This work aims to study the technical and economical feasibility of a new hydrogen transport network by 2035 in France. The goal is to furnish charging stations for fuel cell electrical vehicles with hydrogen produced by electrolysis of water using low-carbon energy. Contrary to previous research works on hydrogen transport for road transport, we assume a more realistic assumption of the demand side: we assume that only drivers driving more than 20,000 km per year will switch to fuel cell electrical vehicles. This corresponds to a total demand of 100 TWh of electricity for the production of hydrogen by electrolysis. To meet this demand, we primarily use surplus electricity production from wind power. This surplus will satisfy approximately 10% of the demand. We assume that the rest of the demand will be produced using surplus from nuclear power plants disseminated in regions. We also assume a decentralized production, namely, that 100 MW electrolyzers will be placed near electricity production plants. Using an optimization model, we define the hydrogen transport network by considering decentralized production. Then we compare it with more centralized production. Our main conclusion is that decentralized production makes it possible to significantly reduce distribution costs, particularly due to significantly shorter transport distances.

Restenosis occurs commonly after aortic coarctation (CoA) repair, usually requiring treatment by balloon dilation. Its effect on physical exercise performance is not documented. A retrospective analysis of exercise testing and echocardiographic assessment was performed in children after CoA repair. A group without restenosis (= group 1) and a group with recoarctation, treated by single balloon dilation (= group 2) were compared by matching for age and gender at a 2:1 ratio. Group 1 included 40 children and group 2 comprised 21 children, with mean age of 12.3 ± 2.3y and 11.9 ± 2.9y (p = 0.536) at evaluation. Group 2 children were younger at CoA repair (group 1: median 32.0d (11.0–188.0)—group 2: 9.0d (5.5–19.0), p = 0.011). Arterial hypertension was frequently noticed (group 1: 23(57.5

We present the case of a 33-year-old patient with a rare combination of a right-sided aortic arch and occluded asymptomatic aberrant left subclavian artery diagnosed after the coincidental finding of an interarm blood pressure difference. Because there were no symptoms of local compression or subclavian steal, conservative management was suggested.

Background Lymphatic disorders such as protein‐losing enteropathy, plastic bronchitis, and chylothorax are important complications of the Fontan circulation and ultimately result in an increased risk of early death. Several European centers are now performing lymphatic procedures. The aim of this study is to map the extent of these lymphatic disorders and treatments provided across European pediatric cardiology centers. Methods and Results A survey was circulated to 49 European pediatric cardiology centers consisting of 37 questions including a mix of binary, categorical, and continuous variables. Thirty‐one centers (63%) participated in the study, performing a median of 250 (interquartile range, 178 ‐ 313) cardiopulmonary bypass procedures per year. Chylothorax emerged as the most prevalent lymphatic disorder followed by protein‐losing enteropathy and plastic bronchitis. The most common diagnostic investigation method was noncontrast magnetic resonance lymphangiography (52%). Eleven centers (35%) conducted lymphatic interventions with a median of 3 (interquartile range, 1 ‐ 4) procedures per year and 12 (interquartile range, 5 ‐ 15) interventions in total per center. Conclusions This study confirms the rarity of and variation in treatment approaches for lymphatic disorders across Europe. With at least 11 centers offering lymphatic interventions, the adoption of these procedures is on the rise in Europe. To improve the quality of care and treatment outcomes for these complex patients, it is crucial to consider evidence‐based lymphatic diagnostics, interventional lymphatic procedures, and the centralization of services in Europe.

Pulmonary arterial hypertension (PAH) is a rare disease affecting the small pulmonary vessels, ultimately leading to right ventricular failure and death. Current treatment options target three different pathways (endothelin, nitric oxide/cGMP and prostacyclin pathways). Despite their demonstrated efficacy, these therapies (commonly used in combination) do not cure the disease which is why novel pathways beyond the traditional 'big three' are being developed. Sotatercept is a ligand trap for multiple proteins within the TGF-β superfamily that was recently approved in the US for the treatment of PAH. Unlike currently available therapies, sotatercept has the potential to act as an anti-remodelling agent rather than a vasodilator. The safety and efficacy of subcutaneous (SC) sotatercept have been established in two multicentre, placebo-controlled randomised-controlled trials. The compound has been shown to consistently improve a variety of measurable endpoints, including exercise capacity, haemodynamics, quality of life and delay of clinical worsening. The drug appears to have an acceptable safety profile, although it is associated with an increased risk in developing telangiectasia and biological changes affecting platelet counts and haemoglobin. This study reviews the current evidence on SC sotatercept and provides a Belgian perspective on its place in the future treatment strategy for PAH.

