Archives of Cardiovascular Diseases最新文献

{"title":"Dapagliflozin in adult patients with failing Fontan (DAPA-FONTAN): A prospective multicentre study","authors":"Mathieu Albertini, Fabien Labombarda, Elise Barre, Valentin Baland, Jelena Radojevic, Fanny Dion, Sylvie Falcon-Eicher, Charlotte Denis, Pauline David, Alexis Barat, Antoine Legendre, Anne-Solene Chaussade, Elie Mousseaux, Sebastien Hascoet, Gilles Soulat, Victor Waldmann, Laurence Iserin","doi":"10.1016/j.acvd.2026.06.007","DOIUrl":"10.1016/j.acvd.2026.06.007","url":null,"abstract":"<div><h3>Introduction</h3><div>Heart failure (HF) is the leading cause of mortality in adults with congenital heart disease, particularly in patients with a univentricular heart and Fontan circulation. This physiology is associated with progressive morbidity and the development of a failing Fontan (FF), most often related to systolic or diastolic ventricular dysfunction. Therapeutic options remain limited. Dapagliflozin, a sodium–glucose cotransporter 2 inhibitor recommended for left-sided HF, may provide functional benefits in this population. We aimed to evaluate the impact of dapagliflozin on functional capacity in adults with FF related to ventricular dysfunction.</div></div><div>Heart failure (HF) is the leading cause of mortality in adults with congenital heart disease, particularly in patients with a univentricular heart and Fontan circulation. This physiology is associated with progressive morbidity and the development of a failing Fontan (FF), most often related to systolic or diastolic ventricular dysfunction. Therapeutic options remain limited. Dapagliflozin, a sodium–glucose cotransporter 2 inhibitor recommended for left-sided HF, may provide functional benefits in this population. We aimed to evaluate the impact of dapagliflozin on functional capacity in adults with FF related to ventricular dysfunction.</div><div><h3>Methods</h3><div>We conducted a 6-month prospective, multicenter, observational study including adults (><!--> <!-->18 years) with FF confirmed by right heart catheterization within the previous 6 months. FF was defined by elevated ventricular filling pressures (ventricular end-diastolic pressure or pulmonary artery wedge pressure<!--> <!-->≥<!--> <!-->15<!--> <!-->mmHg) associated with systolic dysfunction (ejection fraction<!--> <!-->≤<!--> <!-->40%) or diastolic dysfunction (ejection fraction<!--> <!-->≥<!--> <!-->41%). Patients with severe atrioventricular valve regurgitation, elevated pulmonary vascular resistance, Fontan circuit obstruction, pregnancy, or systolic blood pressure<!--> <!--><<!--> <!-->95<!--> <!-->mmHg were excluded. The primary endpoint was change in peak oxygen consumption (VO<sub>2max</sub>) assessed by cardiopulmonary exercise testing between baseline and 6 months. Secondary endpoints included NYHA class, quality of life (KCCQ-12), oxygen saturation, NT-proBNP, ventricular function, HF hospitalizations, diuretic dose, biological parameters, blood pressure, and safety.</div></div><div>We conducted a 6-month prospective, multicenter, observational study including adults (><!--> <!-->18 years) with FF confirmed by right heart catheterization within the previous 6 months. FF was defined by elevated ventricular filling pressures (ventricular end-diastolic pressure or pulmonary artery wedge pressure<!--> <!-->≥<!--> <!-->15<!--> <!-->mmHg) associated with systolic dysfunction (ejection fraction<!--> <!-->≤<!--> <!-->40%) or diastolic dysfunction (ejection fraction<!--> <!-->≥<!--> <!-->41%).","PeriodicalId":55472,"journal":{"name":"Archives of Cardiovascular Diseases","volume":"119 8","pages":"Page S241"},"PeriodicalIF":2.9,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148816085","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Outcome after pulmonary valve remplacement performed at a senior age in patients with repaired tetralogy of Fallot","authors":"Sophie Quennelle, Florence Viau, Anne Claire Casalta, Resseguier Noemie, Caroline Ovaert, Gilbert Habib, Loïc Mace, Marien Lenoir, Philippe Aldebert","doi":"10.1016/j.acvd.2026.06.018","DOIUrl":"10.1016/j.acvd.2026.06.018","url":null,"abstract":"<div><h3>Introduction</h3><div>The optimal timing of pulmonary valve replacement (PVR) in adults with repaired Tetralogy of Fallot (TOF) and severe pulmonary regurgitation remains controversial, particularly in older patients. Current recommendations rely largely on right ventricular volumes assessed by cardiac magnetic resonance imaging (MRI), although their prognostic value remains debated.