Introduction Pulmonary valve (PV) preservation during Tetralogy of Fallot (TOF) repair remains challenging, particularly in patients with small pulmonary annuli. We previously described a standardized and reproducible pulmonary valve–sparing technique based on conservative management of the native valve to preserve its growth potential. We report the mid-term outcomes of the same cohort with extended follow-up. Methods Between July 2015 and December 2019, 23 consecutive children with regular TOF underwent repair using this technique in a single centre. Median age at surgery was 7 months (range: 4.5–150.4; IQR: 4.3) and median weight was 6.7kg (range: 4.8–24.0; IQR: 2.5). Nineteen patients (82%) had a bicuspid pulmonary valve. Median pulmonary annulus diameter was 7.0mm (range: 5.0–14.0; IQR: 2.5), with a median z-score of −2.5 (range: −4.9 to −0.02; IQR: −1.6). The technique combines T-shaped infundibulotomy to release the anterior annulus, extensive commissurotomy following ventricular septal defect closure, and right ventricular outflow tract (RVOT) remodeling using a shield-shaped bovine patch attached to the annulus, creating systolic traction. Results At discharge, all patients had pulmonary regurgitation (PR) less than moderate. After a median follow-up of 60.9 months (range: 8.4–105.1; IQR: 32.6), the median maximal gradient was 21.0mmHg (range: 9.0–46.2; IQR: 11.0), with only one patient having a gradient above 31.0mmHg (46.2mmHg), and all others below 31.0mmHg. At the latest follow-up, severe PR was observed in 4 patients (17%) and moderate PR in 2 patients (8%), while the remaining patients had PR less than moderate. No patient required surgical or transcatheter reintervention on the RVOT. Conclusion This pulmonary valve–sparing approach demonstrates stable mid-term hemodynamic performance despite small annular dimensions at repair. Although progression of PR was observed in a subset of patients, gradients remained acceptable and no RVOT reintervention was necessary.

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.

Purpose: Currently, nearly 90% of patients with congenital heart disease (CHD) reach adulthood in relatively good health. Structured transition programs have emerged to support adolescents and young adults in transitioning to adult care structures, improve their autonomy, and limit healthcare ruptures. The TRANSITION-CHD randomized controlled trial aimed to assess the impact of a transition program on health-related quality of life (HRQoL) in adolescents and young adults with CHD. Methods: From January 2017 to February 2020, 200 subjects with a CHD, aged 13-25 years, were enrolled in a prospective, controlled, multicenter study and randomized in two balanced groups (transition program vs. standard of care). The primary outcome was the change in PedsQL self-reported HRQoL score between baseline and 12-month follow-up, using an intention-to-treat analysis. The secondary outcomes were the change in disease knowledge, physical health (cardiopulmonary fitness, physical activity), and mental health (anxiety, depression). Results: The change in HRQoL differed significantly between the transition group and the control group (mean difference = 3.03, 95% confidence interval (CI) = [0.08; 5.98]; p = .044; effect size = 0.30), in favor of the intervention group. A significant increase was also observed in the selfreported psychosocial HRQoL (mean difference = 3.33, 95% CI = [0.01; 6.64]; p = .049; effect size = 0.29), in the proxy-reported physical HRQoL (mean difference = 9.18, 95% CI = [1.86; 16.51]; p = .015; effect size = 0.53), and in disease knowledge (mean difference = 3.13, 95% CI = [1.54; 4.72]; p < .001; effect size = 0.64). Discussion: The TRANSITION-CHD program improved HRQoL and disease knowledge in adolescents and young adults with CHD, supporting the generalization and systematization of similar preventive interventions in pediatric and congenital cardiology. (c) 2024 Society for Adolescent Health and Medicine. All rights are reserved, including those for text and data mining, AI training, and similar technologies.

