age During follow-up, between six months and 12 months after surgery, moderate to severe growth failure persisted in 16% of the study population and minor growth failure in 24%. In other terms, 40% of our cohort presented a persistent minor to severe growth failure. Fig. 1 , Fig. 2 summarize evolution of growth failure and malnutrition respectively during follow-up from 15 days to 3 years after surgery. Nineteen patients died during follow-up. A significant proportion of patients had impaired somatic growth or malnutrition at the end of the follow-up. Several associated factors of persistent growth failure 6 to 12 months after surgery were identified in univariate analysis: prenatal diagnosis, presence of genetic syndrome, birth weight ≤ 3 kg, complex CHD (CHD with 2 or more significative lesions or double outlet right ventricule or single ventricule physiology), surgery after 30 days from birth, and diuretic therapy before surgery and/or still necessary one month after surgery. The results of multivariate logistic regression analysis are summarized in Table 3 . CHD types were divided in 4 groups regarding anatomy and complexity, using TGA, Fallot and coarctation of Aorta as referent CHD with low impact on infant growth away from the day of surgery. The presence of a feeding tube at the day of surgery was not associated with growth failure 6 to 12 months after. Conclusion Growth failure in infants with CHD who undergo cardiac surgery before the age of one persists between 6 and 12 months after surgery. This should be taken into account as it can have serious consequences on children's health and development. Highlighting predictive factors will allow medical staff to identify vulnerable infants at birth and during the period of surgery and therefore increase nutritional care to combat malnutrition and growth failure.

Objectives This study sought to investigate patient intermediate-term outcomes after transcatheter pulmonary valve replacement (TPVR) with Edwards SAPIEN valve. Background The Edwards SAPIEN valve, initially designed for percutaneous aortic valve replacement, has been approved for TPVR in patients with dysfunctional right ventricular outflow tracts (RVOT), but only short-term follow-up has been reported. Methods From 2011 to 2016, 62 patients undergoing successful TPVR using the SAPIEN XT valve were consecutively included into the study. Primary efficacy and safety endpoints were defined as freedom from valve-reintervention and freedom from infective endocarditis at last follow-up, respectively. Results The primary efficacy outcome was met for 87.1% patients after a mean follow-up of 4.6 ± 1.8 years, corresponding to a freedom of reintervention at 5 years of 89% (95% CI 74.8-95.6%). Reinterventions were exclusively due to recurrent obstruction, no significant valvular regurgitation was observed. One case of infective endocarditis was reported, corresponding to a rate of 0.35% per patient-year (95% CI 0.01-2.00%). At 5 years, freedom from infective endocarditis was 98.4% (95% CI 89.1-99.8%). Six patients died or were transplanted due to advanced cardiac failure, without relationship with TPVR. In univariate analysis, reintervention was associated with young age, a smaller tube-graft, a higher pulmonary valve gradient after the procedure and a ratio of largest implanted stent diameter to invasive balloon conduit diameter over 1.35. Conclusions This study documents the mid-term safety and efficacy of the Edwards SAPIEN XT valve in patients with dysfunctional RVOT, and identifies a patient profile associated with an uncertain benefit-risk balance.

Aims To estimate the effect of prophylactic angiotensin-converting enzyme inhibitors (ACEi) on survival in Duchenne muscular dystrophy (DMD). Methods and results We analysed the data from the French multicentre DMD Heart Registry (ClinicalTrials.gov: NCT03443115). We estimated the association between the prophylactic prescription of ACEi and event-free survival in 668 patients aged 8 to 13 years, with normal left ventricular function, using (i) a Cox model with intervention as a time-dependent covariate, (ii) a propensity-based analysis comparing ACEi treatment vs. no treatment, and (iii) a set of sensitivity analyses. The study outcomes were overall survival and hospitalizations for heart failure (HF) or acute respiratory failure. Among the 668 patients included in the DMD Heart Registry, 576 (mean age 6.1 ± 2.8 years) were eligible for this study, of whom 390 were treated with ACEi prophylactically. Death occurred in 53 patients (13.5%) who were and 60 patients (32.3%) who were not treated prophylactically with ACEi, respectively. In a Cox model with intervention as a time-dependent variable, the hazard ratio (HR) associated with ACEi treatment was 0.49 [95% confidence interval (CI) 0.34–0.72] and 0.47 (95% CI 0.31–0.17) for overall mortality after adjustment for baseline variables. In the propensity-based analysis, 278 patients were included in the treatment group and 834 in the control group, with 18.5% and 30.4% 12-year estimated probability of death, respectively. ACEi were associated with a lower risk of death (HR 0.39; 95% CI 0.17–0.92) and hospitalization for HF (HR 0.16; 95% CI 0.04–0.62). All other sensitivity analyses yielded similar results. Conclusion Prophylactic ACEi treatment in DMD was associated with a significantly higher overall survival and lower rates of hospitalization for HF.

Summary Background Closure of patent foramen ovale is well-managed in adults, but is performed less frequently in children. Aim To analyse all patent foramen ovale closures performed in the past 20 years in French paediatric centres. Methods Retrospective study of patent foramen ovale closures in children without cardiopathy in nine centres between 2000 and 2019. Results Forty-one procedures were carried out in children (median age: 14.9 years). Thirty-one patent foramen ovales were closed after a transient ischaemic attack or stroke, six for a left-to-right shunt and four for other reasons. Transthoracic echocardiography was used for 72.2% of the diagnoses and transoesophageal echocardiography for 27.8%. A substantial degree of shunting was found in 42.9% of patients and an atrial septal aneurysm in 56.2%. General anaesthesia with transoesophageal echocardiography guidance was performed in 68.3% of the procedures; local anaesthesia and transthoracic echocardiography or intracardiac echocardiography was performed in 31.7%. The success rate was 100%. The median fluoroscopy time was 4.14 minutes: 3.55 minutes with transoesophageal echocardiography; and 4.38 minutes with transthoracic echocardiography (P = 0.67). There was only one periprocedural complication (2.4%). Postoperatively, 80,5% of patients were treated with aspirin and 12,2% with an anticoagulant. The rate of complete occlusion was 56.8% immediately after the procedure, 68.6% at 1 year and 92.3% at the last follow-up. There were no delayed complications or cases of recurrent stroke during follow-up (median follow-up: 568 days). Conclusion Closure of patent foramen ovale in children appears to be safe and effective, as we noted a low rate of immediate complications, no delayed complications and no stroke recurrence in this indication.

We report a case of antegrade transcatheter occlusion of a paravalvular leak (PVL) after bioprosthetic pulmonary valve replacement in a 31-year-old patient with tetralogy of Fallot. The leak resulted in severe pulmonary regurgitation with right ventricle volume overload. This case is the first-ever report of a successful percutaneous closure of PVL using the Occlutech paravalvular leak device.

Minimally invasive cardiac surgery is mainly dedicated to acquired left-sided valve diseases. Ministernotomy is widely used for aortic valve repair or replacement, whereas pulmonary valve repair via this approach has been reported only recently. This article aims to describe the use of ministernotomy for pulmonary valve replacement in adult congenital patients.