</div></div><div>The optimal timing of pulmonary valve replacement (PVR) in adults with repaired Tetralogy of Fallot (TOF) and severe pulmonary regurgitation remains controversial, particularly in older patients. Current recommendations rely largely on right ventricular volumes assessed by cardiac magnetic resonance imaging (MRI), although their prognostic value remains debated.</div><div><h3>Methods</h3><div>We conducted a retrospective single-center study including patients aged<!--> <!-->≥<!--> <!-->30 years with repaired TOF who underwent surgical PVR between 2009 and 2018. The primary outcome was a composite endpoint including supraventricular arrhythmia, cardiovascular rehospitalization, or all-cause death during follow-up. Clinical, imaging, and hemodynamic variables were analyzed using univariate and multivariate logistic regression.</div></div><div>We conducted a retrospective single-center study including patients aged<!--> <!-->≥<!--> <!-->30 years with repaired TOF who underwent surgical PVR between 2009 and 2018. The primary outcome was a composite endpoint including supraventricular arrhythmia, cardiovascular rehospitalization, or all-cause death during follow-up. Clinical, imaging, and hemodynamic variables were analyzed using univariate and multivariate logistic regression.</div><div><h3>Results</h3><div>Forty-eight patients (mean age at PVR 41<!--> <!-->±<!--> <!-->9.8 years) were included, with a mean follow-up of 52 months. The primary outcome occurred in 23 patients (48%). Preoperative signs of advanced disease, including right heart failure, left ventricular dysfunction, and a history of syncope or ventricular tachycardia, were associated with adverse outcomes. In multivariate analysis, elevated right ventricular end-diastolic pressure (RVEDP) was the only independent predictor of postoperative morbidity and mortality (OR 1.49, 95% CI 1.01–3.40; <em>P</em> <!-->=<!--> <!-->0.04). Preoperative right ventricular volumes assessed by MRI were not associated with the primary outcome in multivariate analysis.</div></div><div>Forty-eight patients (mean age at PVR 41<!--> <!-->±<!--> <!-->9.8 years) were included, with a mean follow-up of 52 months. The primary outcome occurred in 23 patients (48%). Preoperative signs of advanced disease, including right heart failure, left ventricular dysfunction, and a history of syncope or ventricular tachycardia, were associated with adverse outcomes. In multivariate analysis, elevated right ventricular end-diastolic pressure (RVEDP) was the only independent predictor of postoperative morbidity and mortality ","PeriodicalId":55472,"journal":{"name":"Archives of Cardiovascular Diseases","volume":"119 8","pages":"Page S247"},"PeriodicalIF":2.9,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148815893","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Safety and efficacy of redo percutaneous pulmonary valve implantation after transcatheter valve failure: A multicentre observational study","authors":"Clement Karsenty, Sebastien Hascoet, Peter Kramer, F. Berger, Christophe De Meester, Heike Schneider, Raymond Haddad, Aleksander Kempny, Alain Fraisse, Zakaria Jalal, Patrice Guérin, Ali Houeijeh, Bruno Lefort, Stanimir Georgiev, Sophie-Guiti Malekzadeh Milani","doi":"10.1016/j.acvd.2026.06.046","DOIUrl":"10.1016/j.acvd.2026.06.046","url":null,"abstract":"<div><h3>Introduction</h3><div>Percutaneous pulmonary valve implantation (PPVI) is an established and increasingly used therapy for patients with dysfunctional right ventricular outflow tract (RVOT). We assessed the safety, efficacy, and early outcomes of redo-PPVI performed for dysfunctional transcatheter pulmonary valves.