Objectives To determine the prevalence and phenotypes of children with heritable pulmonary arterial hypertension (PAH) and those with pulmonary hypertension (PH) associated with genetic disorders. Methods We retrospectively analysed 133 paediatric cases with PAH, with a median age of 6 years. Results 83 patients had isolated PAH, 41 had associated congenital heart disease, and 9 had suspected pulmonary veno occlusive disease. The female/male sex ratio was 1.8/1. Affected genes were BMPR2[13], SOX17[10], TBX4[8], ACVRL1[9], EIF2AK4[4], GDF2[2], BMP10[1], and KCNK3[1]. SOX17 variants were more common in children with APAH-CHD (p=0.01). Additionally, 18% of patients had genetic disorders associated with PH, including Chuvash syndrome (n=2), incontinentia pigmenti (n=1), 5 patients with RASopathy (3 Noonan syndromes and 2 neurofibromatosis type 1), Cantu syndrome (n=1), Alagille syndrome (n=1), Bourneville tuberous sclerosis (n=1), mitochondrial diseases (n=5), Myhre syndrome (n=1), and chromosomal rearrangements (n=6). Overall, a significant proportion of patients had either pathogenic variants in known PAH genes or genetic disorders complicated by pulmonary vascular disease. Conclusion The prevalence of genetic disorders and heritable PAH in children appears to be higher than previously reported, affecting over half of the paediatric population. This study emphasizes the importance of systematic genetic testing, including next-generation sequencing panels, in apparently idiopathic PAH and newly diagnosed PH patients. Larger genomic sequencing may be necessary to identify novel genes associated with these conditions when initial testing is negative.

Introduction Transcatheter patent ductus arteriosus (PDA) closure is safe in<2kg infants and in≥6kg patients, but major safety concerns remain when applied to the intermediate weight range. We aimed to assess outcomes of transcatheter PDA closure in 2 to 6kg infants. Methods An international, multicentre, retrospective cohort study was conducted in 31 tertiary hospitals in 17 countries between 2000 and 2023, investigating all infants who underwent attempted transcatheter PDA closure with a procedural weight of 2 to 6kg. Results Attempted transcatheter PDA closure was performed in 1231 infants (median weight: 4747 grams Q1–Q3 [3700–5300]; median age: 132 days Q1–Q3 [83–194]; ex-preterm: n=581 [56.8%]) with a 95.0% success rate. A composite outcome of procedural failure or major adverse events was observed in 173 (14%) patients, including device embolization in 64 (3.7%), device-induced left pulmonary artery stenosis in 47 (2.7%) and procedural death in 2 (0.2%). Logistic regression model analysis identified a 2-to 3.9-kg procedural weight, increased pulmonary artery pressure, and window-type or tubular ductal morphologies as independent predictors of the composite outcome. Based on propensity score matching analysis, 2 to 3.9kg infants had a risk ratio of 2.19 (95%CI, 1.25–3.83) for experiencing the composite outcome, as compared to 4 to 5.9kg infants. Conclusion Transcatheter PDA closure in 2 to 6kg infants was feasible in most patients. Procedural failure or major adverse events occurred in 14% and several independent risk factors were identified, including the 2 to 3.9kg weight range identified as a higher-risk subgroup. These findings may improve risk stratification and decision-making process.

Introduction Among congenital heart diseases, aortic coarctation accounts for 5–8% of patients and is associated with significant morbidity and mortality from childhood to adulthood. Neonatal forms are considered critical congenital heart defects, requiring early management under optimal conditions to ensure a favorable cardiovascular prognosis. Among postoperative complications, aortic recoarctation is the most frequent early complication in these patients. However, to date, the predictive factors for this complication remain poorly understood. The objective of this multicenter study is to describe the morbidity and mortality of aortic coarctation operated on before 1 year of age in a large multicenter cohort and to identify predictive factors for aortic recoarctation. Methods This is a retrospective, multicenter, longitudinal observational study conducted in five French surgical centers. Patients who underwent surgery for aortic coarctation before the age of one year, between January 2011 and December 2025, were included. Patients with complex congenital heart disease and those who initially underwent cardiac catheterization were excluded. The primary endpoint was 6-year survival free from aortic recoarctation. Secondary endpoints were overall survival and survival free from arterial hypertension. Results A total of 908 patients were included (male-to-female ratio: 1.95), with a median follow-up of 4 years. The median age at surgery was 14 days. The surgical technique used was the Crafoord procedure in 35% of cases, extended Crafoord repair in 48%, and aortic arch plasty in 17%. Survival free from recoarctation was 81.5% at 6 years (Figure 1). Overall survival was 97% at 6 years. Survival free from hypertension was 60% at 6 years. Predictive factors for recoarctation identified in univariate analysis included demographic, clinical, and imaging outcomes. Multivariate analysis is ongoing. Conclusion Neonatal aortic coarctation remains a condition associated with substantial morbidity and mortality. The predictive factors for recoarctation identified may help guide surgical decision-making and follow-up strategies. Hypertension remains the main long-term morbidity, affecting up to 40% of children at 6 years of age, although only 8.5% receive treatment. Improving its detection and management is essential to reduce long-term cardiovascular impact in adulthood.

