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.

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.

BACKGROUND:The arterial switch operation has become the treatment of choice for neonates with transposition of great arteries. AIM:This systematic review and meta-analysis aimed to summarize the current evidence regarding neoaortic root dilatation and aortic valve insufficiency as long-term outcomes after the arterial switch operation, and to identify associated risk factors. METHODS:A systematic search of PubMed, EMBASE and Cochrane databases was conducted for human studies published in English or French until May 2024. RESULTS:Out of 1480 identified records, 80 studies were included in the final analysis. The total incidence of moderate-to-severe aortic insufficiency was 588 out of 20,338 patients (2.89%). The total number of neoaortic reinterventions was 306 in 11,291 patients (2.71%). The total number of neoaortic reinterventions for aortic insufficiency was 78 in 4757 patients (1.63%). Distensibility of both the aortic root and the ascending aorta in patients after the arterial switch operation was significantly lower than in the normal population. Several factors were significantly associated with an increased risk of moderate-to-severe aortic insufficiency: ventricular septal defect (P<0.001); pulmonary artery banding (P=0.007); aortopulmonary mismatch (P<0.001); and neoaortic root dilatation (P<0.001). Coronary anomalies showed a borderline association (P=0.055), and the trapdoor technique was significant in one study (P=0.0005). Distensibility of both the aortic root and the ascending aorta was found to be significantly reduced in patients after the arterial switch operation compared with healthy controls, reflecting increased vascular stiffness. CONCLUSIONS:Significant neoaortic valve insufficiency and reintervention remain relatively rare after the arterial switch operation. However, measurable risk factors-including ventricular septal defect, pulmonary artery banding, aortopulmonary mismatch and coronary anomalies-are associated with an increased long-term risk of valve dysfunction or surgical reintervention.

Introduction To evaluate the long-term outcomes of the Ross/Ross-Konno procedure in patients under 16 years of age compared to those over 16, with a focus on the hemodynamic performance of the pulmonary autograft. Method A retrospective analysis was conducted on patients operated between 1992 and 2024. Data were collected from hospital records, with complete follow-up for 90% of patients (10% lost to follow-up abroad). Two groups were formed: children (0–16 years) and adults (>16 years). The primary endpoint was survival. Results Among the 171 patients included, 83 were under 16 years old (median age: 8.5 years) and 88 were adults (median age: 26.4 years). Before surgery, 36.5% of children had aortic stenosis, 26.5% had regurgitation, and 37% had mixed disease, compared to 34%, 37.5%, and 28.5% respectively in adults. Early mortality was 3.6% in children and 1.1% in adults. Seven late deaths were recorded. Twenty-year survival was 87% in patients under 16 and 96% in adults (P=0.2) (Figure 1). Freedom from autograft reoperation at 20 years was 84% in children versus 82% in adults (P=0.67). However, reinterventions on the right ventricular outflow tract were more frequent in younger patients (47% vs. 82% at 20 years, P<0.001). At the end of follow-up, the diameter of the sinus of Valsalva was 37.7±9mm in children compared to 39.7±7mm in adults. Conclusion The Ross procedure provides good autograft growth in younger patients, with performance comparable to adults. However, patients under 16 require more frequent reinterventions on the pulmonary homograft.

Introduction The main risk factors of necrotising enterocolitis (NEC) are prematurity and low birth weight. The aim of our study was to identify risk factors for NEC in patients with duct-dependent congenital heart disease (CHD).Study design Newborns with duct-dependent CHD and NEC were matched 1:1 to those without NEC. Matched criteria were gestational age, birth weight, antenatal versus postnatal diagnosis and type of CHD.Results Twenty-three infants were included in each group. In the NEC group, mortality, length of intensive care unit stay and length of hospital stay were significantly higher (p=0.035; p<0.0001; p<0.0001). Lower diastolic blood pressure (DBP), negative flow balance, peritoneal dialysis and epinephrine-infusion were significantly associated with NEC (respectively, p=0.008, p=0.002, p=0.007, p=0.017). In multivariate analysis, DBP≤30 mm Hg remained the only independent risk factor of NEC (OR=8.70; 95% CI (1.46 to 53.50), p=0.019).Conclusion A DBP lower than 30 mm Hg was in our matched population of newborns with duct-dependent CHD, independently associated with NEC.

