Introduction Adults with congenital heart disease (ACHD) may develop advanced heart failure with associated liver disease from chronic right-sided heart or Fontan failure. Thus, CHD with subsequent irreversible liver dysfunction is an increasing indication for combined heart-liver transplant (CHLT). A systematic liver function assessment is therefore essential prior to listing for heart transplantation (HTx). Objective We aimed to describe clinical characteristics, underlying cardiac defects, surgical history, perioperative issues, and outcomes in a contemporary cohort of ACHD referred for HTx or CHLT. Method We prospectively included all ACHD patients referred to our centre for HTx between August 2021 and February 2025. Liver function and disease were evaluated systematically before listing. Inclusions criteria were 1)CHD; 2)HT/CHLT referral; and 3) age >18years at the time of referral. Results A total of 22 ACHD patients were referred for HT/CHLT to our centre between August 2021 and February 2025. Mean age at referral was 36±24 years. Twelve patients (55%) had univentricular physiology, and all were in New York Heart Association (NYHA) functional class III or IV at the time of referral. Following initial evaluation, 5 patients were listed for CHLT, 7 patients for HTx (including 1 for combined heart-kidney transplantation). The main indications for CHLT were hepatocellular carcinoma and refractory ascites. Three patients died before listing, one of whom received Carmat artificial heart. Prior to transplantation, two patients required inotropic support, two were supported with veno-arterial extracorporeal membrane oxygenation (VA-ECMO) (Fig. 1). The median time from evaluation to listing or death prior to listing was 146 days (IQR 28–598 days). Among listed patients, the median time from listing to transplantation or death was 150 days (IQR 55–210 days). Among the 9 transplanted patients, no one died during the study period. No patients required VA-ECMO after transplantation, and no major post-transplant complications were reported. Conclusion In this contemporary cohort, patients with HF referred for HT/CHLT are heterogeneous also in terms of severity. Results after CHLT in our Centre are encouraging. Times from evaluation to listing and to transplantation are long. This supports the importance of early referral.

Introduction Infective endocarditis (IE) remains a major limitation of prosthetic materials in congenital heart disease (CHD), particularly after repeated interventions. Staphylococcus aureus is associated with severe outcomes, driven by its ability to form biofilms on implanted devices. However, the interaction between patient characteristics, bacterial phenotype and specific cardiac biomaterials remains poorly defined, limiting risk stratification and preventive strategies. Objective To identify bacterial and material determinants of IE by characterizing Staphylococcus aureus biofilm capacity and adhesion across biomaterials used in CHD, with the aim of informing clinical decision-making. Methods This pilot study part of a PhD program will include 50 S. aureus strains isolated from patients with prosthetic IE (with and without CHD), compared with 20 reference strains. Three complementary analyses will be performed: (1) quantitative assessment of biofilm formation, (2) whole-genome analysis to identify virulence and biofilm-associated genes, (3) evaluation of bacterial adhesion and biofilm formation on clinically used materials, including valved conduits, homograft, pericardial tissue, valves and metallic platforms. Expected results We expect to identify high-risk bacterial profiles associated with enhanced biofilm formation and preferential adhesion to specific biomaterials. Differences in colonization between materials may also explain the heterogeneous risk of IE observed in clinical practice. Perspectives This study targets a key unmet need in CHD: understanding why some patients and materials are more prone to IE. By linking bacterial phenotype to biomaterial susceptibility, the results may support: improved selection of valve type and material in high-risk patients, identification of patients requiring reinforced preventive strategies, development of targeted antimicrobial or anti-biofilm approaches.This translational approach aims to move from empirical prevention toward personalized IE risk management in CHD.

The scimitar syndrome is a rare congenital condition including a partial anomalous pulmonary venous return from the right lung into the inferior vena cava (IVC) which leads to a left-to-right shunt. Atypical variants with a dual drainage to both the IVC and the left atrium may be amenable to transcatheter occlusion. We report 3 patients diagnosed with scimitar syndrome with this dual drainage. Explorations confirmed the connection of the scimitar vein (SV) into both the IVC and the left atrium, with a significant left-to-right shunt and a right ventricular dilation. Pulmonary pressures were normal or mildly elevated. A compliant balloon occlusion test was performed at the IVC connection of the SV. All the patients underwent successfully a transcatheter occlusion of SV at the IVC junction using occlusion devices allowing proximal re-rerouting to the left atrium through the fistula. Aberrant systemic artery arising from the abdominal aorta was embolized in 2 cases during the same intervention.