Background: Familial hypercholesterolemia (FH) is characterized by significantly elevated low-density lipoprotein cholesterol (LDL-C) levels, increasing the risk of early-onset atherosclerosis. Ultrasound studies of superficial arteries can non-invasively assess early vascular changes in children. Flow-mediated dilatation (FMD) and arterial stiffness (AS) are more sensitive indicators of early atherosclerosis than intima media thickness (IMT) as they represent the earliest functional alterations in the arterial wall. However, FMD and AS measurements are technically challenging and normative values in pediatrics vary substantially. Here, we review the literature on statin-induced vascular changes in FH.

IntroductionImaging fusion technology is promising as it is radiation and contrast sparing. Herein, we compare conventional biplane angiography to multimodality image fusion with live fluoroscopy using two-dimensional (2D)–three-dimensional (3D) registration (MMIF2D−3D) and assess MMIF2D−3D impact on radiation exposure and contrast volume during cardiac catheterization of patients with congenital heart disease (CHD).MethodsWe matched institutional MMIF2D−3D procedures and controls according to patient characteristics (body mass index, age, and gender) and the seven procedure-type subgroups. Then, we matched the number of tests and controls per subgroup using chronological ordering or propensity score matching. Subsequently, we combined the matched subgroups into larger subgroups of similar procedure type, keeping subgroups with at least 10 test and 10 control cases. Air kerma (AK) and dose area product (DAP) were normalized by body weight (BW), product of body weight and fluoroscopy time (BW × FT), or product of body weight and number of frames (BW × FR), and stratified by acquisition plane and irradiation event type (fluoroscopy or acquisition). Three senior interventionists evaluated the relevance of MMIF2D−3D (5-point Likert scale).ResultsThe Overall group consisted of 54 MMIF2D−3D cases. The combined and matched subgroups were pulmonary artery stenting (StentPUL), aorta angioplasty (PlastyAO), pulmonary artery angioplasty (PlastyPUL), or a combination of the latter two (Plasty). The FT of the lateral plane reduced significantly by 69.6% for the Overall MMIF2D−3D population. AKBW and DAPBW decreased, respectively, by 43.9% and 39.3% (Overall group), 49.3% and 54.9% (PlastyAO), and 36.7% and 44.4% for the Plasty subgroup. All the aforementioned reductions were statistically significant except for DAPBW in the Overall and Plasty (sub)groups. The decrease of AKBW and DAPBW in the StentPUL and PlastyPUL subgroups was not statistically significant. The decrease in the median values of the weight-normalized contrast volume (CMCBW) in all five subgroups was not significant. Cardiologists considered MMIF2D−3D very useful with a median score of 4.ConclusionIn our institution, MMIF2D−3D overall enabled significant AKBW reduction during the catheterization of CHD patients and was mainly driven by reduced FT in the lateral plane. We observed significant AKBW reduction in the Plasty and PlastyAO subgroups and DAPBW reduction in the PlastyAO subgroup. However, the decrease in CMCBW was not significant.

Acute kidney injury (AKI) is common after pediatric cardiac surgery (CS). Several urine biomarkers have been validated to detect AKI earlier. The objective of this study was to evaluate urine CHI3L1, NGAL, TIMP-2, IGFBP7, and NephroCheck® as predictors for AKI ≥ 1 in pediatric CS after 48 h and AKI ≥ 2 after 12 h. Pediatric patients (age < 18 year; body weight ≥ 2 kg) requiring CS were prospectively included. Urine CHI3L1, NGAL, TIMP-2, IGFBP7, and NephroCheck® were measured during surgery and intensive care unit (ICU) stay and corrected for urine dilution. One hundred and one pediatric patients were included. AKI ≥ 1 within 48 h after ICU admission occurred in 62.4% and AKI ≥ 2 within 12 h in 30.7%. All damage biomarkers predicted AKI ≥ 1 within 48 h after ICU admission, when corrected for urine dilution: CHI3L1 (AUC-ROC: 0.642 (95% CI, 0.535–0.741)), NGAL (0.765 (0.664–0.848)), TIMP-2 (0.778 (0.662–0.868)), IGFBP7 (0.796 (0.682–0.883)), NephroCheck® (0.734 (0.614–0.832)). Similarly, AKI ≥ 2 within 12 h was predicted by all damage biomarkers when corrected for urine dilution: uCHI3L1 (AUC-ROC: 0.686 (95% CI, 0.580–0.780)), NGAL (0.714 (0.609–0.804)), TIMP-2 (0.830 (0.722–0.909)), IGFBP7 (0.834 (0.725–0.912)), NephroCheck® (0.774 (0.658–0.865)). After pediatric cardiac surgery, the damage biomarkers urine CHI3L1, NGAL, TIMP-2, IGFBP7, and NephroCheck® reliably predict AKI after correction for urine dilution.