Background Percutaneous treatment of native coarctation (coA), gold standard in adult patients, is still debated in children. We aim to report the French multicentre experience. Methods All patients aged 1 to 18 yrs with native coA, treated percutaneously between 01/2000 and 12/2018, were included in this multcentric (9 centres) retrospective survey. Results One hundred and thirty three children (34 female) were included. Indication for treatment was hypertension in 109, exercise hypertension in 8 and other reasons in 16 patients. Mean age at intervention was 11.5 ± 3.9 years, mean weight 41 ± 18 kg. Procedure consisted of isolated balloon dilatation in 5 (mean age 2,63 yrs, mean weight 12 kg), balloon dilatation followed by stent implantation in 8 (mean age 11 yrs, mean weight 37 kg), and immediate stent implantation in 120 (mean age 12 yrs, mean weight 43 kg). Stents used (n = 124/128) were covered in 43, non-covered in 77, including 38 open cell stents. Invasive gradient across coA dropped from 28 mmHg (range 5 to 64) at baseline (n = 124) to 3 mmHg (range -5 to 20) after intervention (n = 105). Three associated lesions were treated: 1 PDA closure, 1 aortic valvuloplasty and 1 left SVC occlusion. Serious vascular complications occurred in 8 patients (6%) (3 pseudo-aneurysms, 2 dissections, 2 stenosis and 1 occlusion) requiring surgery in 2. Reintervention was performed in 22%: 2 patients had surgery, 28 were treated percutaneously (14 balloon dilatations, 14 stent implantations). Mean follow-up reached 4.9 ± 4.7 years. At latest follow-up, 25% remained hypertensive with 15 patients (11%) on anti-hypertensive medication. MRI, CTscan or fluoroscopy imaging, are available in 52 patients during follow-up (39%). Rate of aneurysm formation, stent fracture and risks factors for reintervention or residual hypertension will be presented. Conclusions Percutaneous treatment of native coarctation was safe and efficient in our french population. Close follow-up is required for longer-term results.

Objectives This study sought to investigate patient intermediate-term outcomes after transcatheter pulmonary valve replacement (TPVR) with Edwards SAPIEN valve. Background The Edwards SAPIEN valve, initially designed for percutaneous aortic valve replacement, has been approved for TPVR in patients with dysfunctional right ventricular outflow tracts (RVOT), but only short-term follow-up has been reported. Methods From 2011 to 2016, 62 patients undergoing successful TPVR using the SAPIEN XT valve were consecutively included into the study. Primary efficacy and safety endpoints were defined as freedom from valve-reintervention and freedom from infective endocarditis at last follow-up, respectively. Results The primary efficacy outcome was met for 87,1% patients after a mean follow-up of 55 ± 21 months, corresponding to a freedom of reintervention at 5 years of 89% (95% CI 74.8–95.6%). Reinterventions were exclusively due to recurrent obstruction, no significant valvular insufficiency was observed. One case of infective endocarditis was reported, corresponding to a rate of 0.35% per patient-year (95% CI 0.01–2.00%). At 5 years freedom from infective endocarditis was 98.4% (95% CI 89.1–99.8%) ( Fig. 1 ). Six patients died or were transplanted due to advanced cardiac failure, without relationship with TPVR. In univariate analysis, reintervention was associated with young age, a smaller tube-graft, a higher pulmonary valve gradient after the procedure and a ratio of largest implanted stent diameter to invasive balloon conduit diameter over 1.35. Conclusions This study confirms the mid-term safety and efficacy of the Edwards SAPIEN XT valve in patients with dysfunctional RVOT, and identifies a patient profile associated with an uncertain benefit-risk balance.

The long-term prospective multi-centre nationwide (French) observational study FRANCISCO will provide new information on perimembranous ventricular septal defect with left ventricular overload but no pulmonary hypertension in children older than 1 year. Outcomes will be compared according to treatment strategy (watchful waiting, surgical closure, or percutaneous closure) and anatomic features of the defect. The results are expected to provide additional guidance about the optimal treatment of this specific population, which is unclear at present. Background The management of paediatric isolated perimembranous ventricular septal defect (pmVSD) with left ventricle (LV) volume overload but no pulmonary arterial hypertension (PAH) remains controversial. Three therapeutic approaches are considered: watchful waiting, surgical closure, and percutaneous closure. We aim to investigate the long-term outcomes of these patients according to anatomic pmVSD characteristics and treatment strategy. Methods The Filiale de Cardiologie Pediatrique et Congenitale (FCPC) designed the FRANCISCO registry, a long-term prospective nationwide multi-centre observational cohort study sponsored by the French Society of Cardiology, which enrolled, over 2 years (2018–2020), patients older than 1 year who had isolated pmVSD with LV volume overload. Prevalent complications related to pmVSD at baseline were exclusion criteria. Clinical, echocardiographic, and functional data will be collected at inclusion then after 1, 5, and 10 years. A core lab will analyse all baseline echocardiographic data to depict anatomical pmVSD features. The primary outcome is the 5-year incidence of cardiovascular events (infective endocarditis, sub-aortic stenosis, aortic regurgitation, right ventricular outflow tract stenosis, tricuspid regurgitation, PAH, arrhythmia, stroke, haemolysis, heart failure, or death from a cardiovascular event). We plan to enrol 200 patients, given the 10% estimated 5-year incidence of cardiovascular events with a 95% confidence interval of ±5%. Associations linking anatomical pmVSD features and treatment strategy to the incidence of complications will be assessed. Conclusions The FRANSCICO study will provide the long-term incidence of complications in patients older than 1 year with pmVSD and LV volume overload. The results are expected to improve guidance for treatment decisions.

International audience; Background The COCCINELLE study is a nationwide retrospective French cohort set up to evaluate the risk of radiation associated cancer in patients who undergone cardiac catheterization (CC) procedures for diagnosis or treatment of congenital heart disease during childhood [1].Material and methods Children who undergone CC procedures from 01/01/2000 to 31/12/2013 before the age of 16 in one of the 15 pediatric cardiology departments which perform pediatric CC in mainland France were included. The follow-up started at the date of the first recorded CC procedure until the exit date, i.e. the date of death, the date of first cancer diagnosis, the date of the 18th birthday, or the 31/12/2015, whichever occurred first. The cohort was linked to the National Childhood Cancer Registry to identify patients diagnosed with cancer and with the French National Directory for the Identification of Natural Persons to retrieve the patients’ vital status. An external comparison was conducted using standardized incidence ratios (SIR). Breslow and Day’s approximation was used to estimate 95% confidence intervals (CI) for the SIRs [2]. Results A total of 17,104 children were included in the cohort and followed for 110,335 person-years, with 22,227 CC procedures collected. Among the patients, 81.6 % received only one procedure. Fifty-nine cancer cases were observed in the cohort. SIRs were increased for all-cancer (SIR = 3.8, 95% CI 2.9, 4.9), leukemia (SIR = 3.3, 95% CI 2.0, 5.4), lymphoma (SIR = 14.9, 95% CI 9.9, 22.5) and solid cancers excluding central nervous system tumors (SIR = 3.3, 95% CI 2.0, 5.5) compared with the general population. Conclusion Increased risks of cancer were observed. Potential explanations include shared genetic or environmental factors, immunosuppression drugs, and exposure to medical ionizing radiation procedures. The dose-response analysis between ionizing radiation doses received during CCs and cancer occurrence will allow to address this question.Keywords: Ionizing radiation, cardiac catheterization, congenital heart disease, cancerCompeting interestsThe authors declare that they have no competing of interest.References1.Baysson H, Nkoumazok B, Barnaoui S, Réhel J, Girodon B, Milani G, Boudjemline Y, Bonnet D, Laurier D, Bernier M (2015) Follow-up of children exposed to ionising radiation from cardiac catheterisation: the Coccinelle study. Radiation protection dosimetry 165:13–162.Breslow NE, Day NE (1987) Statistical Methods in Cancer Research Volume II: The Design and Analysis of Cohort Studies, IARC Scientific Publication. IARC Scientific Publication No. 82, Lyon, France: International Agency for Research on Cancer.NotesCOCCINELLE: French acronym for COhorte sur le risque de Cancer après Cardiologie INterventionnELLECC: Cardiac CatheterizationSIR: Standardized Incidence Ratios CI: Confidence IntervalsCNS: Central Nervous System