Introduction The OPTIVENOSUS study evaluates the efficacy and safety of percutaneous correction of sinus venosus defects (SVD) using covered stents compared with the surgical gold standard. The primary objective was to assess a composite endpoint combining complete shunt closure and absence of major adverse events. Methods This is a prospective, multicenter, comparative study. Surgery was considered first-line treatment, while catheter-based intervention was proposed for patients deemed ineligible for surgery and with favorable anatomy. The primary endpoint at 6 months combined efficacy (complete shunt closure) and safety (absence of death or surgical conversion). Secondary endpoints included reduction in right ventricular end-diastolic volume (RVEDV) and absence of major complications (reintervention, stroke, or conduction disorders requiring pacemaker implantation). Results At 6 months postoperative, fifty-eight patients were analyzed (30 catheter-based, 28 surgical). The primary endpoint was achieved in 93.1% of the catheter group versus 100% of the surgical group, with no significant difference (P=0.49). After adjustment for age, oxygen saturation, arrhythmia history, pulmonary hypertension, and heart failure, no significant difference was observed (P>0.99). Complete shunt closure was obtained in 27 patients in the catheter group and in all patients in the surgical group. A≥20% reduction in RVEDV was observed in 92.6% and 96.2% of patients, respectively (P>0.99). No reintervention, stroke, or conduction disorder requiring pacemaker implantation occurred in 100% of the catheter group and 96.4% of the surgical group (P=0.49), with one reintervention reported in the surgical arm. No death or surgical conversion occurred in the percutaneous group. Residual shunts in the catheter group were infrequent and were related to anatomical constraints at the cavo-atrial junction. Conclusion Surgical repair of SV-ASD demonstrates excellent efficacy and safety outcomes. The percutaneous approach appears to be a safe and effective alternative in the short term, with non-inferior results despite a more comorbid population. This minimally invasive technique represents a promising option, pending longer-term follow-up.

Introduction Sinus venosus defect (SVD) is a rare complex congenital heart defect (1 in 1500 births) that was, until recently, corrected by open-heart surgery with cardiopulmonary bypass. A transcatheter correction of SVD was developed. This medical-economic project is a continuation of a feasibility study on a national cohort “OPTIVENOSUS”, which aimed to evaluate the effectiveness and safety of the transcatheter correction of SVD compared to the standard surgical method in patients deemed uneligible for surgery in a national multi-disciplinary meeting. Objective The primary objective of the ECO-OPTIVENOSUS study is to assess the efficiency of transcatheter correction of SVD in adolescents and adults compared to open-heart surgery. The study will focus on clinical outcomes and economic implications, aiming to demonstrate that the minimally invasive approach is more efficient in patients eligible for both procedures. Methods This multicenter, randomized (2 to 1), prospective, controlled trial will involve 13 expert centers in France (M3C network). A total of 90 patients will be recruited, with 60 undergoing endovascular procedures and 30 receiving surgical treatment. The study will evaluate clinical and economic criteria during the perioperative period and up to six months post-intervention. Key metrics include hospital stay duration, costs, quality of life, and clinical success rates. This study is under review for a PRME grant and aim to start in 2026. Expected results It is anticipated that the endovascular approach will significantly reduce hospital stays (from an average of 9 days for surgery to 3 days for endovascular treatment) and associated costs (estimated at €10,000 for endovascular devices versus €20,688 for open-heart surgery). The study aims to demonstrate comparable or improved clinical outcomes, including quality of life and functional capacity, without compromising safety. Perspectives The findings from this trial are expected to provide critical evidence for redefining treatment algorithms for SVD. The results will inform international guidelines and optimize patient management strategies, potentially leading to a paradigm shift in the treatment of this complex congenital heart defect.