Introduction: Cardiac complications are well-documented in propionic acidemia (PA), and there are a few reported cases of cardiomyopathies in methylmalonic acidemia (MMA). Left-ventricular global longitudinal strain (LV GLS) measurement is known to be able to detect early ventricular dysfunction, leading potentially to cardiomyopathy. The aim of our study was to evaluate left-ventricular global longitudinal strain (LV GLS) in MMA and PA patients and compare it with the pediatric general population. Methods: In this monocentric retrospective study, 26 patients with organic aciduria (OA) were included. Demographic, clinical, electrocardiographic and echocardiographic data were collected. The mean LV GLS in MMA and PA patients was compared with the GLS in the pediatric general population. Results: The left-ventricular ejection fraction (LVEF) was similar between MMA and PA patients and in the normal range (66.27 ± 6.24% vs. 61.41 ± 11.02%; p = 0.182). LV GLS was significantly lower in PA patients than in MMA patients (-15.8 ± 5.67% vs. -20.6 ± 3.19%; p = 0.011). LV GLS was significantly lower in PA patients when compared with the general pediatric population (p = 0.029). Conclusions: Patients with propionic acidemia may have impaired global longitudinal strain even in the presence of normal LVEF. LV GLS might be a useful tool for cardiac follow-up in pediatric patients with OA.

We report on the case of a pediatric patient with homozygous familial hypercholesterolemia (HoFH) who developed significant coronary artery disease (CAD). Percutaneous coronary intervention (PCI) guided by intravascular ultrasound (IVUS) was performed. A 6-year-old female patient was diagnosed with HoFH following the identification of cutaneous xanthomas. Genetic test identified a homozygous mutation in the LDLR gene. Cardiovascular assessment including a coronary CT angiography revealed a severe (70–90

Introduction Transcatheter patent ductus arteriosus (PDA) closure can be challenging in cases of large PDA, complex anatomy, and small patient size. We aimed to assess the feasibility, efficacy and safety of the off-label use of the multifunctional occluder (MFO) in transcatheter PDA closure. Methods A retrospective analysis was conducted on patients who underwent transcatheter PDA closure with the MFO in 14 pediatric cardiology centers (5 countries) from 2018 to 2025. Results A total of 77 procedures were performed on 75 patients, including 5 adults (6.7%). Among the 70 children (93.3%) [median age: 0.8 years (range: 0.1–10 years), median procedural weight: 6.9kg (range: 2.1–32.0kg)], 35 (50.0%) weighed 6kg or less [median procedural weight: 4.0kg (range: 2.1–5.9kg)]. The PDA was large, with a minimal ductal diameter of 3.8mm (range: 1.5–7.0mm), short in 54.5%, and unrestrictive with increased pulmonary artery pressures in 51.6% patients. The morphology was predominantly conical (Krichenko type A: 51.9%), with window-type or complex morphologies representing 26.7% (Type B: 18.2%, Type D: 7.8%). Both arterial and venous femoral accesses were obtained in 87.0% cases, with deployment being antegrade in 77.9% and retrograde in 22.1%. Successful device deployment and release were achieved in 74 cases (96.1%). The rate of major adverse events was 5.2% including 1 early (1.3%) and 3 late complications (3.9%). No other adverse events were reported. Conclusion The off-label use of the MFO device is a promising option for transcatheter PDA closure, demonstrating safety and efficacy in this series.