The aim of this study was to evaluate the frequency of neurodevelopmental disorders (NDD) in children with significant congenital heart disease (CHD) and to determine associated factors to NDD and frequency of follow-up in developmental therapies. Two hundred and ten children with significant CHD aged from 6 to 66 months were enrolled over a period of six months. The AgesStages Questionnaire Third Edition in French (ASQ-3) was used to assess neurodevelopmental domains. NDD were defined if cut-off scores were ≤ - 1SD. - 1SD corresponded to "Monitor" range: children with minor or emerging disorders; - 2SD corresponded to "Refer" range: children exhibiting neurodevelopmental delays. Forty children were in "Monitor" range and 86 in "Refer" range. NDD rate was 60.0% (n = 126, 95% CI, 53.4 to 66.6%). There was no difference regarding CHD severity (p = 0.99). Only the presence of non-cardiac disease (OR = 2.14; 95% CI, 1.11 to 4.20) was associated with NDD. Forty-six children with NDD had no developmental follow-up (among them 21 were in "Refer" range (10%)) despite this being available.Conclusion: Children with significant CHD are at risk for NDD regardless of CHD severity. Systematic and early monitoring in a specific care program is required. Barriers that prevent access of care must be identified.Trial registration: Neurodevelopmental Disorders in Children With Congenital Heart Disease. NeuroDis-CHD. NCT03360370. https://clinicaltrials.gov/ct2/show/NCT03360370 What is Known: • Children with CHD are at risk for neurodevelopmental disorders and behavioural problems impacting their social adaptation, academic achievements and quality of personal and family life even in adulthood. What is New: • Children with CHD are at risk for neurodevelopmental disorders regardless of the complexity of the CHD. • Even with the availability of appropriate developmental services, children with CHD are not correctly followed, highlighting the need of a specific program of care for a better outcome. Local barriers that prevent access of care of those children must be identified.

Between December 2014 and March 2021, 144 patients with aortic (Ao) or mitral (Mi) paravalvular leaks (PVLs) were enrolled at 21 sites in 10 countries. Safety data were available for 137 patients, who were included in the safety analysis fraction (SAF), 93 patients with Mi PVLs and 44 patients with Ao PVLs. The full analysis set (FAS) comprised 112 patients with available stratum (aortic/mitral leak) as well as baseline (BL), 180-day or later assessments (2 years). Procedural success (implantation of the device with a proper closure of the PVL, defined as reduction in paravalvular regurgitation of ≥one grade as assessed by echocardiography post implantation) was achieved in 91.3% of FAS patients with Mi PVLs and in 90.0% of those with Ao PVLs. The proportion of patients suffering from significant or severe heart failure (HF), classified as New York Heart Association (NYHA) class III/IV, decreased from 80% at baseline to 14.1% at 2-year follow-up (FAS). The proportion of FAS patients needing hemolysis-related blood transfusion decreased from 35.5% to 3.8% and from 8.1% to 0% in Mi patients and Ao patients, respectively. In total, 35 serious adverse events (SAEs) were reported in 27 patients (19.7%) of the SAF population. The SAEs considered possibly or probably related to the device included device embolization (three patients), residual leak (two patients) and vascular complication (one patient). During follow-up, 12/137 (8.8%) patients died, but none of the deaths was considered to be device-related. Patients implanted with the Occlutech Paravalvular Leak Device (PLD) showed long-lasting improvements in clinical parameters, including NYHA class and a reduced dependency on hemolysis-related blood transfusions.

Objectives This study sought to assess procedural characteristics, early clinical outcome, and long-term complications after transcatheter closure of atrial septal defect (ASD) in children. Background Transcatheter closure has become the preferred strategy in most cases of isolated secundum ASD. However, reported experience in the pediatric population is limited. Methods A 1998 to 2016 retrospective multicenter study was performed in 9 French tertiary institutions. All children who had an attempt of percutaneous ASD closure with an Amplatzer Septal Occluder were included. Results In 1,326 children (39% males; median age, 9 years [0.7 to 18]; weight, 29 kg [3.6 to 92]), transcatheter ASD closure was performed. Median ASD size was 15 mm (3 to 41); 254 (19.1%) patients had a large ASD (≥20 mm/m2). Procedural success rate was 95.3% (95% confidence interval: 93.9% to 96.3%). No death was observed but periprocedural complications occurred in 24 patients (1.8%). After a median follow-up of 3.5 years (range 6 months to 18 years; 173 patients [13%] followed >10 years), delayed major complications were minimal (n = 12; 1.04%) including no death and/or cardiac erosion. Periprocedural and delayed complications rates were significantly higher in children ≤15 kg (5.2% vs. 1.5%; p = 0.007 and 3.1% vs. 0.7%; p Conclusions Transcatheter ASD closure using Amplatzer Septal Occluder is safe in children with a minimal rate of periprocedural complications and a favorable long-term outcome, especially with no death or cardiac erosion despite a substantial proportion of large defects. Children ≤15 kg and those with large ASDs had a greater risk of complications.

Background Epidemiologic of infective endocarditis (IE) is changing and paediatric population is heterogeneous. We compare clinical characteristics according cardiac status among children. Methods A retrospective multi centric study was conducted over a decade. Clinical presentation, treatment, outcome, microorganism, underlying congenital heart disease data were recorded. Results We identified 64 cases of definite IE between January 2008 to December 2018 median age was 8.0 [0.8–13.2] and 26 were girls, S aureus was the most common pathogen, n = 22 (34%), presented in Table 1 . CHD represented 45 cases of IE and 38 patients had prior cardiac surgery, they were significantly younger and community acquired IE was more common. New cardiac murmur 11 (24%) vs. 11 (58%), P Conclusion CHD patients seem older, require more valve replacement and present community acquired infection when patients without CHD presented more neurological outcome and more hospital acquired infection.

Tetralogy of Fallot is a frequent congenital heart disease that has been repaired since the mid-1950s. The follow-up after repair is good despite a persistent risk of sudden death. The risk factors in long-term follow-up are advanced age at repair, hemodynamic status of the right ventricle, QRS duration≥180ms, left ventricular dysfunction, and existence of sustained or not ventricular tachycardia. In the presence of significant pulmonary regurgitation, it is necessary to perform revalvulation either by classic surgery or cardiac catheterization. To correct the risk of ventricular arrhythmia, some have proposed radiofrequency ablation of critical isthmus, or cryo-application during surgery. However, the use of implantable cardioverter defibrillator is another therapeutic option that is more and more employed as secondary or primary prevention in patients at risk of sudden death.