Background: The prognosis of patients with a functional single ventricle has improved, with better car-diopulmonary fitness, health-related quality of life and survival. Conventional echocardiography remains the first-line technique in single ventricle follow-up. Three-dimensional (3D) echocardiography has shown recent value in congenital cardiology, but its ability to predict functional status in patients with a single ventricle remains unknown.Aim: To evaluate, in patients with a single ventricle, the association between 3D echocardiography variables and functional status determined by cardiopulmonary fitness.Methods: Children and adults with a functional single ventricle were prospectively enrolled in this mul-ticentre study. Cardiopulmonary fitness was assessed by cardiopulmonary exercise test, with measures of maximum oxygen uptake (VO2max) and ventilatory efficiency (VE/VCO2 slope). 3D echocardiography was performed with off-line reproducibility analyses, using TomTec ArenaTM software. Health-related quality of life was assessed using the SF-36 questionnaire.Results: A total of 33 patients were screened, and 3D echocardiography analyses were feasible in 22 sub-jects (mean age 28 +/- 9 years). 3D echocardiography ejection fraction correlated with percent-predicted VO2max (r = 0.64, P < 0.01), VE/VCO2 slope (r = -0.41, P = 0.05), two-dimensional echocardiography ejec-tion fraction (r = 0.55, P < 0.01) and health-related quality of life physical functioning dimension (r = 0.56, P = 0.04). 3D echocardiography indexed end-systolic volume correlated with percent-predicted VO2max (r = -0.45, P = 0.03) and VE/VCO2 slope (r = 0.65, P < 0.01). 3D echocardiography reproducibility was good.Conclusions: Single ventricle ejection fraction and volumes measured by 3D echocardiography correlated with cardiopulmonary fitness, as determined by two main prognostic cardiopulmonary exercise test vari-ables: VO2max and VE/VCO2 slope. Despite good reproducibility, 3D echocardiography feasibility remained limited. 3D echocardiography may be of value in single ventricle follow-up, provided that the technique and analysis software are improved.(c) 2023 Elsevier Masson SAS. All rights reserved.

Abstract This new and easily reproducible pulmonary valve-sparing technique for the correction of Tetralogy of Fallot is based on a conservative management of the native pulmonary valve to preserve its growth potential. From July 2015 to December 2019, 67 children presenting with a Tetralogy of Fallot were operated consecutively in a single centre using this technique in all cases. A T-shaped infundibulotomy is used to release the anterior pulmonary annulus from any muscular attachment. After myocardial resection and ventricular septal defect closure, an extensive commissurotomy is achieved. Finally, the right ventricular outflow tract remodelling is completed by a shield-shaped bovine patch with an oversized square superior edge, attached directly on the pulmonary valve annulus, with an effect of systolic traction. Sixty patients (89.5%) had a Tetralogy of Fallot repair with preservation of the pulmonary valve. To date, with a median follow-up of 38.2 [14–64] months, no patient has needed a surgical or interventional procedure for pulmonary valve stenosis or regurgitation, with low residual gradients. This procedure could provide a significant increase in native pulmonary valve preservation. Long-term studies are needed to assess pulmonary valve growth and the consequent reduction in surgical or interventional reoperations.

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

Residual mitral valve regurgitation is frequent after surgical repair of atrioventricular septal defect (AVSD). We aimed to assess residual mitral valve regurgitation (MVR) severity after surgical repair of AVSD and to correlate it with residual cleft assessed by 3D transthoracic echocardiography (TTE). We included 20 patients who underwent a 3D TTE after surgical repair of AVSD. MVR severity was evaluated on TTE by two different operators. The length of the residual mitral cleft was assessed compared to the total anterior leaflet depth using a multiplanar reformatted (MPR) mode from 3D TTE dataset. The median age at surgery was 1.5 years old (range, 1 month–8 years). Seven patients had ventricular septal defect, including 2 Down syndrome. All patients except one had surgical complete closure of the cleft. Eleven patients had an associated cardiac procedure and commissuroplasty was performed in 4 patients. The median follow-up period was 6.1 years (range, 0.3–20years). The mean MVR severity was mild (1.1; range, 0-3) without significant stenosis. 3D analysis was feasible in 18 patients whom 14 had residual cleft. Residual cleft length was 16.2 mm (range, 0mm–46 mm). Percentage of residual cleft related to the total anterior leaflet depth was 9.5% (range, 0%-41.6%). Correlation between the residual cleft and MVR severity was very good (R = 0.82; P < 0.0001). One patient had mild regurgitation due to dysplastic leaflet and no residual cleft (Fig. 1). Residual cleft assessed by 3D TTE is common after AVSD and correlated to MVR severity. Even after a complete repair, the AVSD may be incompletely corrected, due to either a partially sutured cleft or a dehiscence of the leaflet sutures. The long-term prognosis of the left atrioventricular regurgitation has to be assessed.