Introduction The optimal timing of pulmonary valve replacement (PVR) in adults with repaired Tetralogy of Fallot (TOF) and severe pulmonary regurgitation remains controversial, particularly in older patients. Current recommendations rely largely on right ventricular volumes assessed by cardiac magnetic resonance imaging (MRI), although their prognostic value remains debated. Methods We conducted a retrospective single-center study including patients aged≥30 years with repaired TOF who underwent surgical PVR between 2009 and 2018. The primary outcome was a composite endpoint including supraventricular arrhythmia, cardiovascular rehospitalization, or all-cause death during follow-up. Clinical, imaging, and hemodynamic variables were analyzed using univariate and multivariate logistic regression. Results Forty-eight patients (mean age at PVR 41±9.8 years) were included, with a mean follow-up of 52 months. The primary outcome occurred in 23 patients (48%). Preoperative signs of advanced disease, including right heart failure, left ventricular dysfunction, and a history of syncope or ventricular tachycardia, were associated with adverse outcomes. In multivariate analysis, elevated right ventricular end-diastolic pressure (RVEDP) was the only independent predictor of postoperative morbidity and mortality (OR 1.49, 95% CI 1.01–3.40; P=0.04). Preoperative right ventricular volumes assessed by MRI were not associated with the primary outcome in multivariate analysis. Conclusion In adults aged≥30 years with repaired TOF undergoing PVR, elevated RVEDP is a key predictor of postoperative morbidity and mortality, whereas right ventricular volumes on MRI are not. Invasive hemodynamic assessment may help refine the timing of PVR in this “senior” population.

INTRODUCTION:Transcatheter closure of patent ductus arteriosus (PDA) is expanding due to the fast development of new techniques and new materials. Miniaturization of devices currently allows transcatheter closure of ducts in very small patients. Few published data are however available in the 2 to 6 kilograms population. OBJECTIVE:This study aims to describe and evaluate recent practices and experience in a medium-sized tertiary referral centre. METHODS:We conducted a retrospective observational study comparing percutaneous and surgical duct closure in children weighing between 2 and 6 kilograms at the University Hospital of La Timone, Marseille, France. We analyzed clinical, procedural, and follow-up data, especially success rate of the percutaneous closure and complications related to the procedures. Additionally, the duration of mechanical ventilation, and of hospital stays of each procedure were compared. RESULTS:Between 2014 and 2021, 31 patients weighing 2 to 6 kilos, were referred for transcatheter PDA closure and 22 patients for surgical closure of the PDA. Median age was respectively 5.1 [2.9-6.3] and 2.4 [2-3.2] months. Median weight 4660 [3950-5450] versus 3184 [2287-3775] grams. The success rate was 77.42 % (n = 24/31) for the transcatheter group versus 100 % for the surgical group. No patient died or experienced major adverse event because of the procedure. Minor adverse events occurred in 6/24 patients of the transcatheter group and 2/22 patients of the surgical group. The duration of mechanical ventilation was significantly shorter in the percutaneous group. The length of hospital stay was significantly shorter in the transcatheter group with a median of 2 [2.00-4.00] days compared to 8.5 [5.25-15.50] days in the surgical group (p < 0.001). CONCLUSION:In our experience, percutaneous closure of patent ductus arteriosus is an efficient and safe alternative to surgery in 2 to 6 kg patients. Larger, multicentre studies are needed to confirm this statement.

AIMS:Coarctation of the aorta (CoA) is the most common undiagnosed congenital heart disease (CHD) during pre-natal screening and its overall prognosis relies on the quality of the surgical repair. This study aimed to identify the pre-natal and post-natal factors associated with the type of surgical technique repair in infant CoA. METHODS AND RESULTS:Multicentre, retrospective, observational study in 680 infants (68% males) aged <1 year who underwent CoA surgical repair in 4 CHD surgical centres in France over 11 years. The primary outcome was the surgical repair technique (simple end-to-end repair, extended end-to-end repair, or aortic arch repair). The aortic arch raw diameter, measured by echocardiography just beyond the brachiocephalic arterial trunk, was the most discriminating parameter for the type of surgical repair technique, outperforming the existing Z-score models. Cut-off value of aortic arch diameter ≤3.6 mm in children <1-year old was predictive of an aortic arch repair [area under the curve (95% CI) = 0.76 (0.67;0.86)]. In multivariable analysis, a pre-natal associated cardiac malformation [odd ratio (OR) (95% CI) = 4.39 (1.99; 9.69)] and aortic arch diameter ≤3.6 mm [OR (95% CI) = 3.78 [1.61; 8.62)] were predictive of an aortic arch repair. CONCLUSION:The aortic arch diameter is the most discriminating parameter for the type of surgical repair technique in infant CoA. An aortic arch threshold value of 3.6 mm in infant population is predictive of aortic arch repair and should be considered a practical indicator of the hypoplastic nature of the aortic arch.