</div></div><div>Percutaneous pulmonary valve implantation (PPVI) is an established and increasingly used therapy for patients with dysfunctional right ventricular outflow tract (RVOT). We assessed the safety, efficacy, and early outcomes of redo-PPVI performed for dysfunctional transcatheter pulmonary valves.</div><div><h3>Methods</h3><div>We conducted a retrospective, multicentre observational study including patients who underwent redo-PPVI.</div></div><div>We conducted a retrospective, multicentre observational study including patients who underwent redo-PPVI.</div><div><h3>Results</h3><div>Twenty-four centres enrolled a total of 162 patients (mean age 25.2<!--> <!-->±<!--> <!-->11.5 years). The index valve was a Melody valve in 102 patients (63.0%), a Sapien valve in 59 (37.7%), and a Harmony valve in 1 (0.6%). The redo valve was a Melody valve in 87 patients (53.7%), a Sapien valve in 70 (43.2%), and a Myval valve in 5 (3.1%). The mean time from index PPVI to redo-PPVI was 7.4<!--> <!-->±<!--> <!-->4.0 years. Indications for redo-PPVI were pulmonary regurgitation in 98 patients (60.5%), stenosis in 16 (9.9%), and mixed lesions in 48 (29.6%). Periprocedural complications occurred in 7.4% of patients without relationship with the specific setting of redo-PPVI, such as atrial lead dislocations (<em>n</em> <!-->=<!--> <!-->2) or vascular access complications (<em>n</em> <!-->=<!--> <!-->2). No periprocedural deaths or coronary complications were reported. During follow-up (<em>n</em> <!-->=<!--> <!-->148; median 3.3 years [0.9–6.0]), 6 patients died (3 due to de novo infective endocarditis, 1 due to heart failure) and 29 patients experienced adverse events (2 heart transplantation, 2 cases of heart failure, 9 cases of de novo endocarditis, 1 case of protein-losing enteropathy, 15 cases of sustained tachycardia including 4 ventricular tachycardia, and 11 atrial arrhythmias). Eighteen patients required reintervention: 14 underwent surgical pulmonary valve replacement and 4 underwent balloon dilatation. At last follow-up, none or mild regurgitation was present in 90.7% of patients, and the maximal transvalvular gradient was 26.5<!--> <!-->±<!--> <!-->11.9<!--> <!-->mmHg.</div></div><div>Twenty-four centres enrolled a total of 162 patients (mean age 25.2<!--> <!-->±<!--> <!-->11.5 years). The index valve was a Melody valve in 102 patients (63.0%), a Sapien valve in 59 (37.7%), and a Harmony valve in 1 (0.6%). The redo valve was a Melody valve in 87 patients (53.7%), a Sapien valve in 70 (43.2%), and a Myval valve in 5 (3.1%). The mean time from index PPVI to redo-PPVI was 7.4<!--> <!-->±<!--> <!-->4.0 years. Indications for redo-","PeriodicalId":55472,"journal":{"name":"Archives of Cardiovascular Diseases","volume":"119 8","pages":"Pages S260-S261"},"PeriodicalIF":2.9,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148815900","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Pulmonary atresia in congenital heart defects: Can we deduce embryological mechanisms from morphological phenotype?","authors":"Jeanne Mechin, Cloé Huet, Julie Karila Cohen, Zahra Belhadjer, Damien Bonnet, Lucile Houyel","doi":"10.1016/j.acvd.2026.06.033","DOIUrl":"10.1016/j.acvd.2026.06.033","url":null,"abstract":"<div><h3>Introduction</h3><div>Pulmonary atresia is usually classified into tetralogy of Fallot with pulmonary atresia (TOF-PA) and pulmonary atresia with intact ventricular septum (PA-IVS). It can also be part of more complex cardiac lesions. Our objective was, analyzing fetal and neonatal human heart specimens, to determine the anatomical and pathophysiological mechanisms responsible for the different phenotypes of pulmonary atresia.