BackgroundMore than half of infants with complex congenital heart disease (CHD) will have a neurodevelopmental disorder of multifactorial causes. The preoperative period represents a time-window during which neonates with complex CHD are in a state of hypoxia and hemodynamic instability, which fosters the emergence of brain injuries and, thus, affects early brain networks and neurodevelopmental outcomes. Currently, there is no consensus regarding the optimal age for cardiac surgery in terms of neurodevelopmental outcomes, and its definition is a real challenge. Our aim is to determine the relationship between cardiac surgical timing and long-term neurodevelopmental outcomes for various types of complex CHD.MethodsWe hypothesize that earlier surgical timing could represent a neuroprotective strategy that reduces perioperative white matter injuries (WMIs) and postoperative morbidity, leading to improved neurodevelopmental outcomes in infants with complex CHD. Firstly, our prospective study will allow us to determine the correlation between age at the time of surgery (days of life) and neurodevelopmental outcomes at 24 months. We will then analyze the correlation between age at surgery and (i) the incidence of WMIs (through pre- and postoperative MRIs), (ii) postoperative morbidity, and (iii) the duration of the hospital stay.Implications and DisseminationThis research protocol was registered in the Clinical Trial Registry (National Clinical Trial: NCT04733378). This project aims to help launch the first Neurocardiac Investigation Clinic in Marseille — AP-HM — to propose an overall personalized monitoring and treatment program for patients operated on for complex CHD.

Transposition of the great arteries (TGA) associated with ventricular septal defect (VSD) could present an intracardiac anatomy making the neonatal repair very challenging: multiple/apical VSD, straddling, tortuous intraventricular tunnel repair. To delay the repair, the isolated pulmonary artery banding (PAB) has the disadvantage to endanger the future aortic valve and to create a double obstacle in the frequent association with aortic annulus and arch hypoplasia. We propose a 2-step strategy with an initial palliative arterial switch operation (ASO) associated with PAB (± aortic arch repair) and, later, an intracardiac repair with debanding. The Aim of this retrospective study is to compare this strategy to a classic isolated PAB. Among the 715 neonatal TGA admitted in our institution between 2007 and 2018, 10 complex TGA benefited either from a palliative ASO with PAB (group A, n = 5) or a PAB (group B, n = 5). At palliation, the duration of inotropic support and the length of stay in intensive care unit were significantly shorter in group A (respectively 4.75 ± 1.7 and 5.5 [4.25–6.75] days versus 13 ± 6.9 and 16 [10.5–30] days in group B; P = 0,029 and P = 0,008). At complete repair, age and weight were significantly higher in group A (P = 0,018) and the aortic cross-clamping and bypass times were shorter (P = 0,018). No patient required a delayed sternal closure in group A. Mean length of stay in intensive care unit and hospital duration were respectively of 2 ± 1 days and 5.6 ± 1.53 days in group A versus 21.4 ± 16.9 days and 24.5 ± 19.9 days in the group B (P = 0.018 and P = 0.029), showing reduced morbidity. Differing the neonatal repair for TGA is exceptional. When we should consider it due to particular intracardiac anatomy, it seems legitimate to practice a palliative ASO rather than the classic isolated PAB.

Abnormal origin of left coronary artery from the pulmonary artery (ALCAPA) is one of the most common causes of myocardial ischemia and infarction in childhood. This study aimed to determine the correlation between age at clinical presentation, level of ventricular dysfunction, and post-repair outcomes. This is retrospective study from 1993 to 2018 including thirty-one patients. The study cohort was divided into two groups according to age (< 6 months, > 6 months). The significance level was set at p 0.05.The median follow-up time was 72 [24–168] months. Median age was 4.7 [2.3–16] months. Median weight was 6.2 [4.3–9] kg. There was severe left ventricular (LV) dysfunction (ejection fraction < 35%) in 64.5% of patients. Mitral regurgitation (MR) was moderate to severe in 13 patients (41.9%). Two patients (6.4%) required extracorporeal membrane oxygenation (ECMO) support before surgery and 6 (19.4%) after correction. Age < 6 months was significantly associated with severe clinical presentation, severe LV dysfunction, delayed sternal closure, prolonged respiratory mechanical support, and prolonged length of ICU stay (p = 0.024, p = 0.042, p = 0.002, p = 0.042, p = 0.022, respectively). After surgery, ejection fraction improved to a median of 57% [50.7–60.5]. MR regressed in 12 patients (92.3%). Mortality rate after surgery was 9.7%. All patients were free from reoperation at the last follow-up. Young age at diagnosis was significantly associated with a more severe clinical presentation and poorer outcomes. After re-establishment of a two-coronary circulation, both ventricular function and MR tend to normalize over time regardless of age at repair.