This review is based on interviews with internationally recognized female leaders in congenital cardiac interventions. The discussions explore diverse career pathways and professional experiences across different health care systems. The interviews are structured around 8 core themes: entry into the field, training, key turning points, leadership and professional survival, science and recognition, mentoring and legacy, the “Water or Warrior” paradigm, and messages for the future. Together, these perspectives offer a global view of shared challenges, career development, and the evolving role of women in congenital interventional cardiology.

Introduction Cardiovascular diseases are the leading cause of maternal mortality within 42 days postpartum in France, representing a significant public health concern. Medical and surgical advancements now enable women with grown-up congenital heart (GUCH) to carry a pregnancy to term. However, recent data on maternal and neonatal morbidity and mortality in this population remain limited. This issue is of particular concern given that nearly two-thirds of cardiovascular-related maternal deaths are deemed preventable. This retrospective multicentric study aims to describe maternal and neonatal outcomes in pregnant women with GUCH. Methods A multicenter, retrospective cohort study included women with GUCH who underwent at least one pregnancy between 2015 and 2024 was conducted in three maternity centers in Marseille, France. Results Ninety pregnant women were included yielding data for 129 pregnancies. The most common GUCH were tetralogy of Fallot (18.6%), ventricular septal defect (17.8%), and aortic coarctation (10.9%). No maternal deaths were observed. Only 6 (4,7%) pregnancies were planned. Twenty-three patients (17.8%) experienced at least one cardiovascular event during the pregnancy or within the year following delivery, with heart failure (8.5%) and arrhythmias (8.5%) being the most frequent. Twenty-one (16.3%) cesarean delivery for cardiac indication were performed, including 12 (9.3%) elective procedures. Preterm delivery (<37 weeks of gestation) occurred in 23 pregnancies (17.8%), of which nine (7%) induced for maternal cardiac indication. Conclusion Women with GUCH face a high risk for cardiac, obstetric, and neonatal complications. Optimal management requires a multidisciplinary approach from preconception through postpartum period, which should be recognized as a “fourth trimester” of pregnancy.

BACKGROUND:Children with complex congenital heart disease (CCHD) are at high risk for early neurodevelopmental delays across all domains. Neuromotor delay often emerges first and may impact broader development. Identifying early biomarkers of motor function could capture a critical window for intervention. We assessed the prognostic value of neuron-specific enolase (NSE) and S100B in predicting 4-month motor outcomes in newborns undergoing cardiac surgery with cardiopulmonary bypass (CPB). METHODS:Between December 2021 and October 2024, we conducted a prospective, single-centre study including term neonates with (CCHD) who required cardiac surgery within the first two months of life. NSE and S100B levels were measured at five perioperative time points. Blinded Alberta Infant Motor Scale (AIMS) assessment at four months evaluated motor outcomes. RESULTS:Of 35 newborns, 27 completed follow-up. Preoperative NSE levels were significantly higher in infants with AIMS scores below the 10th percentile (32.7 vs. 20.9 ng/mL, p = 0.044) and negatively correlated with AIMS percentiles (ρ = -0.617, p = 0.006. There was no significant association between motor outcomes, MRI findings or S100B levels. CONCLUSIONS:Higher preoperative NSE levels predict poor early motor outcomes in CCHD and may be a marker for early risk stratification and intervention. IMPACT:Neuron-specific enolase (NSE) may serve as an early biomarker of neuromotor development in newborns with complex congenital heart disease (CCHD). Elevated preoperative NSE levels were associated with poorer motor outcomes at four months. NSE may serve as an additional biomarker within a multimodal risk stratification strategy, complementing clinical, imaging, and electrophysiological assessments to refine prognostic evaluation. These findings highlight the prognostic value of perioperative biomarkers for predicting early motor outcomes and support earlier identification of at-risk newborns, enabling targeted neurodevelopmental interventions. This work adds new evidence to limited literature on biological predictors of motor development after neonatal cardiac surgery.