BACKGROUND:Transcatheter correction of sinus venosus defect provides a less invasive alternative to open-heart surgery. AIM:To evaluate the safety and efficacy of 70-100mm-long partially covered balloon-expandable Optimus-CVS® XXL stents (AndraTec, Koblenz, Germany), specifically designed for sinus venosus defect repair, compared with surgical intervention. METHODS:OPTIVENOSUS is a French nationwide multicentre prospective comparative cohort study of patients with indications for sinus venosus defect correction (May 2023 to February 2031). The study comprises two parts, with a comprehensive shift in patient assignment to the catheter group. Part 1 will enrol 30 adult patients (aged>18years) deemed ineligible for surgery by a multidisciplinary team, who will undergo feasibility assessment for catheter correction, including virtual simulations and three-dimensional-printed bench testing. Part 2 will add 30 adult patients with favourable anatomy, considered directly for stent therapy, whereas three-dimensional-printed bench testing will be reserved for complex cases. Surgical patients (aged ≥12years) will be enrolled continuously throughout the study, with no enrolment cap. All patients will receive standardized follow-up for up to 5years. RESULTS:The primary endpoint is a 6-month composite measure of safety and efficacy, defined by trivial or absent residual shunt and the absence of major adverse events (death or surgical conversion). If no significant difference is found, efficacy (end-diastolic right ventricular volume reduction) and safety (absence of reintervention, stroke, pacemaker implantation or severe arrhythmia) will be further evaluated using hierarchical analysis. CONCLUSION:The OPTIVENOSUS study evaluates the safety and effectiveness of transcatheter sinus venosus defect correction with Optimus-CVS® XXL stents versus surgery (NCT05865119).

BACKGROUND:Transcatheter closure of ostium secundum atrial septal defect (osASD) has been the first-line treatment in most children and adults since the 2000s. Some major adverse events (MAE), such as aortic erosion, have been infrequently reported. AIMS:To report early outcomes in different subgroups by age and investigate risk factors associated with MAE. METHODS:This prospective, single-centre, cohort study included 2253 consecutive patients referred for transcatheter osASD with Amplatzer Septal Occluder (ASO) (Abbott®) between May 1998 and December 2021. Peri-procedural data associated with MAE at 1 month were investigated. RESULTS:Of 2253 patients, 1388 (61.6%) were adults and 865 (38.4%) were children, including 38 (1.7%) who weighed<15kg. Mean±standard deviation osASD diameter was 18±7mm, 22.7% of patients had a deficient aortic rim and 0.9% had a deficient inferior vena cava rim. Procedural success was achieved in 98.1% (95% confidence interval [CI] 97.4-98.6%). A total of 32 peri-procedural MAE occurred in 31 patients (1.4%, 95% CI 0.9--1.9%) including 19 device embolizations (0.8%) and two cardiac erosions (0.1%). No peri-procedural deaths were reported. There were no significant differences in the incidence of MAE between age and weight subgroups. In univariate analysis, MAE were significantly associated with atrial arrhythmia history before the intervention (P=0.013), inferior vena cava rim deficiency (P<0.001), antero-inferior rim deficiency (P=0.004), absence of balloon sizing (P=0.026), larger prosthesis size (P=0.017), maximal atrial septal defect size/body surface area≥20mm/m2 (P=0.008). CONCLUSION:Transcatheter osASD closure using ASO has a high procedural success rate across a broad population of children and adults, reinforcing that transcatheter osASD closure is the intervention of choice for a wide range of patients and osASD morphologies.