OBJECTIVES The aim of this study was to evaluate the relationship between atrioventricular valve and ventricular function in Fontan survivors, including the effect of atrioventricular valve surgery.METHODS Analysis focused on transplant-free survival and the need for atrioventricular valve surgery in single ventricle patients after Fontan completion. Longitudinal echocardiographic examination of long-term valve and ventricular function was performed.RESULTS Fontan completion was performed in 113 patients, having a right univentricular morphology in 33.6%, a left ventricle morphology in 62.8% and ambiguous in 3.6%. Perioperative mortality was 2.7% (n = 3). Within a median follow-up of 16.3 years (interquartile range 10.6-23.6), transplant-free survival was 96.1 +/- 1.9% and 90.4 +/- 5.8% at 10-25 years. Twenty AV valve procedures were performed in 14 (12.4%) children, respectively, pre-Fontan (n = 10), per-Fontan (n = 8) and post-Fontan (n = 2), resulting in a cumulative incidence of AV valve surgery is 5.7 +/- 2.2% and 12.3 +/- 3.2% at 1-5 years. Atrio-ventricular valve function deteriorated over time [hazard ratio (HR) 1.112, 95% confidence interval (CI) 1.089-1.138, P < 0.001], without difference for valve morphology (P = 0.736) or ventricular dominance (P = 0.484). AV valve dysfunction was greater in patients requiring AV valve surgery (HR 20.383, 95% CI 6.223-36.762, P < 0.001) but showed a comparable evolution since repair to those without valve surgery (HR 1.070, 95% CI 0.987-1.160, P = 0.099). Progressive time-related ventricular dysfunction was observed (HR 1.141, 95% CI 1.097-1.182, P < 0.001), significantly less in left ventricle-dominance (HR 0.927, 95% CI 0.860-0.999, P = 0.047) but more after AV valve surgery (HR 1.103, 95% CI 1.014-1.167, P = 0.022).CONCLUSIONS In a homogeneously treated Fontan population, 25-year transplant-free survival is encouraging. Atrio-ventricular valve surgery was necessary in 12.4%, resulting mostly in a durable valve function. However, a slow time-related decline of atrioventricular valve function as of ventricular function is worrisome, evoking a role for additional heart failure therapy.

In the current context of the ban on fossil fuel vehicles (diesel and petrol) adopted by several European cities, the question arises of the development of the infrastructure for the distribution of alternative energies, namely hydrogen (for fuel cell electric vehicles) and electricity (for battery electric vehicles). First, we compare the main advantages/constraints of the two alternative propulsion modes for the user. The main advantages of hydrogen vehicles are autonomy and fast recharging. The main advantages of battery-powered vehicles are the lower price and the wide availability of the electricity grid. We then review the existing studies on the deployment of new hydrogen distribution networks and compare the deployment costs of hydrogen and electricity distribution networks. Finally, we conclude with some personal conclusions on the benefits of developing both modes and ideas for future studies on the subject.