Introduction Patients with congenital heart disease are increasingly reaching adulthood, suggesting a growing exposure to complications, including pulmonary hypertension. Despite improved management, the onset of pulmonary hypertension is a turning point in the evolution of the disease and threatens the prognosis of patients. Method This is a prospective, multicenter cohort study from the ITINERAIR registry including children and adults with congenital heart disease and pulmonary hypertension followed from January 2014 to December 2018.We classified patients into three groups according to the existence of a shunt, whether corrected or persistent, with or without Eisenmenger syndrome, in order to identify prognostic factors for each. Results The prognostic factors for major events in our study were a history of right heart failure and saturation level. The specific risk of mortality or heart and/or lung transplantation is determined by right atrial pressure. Conclusion The prognosis of patients with congenital heart disease complicated by pulmonary hypertension can be assessed using simple tests. Cardiac catheterization appears to play an essential role in the evaluation of this population.Further studies with longer follow-up are needed to confirm our findings and lead to recommendations specific to this patient population.

Introduction and objectives: Balloon atrial septostomy (BAS) improves oxygenation in neonates with transposition of the great arteries (TGA) and restrictive foramen ovale. Currently, there is a global shortage of dedicated BAS catheters, while new unmarked catheters have recently become available at some European centers. This study aimed to characterize BAS outcomes using the currently available BAS catheters in Europe. Methods: A 2-year multicenter observational registry was conducted, including all neonates undergoing BAS for TGA. We report preliminary results (September 2022-February 2024) focusing on BAS characteristics and outcomes. Results: A total of 250 BAS procedures were performed in 29 centers. The median neonatal weight was 3.16 kg, and 88% of neonates had a prenatal diagnosis. Most procedures were performed often on the first day of life during working hours (72.8%), mainly in catheterization laboratories (59.2%). Guidance primarily involved ultrasound with or without fluoroscopy. A guidewire was used in 41.2% of procedures. A total of 290 catheters (286 Z-5 or Z-6) were used, achieving an overall BAS success rate of 96%. Complete procedural failure was associated with the use of the umbilical venous route (OR, 3.62; P = .001) and lower-volume catheters (OR, 7.01; P < .001). The occurrence of significant complications (8%; OR, 9.33; P < .001) was associated with complete procedural failure. For complex procedures, significant risk factors were the absence of fluoroscopy (OR, 3.32; P = .001), use of the umbilical venous route (OR, 2.28; P = .005), and lower-volume catheters (OR, 2.43; P = .03). Conclusions: In the current era, BAS can be challenging, and significant complications and complete failures are not uncommon. The use of the umbilical venous route, low-volume BAS catheters, absence of fluoroscopy guidance, and the occurrence of complications negatively impact procedural outcomes. (c) 2025 Sociedad Espa & ntilde;ola de Cardiolog & iacute;a. Published by Elsevier Espa & ntilde;a, S.L.U. This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/).

BACKGROUND:Transcatheter patent ductus arteriosus (PDA) closure can be challenging in cases of large PDA, complex anatomy, and small patient size. We aimed to assess the feasibility, efficacy and safety of the off-label use of the multifunctional occluder in transcatheter PDA closure. METHODS:A retrospective analysis was conducted on patients who underwent transcatheter PDA closure with the multifunctional occluder in 14 pediatric cardiology centers (5 countries) from 2018 to 2025. RESULTS:A total of 77 procedures were performed on 75 patients, including 5 adults (6.7%). Among the 70 children (93.3%; median age: 0.8 years [range, 0.1-10 years], median procedural weight: 6.9 kg [range, 2.1-32.0 kg]), 35 (50.0%) weighed ≤6 kg (median procedural weight: 4.0 kg [range, 2.1-5.9 kg]). The PDA was large, with a minimal ductal diameter of 3.8 mm (range, 1.5-7.0 mm), short in 54.5%, and unrestrictive with increased pulmonary artery pressures in 51.6% of patients. The morphology was predominantly conical (Krichenko type A: 51.9%), with window-type or complex morphologies representing 26.7% (Type B: 18.2%, Type D: 7.8%). Both arterial and venous femoral accesses were obtained in 87.0% cases, with deployment being antegrade in 77.9% and retrograde in 22.1%. Successful device deployment and release were achieved in 74 cases (96.1%). The rate of major adverse events was 5.2% including 1 early (1.3%) and 3 late complications (3.9%). No other adverse events were reported. CONCLUSIONS:The off-label use of the multifunctional occluder device is a promising option for transcatheter PDA closure, demonstrating safety and efficacy in this series.

