ACVD, Vol.117, Suppl.8-9

Volume 117 – no. 8-9 Supplement – August/September 2024 ISSN 1875-2136 93222 Archives of Cardiovascular Diseases Formerly Archives des maladies du cœur et des vaisseaux

Archives of Cardiovascular Diseases Formerly Archives des maladies du cœur et des vaisseaux Official journal of the French Society of Cardiology Editor in Chef: Ariel A. Cohen Deputy editor: Bernard Iung Editorial board Victor Aboyans Philippe Acar Laurence Amar Denis Angoulvant Franck Boccara Haran Burri Yves Cottin Shinichi Goto Roberto Lang Guillaume Lebreton Christophe Leclercq Gilles Lemesle Paul Milliez Agnès Pasquet Philippe Pibarot Etienne Puymirat Statistical consultant Julien Magne Technical editor Sophie Rushton-Smith How to contact the journal Ariel A. Cohen Service de cardiologie Hôpital Saint-Antoine (pavillon Lemierre) 184, rue du Faubourg-Saint-Antoine, 75571 Paris cedex 12 Tel.: 33 (0)1 49282886 E-mail: clarisse.barille@aphp.fr or ariel.cohen@aphp.fr Scientific committee E. Aliot (France), P. Amouyel (France), M. Böehm (Germany), P. Bonhoeffer (United Kingdom), D. Bonnet (France), E. Bruckert (France), T. Carrel (Switzerland), M. Cohen (United States), A. Cribier (France), N. Danchin (France), J.-C. Daubert (France), J. Davignon (Canada), G. Derumeaux (France), E. Eeckhout (Switzerland), F. Follath (Switzerland), B. Gerber (Belgium), P. Guéret (France), G. Habib (France), A. Hagege (France), M. Komadja (France), B. Kreitmann (France), R. Lang (United States), S. Laurent (France), H. le Marec (France), J. Lima (United States), N. Ludwig (United Kingdom), Z. Mallat (France), G. Marx (United States), J.-L. Monin (France), E. Mousseaux (France), P. Nataf (France), P. Nihoyannopoulos (United Kingdom), G. Parati (Italy), L. Perrault (Canada), L. Pierard (Belgium), B. Prendergast (United Kingdom), S. Priori (Italy), D. Revel (France), V. Roger (United States), R. Rosenhek (Austria), M. Safar (France), M. Sarano (United States), E.J. Schaefer (United States), M. Scherrer Crosbie (United States), J. Schwitter (Switzerland), P. Serruys (Netherlands), M. Simoons (Netherlands), P.G. Steg (France), G. Tomaselli (United States), P. Tornos (Spain), C. Tribouilloy (France), A. Vahanian (France) Archives of Cardiovascular Diseases (ISSN 1875-2136) 2024 (volume 117) One year, 12 issues. Journal manager – Gautier Goffette. E-mail: acvd@elsevier.com Head of Content Solutions – Monika Giergielewicz. E-mail : m.giergielewicz@elsevier.com Partnerships and Supplements – Claire Ebersold. +33 (0)1 71 16 51 14 c.ebersold@elsevier.com Advertising – Nicolas Zarjevski. +33 (0)1 71 16 51 38 n.zarjevski@elsevier.com Subscriptions – Tel.: (33) 01 71 16 55 99. Fax: (33) 01 71 16 55 77. http://www.em-consulte.com/infos Publisher – Anne-Elisabeth Fournié. E-mail: a.fournie@elsevier.com Publishing director – Daniel Rodriguez Archives of Cardiovascular Diseases

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Organisation logistique OVERCOME 13-15 rue des Sablons 75116 Paris France Tél. : +33 (0)1 40 88 97 97 fcpc@overcome.fr Comité scientifique Sébastien Hascoët, Plessis-Robinson (Président de la FCPC) Céline Grunenwald, Nantes (Secrétaire de la FCPC) Sylvie Di Filippo, Monaco Pascal Amedro, Bordeaux Gilles Bosser, Vandoeuvre-lès-Nancy Anne-Claire Casalta, Marseille Nicolas Combes, Toulouse Olivia Domanski, Lille Clément Karsenty, Toulouse Fabien Labombarda, Caen Daniela Laux, Paris Bruno Lefort, Tours Pamela Moceri, Nic Olivier Raisky, Paris Mathieu Albertini, Paris Alexis Barat, Paris Comité local d’organisation - Toulouse Philippe Acar Najeebullah Bina Eric Bruguiere Nicolas Combes Yves Dulac Aitor Guitarte Khaled Hadeed Clément Karsenty Reaksmei Ly Miarisoa Ratsimandresy Nathalie Souletie Olivier Vadhat

Archives of Cardiovascular Disease 117 (2024) iv CONTENTS Abstracted in: Current Contents/Clinical Medicine; MEDLINE/Index Medicus; EMBASE/Excerpta Medica; Pascal (INIST/CNRS); Scopus Available online at www.sciencedirect.com ScienceDirect Editorials Editorial P. Acar, N. Combes and C. Karsenty ............................................................................................................................. S217 Editorial (English version) P. Acar, N. Combes and C. Karsenty .................................................................................................................................. S218 Résumés Communications Orales – Jeudi 19 septembre 2024 de 10 h 00 à 11h00 ........................................................... S219 Communications Orales – Vendredi 20 septembre 2024 de 09h05 à 10h15 ..................................................... S224 Communications Orales (jeunes chercheurs et personnel paramédical) – Vendredi 20 septembre 2024 de 10h45 à 12h15 ................................................................................................................................................................... S228 Session “La valve pulmonaire à tout âge”. Communication Orale – Vendredi 20 septembre 2024 de 10h45 à 12h15 ................................................................................................................................................................... S231 Posters commentés – Vendredi 20 septembre 2024 de 13h45 à 15h00 ............................................................. S232 Posters ............................................................................................................................................................................................ S239 Index .......................................................................................................................................................................................... I7