Interventional cardiac catheterization has a major place in the management of congenital heart disease. Since the Rashkind atrioseptostomy in mid-1960s, many techniques have been developed. For some, it is necessary to close a cardiac or extracardiac shunt using occluder (double disc system, plug, coil…): closure of atrial septal defect, ventricular septal defect or patent arterial duct. For others, it is necessary to treat a valvular or vascular stenosis using a balloon catheter: dilatation of the pulmonary or the aortic valve, dilatation of aortic coarctation. For vascular stenosis, balloon angioplasty may be associated with stent implantation. Moreover, since more than 10 years, valve implantation can be performed: initially for pulmonic valve (the Melody™ valve from Medtronic or the Sapien™ valve from Edwards Lifesciences); but probably, most of the valves in the future could be implanted using appropriate tools and hybrid techniques combining cardiac catheterization and surgery. All these techniques were developed because of progress in fluoroscopy, and more recently association of different imaging techniques (echocardiography, MRI and CT) provides more information about the true anatomy. Interventional cardiac catheterization will continue to increase with use of new tools as 3D printing, tissue engineering and nano-techniques. It seems that from correction with open-heart surgery, many lesions could be repaired in future by hybrid techniques without opening the heart.

Summary Background Transcatheter perimembranous ventricular septal defect (pmVSD) closure remains challenging and is seldom used in France given the risk of atrioventricular block (AVB). pmVSD closure with the Nit-Occlud Le VSD coil was recently introduced in France as an alternative to occluder devices. Aims To study the safety and feasibility of pmVSD closure with the Nit-Occlud Le VSD coil. Methods All consecutives cases of pmVSD closure with the Nit-Occlud Le VSD coil in 20 tertiary French centres were included between January 2015 and December 2018. Results Among 46 procedures in five centres, indications for pmVSD closure were left ventricle overload (76.1%), exertional dyspnoea (17.4%), history of infective endocarditis (4.3%) and mild pulmonary hypertension (2.2%). The median (interquartile [IQR]) age of the patients was 13.9 (5.7–31.8) years. Aneurismal tissue was identified in 91.3% of patients. VSD median (IQR) size was 8 (7–10) mm on the left ventricle side and 5 (4–6) mm on the right ventricle side. Implantation was successful in 40 patients (87.0%; 95% confidence interval [CI] 73.7–95.1%). Severe complications occurred in six patients (13.0%, 95% CI 4.9–26.3%), mainly severe haemolysis (8.7%, 95% CI 2.4–20.8%). One aortic valve lesion required surgical aortic valvuloplasty. Occurrence of severe complications was significantly related to the presence of haemolysis (P = 0.001), residual shunt (P = 0.007) and multi-exit VSD (P = 0.005). Residual shunt was observed in 40% of cases with the implanted device shortly after closure and 15% after a median follow-up of 27 months. No immediate or delayed device embolization or complete AVB was recorded. Conclusion pmVSD closure with the Nit-Occlud Le VSD Coil is feasible in older children and adults. However, residual shunting (leading to haemolysis) is a dreaded complication that should not be tolerated. pmVSD closure with the Nit-Occlud Le VSD as a therapeutic strategy remains controversial and is limited to selected patients.

Decision making for univentricular or anatomical repair remains challenging in children with borderline left ventricle. Unpredictable outcomes have led many caregivers to pursue a single-ventricle strategy. We describe 2 cases of patients with borderline left ventricle initially palliated with univentricular strategy followed by very late conversion to anatomical repair 4 and 16 years later. Anatomical conversion should be considered for these patients even many years later. During the first palliation stages, hemodynamic conditions preserving the potential for growth of the left ventricle should be maintained.

The incidence of diastolic heart failure has increased over time. The evaluation of left-ventricular diastolic function is complex, ongoing, and remains poorly performed in pediatric intensive-care patients. This study aimed to review the literature and to provide an update on the evaluation of left-ventricular diastolic function in adults and children in intensive care. We searched data from PubMed/Medline. Thirty-two studies were included. Four pragmatic questions were identified: (1) What is the physiopathology of diastolic dysfunction? (2) Which tools are required to evaluate diastolic function? (3) What are the echocardiographic criteria needed to evaluate diastolic function? (4) When should diastolic function be evaluated in pediatric intensive care? Early diastole allows characterization of relaxation, whereas compliance assessments and filling pressures are evaluated during late diastole. The evolution of diastolic function differs between adults and children. Unlike in adults, decreased compliance occurs at the same time as delayed relaxation in children. Diastolic function can be evaluated by Doppler echocardiography. The echocardiographic criteria for ventricular relaxation include the E wave, E/A wave ratio, and isovolumic relaxation time. Ventricular compliance can be assessed by the E/e’ wave ratio, atrial volume, and Ap wave duration during pulmonary vein flow. In adult intensive-care patients, the E/e’ ratio can be used as an index of tolerance for volume expansion in septic patients and to adjust the inotropic support. Clinical studies would allow some of these parameters to be validated for use in children in intensive care.

BackgroundThe Occlutech Figulla ASD device series (OFSO) shows an improved device design for interventional ASD closure, larger follow-up series are missing. MethodsWe retrospectively reviewed the feasibility, safety, implantation properties, results, and follow-up of ASD closure using Occlutech devices over a 5 year period by establishing a multi-institutional collaborative result registry with 16 contributing centers from 11 countries (IRFACODE). ResultsIn 1315 patients of all age groups (female 66.9%), successful (98%) ASD closure was performed (mean age 28.9 years, weight 52 kg, height 148.6 cm). Of the defects, 47.9% showed no or only a deficient aortic rim;in 11.9%, there was more than one defect;a septum aneurysm was present in 21.5%;and the mean implanted device size was 20.5 mm. Immediate closure was achieved in 78.6%, at discharge in 83.1%, and 96.4% and 97.3% at 6 and 12 months follow-up, respectively. During a mean follow-up of 2.7 years (in total 3597 patient years), significant complications were minimal (total=8

We report the case of a 14-year-old boy with severe protein-losing enteropathy after Fontan surgery that led to lymphangiectasia, which caused gastrointestinal haemorrhage and required invasive treatment to stop the bleeding. Through this case and a review of the literature on protein-losing enteropathy after Fontan surgery, we highlight a rare and serious presentation of the disease and the difficulties of diagnosis and management.