Melody valve insertion in mitral position is no longer an experimental procedure and is now an acceptable option for severe mitral valve disease in infants and neonates. Although there are more and more publications outlining the outcome, our first and successful case taught us a couple of points that we would like to share. A 20 days-old baby with severe congenital mitral insufficiency underwent Melody valve implantation. The stent was fashioned to avoid left ventricular outflow tract obstruction and expanded with a 14 mm balloon. A small ASD was created to allow further percutaneous balloon dilation. The baby was discharged after 6 weeks and treated with antiplatelet therapy. Postoperative management consisted in close transthoracic echocardiographic follow-up. Two percutaneous balloon dilatations of the stent (14 and 16 mm) were performed, respectively 9 and 16 months after the initial surgery. The patient presented infective endocarditis (Haemophilus influenzae) 26 months after surgery. TTE showed moderate Melody valve regurgitation and stenosis (mean gradient: 11 mmHg) with post-capillary pulmonary hypertension. Mitral valve replacement was performed after 6 weeks of antibiotic therapy. The stent was explanted and a 19 mm mechanical prosthetic valve was inserted. Postoperative course was uneventful. Melody valve in mitral position is now an accepted option but morbidity and mortality remain significant. Post-implantation management is challenging and guidelines are missing. Percutaneous stent dilation, hybrid valve in valve implantation and mechanical mitral valve replacement are 3 possible options in case of valve dysfunction. The per-operative pictures (Fig. 1, Fig. 2) help to understand why, in our case, the first 2 options would have failed. Fig. 1 shows non-calcified and mobile leaflets. One leaflet had a small 2 mm perforation. The stent itself, in contrast, was so thickened that dilatation or valve in valve procedure would have failed at this stage. Major endothelialization of the stent might significantly limit post-implantation management options. We believe that heart teams have to be aware of this potential evolution of the stent in order to establish the best procedural strategy in these patients.

BACKGROUND:Berlin Heart EXCOR (BH) ventricular assist devices provide mechanical long-term circulatory support in children with end-stage heart failure, as a bridge to transplantation or to recovery. Most studies are from large-volume paediatric cardiac centres.AIM:The aim of this study was to analyse the experiences of three French centres and to compare these with available published data.METHOD:We performed a retrospective observational study of three paediatric cardiac intensive care units. All children supported with BH devices were included. Morbidity and mortality data were collected and risk factors analysed.RESULTS:Fifty-four (54) patients (54% male) were included. Survival rate was 73% while on a BH device. Median age at BH device implantation was 17 months (range 2-180 months). The predominant indication was dilated cardiomyopathy (61%). Bi-ventricular assist device was used in 25 (46%) cases. The total length of long-term circulatory support was 3,373 days, with a mean length per patient of 62.5 days (range 5-267 days). Thirty-two (32) patients were transplanted (59%) and seven (13%) were successfully weaned. Type and length of support did not influence morbidity. Main complications were renal dysfunction (57%), bleeding (41%), and infection (39%). In multivariate analysis, a weight <5 kg was significantly associated with higher mortality.CONCLUSIONS:The weight seems to be the most important risk factor of mortality in this precarious condition.

Objectives: Extracorporeal membrane oxygenation has become a gold standard in treatment of severe refractory circulatory and/or pulmonary failure. Those procedures require gathering of competences and material. Therefore, they are conducted in a limited number of reference centers. Emergent need for such treatments induces either hazardous transfers or a mobile pediatric extracorporeal membrane oxygenation team able to remote implantation and transportation. The aim of this work is not to focus on pediatric extracorporeal membrane oxygenation outcomes or indications, which have been extensively discussed in the literature. This study would like to detail the implementation, safety, and feasibility, even in a middle-size pediatric cardiac surgery reference center. Patients: This is a retrospective analysis of a series of patients initiated on extracorporeal membrane oxygenation in a peripheral center and transferred to a reference center. The data were collected from 10 consecutive years: from 2006 to 2016. Results: A total of 57 pediatric patients with a median weight of 6.00 (3.2-14.5) kg and median age of 2.89 (0.11-37.63) months were cannulated in peripheral center and transported on extracorporeal membrane oxygenation. We did not experience any adverse event during transport. The outcomes were comparable to our literature-reported on-site extracorporeal membrane oxygenation series with 42 patients (74%) weaned from extracorporeal membrane oxygenation and a 30-day survival of 60%. Neither patient's age nor weight, indication for extracorporeal membrane oxygenation or length of transport, was statistically significant in terms of outcomes. Conclusion: Offsite extracorporeal membrane oxygenation implantation and ground or air transport for pediatric patients on extracorporeal membrane oxygenation appeared to be safe when performed by a dedicated and experienced team, even within a mid-size center.