Archives of Cardiovascular Disease 117 (2024) S217 Disponible en ligne sur ScienceDirect www.sciencedirect.com Éditorial « Parfois au fond de moi se raniment l’eau verte du canal du Midi. Et la brique rouge des Minimes ô mon païs, ô Toulouse, ô Toulouse » Claude Nougaro Cher(e)s ami(e)s, cher(e)s collègues, C’est avec une grande joie que nous vous invitons à Toulouse pour le 20e congrès médico-chirurgical de la Filiale de cardiologie pédiatrique et congénitale de la Société franc¸ aise de cardiologie, qui se tiendra du mercredi 18 au vendredi 20 septembre 2024. Douze ans après l’édition 2012, la Ville Rose accueillera l’évènement de nouveau en ce beau lieu convivial des espaces Vanel, situé en centre-ville. Le congrès débutera le mercredi 18 septembre par une journée de DPC médical consacrée aux aortopathies, et qui se déroulera à la clinique Pasteur. Suivront deux jours de sessions scientifiques où, comme de coutume dans notre société savante, les travaux de nos équipes francophones, et en particulier de nos jeunes collègues, seront mis à l’honneur et valorisés. Les sessions à thèmes alterneront avec des sessions interactives de cas cliniques, d’ateliers, de programme de simulation, toujours au plus près de l’innovation médico-chirurgicale diagnostique et thérapeutique, médicamenteuse et interventionnelle. Nous aurons à cœur de débattre de tous les sujets d’actualité de notre spécialité, en particulier de la rythmologie, du cathétérisme et de l’imagerie. Toulouse présentera aussi un programme paramédical riche en sujets remarquables, de la prise en charge anténatale jusqu’à celle des adultes avec cardiopathie congénitale. Le programme social vous fera découvrir ou redécouvrir tous les charmes de notre belle ville. Merci à Bernard Cadène, peintre toulousain, d’avoir réalisé l’affiche du congrès. Au plaisir de se retrouver tous ensemble ! Le Comité d’organisation local Philippe Acar Nicolas Combes Clément Karsenty , au nom de toute l’équipe médico-chirurgicale Toulousaine France https://doi.org/10.1016/j.acvd.2024.09.001 1875-2136/© 2024 Publie´ par Elsevier Masson SAS.

Archives of Cardiovascular Disease 117 (2024) S218 Disponible en ligne sur ScienceDirect www.sciencedirect.com Editorial Dear friends, dear colleagues, It is with great joy that we invite you to Toulouse for the 20th medical-surgical congress of the Pediatric and Congenital Cardiology Branch of the French Society of Cardiology, which will be held from Wednesday September 18 to Friday September 20, 2024. Twelve years after the 2012 edition, the Pink City will host the event again in the beautiful and friendly Vanel spaces, located in the city center. The congress will begin on Wednesday September 18 with a day of medical CPD devoted to aortopathies, which will take place at the Pasteur Clinic. Two days of scientific sessions will follow where, as is customary in our Learned Society, the work of our French-speaking teams, and in particular our young colleagues, will be honored and valued. Thematic sessions will alternate with interactive sessions of clinical cases, workshops, simulation programs, always as close as possible to diagnostic and therapeutic, medicinal and interventional medical-surgical innovation. We will be keen to discuss all current topics in our specialty, in particular rythmology, catheterization and imaging. Toulouse will also present a paramedical program rich in remarkable subjects, from antenatal care to that of adults with congenital heart disease. The social program will make you discover or rediscover all the charms of our beautiful city. Thanks to Bernard Cadène, painter from Toulouse, for creating the conference poster. Looking forward to seeing you all together! Philippe Acar Nicolas Combes Clément Karsenty , On behalf of The local Organizing Committee the entire Toulouse medical-surgical team , https://doi.org/10.1016/j.acvd.2024.09.002 1875-2136/© 2024 Published by Elsevier Masson SAS.

Archives of Cardiovascular Disease 117 (2024) S219–S223 Disponible en ligne sur ScienceDirect www.sciencedirect.com Communications Orales – Jeudi 19 septembre 2024 de 10 h 00 à 11h00 00001 Pulmonary hypertension (PH) in the first twelve-months of live: Long-term outcome in relation to diagnosis and invasive hemodynamic A. Callegari , J. Grynblat , M. Meot , I. Szezepanski , S. Malekzadeh-Milani , D. Bonnet Centre de Référence Malformations Cardiaques Congénitales Complexes (M3C), Hôpital Universitaire Necker-Enfants–Malades, Assistance publique–Hôpitaux de Paris, Paris, France Introduction Some patients develop pulmonary hypertension (PH) in the first months of life. Objective Knowledge gaps remain on their characteristics and long-term outcome. Methods In total, 563 consecutive patients (mean follow-up 4years) with mPAP > 20 mmHg between 1–12 months of life were included and their history examined. Results Age was mean (SD) 154 (95) days, mPAP 40 (14) mmHg, transpulmonary gradient 31 (14) mmHg, pulmonary vascular resistance index (PVRi) 4.4 (3.9) WU.m2. Genetic disease was found in 122 (19%). Most frequent indications for cath were PH on TTE (220, 39%), shunt calculation/closure (256, 45%), or suspected pulmonary veins anomaly (51, 9%). Majority of patients (404, 72%) had an open shunt, 37 (6%) repaired CHD, 57 (10%) postcapillary PH and 66 (12%) normal cardiac anatomy but lung development disease (36/66), severe systemic disease (9/66), drug induced (16/66), or heritable (4/66) PH. In open shunt patients PVRi was < 3 WU.m2 in 99 (41%), 3–6 in 89 (37%), > 6 in 52 (22%). Thirteen (3%) patients were considered not operable due to high PVRi (3/13), combined lung diseases (5/13), and/or severe chromosomal abnormalities (6/13). PH related to open shunt was protective for persistent PH at follow-up (P<0.001) and survival (P< 0.001, Fig. 1). Overall PH at follow-up was found in 84 (15%) and independent risk factors for persistence of PH were lung disease (P< 0.001), arterial switch for TGA (P< 0.008), PVRi > 6 WU.m2(P< 0.001), idiopathic PH (P= 0.009), and low pulmonary vein saturation (P=0.05). Mortality was 10% and survival was significantly reduced in patients with concomitant lung disease (P= 0.001, Fig. 2), normal cardiac anatomy (P= 0.001), and cath for suspected PH at TTE (P=0.014). Fig.1 Kaplan–Meier survival analysis in patients with open shunt (green) vs. no open shunt (blue) (P<0.001). Fig. 2 Kaplan–Meier survival analysis in patients with lung disease (green) vs. no lung disease (blue) (P<0.001). Conclusion Open shunt normally causes PH in early life, but hardly impacts operability and isolated open shunt rarely results in PH or reduced survival at follow-up. Patients with normal cardiac anatomy and lung disease have higher PH-related mortality and prevalence of PH at follow-up. Disclosure of interest The authors have not supplied their declaration of competing interest. https://doi.org/10.1016/j.acvd.2024.07.002 1875-2136/