</div></div><div>Pulmonary atresia is usually classified into tetralogy of Fallot with pulmonary atresia (TOF-PA) and pulmonary atresia with intact ventricular septum (PA-IVS). It can also be part of more complex cardiac lesions. Our objective was, analyzing fetal and neonatal human heart specimens, to determine the anatomical and pathophysiological mechanisms responsible for the different phenotypes of pulmonary atresia.</div><div><h3>Methods</h3><div>We analyzed macroscopically 151 specimens with pulmonary atresia from the anatomic collection of the French Reference Center for Complex Congenital Heart Defects. Measurements included the height and width of the subpulmonary atrium, the diameter of the tricuspid valve, the size and thickness of the subpulmonary ventricle, the location of the outlet septum, the nature of the pulmonary atresia (muscular or valvar), the diameter of the pulmonary arteries.</div></div><div>We analyzed macroscopically 151 specimens with pulmonary atresia from the anatomic collection of the French Reference Center for Complex Congenital Heart Defects. Measurements included the height and width of the subpulmonary atrium, the diameter of the tricuspid valve, the size and thickness of the subpulmonary ventricle, the location of the outlet septum, the nature of the pulmonary atresia (muscular or valvar), the diameter of the pulmonary arteries.</div><div><h3>Results</h3><div>Hearts were classified in six groups: TOF-PA, 53 (35%); PA-IVS, 43 (28%): tricuspid anomalies, 17 (11%); anomalous ventriculo-arterial connections, 27 (18%); univentricular hearts, 11 (7%). Pulmonary atresia was mostly valvar in tricuspid anomalies and PA-IVS and muscular in the other groups. Pulmonary arteries were larger in PA-IVS. Outlet septum was incorporated to ventricular septum in PA-IVS and tricuspid anomalies, malaligned in TOF-PA, variable in other groups. The right ventricular wall was thicker in PA-IVS and thinner in tricuspid anomalies. The tricuspid valve was, by definition, abnormal in tricuspid anomalies and was also abnormal in most of PA-IVS specimens. The right atrium was larger in tricuspid anomalies.</div></div><div>Hearts were classified in six groups: TOF-PA, 53 (35%); PA-IVS, 43 (28%): tricuspid anomalies, 17 (11%); anomalous ventriculo-arterial connections, 27 (18%); univentricular hearts, 11 (7%). Pulmonary atresia was mostly valvar in tricuspid anomalies and PA-IVS and muscular in the other groups. Pulmonary arteries were larger in PA-IVS. Outlet septum was incorporated to ventricular septum in P","PeriodicalId":55472,"journal":{"name":"Archives of Cardiovascular Diseases","volume":"119 8","pages":"Page S254"},"PeriodicalIF":2.9,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148815965","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"French ISACC Registry: Impact of sacubitril/valsartan in adults with complex congenital heart disease (systemic right ventricle and single ventricle)","authors":"Claire Massardier, Johanne Auriau, Hélène Bouvaist, Stéphanie Douchin, Matthias Lachaud","doi":"10.1016/j.acvd.2026.06.014","DOIUrl":"10.1016/j.acvd.2026.06.014","url":null,"abstract":"<div><h3>Introduction</h3><div>Currently, 90% of patients with congenital heart disease survive into adulthood. In this population, heart failure (HF) represents a major cause of morbi-mortality, and patients with a systemic right ventricle (SRV) or a single ventricle (SV) are particularly at risk. Sacubitril/Valsartan is an established therapy for heart failure in adults; however, data regarding its use in congenital cardiology remain limited.</div></div><div>Currently, 90% of patients with congenital heart disease survive into adulthood. In this population, heart failure (HF) represents a major cause of morbi-mortality, and patients with a systemic right ventricle (SRV) or a single ventricle (SV) are particularly at risk. Sacubitril/Valsartan is an established therapy for heart failure in adults; however, data regarding its use in congenital cardiology remain limited.</div><div><h3>Methods</h3><div>This prospective, multicenter, observational study conducted over 12 months, aimed to evaluate efficacy and safety of Sacubitril/Valsartan in patients with a SRV or a SV dysfunction (systemic ventricular ejection fraction<!