BACKGROUND:Superior sinus venosus defect (SVD) is a complex congenital heart disease (CHD) with a wide spectrum of anatomical variants. The innovative transcatheter correction of SVD (TCSVD) is feasible in selected cases. Patient selection requires a detailed anatomical evaluation. This study aims to provide an anatomical classification of SVD using 3D models. METHODS:Computed tomography (CT) scans of hearts with superior SVD were 3D-modeled using semi-automatic segmentation. Key parameters such as superior vena cava (SVC) size, SVC overriding, caudal defect extension, and size/orientation of the anomalous pulmonary vein (APV) were assessed in this single-center cohort study. RESULTS:We included 197 patients with superior SVD. SVC overriding was absent in 38 ​% of cases and exceeded 50 ​% in 7 ​% of cases. A single APV ostium was identified in 52 ​% of patients and additional ostia in 48 ​% of patients. Among children older than 12 years, 83 ​% had an SVC diameter greater than 14 ​mm (at the first quartile in adults). Based on the amount of SVC overriding and caudal extension of the defect, we classified the SVDs into two types: fenestration (30 ​%) and cavo-atrial (70 ​%). Associated lesions included left superior vena cava (15 ​%) and ostium secundum atrial septal defect (8 ​%). CONCLUSION:A standardized 3D evaluation of SVDs provided a new anatomical classification that should prove useful for tailoring TCSVD strategies.

BACKGROUND:Infective endocarditis (IE) after transcatheter atrial septal defect (ASD) closure is rare. CASE SUMMARY:We report the case of a 6-year-old girl who developed methicillin-resistant Staphylococcus aureus IE 19 months after ASD closure with an Amplatzer device. She presented with severe sepsis, a large vegetation on the right atrial side of the occluder, and moderate tricuspid regurgitation. Despite empirical antibiotic therapy, persistent bacteremia mandated surgical removal of the device, ASD closure with a pericardial patch, and tricuspid valve repair. Six weeks of targeted antibiotics led to a favorable 1-year outcome. DISCUSSION:This case, alongside other reports of late-onset IE after transcatheter ASD closure, highlights that although rare, such infections can occur well beyond 6 months postprocedure. This observation questions current guidelines restricting prophylactic measures to the early postimplant period. TAKE-HOME MESSAGE:Patients with septal occluders may require prolonged vigilance and reinforced education on skin and dental hygiene to minimize the risk of late IE.

OBJECTIVES:The goal of this retrospective multicentre study was to present late surgical outcomes of the treatment of children with double outlet right ventricle (DORV) coming from emerging countries. METHODS:The Mécénat Chirurgie Cardiaque brings to France for surgery selected children with simple and complex congenital diseases, including DORV. The patients are operated on in 9 hospitals that specialize in paediatric cardiac surgery. Data are collected from the Mécénat Chirurgie Cardiaque comprehensive database, with a strict postoperative follow-up. The patients included only those who had biventricular repair of DORV with 2 viable ventricles. According to the classification of the Eleventh Revision of the International Classification of Diseases, DORV was defined as a congenital cardiovascular malformation in which both great arteries arise entirely or predominantly from the morphologically right ventricle. RESULTS:From January 1996 to January 2022, a total of 81 consecutive DORV biventricular repair operations were performed. There were 6 subtypes of DORV divided into 2 groups: DORV-committed ventricular septal defect (VSD): DORV-VSD (n = 25), DORV-Fallot (n = 34), DORV-transposition of the great arteries (n = 5); and DORV-non-committed (nc) VSD: DORV-ncVSD-no pulmonary stenosis (PS) (n = 7), DORV-ncVSD-PS (n = 5) and DORV-atrioventricular septal defect (AVSD)-PS (n = 5). Four Fontan patients were excluded. Three patients were lost to follow-up (3.4%). The overall perioperative mortality was 7.4% ± 2.6%, 6/81 (95% confidence interval: 2.8%-15.4%) ranging from 0% in DORV-AVSD-PS to 14% for DORV-ncVSD-no PS. The overall 10-year survival was 86%. The early mortality of DORV-ncVSD at 5.9% ± 2.4% (1/17) was similar to that of DORV-committed VSD at 7.8% ± 2.7% (5/64) (P = 0.79). There was a trend towards an optimal outcome for the arterial switch operation and the DORV-AVSD-PS repair. VSD enlargement was significantly more frequent in DORV-ncVSD at 42% (5/12) (P = 0.001). There were low numbers in the complex groups. The number of Fontan cases was noticeably low. The aorta located entirely on the right ventricle represents the fundamental anomaly and the surgical challenge of DORV. CONCLUSIONS:Overall survival at 10 years was 86%. This study shows a trend towards satisfactory early and late outcomes in BVR of simple DORV with committed VSD, compared to complex DORV with ncVSD.