Communications Orales – Jeudi 19 septembre 2024 de 10 h 00 à 11 h 00 Archives of Cardiovascular Disease 117 (2024) S219–S223 00003 Pulmonary pressure in the first twelve-months of live: What should weexpect? A. Callegari , J. Grynblat , M. Mathilde , I. Szezepanski , S. Malekzadeh-Milani , D. Bonnet Centre de Référence Malformations Cardiaques Congénitales Complexes (M3C), Hôpital Universitaire Necker-Enfants–Malades, Assistance publique–Hôpitaux de Paris, Paris, France Introduction Mean pulmonary artery pressure (mPAP)≥20 mmHg defines pulmonary hypertension (PH). Objective Critically evaluate this cut-off in very young patients. Methods In total, 1129 consecutive patients had a reliable mPAP measurement between 1–12 months of life. These invasive measurements and their clinical history were reviewed. Results Age was mean±SD 158±100 days, weight 5.5±1.9kg. Patients with a palliated single ventricle anatomy 129/1129, peripheral PA-branch stenosis or MAPCAs 45/1129, PA-banding for open shunt 30/1129, or severe RVOTO 185/1129 were excluded. Of the remaining 726 patients 163/726 (22%) had mPAP < 20 mmHg while 563 (78%) had PH with mPAP≥20mmHg. Overall, PH at TTE was the reason for invasive mPAP measurement in 236 patients and only 16/236 patients (7%) had a mPAP < 20 mmHg but 12/16 (75%) had a mPAP≥18 mmHg. In the group with mPAP≥20 mmHg PH persisted at 6-months in 109/236 (46%) (Fig. 1A) and in 68/236 (28%) at long-term follow-up (Fig. 1B). In the group with mPAP between 18 and 20 mmHg PH persisted at 6-months in 9/12 (75%) and in 3/12 (25%) at long-term follow-up. None of the patients with mPAP < 18 mmHg had PH at follow-up. In patients with less than 3 months of age, PH at TTE was the reason for invasive mPAP measurement in 73/306 (23%) and only 3/73 patients (4%) had a mPAP < 20 mmHg, but 2/3 (75%) had amPAP≥18 mmHg. In the group with mPAP≥20mmHg PH persisted at 6-months in 34/70 (48%) (Fig. 2A) and in 34/70 Fig. 1 Fig. 2 (48%) at long-term follow-up (Fig. 2B). In the group with mPAP between 18 and 20 mmHg PH persisted at 6-months in 2/2 (100%) and in 2/2 (100%) at long-term follow-up. None of those with mPAP < 18 mmHg had PH at follow-up. Conclusion Pulmonary hypertension defined by mPAP≥20 mmHg is very common in this cohort of young patients, especially in case of suspected PH at echo. A different cut-off defined as 18 mmHg should be discussed in patients with less than 12-months. Disclosure of interest The authors have not supplied their declaration of competing interest. https://doi.org/10.1016/j.acvd.2024.07.003 00013 Accurate detection of atrioventricular septal defect (AVSD) in fetal ultrasound using artificial intelligence B. Stos1, M. Lévy1, E. Héry2, I. Durand3, E. Askinazi4, V. Thorey4, M. De Boisredon4, C. Gardella4 1 UE3C, Paris, France 2 Cabinet de Cardiologie, Osny, France 3 Cardiologie Fœtale, Clinique Saint-Hilaire, Rouen, France 4 Brightheart, Paris, France Introduction A deep neural network could accurately detect AVSD in 2nd trimester fetal heart ultrasound video clips. Objective AVSDs are often undetected before birth, with an impact on morbidity and mortality. In addition, detecting AVSD may allow diagnosing genetic disorders (such as Down syndrome), which are frequently associated. Here, we aim at evaluating whether a deep neural network (DNN) could identify AVSD in 2nd trimester (2T) fetal ultrasound video clips. S220