Abstract Background and aim: Noonan syndrome (NS) is associated with different types of heart defects of which a supravalvular pulmonary stenosis ((SV)PS) is the most frequent. Possible treatment options are percutaneous balloon pulmonary valvuloplasty (BVP) or surgical intervention. Anatomical location of the PS may help predict BVP failure. We aimed to identify factors predicting treatment outcome and reintervention rate of BVP in PS, in children with NS. Methods: Medical records of children with a diagnosis of NS and in follow-up at Antwerp- and Ghent University Hospitals from 2000 to 2022 were retrospectively reviewed. Results: 32 children were identified with a SVPS, either isolated or in combination with other heart defects. 69% of children with PS had SVPS. The prevalence of PS and SVPS was similar for all genes. An isolated SVPS was identified as a risk factor for intervention. An intervention was necessary in 17/32 patients (53%). All but 2 children with pulmonary valve stenosis had SVPS. Only 2 of 17 patients had primary surgical repair. The remaining 15 (13 with SVPS) underwent BVP, of which 10 (67%) needed a second intervention, but all of them ultimately needed surgical repair. The global success rate of BVP was (31,1%). Conclusion: SVPS is the most frequent heart defect in children with NS. The prevalence of SVPS was similar for all genes. Isolated SVPS is a risk factor for intervention. The success rate of BVP in patients with NS is low. BVP might still be useful in selected cases and might be considered to clarify the anatomical location of PS.

Introduction:Multisystem inflammatory syndrome in children (MIS-C) is associated with important cardiovascular morbidity during the acute phase. Follow-up shows a swift recovery of cardiac abnormalities in most patients. However, a small portion of patients has persistent cardiac sequelae at mid-term. The goal of our study was to assess late cardiac outcomes of MIS-C. Methods:A prospective observational multicenter study was performed in children admitted with MIS-C and cardiac involvement between April 2020 and March 2022. A follow-up by NT-proBNP measurement, echocardiography, 24-h Holter monitoring, and cardiac MRI (CMR) was performed at least 6 months after MIS-C diagnosis. Results:We included 36 children with a median age of 10 (8.0-11.0) years, and among them, 21 (58%) were girls. At diagnosis, all patients had an elevated NT-proBNP, and 39% had a decreased left ventricular ejection fraction (LVEF) (<55%). ECG abnormalities were present in 13 (36%) patients, but none presented with arrhythmia. Almost two-thirds of patients (58%) had echocardiographic abnormalities such as coronary artery dilation (20%), pericardial effusion (17%), and mitral valve insufficiency (14%). A decreased echocardiographic systolic left ventricular (LV) function was detected in 14 (39%) patients. A follow-up visit was done at a mean time of 12.1 (±5.8) months (range 6-28 months). The ECG normalized in all except one, and no arrhythmias were detected on 24-h Holter monitoring. None had persistent coronary artery dilation or pericardial effusion. The NT-proBNP level and echocardiographic systolic LV function normalized in all patients, except for one, who had a severely reduced EF. The LV global longitudinal strain (GLS), as a marker of subclinical myocardial dysfunction, decreased (z < -2) in 35%. CMR identified one patient with severely reduced EF and extensive myocardial fibrosis requiring heart transplantation. None of the other patients had signs of myocardial scarring on CMR. Conclusion:Late cardiac outcomes after MIS-C, if treated according to the current guidelines, are excellent. CMR does not show any myocardial scarring in children with normal systolic LV function. However, a subgroup had a decreased GLS at follow-up, possibly as a reflection of persistent subclinical myocardial dysfunction.

Background: Coarctation of the Aorta (CoA) leads to increased morbidity and mortality later in life despite early surgical or percutaneous treatment. Many long-term complications are related to hypertension (HT) which is a common finding late after coarctation repair. Methods: A systematic Review was performed including articles published between February 2012 to December 2020. Systematic searches were conducted on PubMed and the Cochrane Controlled Trials Register to look for studies on HT after aortic CoA-repair. PRISMA guidelines were used. Results: In this systematic review on HT after CoA Repair the mean prevalence of HT was 47.3% (20- 70%). A progressive character was of the HT was found, furthermore if only studies are included with 24 h blood pressure (BP) recording in addition to standard BP measurements, the incidence of HT rose to 57.8%. Discussion: Most clinical studies look at complications, mortality rate and residual pressure gradient rather than correlating hemodynamic indices with long-term outcome. Although HT is commonly based in measurement of peripheral BP, it has been shown that peripheral BP in CoA patients has a poor correlation with central aortic pressure. Central aortic hemodynamics are significantly altered in patients with repaired CoA, which can now adequately be investigated non-invasively. At the present time there are no studies linking long-term outcome with abnormal central hemodynamics.