Summary OBJECTIVES This retrospective multicentre study aims to present late surgical outcomes of Double Outlet Right Ventricle (DORV) coming from emerging countries. METHODS Mécénat Chirurgie Cardiaque brings to France for surgery selected children with simple and complex congenital diseases, including DORV. The patients are operated in nine hospitals, specialized in paediatric cardiac surgery. heart Data are collected from the MCC comprehensive database, with a strict post-operative follow-up. Patients included only biventricular repair of DORV with 2 viable ventricles. According to the classification of Eleventh Revision of the International Classification of Diseases (ICD11), DORV was defined as a congenital cardiovascular malformation in which both great arteries arise entirely or predominantly from the morphologically right ventricle. RESULTS From Jan. 1996 to Jan. 2022, 81 consecutive DORV biventricular repair (BVR) were performed. There were 6 subtypes of DORV divided in two groups:—DORV-committed VSD: DORV-VSD (n:25), DORV-Fallot (n:34), DORV-TGA (n:5);—and DORV-ncVSD: DORV-nc-VSD-no PS (n: 7), DORV-nc-VSD-PS (n: 5), and DORV-AVSD-PS (n: 5). Four Fontan patients were excluded. Three patients were lost to follow-up, (3.4%). The overall peri-operative mortality was 7.4% ± 2.6%, 6/81, [95% CI: 2.8% - 15.4%] ranging from 0% in DORV-AVSD-PS to 14% for DORV-ncVSD-no PS. The overall 10Y survival was 86%. The early mortality of DORV-non committed VSD at 5.9% ± 2.4%, (1/17) was similar to DORV-committed VSD at 7.8% ± 2.7%, (5/64), p: 0.79 There was a trend for optimal outcome for arterial switch operation and DORV-AVSD-PS repair. VSD enlargement was significantly more frequent in DORV-non committed VSD, 42% (5/12), p: 0.001. There were low numbers in complex groups. The number of Fontan is noticeably low. The fundamental anomaly and surgical challenge in DORV is the aorta standing entirely on the right ventricle. CONCLUSIONS Overall survival at 10Y was 86%. This study shows a trend towards satisfactory early and late outcomes in biventricular repair of simple DORV with committed VSD, compared to complex DORV with non-committed VSD.

Objectives . Aortic coarctation in neonates remains a clinical challenge. Low weight, arch hypoplasia and ductal dependence have been identified as risk factors for recurrent coarctation. We hypothesized that a tailored surgical technique may prevent recurrence. Methods . Retrospective evaluation of neonates treated for coarctation through thoracotomy was done. No primary percutaneous procedure was performed and repairs through sternotomy were excluded. Aortic hypoplasia was defined as a ratio arch diameter (mm)/patient’s weight (kg) < 1. Extended end-to-end anastomosis (EEEA), subclavian flap (Waldhausen) and Amato aortoplasty were performed. Mortality and recurrent obstruction requiring re-intervention were assessed. Results . Records of 340 consecutive patients (2003–2019) were analyzed. Preoperative median age and weight were, respectively, 10 days (1–30) and 3080 grams (1400–5180). Arch hypoplasia was documented in 31 patients (9.1%). Prostaglandin was infused in 220 (65.3%). Critical preoperative status was documented in 35 (10.8%). EEEA repair was performed in 273 (80.3%), Waldhausen was performed in 42 (12.4%), and Amato was performed in 25 (7.4%). The last two were more likely to be performed in the presence of arch hypoplasia ( p  < 0.0001). Hospital mortality occurred in 2 patients (0.6%). Thirty-six procedures (31 percutaneous/5 surgical) were performed for recurrent arch obstruction in 33 patients. Three late deaths occurred. Low-weight, hypoplastic arch, and ductal dependency did not influence the outcome. All survivors were free from residual coarctation at a mean follow-up of 3.6 ± 3.4 years postoperatively. Conclusions . Surgical repair remains the procedure of choice for neonatal coarctation. A tailored approach using alternative techniques seemed to offer comparable results even in presence of associated risk factors.