Communications Orales – Jeudi 19 septembre 2024 de 10 h 00 à 11 h 00 Archives of Cardiovascular Disease 117 (2024) S219–S223 Fig. 1 Receiver operating characteristics (ROC) curve of the deep neural network (DNN) for atrioventricular septal defect (AVSD) detection. Methods Patients with single pregnancy who had an echocardiography performed at one center (18–25 weeks GA) were included retrospectively starting from Jan 1, 2021. Based on clinical records, we included consecutive cases of partial or complete AVSD, and consecutive negative cases referred due to family history. This inclusion criterion was used for negative cases to be more representative of the general population, in a center with a high prevalence of CHD since it only receives patients referred for echocardiography. Cases were reviewed by one of two fetal echocardiography experts to confirm that the presence or absence of AVSD was documented in at least one video clip. Patients with no such video clip were excluded. The DNN takes as input all the recorded video clips of a given examination and outputs the absence or presence of AVSD, or an “inconclusive” output if its confidence is low. The DNN was trained to detect AVSD, as seen on the four-chamber view, on patients not included in the evaluation. Results We included 26 cases with AVSD and 129 cases without. The DNN achieved an AUC of 97.1%, a sensitivity of 86.4% (95% CI: 66.7–95.3) and a specificity of 95.2% (95% CI: 90.0–97.8), after excluding inconclusive diagnosis. The DNN predicted a conclusive diagnosis in 95.5% of cases (Fig. 1). Conclusion A DNN could accurately identify AVSD in 2T fetal echocardiography. These results establish the groundwork for efficient and accurate AI-assisted fetal ultrasound heart screening. Acknowledgements The study was supported by Brightheart. Disclosure of interest The authors have not supplied their declaration of competing interest. https://doi.org/10.1016/j.acvd.2024.07.004 00020 Infant mitral valve surgery: Comparison between valvuloplasty and replacement S. Bernheim , M. Pontailler , A. Haydar , D. Bonnet , O. Raisky Necker-Enfants–Malades, Paris, France Introduction Mitral valve replacement in infants is associated with high mortality and a significant rate of re-intervention. There is limited research examining the long-term consequences of valvuloplasty in this particular age group. Objective Evaluate patient outcomes with mitral disease (stenosis, regurgitation or both) who had mitral valvuloplasty or replacement in the first year of life. Methods Descriptive monocentric retrospective study including all children with mitral valve repair or replacement under 1 year of age over a period of 22 years (2001–2023). The outcomes assessed were: early mortality (at 30 days), late mortality, need for reintervention. Results Fifty-six patients were identified. Median age at surgery was 147 days and median weight was 5.1 kg. Thirty-nine patients underwent mitral valve repair and 17 mitral valve replacement. Mean duration of ICU stay was 7 days. Overall median hospital stay was 11 days. Mortality rate was 17.9% (7.1% before discharge). Overall survival was 96% at 30 days, 86% at 1 year 82% at 3 years. Overall survival was significantly higher in patient with mitral valve repair compared to mitral valve replacement (P= 0.039). The only mortality risk factor identified was replacement of the mitral valve compare to repair, HR: 9 (1.3–94.8; P= 0.038). Twenty-three patients (41.1%) needed re-intervention. The re-intervention free survival rate after repair was 81% at 1 year; 65% at 5 years and 46% at 10 years. The re-intervention free survival rate after replacement was 74% at 1 year; 55% at 5 and 10 years. A log-rank test showed no difference in re-intervention free survival between mitral valve repair or replacement. Conclusion Mitral valve surgery in infants carries particularly high risks and is associated with a high rate of re-interventions. While mitral valvuloplasty demonstrates superior outcomes in term of mortality, it frequently serves as a temporary measure, postponing the need for valve replacement. Disclosure of interest The authors have not supplied their declaration of competing interest. https://doi.org/10.1016/j.acvd.2024.07.005 00032 Acute myocarditis according to age: Presentation, management, and early outcomes C. Karsenty1, P. Vignaud-Marighetto1, C. Brusq1, P.Moceri2, P. Lim3, C. Ovaert4, S. Di Filippo5, C. Delmas1 1 CHU de Toulouse, Toulouse, France 2 CHU de Nice, Nice, France 3 AP–HP, Créteil, France 4 AP–HM, Marseille, France 5 CHU de Lyon, Lyon, France Introduction Acute myocarditis (AM) is a rare but severe disease affecting patients of all age. Large multicentric data comparing children and adults are lacking. Objective We aimed to elucidate differences in presentation, management, and outcomes of AM across age groups. Methods A comprehensive French national cohort study, encompassing 53 pediatric and adult units from March 2020 to November 2021, was analysed. Baseline characteristics and evolution, management and in-hospital complications were collected. Major cardiovascular events (MACE) within 30 days included all-cause death, cardiogenic shock, cardiac arrest, ventricular arrhythmias, or complete AV block. Results We included 745 AM patients (328 children and 417 adults), mainly male (73.4%) with a median age of 19.8 years [IQR: 12.5–30.9]. Multisystem inflammatory syndrome (MIS) was more prevalent among pediatric cases (69.8%), whereas infectious aetiology dominated in adults (13.4 vs. 52.4%). Children exhibited a more severe clinical presentation, with increased risk of heart failure (15.9 vs. 7.2%) and cardiogenic shock (14.4 vs. 6.9%), requiring higher use of inotropes (25.0 vs. 9.4%), vasopressors (12.0 vs. 6.2%), and ventilatory support (12.5 vs. 7.7%). Cardiac treatments S221