AIMS:The natural history of congenital or childhood non-immune, isolated atrioventricular block (AVB) is poorly defined. We aimed at clarifying its long-term outcomes. METHODS AND RESULTS:We retrospectively studied 385 children with isolated, non-immune AVB diagnosed from in utero or up to 18 years of age, at 29 French medical centres, between 1980 and 2022. Patients with structural heart disease, endomyocardial fibrosis, or maternal antibodies were excluded. Atrioventricular block was asymptomatic in 314 (81.6%) and complete in 263 (68.3%) patients at the time of diagnosis. There was progression to complete AVB in 84/122 (68.8%) patients with incomplete AVB over 12 years (7-17). A total of 286/385 patients (74.3%) received a permanent pacemaker, implanted in the first year of life in 39 (14%) and before 10 years of age in 172 (60%) children. The pacing indication was prophylactic in 203 children (71%). Genetic screening was performed in 133/385 patients (34.5%), leading to the identification of a clinically actionable variant in 11 (8.3%) patients. After a median follow-up of 10 years (5-17), no patient died or developed endomyocardial fibrosis or dilated cardiomyopathy. CONCLUSION:In this large nationwide study, the long-term outcome of congenital or childhood non-immune, isolated AVB was excellent. Most children required pacemaker implantation over time, albeit often as a prophylactic measure.

Introducción y objetivos La atrioseptostomía con balón (ASB) facilita la oxigenación en los recién nacidos con transposición de grandes arterias (TGA) con foramen oval restrictivo. Ante la escasez mundial de catéteres específicos, se están utilizando nuevos catéteres sin marcado de la Comunidad Europea. El objetivo de este estudio es mostrar los resultados de la ASB con los catéteres actualmente disponibles en Europa. Métodos Estudio observacional multicéntrico de 2 años de los neonatos con TGA tratados con ASB. Presentamos los resultados preliminares (septiembre de 2022-febrero de 2024) describiendo las características y resultados de la ASB. Resultados Se efectuaron 250 ASB en 29 centros. El peso neonatal mediano fue de 3,16kg, y el 88% de los recién nacidos tuvieron un diagnóstico prenatal. La mayoría de las ASB se realizaron principalmente durante el primer día de vida (70,4%), en la sala de hemodinámica (59,2%) y mayoritariamente guiados por ecocardiografía con o sin fluoroscopia. Se utilizó una guía en el 41,2% de los procedimientos. Se utilizaron un total de 290 catéteres (286 Z-5 o Z-6), con los que se logró una tasa de éxito global de la ASB del 96%. El fracaso del procedimiento se asoció con la vía umbilical (OR=3,62; p=0,001), catéteres de bajo volumen (OR=7,1; p<0,001) y complicaciones significativas (8%; OR=9.33; p<0,01). Los factores de riesgo de los procedimientos complejos fueron la ausencia de fluoroscopia (OR=3,32; p=0,001), la vía umbilical (OR=2,28; p=0,005) y los balones de bajo volumen (OR=2,43; p=0,03). Conclusiones Actualmente la ASB puede ser un procedimiento complejo con una tasa significativa de complicaciones y fracaso del procedimiento. El uso de la vía umbilical y de catéteres de bajo volumen, la no utilización de fluoroscopia y la presencia de complicaciones tienen un impacto negativo en los resultados.