Introduction and objectives: Diagnosis, management, and surgical decision-making in children and adults with congenital heart disease are largely based on echocardiography findings. A recent development in cardiac imaging is fusion of different imaging modalities. Our objective was to evaluate the feasibility of computed tomography (CT) and 3-dimensional (3 D) transthoracic echocardiography (TTE) fusion in children and adults with congenital heart disease.Methods: We prospectively included 14 patients, 13 of whom had congenital heart disease, and who underwent both CT and 3 D TTE as part of their usual follow-up. We described the steps required to complete the fusion process (alignment, landmarks, and superimposition), navigation, and image evaluation.Results: Median age was 9.5 [2.7-15.7] years, 57% were male, and median body surface area was 0.9 m2 [0.6-1.7]. Congenital heart disease was classified as simple (n = 4, 29%), moderate (n = 4, 29%), or complex (n = 6, 42%). 3 D TTE-CT fusion was successful in all patients. Median total time to complete the fusion process was 735 [628-1163] seconds, with no significant difference according to the degree of complexity of the defects. Landmarks were significantly modified in complex congenital heart disease.Conclusions: We established the feasibility and accuracy of 3 D TTE-CT fusion in a population of children and adults with a variety of congenital heart diseases. The simultaneous visualization of many intracardiac structures may help to understand the anatomical features of congenital heart disease without limitations regarding age, weight, or complexity of the congenital defects. ⠂C 2022 Sociedad Espanola de Cardiologia. Published by Elsevier Espana, S.L.U. All rights reserved.

Transcatheter therapy of congenital heart disease has seen major advances over the last 40 years with the development of prostheses for cardiac shunts occlusion, followed by the development of stents, particularly valved stents for transcatheter pulmonary valve replacement. More recently, transcatheter correction of sinus venosus defect (SVD) has emerged, taking minimally invasive treatment options for congenital heart disease to a new level.

Background: Anomalous aortic origin of a coronary artery (AAOCA) is a congenital heart disease with a 0.3%-0.5% prevalence. Diagnosis is challenging due to nonspecific clinical presentation. Risk stratification and treatment are currently based on expert consensus and single-center case series. Methods: Demographical and clinical data of AAOCA patients from 17 tertiary-care centers were analyzed. Diagnostic imaging studies (Bidimensional echocardiography, coronary computed tomography angiography [CCTA] were collected. Clinical correlations with anomalous coronary course and origin were evaluated. Results: Data from 239 patients (42% males, mean age 15 y) affected by AAOCA were collected; 154 had AAOCA involving the right coronary artery (AAORCA), 62 the left (AAOLCA), 23 other anomalies. 211 (88%) presented with an inter-arterial course. Basal electrocardiogram (ECG) was abnormal in 37 (16%). AAOCA was detected by transthoracic echocardiography and CCTA in 53% and 92% of patients, respectively. Half of the patients reported cardiac symptoms (119/239; 50%), mostly during exercise in 121/178 (68%). An ischemic response was demonstrated in 37/106 (35%) and 16/31 (52%) of patients undergoing ECG stress test and stress-rest single positron emission cardiac tomography. Compared with AAORCA, patients with AAOLCA presented more frequently with syncope (18% vs. 5%, P = 0.002), in particular when associated with inter-arterial course (22% vs. 5%, P < 0.001). Conclusion: Diagnosis of AAOCA is a clinical challenge due to nonspecific clinical presentations and low sensitivity of first-line cardiac screening exams. Syncope seems to be strictly correlated to AAOLCA with inter-arterial course.