Communications Orales – Jeudi 19 septembre 2024 de 10 h 00 à 11 h 00 Archives of Cardiovascular Disease 117 (2024) S219–S223 such as beta-blockers (33.4 vs. 84.4%) or ACE/ARB (37.3 vs. 63.1%) were less often used in children whereas corticosteroids (68.3 vs. 14.3%) and immunomodulators (65.1 vs. 4.5%) were more often used. MACE occurrence was substantial but not significantly different between children and adults (18.1 vs. 13.4%). Extra-cardiac manifestations at admission were significant predictors of MACE (aOR: 2.40 [1.43–4.38]), regardless of multisystem inflammatory syndrome (MIS) status. Conclusion AM exhibits variations in presentation, aetiologies, and management, but shares a comparable 30-day prognosis in children and adults. Disclosure of interest The authors have not supplied their declaration of competing interest. https://doi.org/10.1016/j.acvd.2024.07.006 00035 Exercise stress echocardiography in coarctation of the aorta R. Ly1, S. Hascoet2, N. Combes3, P. Di Marco4, C. Karsenty5, I. Van Aershot2, L. Guirgis2, M. Ratsimandresy1, J. Radojevic6 1 Clinique Pasteur, Toulouse, France 2 Hôpital Marie-Lannelongue, Le Plessis-Robinson, France 3 Clinique Pasteur, Hôpital Marie-Lannelongue, Toulouse, Le Plessis-Robinson, France 4 Clinique Rhena, Strasbourg, France 5 CHU Toulouse-Purpan, Toulouse, France 6 Clinique Rhena, Hôpital Marie-Lannelongue, Strasbourg, Le Plessis-Robinson, France Introduction Aortic coarctation (COA) is frequent congenital heart disease. It can be difficult to assess the indication for intervention or re-intervention in some cases. Exercise stress echocardiography (ESE) can be helpful for hemodynamic evaluation in patients with COA or reCOA. Objective We aimed to determine ESE parameters predictive of intervention (angioplasty or surgery). Methods We retrospectively reviewed 94 ESE performed in children (n= 14) and adults with native or repaired CoA and unclear indication for intervention in three centers in France. Exercise test was performed concomitantly to echocardiography on the e-bike in semi-lying position. The protocol was adapted according to the age and the physical condition. Echocardiography protocol included left ventricular adaptation (LVA) to effort and peak systolic gradient (PSG) at isthmus and appearance of diastolic tail during effort. We investigated risk marker associated with subsequent indication for intervention. Results Median age (min–max) was 26 years (10–72). Intervention was subsequently performed in thirteen patients (14.7%). Fifty-six patients (60.2%) had simple COA and 37 (39.8%) had complex anatomy, 24 (25%) had hypertension. Poor LVA was present in 13 (14%). Diastolic tail during effort appeared in 65 cases. The mean pic isthmus gradient 50±21 mmHg (min: 15; max: 124). Poor LVA was associated with more interventions (log-rank, P= 0.004) (Fig. 1). On univariate analysis presence of antihypertensive drugs (HR: 4.13, 95% CI [1.35–12.65]; P= 0.013), systolic blood pressure at rest (HR: 1.04, 95% CI [1.011–1.072]; P= 0.007); the lower exercise power (Watts) (HR: 0.98; 95% CI [0.96–0.99]; P= 0.001); poor LVA (HR: 5.95, 95% CI [1.56–22.65]; P= 0.009); peak systolic gradient at rest and on effort at isthmus (HR: 1.06, 95% CI [1.03–1.10] and HR: 1.04, 95% CI [1.02–1.06]) were significantly predictive of interventions (Table 1). Conclusion ESE is a useful tool for hemodynamic evaluation of COA. More severe forms that needed intervention showed less well adaption of the LV to effort and increase in the cardiac output and afterload. Fig. 1 Kaplan–Meier and log-rank test: comparison outcomes between low and not low contractile reserve and systolic function of left ventricle. https://overcome.key4events.com/api.aspx?e =509&img=Table+and+figure Coa+and+effort+test Abstract.bmp &ai=15377&op=getabstractimg&dirN=0. Table1 Predictive factors associated with outcomes. Disclosure of interest The authors have not supplied their declaration of competing interest. https://doi.org/10.1016/j.acvd.2024.07.007 00061 The pre-Fontan cyanosis period: Key factor in post-Fontan exercise cardiac output in young patients P. David1, A.S. Chaussade2, L. Iserin2, S. Malekzadeh-Milani1, F. Bajolle1, D. Bonnet1, D. Khraiche1, A. Legendre1,2 1 CHU Necker, Cardiologie Pédiatrique, AP-HP, Paris, France 2 Hôpital Européen Georges-Pompidou, Adult Congenital Heart Disease, AP-HP, Paris, France Background Most patients with Fontan circulation struggle to increase cardiac output during exercise affecting aerobic capacity, response to training and quality of life. This poor cardiac performance affects their aerobic capacity, their response to training and their quality of life. We sought to identify the pre-Fontan and managerial factors that influence their exercise cardiac performance. Methods We retrospectively collected pre and post-Fontan anatomical, anamnestic and hemodynamic data in 57 consecutive young Fontan patients (median age 14.7 years) who underwent cardiopulmonary exercise test. We studied their exercise cardiac performance using a thoracic bioelectrical impedance device that allows measurement of the peak cardiac index (pCI). Results The median age at Fontan was 6.4±3 years. In multivariable models, pCI was negatively influenced by cyanosis duration (B=−0.240; P= 0.0002). Peak indexed stroke volume (pSVi) was negatively influenced by Norwood procedure (B = -8.415; P=0.005) and cyanosis duration (B =−1.591; P= 0.0002) and peak heart rate (pHR) by pre-Fontan peripheral oxygen saturation (B = 0.602 P= 0.009). Cyanosis duration≤6.9 years predicted a pSVi ≥45 mL/m2 (AUC = 0.747; P= 0.001). Furthermore, pSVi and pHR were correlated with post-Fontan ventricular filling pressure (post-VFP) (respectively rp =−0.539, p = 0.012 and rp =−0.552; P= 0.010) and S222

Communications Orales – Jeudi 19 septembre 2024 de 10 h 00 à 11 h 00 Archives of Cardiovascular Disease 117 (2024) S219–S223 post-VFP was correlated with bidirectional cavopulmonary shunt duration (rs = 0.498, P=0.023). Conclusion In young Fontan patients, the duration of cyanosis and low peripheral oxygen saturation prior to the Fontan procedure affect both components of cardiac exercise performance probably through increased ventricular filling pressure. Shortening the duration of cyanosis could help preserve cardiac performance in the long term. Disclosure of interest The authors have not supplied their declaration of competing interest. https://doi.org/10.1016/j.acvd.2024.07.008 S223