Background The Coaguchek INRange® is a self-testing meter to measure the INR, that is reimbursed in France since August 2017 for patients with mechanical valve. Methods We evaluated the use of this anticoagulation method in a French population of GUCH (Grown-Up patients with Congenital Heart Disease) with mechanical valve. Results Since September 2018, 52 patients of 37 ± 11 years old were asked to attend a training course of 2 hours on anticoagulation and Coaguchek INRange® use, provided by specialized nurses, before getting home with the device. Patients had to attend a 3 months’ medical re-evaluation appointment. 29 (56%) patients had an aortic mechanical valve, 12 (23%) a mitral one, 8 (15%) a double aortic and mitral one, 2 (4%) a double aortic and pulmonary one and 1 (2%) a tricuspid one. 28 patients (54%) had a mechanical valve for ≥ 10 years (group 1). In group 1, patients were older (41 ± 10 years old vs. 34 ± 10, P = 0.01). Fluindione was the preferred oral anticoagulant (30 patients, 57%), and was more frequent in group 1 (18 patients, 64%). In group 1, 61% of patients usually managed themselves dosage adjustment whereas in the other group, 52% of patients referred to their doctor. Thirty-one patients (62%) had a higher target of INR than recommended in last European guidelines. Concerning follow-up, 26 patients (50%) did not attend the 3 months appointment and had to be rescheduled or contacted by phone. 8 patients (15%) did not use the device at 3 months: 5 for variation > 15% compared to laboratory plasma technique and 3 for not trusting the new device. INR at 3 months was obtained in only 40 (77%) patients, and was in the attended target in 62% of cases. Conclusion GUCH patients with mechanical valves, especially when present for ≥ 10 years, seem to validate Coaguchek, even though evaluation remains hard since a great proportion of them did not attend follow-up appointment, stick to their old INR target and did not use the device as often as recommended.

Objectives The short-term efficacy and safety of transcatheter closure (TCC) for the management of coronary artery fistulas (CAFs) was investigated in pediatric patients. Background CAFS are rare with potentially severe complications and their management is still a matter of debate. Percutaneous closure appears to be the treatment of choice in anatomically suitable vessels but limited data are available in the pediatric population. Methods This retrospective, observational, multicenter, national study included patients under 16 years of age who underwent TCC of a congenital CAF. Patients with additional congenital heart defect were excluded. Results 61 patients (36 girls, 25 boys) with a median age of 0.6 year [0–15.4] at diagnosis and 3.9 years [0–16] at procedure were included. The CAF was large in 48 patients (79%); it was distal in 23 (38%) and proximal in 22 (36%). Most patients (77%) were asymptomatic at diagnosis. Clinical signs of congestive heart failure were present in seven patients (11%). Perioperative complications included three cases of ST elevation myocardial infarction (exclusively during attempted closure of a distal CAF), three devices migrations, and one case of leg ischemia. One patient died after surgical closure of a large distal CAF that could not be closed by TCC. Follow-up data were collected for 43 patients (70%) for a median of 91 days [0–4,824]. The Kaplan-Meyer estimate for complete occlusion at 2 years was 73 ± 7.6%. Conclusion TCC in the pediatric population appears to be effective and associated with few complications. © 2015 Wiley Periodicals, Inc.

The aim of this study was to describe and analyze data from patients treated in France with the Edwards SAPIEN transcatheter heart valve (Edwards Lifesciences LLC, Irvine, California) in the pulmonary position.The Edwards SAPIEN valve has recently been introduced for percutaneous pulmonary valve implantation (PPVI).From April 2011 to May 2017, 71 patients undergoing PPVI were consecutively included.The median age at PPVI was 26.8 years (range 12.8 to 70.1 years). Primary underlying diagnoses were conotruncal malformations (common arterial trunk, tetralogy of Fallot and variants; n = 45), Ross procedure (n = 18), and other diagnoses (n = 8). PPVI indication was pure stenosis in 33.8% of patients, pure regurgitation in 28.1%, and mixed lesions in 38.1%. PPVI was successfully implemented in 68 patients (95.8%). Pre-stenting of the right ventricular outflow tract was performed in 70 patients (98.6%). Early major complications occurred in 4 subjects (5.6%), including 1 death, 1 coronary compression, and 2 pulmonary valve embolizations. Three of the 4 major complications occurred in the first 15 operated patients. No significant regurgitation was recorded after the procedure. Transpulmonary gradient was significantly reduced from 34.5 to 10.5 mm Hg (p 0.0001). No patient died during a 1-month follow-up period. At 1-year follow-up, the death rate was 2.9%, and 3 patients had undergone surgical reintervention (44%).Early results with the Edwards SAPIEN valve in the pulmonary position demonstrate an ongoing high rate of procedural success.

This study sought to assess procedural characteristics, early clinical outcome, and long-term complications after transcatheter closure of atrial septal defect (ASD) in children.Transcatheter closure has become the preferred strategy in most cases of isolated secundum ASD. However, reported experience in the pediatric population is limited.A 1998 to 2016 retrospective multicenter study was performed in 9 French tertiary institutions. All children who had an attempt of percutaneous ASD closure with an Amplatzer Septal Occluder were included.In 1,326 children (39% males; median age, 9 years [0.7 to 18]; weight, 29 kg [3.6 to 92]), transcatheter ASD closure was performed. Median ASD size was 15 mm (3 to 41); 254 (19.1%) patients had a large ASD (≥20 mm/mTranscatheter ASD closure using Amplatzer Septal Occluder is safe in children with a minimal rate of periprocedural complications and a favorable long-term outcome, especially with no death or cardiac erosion despite a substantial proportion of large defects. Children ≤15 kg and those with large ASDs had a greater risk of complications.

Background Congenital heart disease (CHD) represents a predisposing condition for the development of infective endocarditis (IE). In recent years, the reduction in the incidence of rheumatic heart disease, advances in cardiac surgery and the increased use of long-term central venous catheters (CVC) in subjects without CHD have changed significantly the epidemiology of the disease. We sought to evaluate the epidemiology of IE in children in the north of France. Method We retrospectively included all children Results We identified 64 episodes of IE in 60 children. Incidence was 3,07/10 000 paediatric hospitalisations. Median age was 8 years [0–13] and distribution was bimodal with peaks in infancy and adolescence. An organism pathogen was isolated in 87% of cases. The most common was Staphylococcus aureus (34%) then streptococcus (25%). At echocardiography, anomalies were vegetations (n = 32), new valvular regurgitations (n = 10), peri valvular abscesses (n = 2). Among cohort, 45 had a CHD (70%), 7 were pre-term birth (11%), 2 had long-term CVCs and 10 no predisposition. Non-CHD patients were significantly younger than CHD patients ( Table 1 ). Anomalies at echocardiography and identification of pathogen were more common in non-CHD patients. Among CHD patients, 38 (84%) had previous heart surgery and 10 presented an IE Conclusion IE mostly occurred in predisposed children. IE in CHD and non-CHD patients has different features, diagnosis is more arduous and needs more investigation as nuclear medicine imaging.

Background Congenitally corrected transposition of the great arteries (ccTGA) is rare congenital heart disease (CHD) with an estimated prevalence of less than 1% among all CHDs. Long time prognosis is dependent on the systemic right ventricule function, presence of heart block and surgical correction. Methods In this retrospective study, all cases of ccTGA from 2 tertiary centers (one university hospital, one private pratice) were analyzed starting from 1980. Diagnosis circumstances, associated lesions, apparition of conduction blockage, surgery (anatomical versus arterial switch), systemic ventricular dysfunction, cardiac magnetic Imaging (CMR) and cardiopulmonary exercise testing (CET) were retrieved from patient records. Results Eighty-two patients were included with a mean follow up time of 23.3 years (Interquartile range (IQR) = 25.6 years). Survival rate was 99% CI95% [96–100] at 5 years, 97% CI95% [93–100] at 10 years and 90% CI95% [81–98] at 20 years. Patients had complete heart block after a mean time of 9.7 years (IQR 14.3 years) with a prevalence of 28% CI95% [8.4–44] at 30 years. Patients with systemic right ventricule had heart failure after a mean time of 52.7 years CI95% [42.6–100]. The ejection fraction of the systemic right ventricule based on CMR was correlated with VO2 uptake (ρ=0.49, P = 0.05) at CET. Conclusion Lifetime follow-up is necessary for the diagnosis of complications of ccTGA, specifically complete heart block and systemic right ventricle dysfonction. Right heart ejection fraction with CMR is correlated and could be used as a surrogate of peak VO2 uptake at CET.