--> <!-->≤<!--> <!-->40%) and New York Heart Association (NYHA) class II–III despite optimal HF therapy. The primary endpoint was the comparison of maximal oxygen uptake (peak VO<sub>2</sub>) before initiation and after 12 months of treatment.</div></div><div>This prospective, multicenter, observational study conducted over 12 months, aimed to evaluate efficacy and safety of Sacubitril/Valsartan in patients with a SRV or a SV dysfunction (systemic ventricular ejection fraction<!--> <!-->≤<!--> <!-->40%) and New York Heart Association (NYHA) class II–III despite optimal HF therapy. The primary endpoint was the comparison of maximal oxygen uptake (peak VO<sub>2</sub>) before initiation and after 12 months of treatment.</div><div><h3>Results</h3><div>A total of 50 patients were included, 33 men (71.7%), 41.3<!--> <!-->±<!--> <!-->9.6years. Hence, 43 patients (86%) had a SRV, including 32(64%) D-transposition of the great arteries and 11 (22%) congenitally corrected transposition of the great arteries. Among the 7 patients (14%) with a SV, 4 (8%) had a left ventricle. After 12 months of treatment at the maximum tolerated dose, no improvement in peak VO<sub>2</sub> was observed: 17.3<!--> <!-->mL/kg/min [14–21.6] vs 17.5<!--> <!-->mL/kg/min [14.4–21.8] (<em>P</em> <!-->=<!--> <!-->0.656). However, improvement was noticed in NYHA class (2.19<!--> <!-->±<!--> <!-->0.6 vs 1.72<!--> <!-->±<!--> <!-->0.6; <em>P</em> <!-->≤<!--> <!-->0.001), NT-proBNP (998<!--> <!-->pg/mL [451–1751] vs 639<!--> <!-->pg/mL [365.5–1174.5]; <em>P</em> <!-->=<!--> <!-->0.002), right ventricular fractional area change (27.71<!--> <!-->±<!--> <!-->6.9% vs 31.6<!--> <!-->±<!--> <!-->6.9%; <em>P</em> <!-->≤<!--> <!-->0.001), and tricuspid regurgitation grade (2.13<!--> <!-->±<!--> <!-->0.9 vs 1.70<!--> <!-->±<!--> <!-->0.7; <em>P</em> <!-->≤<!--> <!-->0.001). Sacubit","PeriodicalId":55472,"journal":{"name":"Archives of Cardiovascular Diseases","volume":"119 8","pages":"Page S245"},"PeriodicalIF":2.9,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148816004","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Associated factors to recoarctation in a large cohort of infant operated of aortic coarctation before 1 year of age","authors":"Oscar Werner, Charlotte Duseigneur, Clement Karsenty, Camille Velly, Marie-Christine Picot, Helena Huguet, Alban-Elouen Baruteau, Virginie Fouilloux, Caroline Ovaert, Bruno Lefort, Jean-Marc El Arid, Marie Vincenti, Sophie Guillaumont, Pascal Amédro","doi":"10.1016/j.acvd.2026.06.063","DOIUrl":"10.1016/j.acvd.2026.06.063","url":null,"abstract":"<div><h3>Introduction</h3><div>Among congenital heart diseases, aortic coarctation accounts for 5–8% of patients and is associated with significant morbidity and mortality from childhood to adulthood. Neonatal forms are considered critical congenital heart defects, requiring early management under optimal conditions to ensure a favorable cardiovascular prognosis. Among postoperative complications, aortic recoarctation is the most frequent early complication in these patients. However, to date, the predictive factors for this complication remain poorly understood. The objective of this multicenter study is to describe the morbidity and mortality of aortic coarctation operated on before 1 year of age in a large multicenter cohort and to identify predictive factors for aortic recoarctation.</div></div><div>Among congenital heart diseases, aortic coarctation accounts for 5–8% of patients and is associated with significant morbidity and mortality from childhood to adulthood. Neonatal forms are considered critical congenital heart defects, requiring early management under optimal conditions to ensure a favorable cardiovascular prognosis. Among postoperative complications, aortic recoarctation is the most frequent early complication in these patients. However, to date, the predictive factors for this complication remain poorly understood. The objective of this multicenter study is to describe the morbidity and mortality of aortic coarctation operated on before 1 year of age in a large multicenter cohort and to identify predictive factors for aortic recoarctation.