Archives of Cardiovascular Disease 117 (2024) S224–S227 Disponible en ligne sur ScienceDirect www.sciencedirect.com Communications Orales – Vendredi 20 septembre 2024 de 09 h 05 à 10h15 00005 Which classification best predicts functional prognosis in children with congenital heart disease? A. Gavotto1, P. Amedro2, I. Ouhab1, S. Guillaumont1, I. Liard3, H. Huguet1, M.C. Picot1 1 CHU de Montpellier, Montpellier, France 2 CHU de Bordeaux, Bordeaux, France 3 CHU de Nîmes, Nîmes, France Introduction Despite these advances in paediatric cardiology, the stratification of CHD severity using a simple and reproducible classification has not been established, as can be the NYHA functional class in adult heart failure. Various CHD classifications have been used in CHD, focusing on anatomical lesions, complexity of care, or physiological status, but their prognostic value has not been determined. Objective We aimed to compare the accuracy of the main existing CHD classifications (Uzark, Stout and Bethesda classifications), in the prediction of functional status in children with CHD, as determined by cardiopulmonary fitness. Methods Longitudinal cohort study. Results The CHD population having had 2 CPET included 296 subjects (n= 129 female). The time between the first (T1) and second CPET (T2) assessments was 4.1±1.6 years. The performance of classifications according to VO2max at T1 was better for Uzark classification. The VO2max Z-score decreased significantly according to the severity group (groups 1 and 2 > group 3 > group 4) and group 4 had a significant VO2max decrease of −6.68 [−10.69; −2.67] mL/kg/min compared to group 1. The prediction of classifications at T2 according to VO2max was better for Uzark classification with AUC values of 0.62 [0.55–0.69], compared to 0.59 [0.51–0.66] for Stout and 0.55 [0.48–0.62] for Bethesda (Fig. 1). Conclusion Among the existing cardiovascular risk classifications for CHD, the Uzark classification appeared to be the most reliable for discriminating the severity of CHD according to exercise capacity and for predicting the VO2max impairment than the other classifications tested. This longitudinal study also showed the continued decline in exercise capacity, whatever the CHD, and recalls the interest of regular monitoring to offer care adapted to these patients (such as rehabilitation programs) for primary prevention of the added cardiovascular risks of adulthood. Fig. 1 1875-2136/

Communications Orales – Vendredi 20 septembre 2024 de 09 h 05 à 10 h 15 Archives of Cardiovascular Disease 117 (2024) S224–S227 Disclosure of interest The authors have not supplied their declaration of competing interest. https://doi.org/10.1016/j.acvd.2024.07.010 00009 Safety and efficacy of dapagliflozin in patients with systemic right ventricular dysfunction: Preliminary results M. Albertini1, V. Waldmann1, P. David1, A. Barat1, A.S. Chaussade1, L. Iserin1, M. Ladouceur2 1 Centre de Référence des Malformations Cardiaques Congénitales Complexes (M3C), Adult Congenital Heart Disease Unit, Hôpital Européen Georges-Pompidou, AP–HP, Paris Descartes University, Centre de Recherche Cardiovasculaire de Paris, Inserm U970, Paris, France 2 Division of Cardiology, University Hospital Geneva, Geneva, Switzerland Introduction Heart failure (HF) is the leading cause of death in adults with a systemic right ventricle (sRV). Dapagliflozin has been shown to reduce hospitalization for HF and all-cause death in patients with HF and reduced ejection fraction. The impact of dapagliflozin in patients with a sRV remains unknown. Objective We aimed to evaluate the efficacy and safety of dapagliflozin in patients with sRV. Methods This was a prospective, observational, single-center study. All symptomatic (NYHA≥2) patients with a sRV dysfunction despite optimal medical treatment were included from March 2023 to March 2024. Patients were assessed at baseline and 3, 6 and 12 months after dapagliflozin introduction. The primary endpoint was the 6-minute walk distance. Secondary endpoints included NT-proBNP, quality of life (KCCQ-12), NYHA class, systemic and sub-pulmonary ventricular systolic function, and potential treatment-related side effects. Expected results A total of 32 patients were included. Mean age was 48 years (range: 19–79), 20 (62%) of patients were male, 12 (38%) had congenitally corrected transposition of the great arteries and 20 (62%) had transposition of the great arteries with atrial switch. Preliminary results at 6 months were available in 20 patients. There was no statistically significant improvement in 6minute walk distance (558.0 m vs. 599.5, P= 0.11) nor NT-proBNP (355.5 pg/mL vs. 293.5, P= 0.81). However, the quality of life of patients (80.5 vs. 92.0, P< 0.01) and the right ventricle global longitudinal strain (−11.2%vs. −12.9, P< 0.01) significantly improved. The drug was well tolerated with no side effects reported (Fig. 1). Perspectives These preliminary results suggest that dapagliflozin is well tolerated and associated with improved quality of life and Fig. 1 right ventricle global longitudinal strain in patients with sRV dysfunction. Final results will bring important data on long-term outcomes associated with dapagliflozin use in this population. Disclosure of interest The authors have not supplied their declaration of competing interest. https://doi.org/10.1016/j.acvd.2024.07.011 00010 Independent external evaluation of pediatric HCM Risk Scores in predicting severe ventricular arrhythmias M. Wilkin , V. Waldmann Hôpital Necker-Enfants–Malades, AP–HP, Paris, France Introduction Sudden cardiac death (SCD) is the most common cause of death in childhood hypertrophic cardiomyopathy (HCM). Recently, two risk scores have been developed to estimate the 5year risk of SCD. Objective We aimed to assess their respective performances in an independent cohort of primary prevention children with HCM. Methods All patients with HCM < 18-year-old from a singlecenter were retrospectively included between 2003 and 2023. Secondary and syndromic causes of HCM were excluded as well as children with inaugural sustained ventricular arrythmias. HCM Risk-Kids and PRIMaCY risk scores were calculated at diagnosis and during follow-up. The primary composite outcome included sustained ventricular arrhythmia, appropriate ICD therapy, aborted cardiac arrest, or SCD. Results Hundred primary prevention children were included (mean age 7.1±5.6 years, 59.0% males), with a mean follow-up of 8.6±5.5 years.13 (13.0%) patients experienced the primary composite outcome. When only considering events during the 5 first years, Harrel’s C index was 0.52 (95% CI: 0.27–0.77) for HCM RiskKids (≥6%) and 0.70 (95% CI: 0.59–0.80) for PRIMaCY (> 8.3%), with 1 patient potentially treated by ICD for every 25 ICDs implanted for HCM Risk Kids and 1 for every 14 ICDs implanted for PRIMaCY. When risk scores were repeated and all primary outcomes during follow-up considered, all events except one (93.2%) were correctly identified using both risk scores, with 1 patient potentially treated by ICD for every 5.6 ICDs implanted for HCM Risk Kids and 1 for every 5.3 ICDs implanted for PRIMaCY. Among 44 (44.0%) patients implanted with an ICD, all primary prevention patients whohad≥one appropriate ICD therapy during follow-up had HCM Risk-Kids≥6% and PRIMaCY > 8.3% at implantation. Conclusion Our findings suggest imperfect discrimination between low and high-risk HCM patients using these two risk scores. The performance or risk scores was substantially improved by periodic re-assessment during follow-up. Disclosure of interest The authors have not supplied their declaration of competing interest. https://doi.org/10.1016/j.acvd.2024.07.012 S225