Objectives Kawasaki Disease (KD) is a child vasculitis. The prognosis is associated with a higher risk of coronary artery aneurysm (CAA). Currently the main goal of treatment consists of preventing CAA. At first the treatment consists on immunoglobulin (IVIG). The last American 2016 guidelines recommends echographical coronary diameter express as z-score. The epidemiology of KD is not well known in France. The aim of this study was to describe the population of the children in a region of North of France and to look for risk factors of CAA. Methods We included patients with KD who were admitted in hospital centers of the region from 2006 to 2016. We reviewed retrospectively the medical, biological and echographical records and their monitoring data. We compared patients in group with and without CAA at 4 weeks from the diagnosis. Results We included 240 children from 6 hospital centers. The median age was 28 months (14–50), 20% were less than 1 year old. The male-to-female ratio was 1.8. Diagnosis was done after 7 days of fever at mean. We found 87 children with initial z-score ≥ 2DS and 28 with CAA. Patients were treated with IVIG and 35 get more than one cure, 95% get aspirin for anti-inflammatory then 87% as antiplatelet therapy. Five received corticosteroids, 1 an anti-TNFα and 1 an anti-IL1. Risk scores of CAA from Kobayashi, Egami and Sano present low sensitivity and low specificity. Several risk factors were associated with CAA: age Conclusion The Japanese risk scores were not significant in the French population, as previously shown in North American or English populations. The initial z-score ≥ 2 DS is a good risk factor of CAA so is the Pq ≥ 444 G/l after the day 7th of fever in our population.

The authors describe a case of Kawasaki disease who presented fever, coronary artery aneurysm, and ischaemic intestinal obstruction requiring stage resections. A review of literature of such association is performed. Atypical Kawasaki disease is more complex to diagnose and in combination of persistent fever with no definite cause and surgical abdominal pain, Kawasaki disease should be considered in the differential diagnosis.

International audience; Background: The relationship between pulmonary arterial hypertension-specific drug therapy (PAH-SDT) and mortality in Eisenmenger syndrome (ES) is controversial.Aims: To investigate outcomes in patients with ES, and their relationship with PAH-SDT.Methods: Retrospective, observational, nationwide, multicentre cohort study.Results: We included 340 patients with ES: genetic syndrome (n = 119; 35.3%); pretricuspid defect (n = 75; 22.1%). Overall, 276 (81.2%) patients received PAH-SDT: monotherapy (endothelin receptor antagonist [ERA] or phosphodiesterase 5 inhibitor [PDE5I]) 46.7%; dual therapy (ERA + PDE5I) 40.9%; triple therapy (ERA + PDE5I + prostanoid) 9.1%. Median PAH-SDT duration was 5.5 years [3.0–9.1 years]. Events (death, lung or heart-lung transplantation) occurred in 95 (27.9%) patients at a median age of 40.5 years [29.4–47.6]. The cumulative occurrence of events was 16.7% [95% confidence interval 12.8–21.6%] and 46.4% [95% confidence interval 38.2–55.4%] at age 40 and 60 years, respectively. With age at evaluation or time since PAH diagnosis as time scales, cumulative occurrence of events was lower in patients taking one or two PAH-SDTs (P = 0.0001 and P = 0.004, respectively), with the largest differences in the post-tricuspid defect subgroup (P < 0.001 and P < 0.02, respectively) versus patients without PAH-SDT. By multivariable Cox analysis, with time since PAH diagnosis as time scale, New York Heart Association/World Health Organization functional class III/IV, lower peripheral arterial oxygen saturation and pretricuspid defect were associated with a higher risk of events (P = 0.002, P = 0.01 and P = 0.04, respectively), and one or two PAH-SDTs with a lower risk of events (P = 0.009).Conclusions: Outcomes are poor in ES, but seem better with PAH-SDT. ES with pretricuspid defects has worse outcomes despite the delayed disease onset.; Contexte: L’intérêt du traitement médical spécifique (TMS) de l’hypertension artérielle pulmonaire (HTAP) dans le syndrome d’Eisenmenger (SE) est controversé.Objectifs: Étudier le pronostic à long terme des patients ayant un SE et la relation avec le TMS.Méthodes: Une cohorte observationnelle longitudinale multicentrique rétrospective historique française de 340 SE a été constituée.Résultats: Le shunt était prétricuspide dans 75 cas (22,1 %). Au total, 276 (81,2 %) patients étaient sous TMS (monothérapie 46,7 % ; bi-thérapie 40,9 % ; tri-thérapie 9,1 %). La durée médiane de TMS était de 5,5 ans [3,0–9,1]. Un événement clinique majeur (ECM : décès, transplantation cardiopulmonaire ou bipulmonaire) a été observé dans 95 (27,9 %) cas à un âge médian de 40,5 [29,4–47,6] ans. La survenue cumulée d’un ECM était de 16,7 % [IC 95 % 12,8–21,6 %] et 46,4 % [IC 95 % 38,2–55,4 %] à l’âge de 40 et 60 ans. Avec l’âge ou le délai depuis le premier examen comme échelle temporelle, la survenue cumulée des ECM était moindre chez les patients sous un ou deux TMS (p = 0,0001 et p = 0,004), en particulier chez les patients avec un shunt post-tricuspide (p < 0,001 et p < 0,02) comparée aux patients sans TMS. Une analyse multivariée de Cox avec le délai depuis le diagnostic de l’HTAP comme échelle temporelle a montré qu’une classe fonctionnelle III ou IV de la NYHA/WHO, une saturation périphérique en oxygène basse (en variable continue), un shunt pré-tricuspide et l’absence de TMS étaient associés à un risque augmenté d’ECM (p = 0,002 ; p = 0,01 ; p = 0,04 and p = 0,009, respectivement).Conclusions: Le TMS dans le SE semble associé à un meilleur pronostic. Néanmoins, même avec un traitement médical palliatif, le pronostic du SE reste altéré. Les patients avec un shunt prétricuspide ont un profil clinique et un pronostic plus sombre malgré une survenue plus tardive de l’HTAP.

We report the case of a 2-year-old boy with severe Langerhans cell histiocytosis who had tricuspid endocarditis caused by Staphylococcus lugdunensis and required surgery despite appropriate antimicrobial therapy. Through this case and literature review of endocarditis caused by S. lugdunensis in children, we highlight pitfalls and mistakes to be avoided in the management of this rare but serious infection.