Introduction: Medical and para-medical education is one of the key points of healthcare strategy. Training and education based on high-fidelity simulation is one of the gold standards in modern healthcare institutions. We describe a model of training dedicated to ICU nurses in charge of patients with ECMO. The aim of our educational tool was to teach ICU nurses ECMO basic knowledge and skills. Methods: An ECMO Specialist Course Committee implemented the training programme. It was on two consecutive days and consisted of theoretical, practical and high-fidelity, simulation-based teaching. A content expert implemented each scenario and learning objectives were defined. Participants were assessed pre- and post-test (Group 1 and Group 2). Results: In two years, seven sessions took place and 40 volunteers were enrolled. High-fidelity, simulation-based teaching consisted of seven scenarios. There was a significant improvement in mean score between pre- and post-test. Moreover, we noticed that the basic level (pre-test) of participants was improving over the time. The mean pre-test scores of Group 2 were significantly higher than Group1. Conclusion: The implementation of education and training course for ICU nurses in charge of patients on ECMO is feasible and reliable. It improves nurse personal levels, but also shares in improving the global level of the team to which they belong.

Abnormal origin of coronary artery from the pulmonary artery (ACAPA) is one of the most common causes of myocardial ischemia and infarction in childhood. Immediate surgical correction can provide excellent results. This study aimed to determine the surgical outcome of ACAPA. From 1993 to 2016, 31 consecutive patients underwent coronary re-implantation. Concomitant mitral valvuloplasty was performed in two. The study cohort was divided into two groups according to age (group 1: ≤ 6 months and group 2: > 6 months). Data from demographic characteristics, electrocardiography, echocardiography, surgery, intensive care unit stay and follow-up were evaluated. The study included 29 patients with anomalous left coronary artery from the pulmonary artery and 2 with anomalous right coronary artery from the pulmonary artery. The median age at repair was 4.75 [2.3–16.3] months and the median weight was 6.2 [4.3–9.4] kg. Preoperative echocardiography showed severe LV dysfunction in 56.7% of patients. The median preoperative LVEF was 33.5% [20–55]. Mitral regurgitation (MR) was moderate to severe in 13 patients (41.9%). Requirement to extracorporeal membrane oxygenation (ECMO) support systems was necessary in 2 cases (6.4%) before surgery and 5 cases (16.1%) after correction. Postoperative mortality rate was 9.7%. Younger age (< 6 months) was significantly associated with severe clinical presentation, severe LV dysfunction, requirement to ECMO, ICU stay, and mortality (P = 0.007, P = 0.011, P < 0.0001, P < 0.0001, P < 0.0001 respectively). The median follow-up time was 72 [36–168] months. Six months after the operation (n = 28), ejection fraction improved to a median of 59% [54–65]. Recovery was more progressive (30 months) in one patient. MR regressed in 12 patients (92.3%). There were no early or late reoperations. Young age is significantly associated with a more severe presentation, more ECMO requirement and mortality. Lesser development of coronary collateral circulation can explain these findings. After establishment of a two-coronary circulation both ventricular function and MR tend to be normalized over time. This ‘recovery’ usually occurs within 6 months but may require more time (maximum of thirty months in our population).

A three-year-old boy was referred for persistent arterial duct. Transthoracic echocardiography showed a right aortic arch and an unusual Doppler flow in the arch vessels and the pulmonary artery. The tomodensitometry showed a right-sided aortic arch, with successive origin of the right common carotid, the right subclavian artery, and an aberrant (lusoria) left subclavian artery. The left common carotid took origin from the pulmonary trunk. During surgery, a fibrous cord independent from the anomaly was identified. An end-to-side anastomosis between the left carotid and the ascending aorta was done and the fibrous cord was divided. Was this fibrous cord a ductal ligament?

Surgical approach for persistent ductus arteriosus ligation is typically a left lateral thoracotomy opening the pleural-space with left lung retraction. We describe an alternative approach, with a minimally invasive anterior parasternal incision. This is particularly adapted to preterm infants weighing less than 1.5 kg. This approach ensures a good exposure of vessels. We believe that it is safe, reliable and reproducible. The learning curve should not be an issue for surgeons used to manage low weight patients.

Objective: To report our experience with perventricular closure of muscular (apical) ventricular septal defects (VSDs) in small infants, with echocardiographic guidance only, in a nonhybrid suite. Methods: Eight infants with nine large muscular (apical) VSDs underwent perventricular device closure in a nonhybrid operating room, with transesophageal and epicardial echocardiography guidance, at a mean age and weight of 3.07 (0.3-7.28) months and 3.7 (2.5-6.2) kg, respectively. Five patients had multiple VSDs. Four had associated cardiac defects. Results: Nine Amplatzer muscular VSD devices with a mean size of 10 (4-14) mm were deployed. Seven patients were discharged from the intensive care unit with a mean length of stay of 8.6 days. Four patients had minimal postprocedural residual shunt; no one had a residual shunt at six-month follow-up. Mid-term results are excellent. Conclusion: Perventricular closure is feasible under echocardiographic guidance only in small patients, even without hybrid suite. This may be a good approach for very symptomatic low-weight infants with apical VSD and may also be useful, in any center, at any time, and in any operating room, to treat an associated apical VSD, even unexpected.