Communications Orales – Vendredi 20 septembre 2024 de 09 h 05 à 10 h 15 Archives of Cardiovascular Disease 117 (2024) S224–S227 00025 Clinical and paraclinical evolution of term and near term neonates with persistent pulmonary hypertension, treated with treprostinil and/or epoprostenol, hospitalized in NICU C.Mazepa1, S.Mur2, G. Gascoin3, L. Storme2, N. Joram4, C. Viard5, Y. Dulac6, M. Butin6, S. Breinig1 1 Neonatal and Pediatric Intensive Care Unit, Children’s Hospital, CHU de Toulouse, Toulouse, France 2 Neonatal Intensive Care Unit, Lille University Hospital, Lille, France 3 Neonatal Intensive Care, University Hospital Centre Toulouse, Toulouse, France 4 Pediatric Intensive Care Unit, Nantes University Hospital, Nantes, France 5 Pediatric Pharmacy, Children’s Hospital, CHU de Toulouse, Toulouse, France 6 Department of Pediatric Cardiology, Children’s Hospital, CHU de Toulouse, Toulouse, France Introduction Persistent pulmonary hypertension of the newborn (PPHN) is a serious disease that occurs in 1.9 per 1000 live births. Epoprostenol and treprostinil, witch are prostacyclin analogues, are used by some care teams in the treatment of PPHN, in absence of established proof of their efficacy in this indication. Objective The main objective of this retrospective multicenter study was to evaluate clinical and paraclinical evolution of newborns treated with treprostinil and/or epoprostenol during neonatal period. Methods Inclusion of neonates≥34 SA and aged≤28 days, with clinical signs and≥1 ultrasound sign of pulmonary hypertension, treated with treprostinil and/or epoprostenol between 01/01/17 and 31/12/22 in 4 French teaching hospitals. Data collected included clinical, biological and ultrasound parameters. Results Seventy patients were included, with a mean age of 39 SA + 2.5 days and a mean birth weight of 3200 g, including 40 congenital diaphragmatic hernia (57%). On arrival in NICU over 90% of newborns had iso or supra-systemic pulmonary hypertension. The introduction of prostacyclin analogues appears to have a beneficial effect on the pre- and post-ductal saturation differential as well as on the echocardiographic evolution of pulmonary hypertension. The temporality of evolution of pulmonary hypertension seems to depend on the underlying etiology due to the different pathophysiological mechanisms. Moreover, our study shows that epoprostenol and treprostinil can be used in neonates suffering from iNO refractory PPHN without significant adverse effects. Conclusion We report few adverse effects of epoprostenol and treprostinil in neonates treated for in iNO refractory PPHN. However, the heterogeneity of practices between centers requires further studies to establish recommendations for the use of these molecules in severe neonatal pulmonary hypertension. Disclosure of interest The authors have not supplied their declaration of competing interest. https://doi.org/10.1016/j.acvd.2024.07.013 00027 A 3D analysis of ventricle position in patients with heterotaxy shows high frequency of ventricle malposition in D-Loop hearts D.Madec1, S. Bernheim1, A. Desgrange2, N. Boddaert3, O. Raisky4 1 M3C-Necker, Institut Imagine, Paris, France 2 Institut Imagine, Paris, France 3 Necker, Paris, France 4 M3C-Necker, Paris, France Introduction Congenital heart defects in the context of heterotaxy are severe, with a complex anatomy. These defects are described using the binary concept of clinical loop (D-Loop or LLoop), which implies that ventricle position is determined during heart looping. Recent work in the mouse has shown unexpected plasticity of ventricle position after heart looping. Based on a analysis of Nodal mutants with heterotaxy and right bronchus isomerism, 27% of D-Loop mutants at birth underwent leftward embryonic heart looping. These mice with a revertant loop, all display abnormal ventricle position. Objective Analysis of 3D ventricle position in human patients with heterotaxy and right bronchus isomerism, using the strategy developed in the mouse based on the 3D orientation of the interventricular septum. Methods Constitution of an heterotaxy cohort from the Necker database. Controls were selected as patients with transposition of the great arteries who had a systematic CT scan at 6 years. Three dimensions reconstructions of CT scans and quantitative analyses were performed using the Imaris software. Expected results Control patients had their right ventricle-left ventricle axis in the expected anteroposterior and left-right orientation. We collected 506 patients with heterotaxy syndrome, 186 of whom had CT scan. Forty patients had right bronchial isomerism. Among them, we found 29 (72%) patients who had an orientation of the interventricular septal axis similar to control, 7 (18%) patients with abnormal supero-inferior ventricles and 4 (10%) patients with abnormal left-right ventricles (Fig. 1). In total, 32% of patients in D-Loop have a malpositioned interventricular septum whereas 0 of patients in L-Loop. Perspectives There is a high proportion of malpositioned ventricles in patients with D-Loop (32%), similar to what is observed in Nodal mouse mutants. This result supports conserved mechanisms in the mouse and human, including plasticity of ventricle position after heart looping. Fig. 1 3D quantitative analysis of ventricle position in 40 patients with heterotaxy syndrome and correlation with clinical loop. A. Determination of ventricle position in 3D as the perpendicular to the interventricular septum (IVS) in patients compared to controls. B and C. 3D vector corrdinates of ventricle position in cases and controls, according to the clinical loop class. S226