</div><div><h3>Methods</h3><div>This is a retrospective, multicenter, longitudinal observational study conducted in five French surgical centers. Patients who underwent surgery for aortic coarctation before the age of one year, between January 2011 and December 2025, were included. Patients with complex congenital heart disease and those who initially underwent cardiac catheterization were excluded. The primary endpoint was 6-year survival free from aortic recoarctation. Secondary endpoints were overall survival and survival free from arterial hypertension.</div></div><div>This is a retrospective, multicenter, longitudinal observational study conducted in five French surgical centers. Patients who underwent surgery for aortic coarctation before the age of one year, between January 2011 and December 2025, were included. Patients with complex congenital heart disease and those who initially underwent cardiac catheterization were excluded. The primary endpoint was 6-year survival free from aortic recoarctation. Secondary endpoints were overall survival and survival free from arterial hypertension.</div><div><h3>Results</h3><div>A total of 908 patients were included (male-to-female ratio: 1.95), with a median follow-up of 4 years. The median age at surgery was 14 days. The surgical technique used was the Crafoord procedure in 35% of cases, extended Crafoord repair in 48%, and aortic arch plasty in 1","PeriodicalId":55472,"journal":{"name":"Archives of Cardiovascular Diseases","volume":"119 8","pages":"Pages S269-S270"},"PeriodicalIF":2.9,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148816013","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Prolonged hypoxemia before tetralogy of Fallot surgery: Harmful or harmless?","authors":"Nolwenn Tessier, Martin Bouaud, Estelle Ménard, Elina Deneele, Angelique Erraud, Eva Papin, Eva Mélenchon, Olivier Baron, Mohamedou Ly, Pierre Maminirina, Benjamin Lauzier, Manon Denis","doi":"10.1016/j.acvd.2026.06.066","DOIUrl":"10.1016/j.acvd.2026.06.066","url":null,"abstract":"<div><h3>Introduction</h3><div>Tetralogy of Fallot (TOF) is the most common cyanotic heart disease (CHD). The associated cardiac abnormalities disrupt oxygenation and depending on disease severity, infants with TOF syndrome are classified into two groups. Patients with normal oxygen saturation (SaO2<!--> <!-->><!--> <!-->94%) are referred to as non-cyanotic TOF, whereas those with reduced oxygen saturation (<<!--> <!-->90%) are classified as cyanotic TOF. Despite low oxygen saturation, these patients often present with a relatively good clinical condition; however, significant postoperative complications may still occur. The optimal timing of surgical repair therefore remains controversial, with conflicting evidence regarding early <em>versus</em> delayed intervention.</div><div>The present study aims to evaluate the impact of preoperative hypoxemia on perioperative parameters in patients undergoing TOF repair and to explore whether the duration of hypoxemia is associated with postoperative complications and recovery.</div></div><div>Tetralogy of Fallot (TOF) is the most common cyanotic heart disease (CHD). The associated cardiac abnormalities disrupt oxygenation and depending on disease severity, infants with TOF syndrome are classified into two groups. Patients with normal oxygen saturation (SaO2<!--> <!-->><!--> <!-->94%) are referred to as non-cyanotic TOF, whereas those with reduced oxygen saturation (<<!--> <!-->90%) are classified as cyanotic TOF. Despite low oxygen saturation, these patients often present with a relatively good clinical condition; however, significant postoperative complications may still occur. The optimal timing of surgical repair therefore remains controversial, with conflicting evidence regarding early <em>versus</em> delayed intervention.