We developed a passenger transport model for the North of France and used it to discuss the impacts of some policies focusing on the limitations of polluting gas emissions and congestion. The model is calibrated for the North of France and includes both urban and intercity trips. Four transport modes are considered: walking, biking, public transport and private cars. To some extent, the combination of these modes is possible. The model is calibrated to match mode shares and the dynamic of congestion along a full day. The simulations are conducted within the MATSim framework. We evaluate the impacts, on traffic flows and polluting gas emissions, of two pricing reforms: free public transport and road pricing in city center of Lille (the main metropolitan area in the study region). Free public transport yields a significant modal shift towards public transport, resulting in a reduction in the usage of private cars. The road pricing scheme we have considered results in similar impacts but with limited magnitude. Overall, a significant reduction in congestion and emissions of pollutant gases can be obtained by applying convenient pricing reforms. Since we use an agent-based model, we are able to identify the specific location of the main impacts on the network.

AbstractBackground:Limited data exist on training of European paediatric and adult congenital cardiologists.Methods:A structured and approved questionnaire was circulated to national delegates of Association for European Paediatric and Congenital Cardiology in 33 European countries.Results:Delegates from 30 countries (91%) responded. Paediatric cardiology was not recognised as a distinct speciality by the respective ministry of Health in seven countries (23%). Twenty countries (67%) have formally accredited paediatric cardiology training programmes, seven (23%) have substantial informal (not accredited or certified) training, and three (10%) have very limited or no programme. Twenty-two countries have a curriculum. Twelve countries have a national training director. There was one paediatric cardiology centre per 2.66 million population (range 0.87–9.64 million), one cardiac surgical centre per 4.73 million population (range 1.63–10.72 million), and one training centre per 4.29 million population (range 1.63–10.72 million population). The median number of paediatric cardiology fellows per training programme was 4 (range 1–17), and duration of training was 3 years (range 2–5 years). An exit examination in paediatric cardiology was conducted in 16 countries (53%) and certification provided by 20 countries (67%). Paediatric cardiologist number is affected by gross domestic product (R2 = 0.41).Conclusion:Training varies markedly across European countries. Although formal fellowship programmes exist in many countries, several countries have informal training or no training. Only a minority of countries provide both exit examination and certification. Harmonisation of training and standardisation of exit examination and certification could reduce variation in training thereby promoting high-quality care by European congenital cardiologists.

Congenital heart defects (CHD) are the most common congenital anomalies in liveborn children. In contrast to syndromic CHD (SCHD), the genetic basis of isolated CHD (ICHD) is complex, and the underlying pathogenic mechanisms appear intricate and are incompletely understood. Next to rare Mendelian conditions, somatic mosaicism or a complex multifactorial genetic architecture are assumed for most ICHD. We performed exome sequencing (ES) in 73 parent–offspring ICHD trios using proband DNA extracted from cardiac tissue. We identified six germline de novo variants and 625 germline rare inherited variants with ‘damaging’ in silico predictions in cardiac-relevant genes expressed in the developing human heart. There were no CHD-relevant somatic variants. Transmission disequilibrium testing (TDT) and association testing (AT) yielded no statistically significant results, except for the AT of missense variants in cilia genes. Somatic mutations are not a common cause of ICHD. Rare de novo and inherited protein-damaging variants may contribute to ICHD, possibly as part of an oligogenic or polygenic disease model. TDT and AT failed to provide informative results, likely due to the lack of power, but provided a framework for future studies in larger cohorts. Overall, the diagnostic value of ES on cardiac tissue is limited in individual ICHD cases.

We extend a multimodal transport model to simulate an increase of the market share of electric vehicles. The model, which is described in detail in Kilani et al. (Sustainability 14(3):1535, 2022), covers the north of France and includes both urban and intercity trips. It is a multi-agents simulation based on the MATsim framework and calibrated on observed traffic flows. We find that the emissions of pollutant gases decrease in comparable proportion to the market share of the electric vehicles. When only users with shorter trips switch to electric vehicles, the impact is limited and demand for charging stations is small since most users will charge by night at home. When the government is able to target users with longer trips, the impact can be higher by more than a factor of two. But, in this case, our model shows that it is important to increase the number of charging stations with an optimized deployment for their accessibility.