Communications Orales – Vendredi 20 septembre 2024 de 09 h 05 à 10 h 15 Archives of Cardiovascular Disease 117 (2024) S224–S227 Disclosure of interest The authors declare that they have no competing interest. https://doi.org/10.1016/j.acvd.2024.07.014 00043 Restrictive LV-PA conduit in ccTGA with VSD and LVOTO A. Moiroux-Sahraoui , N. Derridj , R. Gaudin , M. Pontailler , P. Vouhe , D. Bonnet , O. Raisky Hôpital Necker-Enfants–Malades, Paris, France Introduction Congenitally corrected transposition of the great arteries (ccTGA) is a rare, complex and challenging structural heart disease. Therapeutic management remains controversial, particularly in patients with left ventricle outflow tract obstruction (LVOTO). As the natural history of ccTGA with LVOTO is overall very satisfactory and interventions that increase sub-pulmonary left ventricle (LV) pressure load are associated with a reduction of tricuspid regurgitation (TR) and systemic right ventricle (RV) dysfunction, we began to use restrictive left ventricle-to-pulmonary artery (LV-PA) conduit without ventricular septal defect (VSD) closure as part of the physiological repair of ccTGA/VSD/LVOTO. Objective To report on the outcome of ccTGA/VSD/LVOTO patients treated with restrictive LV-PA conduit without VSD closure. Methods Between 1979 and 2024, 9 consecutive patients with ccTGA/non-restrictive VSD/LVOTO underwent restrictive LV-PA conduit interposition without VSD closure at Necker Sick Children’s Hospitals (Paris, France). Results Six (66.7%) patients had pulmonary stenosis and 3 (33.3%) patients had pulmonary atresia. Median age and weight at surgery were 5 (IQR: 16.6) years and 18 (IQR: 36.5) kgs. Five (55.6%) patients had previously undergone surgical palliation. No patient died after surgery with a median follow-up of 9.8 (IQR: 13.4) years. No patient required permanent pacemaker implantation for complete AV block. Freedom from reintervention was 62.5% (95% CI: 22.9–86.1) at 10 years. Late reintervention consisted in LV-PA conduit replacement (n= 3), PA stenting (n= 1) and one and half ventricle repair (hemi-Senning, Glenn, Rastelli, n= 1). At last followup, only one patient was significantly limited in physical activity (NYHA class III). No patient developed atrial or ventricular arrythmia. No patients developed moderate or severe TR and only 2 (22.2%) patients had decreased RV function and dilatation. Conclusion We believe that restrictive LV-PA conduit placement without VSD closure represents a worthwhile biventricular alternative strategy to anatomic repair when it is deemed too risky and/or impractical. Disclosure of interest The authors have not supplied their declaration of competing interest. https://doi.org/10.1016/j.acvd.2024.07.015 00059 Morphological risk markers for major adverse events following transcatheter closure of ostium secundum atrial septal defects in 2253 children and adults G. Albenque , E. Valdeolmillos , C. Foray , M. Jaber , F. Lecerf , E. Belli , C. Batteux , J. Petit , S. Hascoët Hôpital Marie-Lannelongue, Le Plessis-Robinson, France Fig. 1 Introduction Since the 2000s, transcatheter closure has been the primary treatment for ostium secundum atrial septal defect (osASD) in children and adults. Objective This study aims to identify factors associated with short-term adverse outcomes following this procedure in a large cohort. Methods A prospective, single-center cohort study included 2,253 consecutive patients (median age 28 years; children: n= 865, 38.4%) who underwent transcatheter ASD closure with the Amplatzer Septal Occluder (ASO; AmplatzerTM Atrial Septal Occluder Device, Abbott, Chicago, USA) from May 1998 to December 2021. Peri-procedural data associated with major adverse events were investigated retrospectively. Results The mean ASD diameter, as measured by transthoracic echocardiography, was 18 mm. About 8.9% of patients had an ASD size-to-body surface area (BSA) ratio of≥20mm/m2. Deficient rims (< 5 mm) were identified in 27.9% of patients, with retroaortic rim deficiency in 22.7% and inferior rim deficiency in 0.9%. The median ASO diameter was 24 mm, with a procedural success rate of 98.2%. ASD/BSA≥20mm/m2 was associated with procedural failure, while age and weight were not. Major peri-procedural adverse events occurred in 31 patients (1.4%), with 19 device embolizations and 2 cardiac erosions. No peri-procedural deaths were reported. Multivariate analysis showed that deficiency of the inferior vena cava rim and an ASD size/BS ratio≥20mm/m2 were significantly associated with major adverse events (P=0.002 and P= 0.035, respectively) (Fig. 1). Conclusion Transcatheter osASD closure using ASO is safe and effective in a large spectrum population with low rate of periprocedural adverse events and favorable short-term outcomes. ASD size-to-body surface area ratio (≥20mm/m2) and inferior rim deficiency are key morphological risk markers for major adverse events following this procedure. Disclosure of interest The authors have not supplied their declaration of competing interest. https://doi.org/10.1016/j.acvd.2024.07.016 S227

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