</div><div>The present study aims to evaluate the impact of preoperative hypoxemia on perioperative parameters in patients undergoing TOF repair and to explore whether the duration of hypoxemia is associated with postoperative complications and recovery.</div><div><h3>Methods</h3><div>This study was a single-center retrospective study conducted at Nantes University Hospital between January 2020 and December 2024 in children who had undergone corrective cardiac surgery for Tetralogy of Fallot. All presented data were extracted from the database of the pediatric intensive care and cardiac surgery unit.</div></div><div>This study was a single-center retrospective study conducted at Nantes University Hospital between January 2020 and December 2024 in children who had undergone corrective cardiac surgery for Tetralogy of Fallot. All presented data were extracted from the database of the pediatric intensive care and cardiac surgery unit.</div><div><h3>Results</h3><div>A total of 176 patients were included, 70 in the normoxemic group (Median [range] SaO2, 97.0 [90.0–100.0]%) and 106 in the hypoxemic group (76.0 [54.0–88.0]%; <em>P</em> <!--><<!--> <!-->0.001), with an equal p","PeriodicalId":55472,"journal":{"name":"Archives of Cardiovascular Diseases","volume":"119 8","pages":"Page S271"},"PeriodicalIF":2.9,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148816074","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Integrated echocardiographic right ventricular myocardial work and cardiopulmonary exercise testing in pediatric precapillary pulmonary arterial hypertension: A pilot study","authors":"Ramona Ghenghea, Aitor Guitarte, Paul Vignaud, Yves Dulac, Philippe Acar, Khaled Hadeed, Clement Karsenty","doi":"10.1016/j.acvd.2026.06.035","DOIUrl":"10.1016/j.acvd.2026.06.035","url":null,"abstract":"<div><h3>Introduction</h3><div>Right ventricular (RV) adaptation is the main determinant of functional limitation in precapillary pulmonary arterial hypertension (PCPH). Resting hemodynamics may remain preserved despite early impairment of RV mechanical and energetic performance. The relationship between RV myocardial work (MW) and cardiopulmonary exercise testing (CPET) parameters in pediatric PCPH is not well defined. The aim was to investigate the associations between RV MW indices and exercise ventilatory efficiency in children with PCPH.</div></div><div>Right ventricular (RV) adaptation is the main determinant of functional limitation in precapillary pulmonary arterial hypertension (PCPH). Resting hemodynamics may remain preserved despite early impairment of RV mechanical and energetic performance. The relationship between RV myocardial work (MW) and cardiopulmonary exercise testing (CPET) parameters in pediatric PCPH is not well defined. The aim was to investigate the associations between RV MW indices and exercise ventilatory efficiency in children with PCPH.</div><div><h3>Methods</h3><div>Nine pediatric patients with precapillary PCPH were prospectively evaluated with median age of 15.4 years (range: 7.7–17.9; IQR: 6.3) and median weight of 52.8<!--> <!-->kg (range: 21.0–74.6; IQR: 42.3). Etiology included idiopathic PCPH (<em>n</em> <!-->=<!--> <!-->6), BMPR2 mutation–associated PCPH (<em>n</em> <!-->=<!--> <!-->2), and GDF2 mutation–associated PCPH (<em>n</em> <!-->=<!--> <!-->1). Two patients had a Potts shunt. All patients underwent comprehensive 2D and 3D transthoracic echocardiography including speckle-tracking-derived RV strain and MW analysis, together with CPET.</div></div><div>Nine pediatric patients with precapillary PCPH were prospectively evaluated with median age of 15.4 years (range: 7.7–17.9; IQR: 6.3) and median weight of 52.8<!--> <!-->kg (range: 21.0–74.6; IQR: 42.3). Etiology included idiopathic PCPH (<em>n</em> <!-->=<!--> <!-->6), BMPR2 mutation–associated PCPH (<em>n</em> <!-->=<!--> <!-->2), and GDF2 mutation–associated PCPH (<em>n</em> <!-->=<!--> <!-->1). Two patients had a Potts shunt. All patients underwent comprehensive 2D and 3D transthoracic echocardiography including speckle-tracking-derived RV strain and MW analysis, together with CPET.