Tetralogy of Fallot is a frequent congenital heart disease that has been repaired since the mid-1950s. The follow-up after repair is good despite a persistent risk of sudden death. The risk factors in long-term follow-up are advanced age at repair, hemodynamic status of the right ventricle, QRS duration≥180ms, left ventricular dysfunction, and existence of sustained or not ventricular tachycardia. In the presence of significant pulmonary regurgitation, it is necessary to perform revalvulation either by classic surgery or cardiac catheterization. To correct the risk of ventricular arrhythmia, some have proposed radiofrequency ablation of critical isthmus, or cryo-application during surgery. However, the use of implantable cardioverter defibrillator is another therapeutic option that is more and more employed as secondary or primary prevention in patients at risk of sudden death.

Interventional cardiac catheterization has a major place in the management of congenital heart disease. Since the Rashkind atrioseptostomy in mid-1960s, many techniques have been developed. For some, it is necessary to close a cardiac or extracardiac shunt using occluder (double disc system, plug, coil…): closure of atrial septal defect, ventricular septal defect or patent arterial duct. For others, it is necessary to treat a valvular or vascular stenosis using a balloon catheter: dilatation of the pulmonary or the aortic valve, dilatation of aortic coarctation. For vascular stenosis, balloon angioplasty may be associated with stent implantation. Moreover, since more than 10 years, valve implantation can be performed: initially for pulmonic valve (the Melody™ valve from Medtronic or the Sapien™ valve from Edwards Lifesciences); but probably, most of the valves in the future could be implanted using appropriate tools and hybrid techniques combining cardiac catheterization and surgery. All these techniques were developed because of progress in fluoroscopy, and more recently association of different imaging techniques (echocardiography, MRI and CT) provides more information about the true anatomy. Interventional cardiac catheterization will continue to increase with use of new tools as 3D printing, tissue engineering and nano-techniques. It seems that from correction with open-heart surgery, many lesions could be repaired in future by hybrid techniques without opening the heart.

Objective assessment of maximal aerobic capacity using peak oxygen consumption (peak VO2) can be helpful in the management of patients with asymptomatic aortic stenosis (AS). The relationship between peak VO2 and AS severity criteria derived from rest and supine exercise echocardiography (SEE) has never been explored.We aimed to determine whether low peak VO2 (85% of predicted value) is associated with severity parameters in SEE, and poor clinical outcome.Fifty one asymptomatic patients (mean age of 54±21years) with moderate to severe aortic stenosis (Vmax3m/s) and left ventricle ejection fraction50% prospectively underwent resting and SEE and cardiopulmonary exercise testing (CPX).Peak VO2 was lower than expected (21.9±7.4mL/kg/min), i.e.85% of predicted value in 57% patients, secondary to cardiac limitation in most of them (69%). In multiple regression analysis, age, BMI and female gender were the only independent determinants of peak VO2. Interestingly no parameter derived from SEE was associated with peak VO2. After 21±7month follow-up, no patient died, 20 underwent cardiac surgery. Peak VO285% of predicted value was associated with lower event free survival compared to normal peak VO2 (57%±11% vs 93±6%, p=0.036) whereas no exercise echocardiographic parameter could predict such events. Peak VO2≥85% had a negative predictive value of 97%.CPX detects a high proportion of false asymptomatic AS patients with poorer outcome that cannot be predicted by SEE markers of AS severity. Assessment of aerobic capacity should be part of current approach within a "watchful waiting" strategy.

Introduction Pulmonary hypertension (PH) affects 16–25% of premature infants with bronchopulmonary dysplasia (BPD), contributing significantly to perinatal morbidity and mortality. Inflammation is highly implicated in the pathogenesis of PH and BPD. Polyunsaturated fatty acids ω-3 (PUFA ω-3) can improve vascular remodeling, angiogenesis, and inflammation under pathophysiological conditions. However, the effects of PUFA ω-3 supplementation in BPD-associated PH are unknown. Objective Evaluate the effects of PUFA ω-3 on pulmonary vascular remodeling, angiogenesis, and inflammatory response in a hyperoxia-induced rat model of PH. Methods From embryonic day 15, pregnant Spague-Dawley rats were supplemented daily with PUFA ω-3, PUFA ω-6, or normal saline (0.2 ml/day). After birth, pups were pooled, assigned as 12 per litter, and randomly to either in air or continuous oxygen exposure (FiO2=85%) for 20 days, then sacrificed for pulmonary hemodynamic, morphometric, and immunohistological analysis. Results PUFA ω-3 supplementation improved survival, decreased right ventricular systolic pressure and RVH caused by hyperoxia, and significantly improved alveolarization, vascular remodeling, and vascular density. PUFA ω-3 supplementation produced a higher level of total ω-3 in lung tissue and breast milk, and was found reversing the reduced levels of VEGFA, VEGFR-2, ANGPT-1, TIE-2, eNOS, and no concentrations in lung tissue, and the increased ANGPT-2 levels in hyperoxia-exposed rats. The beneficial effects of PUFA ω-3 in improving lung injuries were also associated with an inhibition of leukocyte infiltration, and reduced expression of proinflammatory cytokines IL-1β, IL-6 and TNF-α. Conclusion These data indicated that maternal PUFA ω-3 supplementation strategies could effectively protect against infant PH induced by hyperoxia.

Introduction Fontan patients can develop veno-venous shunts with time, which are usually closed percutaneously when they reduce oxygen saturation. But the significance of these shunts regarding the type of congenital heart disease or surgical repair and their clinical relevance is not well known. Objective The purpose of this study was to evaluate the proportion of Fontan patients presenting veno-venous shunt and to evaluate the impact on aerobic capacity. Results Twenty-six Fontan patients (mean age 23 ± 9 years) who underwent cardiopulmonary exercise testing with blood gas measurement between January 2016 and January 2018 were included. Twenty patients had total cavopulmonary connection with extracardiac conduit surgery and 6 had atriopulmonary connection. Mean oxygen saturation at rest was 94.9 ± 2.1%. A resting shunt fraction, defined as PaO2 + PaCO2 Conclusion The importance of shunt fraction in Fontan patients seems correlated to aerobic capacity. Further studies are needed to confirm these results. Whether, percutaneous closing of veno-venous shunts in Fontan patients can reduce this shunt fraction and could improve maximal oxygen consumption still remains to be proved.