El diagnóstico y manejo terapéutico de pacientes con cardiopatías congénitas se basa en gran medida en los resultados de la ecocardiografía. Las diferentes técnicas de fusión de imagen han supuesto un reciente avance en la obtención de imágenes cardíacas. Nuestro objetivo ha sido el de evaluar la viabilidad de la fusión de la tomografía computarizada (TC) y la ecocardiografía transtorácica (ETT) 3 D en niños y adultos con cardiopatías congénitas. Se incluyeron de forma prospectiva 13 pacientes con cardiopatías congénitas y 1 paciente con corazón normal los cuales se sometieron a TC y ETT 3 D como parte de su seguimiento habitual. Se describieron los pasos necesarios para completar el proceso de fusión (alineación, puntos de referencia y superposición), la navegación y la evaluación de las imágenes. La edad media fue de 9,5 años [2,7-15,7], el 57% eran varones y la superficie corporal media fue de 0,9 m2 [0,6-1,7]. Se clasificaron las cardiopatías como simples (n = 4, 29%), moderadas (n = 4, 29%) o complejas (n = 6, 42%). La fusión 3 D ETT-TC fue exitosa en todos los pacientes. La mediana del tiempo total para completar el proceso de fusión fue de 735 segundos [628-1163], sin diferencias significativas según el grado de complejidad de la cardiopatía. Los puntos de referencia se modificaron significativamente en las cardiopatías congénitas complejas. La técnica de fusión 3 D ETT-TC en una población de niños y adultos con diversas cardiopatías congénitas es viable y precisa según demostramos. La visualización simultánea de diferentes estructuras cardíacas podría ayudar a comprender las características anatómicas de las cardiopatías congénitas sin limitaciones en cuanto a la edad, el peso o la complejidad. Diagnosis, management, and surgical decision-making in children and adults with congenital heart disease are largely based on echocardiography findings. A recent development in cardiac imaging is fusion of different imaging modalities. Our objective was to evaluate the feasibility of computed tomography (CT) and 3-dimensional (3 D) transthoracic echocardiography (TTE) fusion in children and adults with congenital heart disease. We prospectively included 14 patients, 13 of whom had congenital heart disease, and who underwent both CT and 3 D TTE as part of their usual follow-up. We described the steps required to complete the fusion process (alignment, landmarks, and superimposition), navigation, and image evaluation. Median age was 9.5 [2.7-15.7] years, 57% were male, and median body surface area was 0.9 m2 [0.6-1.7]. Congenital heart disease was classified as simple (n = 4, 29%), moderate (n = 4, 29%), or complex (n = 6, 42%). 3 D TTE-CT fusion was successful in all patients. Median total time to complete the fusion process was 735 [628-1163] seconds, with no significant difference according to the degree of complexity of the defects. Landmarks were significantly modified in complex congenital heart disease. We established the feasibility and accuracy of 3 D TTE-CT fusion in a population of children and adults with a variety of congenital heart diseases. The simultaneous visualization of many intracardiac structures may help to understand the anatomical features of congenital heart disease without limitations regarding age, weight, or complexity of the congenital defects.

Background: Children with severe congenital heart disease (CHD) are rarely treated in developing countries and have very little to no chance to survive in their local environment. Mecenat Chirurgie Cardiaque (MCC) flies to France children with CHD from developing countries. This report focuses on the early, mid, and late outcomes of 531 children with severe CHD sent to MCC for surgery from 1996 to 2019. Methods: The inclusion criteria were based on diagnosis and not on procedure. MCC is present in 66 countries and has developed a robust staff, including 12 permanent employees and 700 volunteers, with 350 host families based in France, 120 local correspondents, and 100 local physicians. Since 1996, MCC has organized a basic training of local pediatric cardiologists yearly, offering a free 1-month training course. Over time, MCC could count on a pool of doctors trained in basic pediatric cardiology. Flights were secured by the Aviation Sans Frontieres Foundation. Nine French centers performed the surgeries. A robust follow-up was conducted in all the nations where MCC operates. Results: The most frequent pathologies were single ventricle (n = 126), double-outlet right ventricle (n = 116), pulmonary atresia with ventricular septal defect (n = 68), transposition of the great arteries with ventricular septal defect and transposition of the great arteries with intact ventricular septum (n = 61), arterial trunk (n = 39), transposition of the great arteries with ventricular septal defect and left ventricle outflow tract obstruction (n = 35), complete atrioventricular septal defect (n = 18), congenitally corrected transposition of the great arteries (n = 16), and so on. The median age was 5.4 years (range, 1 month-26 years). The mean perioperative mortality was 5.5% (29 out of 531) (95% confidence limit, 3.5%-7.4%). The follow-up was 91.3%, with a mean follow-up of 5.1 years. The global actuarial survival at 5, 10, and 15 years was, respectively, 85%, 83%, and 74%. There was a significant higher late mortality for patients surviving only with a Blalock-Taussig shunt (P = .001). Conclusions: Operating on 531 children with severe CHD from developing nations was achieved with satisfactory early and long-term results. Children with severe CHD are rarely operated on in developing nations. Programs like MCC's offer a viable option to save these children born with severe CHD.

