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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.

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). 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

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 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.

Aim 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. Methods 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). Results 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. Conclusion 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.

Background Management of patients with complex congenital heart defects (CHD) requires 3D imagination skills for the spatial evaluation of cardiac structures. Current 3D imaging [echocardiography, MRI, computed tomography (CT)], projected on a 2D screen, limits the appreciation of the relief and the interaction of anatomical structures. Purpose The contribution of 3D stereoscopic relief imagery (3D S/R), built by triangle-based mesh generation, is more qualitative than quantitative, which is why its use, rarely reported in the literature, must be evaluated. Methods 3D S/R was used from 2009 to 2019, as much as the CT made it possible to define a Region of Interest. Data and segmentation processing have been used to obtain four different types of reconstructions: MultiPlanar (MPR), Volume Rendering Technique (VRT), Surface Rendering Technique (SRT), and 3D S/R. The anaglyph 3D S/R effect requires the wearing of glasses (red - cyan blue filters) distributed during the session ( Fig. 1 ). Five complex CHD will be presented in MPR, VRT, then in SRT and 3D S/R: – Ventriculo-Arterial Malposition, – multiple Ventricular Septal Defects, – Anomalous Aortic Origin of a Coronary Artery, – Coarctation and hypoplastic Aortic Arch, – abnormal Pulmonary Arterial Arborization with Major Aorto-Pulmonary Collateral Arteries. Results The qualitative evaluation [MPR + VRT versus SRT and 3D S/R] will be carried out by questions/answers [Wooclap™ interactive system - voting on smartphone, in streaming, using a code associated with the presentation], allowing a global rating of the five CHD, concerning: – the perception of the relief, – the understanding of the cardiopathy, – the probability of appropriation of this 3D S/R imagery. Conclusions This presentation will allow: – to assess the potential influence of 3D S/R on the management strategy of CHD, – to compare these imaging studies with mesh generation techniques leading to the more commonly reported 3D printing.

Objective Percutaneous closure of interatrial communications (ASD) has become the standard for therapeutic management. However, some patients are ineligible for this technique due to the morphology of the ASD, thus requiring conventional surgical closure by sternotomy or lateral thoracotomy. Minimally invasive surgery (MIS) has been introduced in several adult heart surgery centers especialy for mitral surgery. The purpose is to present the preliminary experience of ASD closure by MIS. Material and methods We analyzed all of the patients (n = 5) treated from March 2019 (start date of the MIS program) to December 2019. The MIC was performed by an anterior mini thoracotomy of approximately 5 cm with femoral cannulation ( Fig. 1 ). The pre, per, and postoperative data were prospectively collected. All patients underwent postoperative vascular Doppler ultrasound of the femoral cannulation site. Results Average age at intervention was 27.3 years (range: 14.5–41), weight was of 60 kg (43–80) (4 women for 1 man). The median duration of CPB was 108 min (101–139), the duration of aortic cross clamping 45 min (36–61). There were no hospital deaths or major complication. One patient had a pneumothorax on D3. The median duration of extubation was 3.5 hours (3–6), the median duration of hospitalization was 8 days (6–11). Postoperative echocardiography did not show any residual ASD or pericardial effusion. All patients were sinus rhythm. No venous or arterial stenosis was observed at the cannulation sites. The average systolic pulmonary arterial pressure, was measured 34 mmHg (15–74) preoperatively, and estimated 30 mmHg (20–55) postoperatively. Conclusion This clinical study shows the preliminary experience of MIS program for the closure of ASDs. This approach appears to be safe and effective, without major vascular complications. longer term follow-up is required to determine the specific role of MIS approach in the management of this patient population.

Introduction Patent ductus arteriosus (PDA) is common in preterm infants and contributes to prolonged mechanical ventilation, renal failure, necrotizing enterocolitis and periventricular leukomalacia. Several studies have shown the feasibility and safety of percutaneous PDA closure. Minimally invasive surgical ligation by anterior thoracotomy is an alternative, bedside technique for PDA closure in extremely low birth weight preterm infants. Purpose Our study aimed to compare short-term morbi-mortality between surgical PDA ligation by anterior mini-thoracotomy and transcatheter PDA closure. Methods From 2010 to 2020, 92 preterm babies weighing Results Preoperative characteristics were similar between the 2 groups after propensity matching (mean weight at procedure, 1171 ± 183 gr; P = 0.8). PDA closure was successful in all cases, except 1 in the transcatheter group. Mean time to extubation was similar: 10 ± 15 days in group 1 versus 9 ± 13 d. in group 2 (P = 0.9). Mean non-invasive ventilation duration was similar (P = 0.96). Mean age at hospital discharge was 114 ± 29 days in group 1 versus 105 ± 19 d. in group 2 (P = 0.2). 2 deaths occurred in group 1 (9%) and 1 in group 2 (4.5%) (logRank = 0.61). 5 complications (pneumothorax n = 2, chylothorax n = 2, phrenic nerve injury n = 1) occurred in 3 patients in group 1 (13%). 3 complications (chylothorax n = 1, endocarditis n = 1, renal vein thrombosis n = 1) occurred in 2 patients of group 2 (9%) (P = 0.63). Conclusion Equivalent efficiency and safety of surgical mini-invasive versus transcatheter PDA closure in extremely low birth weight preterm infants are in favour of applying these alternative techniques according to center's facilities and competences.

Objective 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. Methods 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. Results 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 ( Conclusions 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).

Background Extracorporeal membrane oxygenation (ECMO) has become a gold standard in treatment of severe refractory circulatory and/or pulmonary failure. The aim of this work is to give key points for pediatric mobile ECMO team implementation and to demonstrate its safety and feasibility, even in a middle size pediatric cardiac surgery reference center. Methods This is a retrospective analysis of our series of patients initiated on ECMO in a peripheral center and transferred to our center was conducted. Our data were collected from 10 consecutive years: from 2006 to 2016. Results Fifty-seven pediatric patients were cannulated in peripheral center and transported on ECMO. Forty-two patients (73,7%) were weaned from ECMO. We did not experience any adverse event during transport. Up to 40% of our population were neonates (age Conclusion Offsite ECMO Implantation and transport of pediatric patients on ECMO can be safe, when performed by a dedicated and experienced team.

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.

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?