RésuméJustificationLes données décrivant l’hypertension artérielle pulmonaire chez l’enfant sont peu nombreuses.ObjectifsL’objectif a été de caractériser l’épidémiologie, la prise en charge, l’impact sur la qualité de vie et les conséquences de l’hypertension artérielle pulmonaire de l’enfant en excluant les patients avec une hypertension artérielle pulmonaire persistante du nouveau-né et ceux dont l’hypertension artérielle pulmonaire était causée par une cardiopathie congénitale.MéthodesDans cette étude non interventionnelle multicentrique, des enfants atteints d’hypertension artérielle pulmonaire ont été inclus et suivis prospectivement pendant 2 ans dans 21 centres français. La classe fonctionnelle OMS, la distance de marche de 6 minutes, la qualité de vie (questionnaire CHQ-PF50), les paramètres hémodynamiques et les échodopplers réalisés ont été évalués.RésultatsCinquante enfants d’âge moyen de 8,9 ± 5,4 ans ont été inclus de mai 2005 à juin 2006. La prévalence de l’hypertension artérielle pulmonaire a été estimée à 3,7 cas/million. Les patients avaient une hypertension artérielle pulmonaire idiopathique (60 %), familiale (10 %), associée avec une cardiopathie congénitale qui n’était pas la cause de l’hypertension artérielle pulmonaire (24 %), associée à une connectivite (4 %) ou à une hypertension portale (2 %). Pendant le suivi, le nombre d’associations de médicaments spécifiques de l’hypertension artérielle pulmonaire prescrit a augmenté (44 % des patients contre 22 % à l’inclusion). La majorité des patients est restée stable en ce qui concerne l’état clinique, le test de marche de 6 minutes et la qualité de vie. La survie à 1 et 2 ans a été estimée à 86 % (intervalle de confiance à 95 % : [76, 96]) et 82 % (95 % intervalle de confiance : [71, 93]).ConclusionsChez l’enfant, dans la majorité des cas, les hypertensions artérielles pulmonaires sont idiopathiques ou familiales. Un groupe spécifique d’hypertension artérielle pulmonaire concomitante d’une cardiopathie congénitale a été identifié et ressemble à une hypertension artérielle pulmonaire idiopathique. Les associations de traitements spécifiques de l’HTAP peuvent avoir contribué à la stabilité de la maladie et à une meilleure survie.SummaryJustificationThere is few data describing pulmonary arterial hypertension in children.ObjectivesThe objective was to describe the epidemiology and management of pulmonary arterial hypertension (PAH) in children, as well as its effects on the quality of life and its consequences, excluding patients with persistent pulmonary hypertension of the newborn and those with pulmonary arterial hypertension due to congenital heart disease.MethodsThis multicenter non interventional study included children with pulmonary arterial hypertension who were prospectively followed for two years in 21 French centres. The WHO functional classification, the 6-minute walk distance, the quality of life (CHQ-PF50 questionnaire), the hemodynamics parameters and echodoppler performed were evaluated.ResultsFifty children with a mean age of 8.9 ± 5.4 years were included from May 2005 to June 2006. The prevalence of pulmonary arterial hypertension was estimated at 3.7 cases per million. The patients had the following types of pulmonary arterial hypertension: idiopathic (60%), familial (10%), associated with congenital heart disease that was not the cause of the pulmonary hypertension (24%), associated with a connective-tissue disease (4%) or with portal hypertension (2%). During follow-up there was an increase in the number of drugs prescribed specifically for pulmonary arterial hypertension (44% patients versus 22% at inclusion). The clinical status, 6-minute walk test and quality of life of the majority of patients remained stable. The survival at one and at two years was estimated at 86% [95% confidence interval (76, 96)] and 82% [95% confidence interval (71, 93)].ConclusionsThe majority of cases of pulmonary arterial hypertension in children are idiopathic / familial. A specific group of pulmonary arterial hypertension occurring with congenital heart disease has been identified and resembles idiopathic pulmonary arterial hypertension. The use of specific treatments for PAH may contribute to the stability of the disease and to better survival.

Objective Off-pump valve replacement using self-expandable stents is an emerging technique for pulmonary valve disease. However, significant limitations are the lack of easily available valve substitute to be inserted within the stent and, in the setting of repaired tetralogy of Fallot, the existence of huge pulmonary trunk. We report the first experimental results of a transventricular approach using a decellularized porcine xenograft mounted in a self-expandable stent. Methods Pulmonary valve replacement was realized in 15 lambs by direct access of the infundibulum through a left thoracotomy, combined with pulmonary artery banding. Animals were followed by transthoracic echocardiography and, after control hemodynamic study, were electively killed either at day 7, month 1, or month 4 after implantation. Results Implantation succeeded in all lambs. Two animals died after implantation (1 pneumothorax and 1 endocarditis). Doppler echocardiographic follow-up did not show any significant transvalvular gradient and showed only mild pulmonary regurgitation. The hemodynamic control before termination revealed a systolic pulmonary valve gradient of 18.5 ± 12.4 mm Hg at 1 week (n = 4), 13.5 ± 10.6 mm Hg at 1 month (n = 4), and 4.3 ± 4.9 mm Hg at 4 months (n = 5). Gross examination demonstrated the presence of connective tissue between the valved stent and pulmonary wall, which increased with time. Conclusion Fifteen lambs underwent successful deployment of a self-expandable valved stent in the pulmonary position using a transventricular approach. This technique combined with pulmonary artery banding could be a therapeutic option for pulmonary insufficiency after repair of tetralogy of Fallot with a transannular patch.

Purpose To review the use of thoracic endovascular aortic repair (TEVAR) for late pseudoaneurysm formation after surgical repair of aortic coarctation. Methods From May 2001 to May 2005, 8 patients (5 men; mean age 47.6 years, range 18-73) with a history of aortic coarctation repairs 17 to 40 years prior were referred to our institution for an anastomotic thoracic pseudoaneurysm. TEVAR was performed successfully in 7 patients; 1 died of suspected aneurysm rupture before the scheduled procedure. A carotid-subclavian bypass was performed in 3 patients. Results All the procedures were immediately successful. No type I endoleaks were seen on the final control angiogram, but 2 of the patients with carotid-subclavian bypasses required additional left subclavian artery embolization due to type II endoleak. One of these patients died before embolotherapy on the 5th postoperative day from presumed aneurysm rupture (14% 30-day mortality rate). Over a follow-up period ranging from 15 to 72 months (mean 37), all the false aneurysms have remained thrombosed and the mean diameter has decreased from 44 to 23 mm. No endograft-related complications have occurred, and no further interventions have so far been necessary. Conclusion TEVAR is a feasible alternative treatment for patients who have already undergone surgical repair of aortic coarctation. Technical issues regarding the endovascular strategy should be discussed with a multidisciplinary team to define the correct interventional plan.

Introduction Infantile haemangioma (IH) is a very common vascular tumour that affects up to 10% of newborns. Since 2008, oral propranolol is used to treat complicated IH, like haemangioma that obstruct vital structures or ulcerated haemangioma. Objective The aim of this study was to investigate, by a retrospective review, the therapeutic results and effects of propranolol on cardiovascular and biological parameters in infants treated for complicated infantile haemangioma and to assess its safety. Results All paediatric patients with complicated IH who started systemic propranolol from February 2009 to December 2014 were included. 218 patients (155 girls and 63 boys) were treated by propranolol. The mean weight was 6780g (2115g to 20000g). Median age at beginning of treatment was 4.7 months (10 days to 6 years). The most frequent localisation of IH was facial (63 patients), palpebral (52 patients), perineal (20 patients), labial (14 patients), airway obstruction (8 patients) and 1 PHACE syndrome. Median length of therapy was 7.5 months for facial IH, 6 months for palpebral, 5.6 months for perineal IH and 7 months for subglottic localisation. Adverse events were observed: hypoglycemia (n=11 patients aged less 6 months), arterial hypotension (n=103 patients, especially at the second and third dose with dose titration), bradycardia (n=120). Transthoracic echocardiography was realised in 158 patients: 19 pathologies were found (8 PDA, 4 ASD, 4 mitral regurgitations, 2 VSD and 1 coarctation). Other adverse events occurred in 49 patients (wheezing, acrocyanosis, diarrhoea, sleep disturbance) leading to modification in management. Complete response was observed for all but 11 (partial or no response). Conclusion In appropriated patients, propranolol therapy is effective for severe or complicated IH. We must be aware of frequent adverse events under beta-blocker in these patients.