Understanding of cardiac structure has been improved by 3D cardiac echocardiography. The anatomy and geometry of the mitral valve annulus (MVA) has been previously described with advanced imaging techniques, allowing for a better comprehension of valve dysfunction and providing significant information for the surgical repair. To apply transthoracic 3D echocardiography (3DTTE) for quantitative and qualitative assessment and comparison of the pathologic MVA in congenital cardiopathies (CC) of the valve with a normal group. Patients with repaired atrioventricular septal defect (AVSD) attending consultation were prospectively included and matched with a control group of healthy children by sex, age and body surface area (BSA). 3DTTE full volume and 3D zoom acquisitions were performed on a Philips EPIQ7. The 3D shape of the annuli was reconstructed and analysed with TOMTEC™ 4D MV-ASSESSMENT© (Fig. 1). The assessed and compared parameters were annular area (2D & 3D), circumference, high-low distances (height), anterolateral-posterolateral (ALPM), and anteroposterior (AP) axes, non-planar angle (NPA), sphericity index, angle between the aortic annulus and AP axe (AAoAP angle). All measurements were indexed by BSA. A total of 18 participants were recruited. Both groups were comparable in age (10.67 ± 3.45 years) and BSA (1.13 ± 0.27 m2). All acquisitions could be analysed with need of minimal editing of the annulus after automated detection, leaflets couldn’t be analysed in the AVSD group. Significative differences between both groups were found in the NPA with an increase in the nonplanarity for the patients with a repaired AVSD (144.5 vs. 132.9; P ˂ 0.01) and a more circular shape represented by a higher sphericity index (0.99 vs. 0,85; P = 0.01). No difference was found in indexed height, area and diameters were similar between both groups. The specific and exclusive shape of the MVA in patients with repaired AVSD was revealed in the patients studied, resulting in a loss of the saddle shape structure. Those results could help in the comprehension of anatomic changes of the mitral valve annulus occurring after AVSD surgery.

Background Pleuroblastoma (PPB) is a rare pediatric tumor which, in 30% of cases, is associated with cystic nephroma. It has been recently linked to the DICER1 mutation as part of a predisposition syndrome for various tumors. However, if DICER 1 anomalies have been reported in patients with Wilms tumor (WT), to date, no cases of PPB, WT, and DICER1 mutations have been reported in the same patient. Case presentation We report the case of a 3-year-old patient, initially managed for metastatic WT. During his clinical course, the diagnosis of a PPB was made after detecting the DICER1 mutation and subsequent management was therefore modified. Conclusion This case highlights that in case of simultaneous discovery of a renal tumor and a pulmonary lesion in a child, the DICER 1 mutations should be looked for as these could help adapt management and schedule the surgical procedures.

We report a rare and serious complication of cardiac strangulation arising from the implantation of epicardial pacing leads in a newborn. Patient's follow-up 9-month postsurgery revealed compression under the pulmonary valve annulus by a pacemaker lead, causing progressive stenosis of the right ventricular outflow tract. The epicardial leads were replaced to relieve compression, and stenosis of the right ventricular outflow tract was rectified. Pacemaker implantation in newborns is not without challenges; epicardial leads should be carefully positioned to avoid any compression of cardiac structures.

We describe the case of a 32-month-old patient from a developing country with tetralogy of Fallot associated with a severe biventricular dysfunction. This association is rare but makes the surgical strategy complex and potentially contraindicated. An acute severe hypoxic episode led us to perform palliative rescue intervention involving the placement of an undersized systemic-to-pulmonary shunt. This surgery was well tolerated and allowed a fast and impressive recovery of the ventricular function, making complete repair possible.