Percutaneous pulmonary valve implantation (PPVI) remains limited in patients with large native right ventricle outflow tract (RVOT). New 3D post-processing analysis derived from cardiac tomography (CT) may improve pre-procedural assessment. We retrospectively selected 15 patients who had invasive balloon sizing (IBS) and printed cardiac models with thermoplastic polyurethane (TPU) by laser sintering before PPVI. RVOT long-axis curvilinear reconstruction (LACR) and 3D volume rendering images were produced from CT scan by interventionists blinded to outcome, to assess minimal diameter and shape of the expected valve landing zone (proto-meso diastolic phase in 13 patients and systolic phase in two, Aquarius 3D, Tokyo) (Fig. 1). Median IBS was 26.0 [24.4–27.9] mm. LACR and IBS diameters were well correlated (r = 0.67, P = 0.007; r2 = 0.55, P = 0.002) with a mean bias of 2.8 mm. Printed model and IBS diameters were moderately correlated (r = 0.55, P = 0.04, r2 = 0.50, P = 0.003) with a mean bias of 0.9 mm (Fig. 2). Assessing size and shape of the models, 3 interventionists rated the expected complexity of PPVI from non-feasible (n = 3), challenging (n = 3), and straightforward (n = 9). Analysis of outcome confirmed non-feasible in 3, challenging procedures in 1, and uneventful in 11 (r = 0.85, P = 0.0001). Median fluoroscopy time was 18.6 min [16.9–26.2]. Expected complexity was correlated with fluoroscopy time (r = 0.76, P = 0.003). RVOT CT scan 3D reconstruction is feasible by interventionists and the cardiology team before PPVI and offered assessment of landing zone shape and diameter. CT scan acquisition in diastole underestimated the RVOT diameter. Nevertheless, it allowed accurate predicting of feasibility and complexity of PPVI.

Anatomical repair by double switch procedure (DS) for patients with congenitally corrected transposition of the great arteries (ccTGA) remains under debate. The outcomes after DS for ccTGA without LVOTO, with specific focus on the left ventricle (LV) performance in patients with intact ventricular septum (IVS) were assessed. Since 1994, 39 patients underwent DS: 9 had an IVS, and 30 had an associated VSD. Palliative procedure consisted in pulmonary artery banding (PAB) for 31 patients (79%): 9 (twice in 5) for tricuspid regurgitation (TR) and 22 for hemodynamically significant associated VSD. Survival and LV performance in follow up were analyzed. PAB was efficient to diminish TR significantly in all 9. The median age at repair was 22 months (12 days–13 years). Associated VSD closure was performed in 76% of patients (n = 30). Four patients required peri-operative pacemaker implantation. Hospital mortality occurred in 1 (2.5%: 70% CI 1–8%) neonate who underwent DS and aortic arch repair: he died because of low output syndrome. Two (5%) patients required early reoperation for pulmonary venous pathway stenosis release. Median follow up was 13.7 years (range: 3 months–26 years). Two late deaths occurred 16 and 84 months postoperatively because of refractory LV dysfunction: both patients had IVS and benefited from LV retraining. Six patients (16%) required 7 reoperations during follow up. At last visit, 5 patients (14%) presented with LV dysfunction (LV ejection fraction < 50%) requiring medical treatment while remaining 31 (84%) were asymptomatic. Actuarial survival curves are shown in Fig. 1. Mortality was significantly higher in the IVS group compared to the VSD group (33% vs. 0%, P = 0.01). The DS appeared to be the procedure of choice for ccTGA associated with VSD. PAB was efficient for the management of TR in early life. Our Results suggest that the strategy of LV retraining by PAB to achieve DS at any price remains questionable.