In pediatric practice, nalbuphine hydrochloride can be administered by continuous infusion through a multiport manifold or Y-site connection over 24 h in a 60 mL polypropylene syringe unprotected from light at concentrations ranging from 52.1 µg.mL-1 to 333.3 µg.mL-1 in normal saline (NS). To limit the need for multiple injections, nalbuphine hydrochloride may be co-administered with other drugs. Its stability and compatibility were already studied at concentration ranges equal or higher than 1.0 mg.mL-1. However, when nalbuphine hydrochloride needs to be diluted for a pediatric administration its stability in NS over 24 h of light exposure at ambient temperature and its compatibility with other drugs have never been studied before. A novel chromatographic method using high-performance liquid chromatography (HPLC) was validated by the International Council for Harmonisation (ICH) Q2 (R1) guidelines. The stability of nalbuphine hydrochloride and the appearance of degradation products under five experimental conditions (light, heat, oxidation, basicity, and acidity) were monitored by HPLC-UV for 24 h at ambient temperature at three concentrations administered in pediatric departments (52.1 µg.mL-1, 166.7 µg.mL-1 and 333.3 µg.mL-1). The physical compatibility of nalbuphine hydrochloride with 1:1 (v/v) mixtures of selected drugs used in pediatrics was evaluated by visual inspection and with a 10 µm and 25 µm sub-visible particle counter. Our nalbuphine hydrochloride quantification method has been validated and was stability-indicating. The stability of nalbuphine hydrochloride and the forced degradation assay (light, heat, oxidation, basicity, and acidity) studied for the three concentrations of nalbuphine hydrochloride diluted in 48 mL of NS and stored in a 60 mL polypropylene syringe unprotected from light were compliant for at least 24 h at ambient temperature. Nalbuphine hydrochloride in NS was found to be compatible with several drugs, but was incompatible with furosemide and amphotericin B. Nalbuphine hydrochloride can be administered in pediatric practice using a syringe pump for 24 h. However, drug-drug incompatibilities need to be considered when it is administered through a multiport manifold or Y-site connection.

Introduction and objectives: Transcatheter patent ductus arteriosus (PDA) closure is safe in   2-kg infants and in ≥ 6-kg patients, but major safety concerns remain when applied to the intermediate weight range. We aimed to assess outcomes of transcatheter PDA closure in 2- to 6-kg infants.Methods: An international, multicenter, retrospective cohort study was conducted in 31 tertiary hospitals in 17 countries between 2000 and 2023, investigating all infants who underwent attempted transcatheter PDA closure with a procedural weight of 2-to-6 kg.Results: Attempted transcatheter PDA closure was performed in 1231 infants (median [Q1-Q3] weight, 4747 [3700-5300] g; median age, 132 [83-194] days; ex-preterm, n = 581 [56.8%]) with a 95.0% success rate. A composite outcome of procedural failure or major adverse events was observed in 173 (14%) patients, including device embolization in 64 (3.7%), device-induced left pulmonary artery stenosis in 47 (2.7%), and procedural death in 2 (0.2%). Logistic regression model analysis identified a 2- to 3.9-kg procedural weight, increased pulmonary artery pressure, and window-type or tubular ductal morphologies as independent predictors of the composite outcome. Based on propensity score matching analysis, 2- to 3.9-kg infants had a risk ratio of 2.19 (95%CI, 1.25-3.83) for experiencing the composite outcome, compared with 4- to 5.9-kg infants.Conclusions: Transcatheter PDA closure in 2- to 6-kg infants was feasible in most patients. Procedural failure or major adverse events occurred in 14% and several independent risk factors were identified, including the 2- to 3.9-kg weight range identified as a higher-risk subgroup. These findings may improve risk stratification and the decision-making process.

BACKGROUND:A widely accepted protocol for the diagnosis and follow-up of Fontan Associated Liver Disease (FALD) is currently lacking, thus we undertook a European survey with the dual aim of examining the current status quo and developing a shared screening algorithm. METHODS:In this cross-sectional qualitative study, 39 Adult Congenital Heart Disease (ACHD) centres in Europe were invited (between August 2023 and April 2024) to participate in a survey regarding their approach to FALD diagnosis and surveillance. RESULTS:Twenty-two (54.5 %) centres from 11 Countries responded to the survey. Patients with a Fontan circulation had annual liver assessment in the majority of centres, with review of selected case by a specialist hepatologist. Different scoring systems were employed to quantify the severity of FALD (Child-Pugh score, MELD XI, FIB4). Ultrasound was the first-line imaging modality, followed by magnetic resonance imaging and computed tomography. Liver fibrosis and portal hypertension were assessed with different modalities and at variable time intervals ranging from 1 to 3 years, with the most used technique being the Fibroscan (74 %). Half of the centres would consider transplant referral in the presence of FALD. Combined heart-liver transplantation was an option in 8 centres. CONCLUSIONS:Our data suggests universal agreement amongst European ACHD centres on the need for regular assessment of adult patients for FALD, often with the involvement of a hepatologist, though the approaches varied widely between centres. We propose herewith a FALD surveillance algorithm to introduce a uniform approach to this complex entity which impacts on the growing number of patients with a Fontan circulation.