</div><div><h3>Results</h3><div>Median RV ejection fraction was 51.1% (range: 38.7–59.4; IQR: 13.9), demonstrating heterogeneous systolic impairment despite preserved resting hemodynamics. Pulmonary artery systolic pressure was elevated at 66<!--> <!-->mmHg (range: 38–122; IQR: 59). Resting cardiac index was preserved in all but one patient (1.7<!--> <!-->L/min/m<sup>2</sup>). CPET revealed impaired ventilatory efficiency with VE/VCO<sub>2</sub> slope 42.9 (range: 38.5–49.8; IQR: 6.2) and reduced minimal PETCO<sub>2</sub> 23.3<!--> <!-->mmHg (range: 17.1–31.7; IQR: 4.1), with a downward PETCO<sub>2</sub> trajectory during exercise in all patients. Exercise capacity was moder","PeriodicalId":55472,"journal":{"name":"Archives of Cardiovascular Diseases","volume":"119 8","pages":"Page S255"},"PeriodicalIF":2.9,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148816078","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Neuropsychological and structural brain abnormalities in adults with d-transposition of the great arteries: The Boston Circulatory Arrest Study","authors":"Johanna Calderon, Ai Wern Chung, Rofeberg Valerie, Wypij David, Jane Newburger, Bellinger David, Gurvitz Michelle","doi":"10.1016/j.acvd.2026.06.008","DOIUrl":"10.1016/j.acvd.2026.06.008","url":null,"abstract":"<div><h3>Introduction</h3><div>Neurocognitive impairments are frequent in congenital heart disease. The Boston Circulatory Arrest Study (BCAS) is a randomized trial comparing the developmental and neurological outcomes of newborns born with d-transposition of the great arteries (d-TGA) who underwent the arterial switch operation (ASO) with predominantly either deep hypothermic circulatory arrest (DHCA) or continuous low-flow bypass (LFBP) as the method of vital organ support. We report the neuropsychological and structural brain MRI findings of this cohort at adulthood.</div></div><div>Neurocognitive impairments are frequent in congenital heart disease. The Boston Circulatory Arrest Study (BCAS) is a randomized trial comparing the developmental and neurological outcomes of newborns born with d-transposition of the great arteries (d-TGA) who underwent the arterial switch operation (ASO) with predominantly either deep hypothermic circulatory arrest (DHCA) or continuous low-flow bypass (LFBP) as the method of vital organ support. We report the neuropsychological and structural brain MRI findings of this cohort at adulthood.</div><div><h3>Methods</h3><div>Adults with d-TGA enrolled at birth in the BCAS study underwent a neuropsychological evaluation including IQ, executive function, social cognition, and memory functioning. A structural brain MRI was conducted on a Siemens Skyra 3<!--> <!-->T with a 64-channel head coil. Focal and multifocal brain abnormalities were recorded including brain mineralization/iron deposits and abnormal T2 hyperintensities (≥<!--> <!-->3<!--> <!-->mm) in subcortical grey, periventricular, and deep white matter. d-TGA outcomes were compared to population norms or to a control group.</div></div><div>Adults with d-TGA enrolled at birth in the BCAS study underwent a neuropsychological evaluation including IQ, executive function, social cognition, and memory functioning. A structural brain MRI was conducted on a Siemens Skyra 3<!--> <!-->T with a 64-channel head coil. Focal and multifocal brain abnormalities were recorded including brain mineralization/iron deposits and abnormal T2 hyperintensities (≥<!--> <!-->3<!--> <!-->mm) in subcortical grey, periventricular, and deep white matter. d-TGA outcomes were compared to population norms or to a control group.</div><div><h3>Results</h3><div>Eighty-seven adults (28.9<!--> <!-->±<!--> <!-->1.3 y, mean age<!--> <!-->±<!--> <!-->SD) participated. Their mean scores were not significantly lower compared to population norms except for working memory (9.2<!--> <!-->±<!--> <!-->2.6, <em>P</em> <!-->=<!--> <!-->0.01), visual social cognition (9.1<!--> <!-->±<!--> <!-->2.9, <em>P</em> <!-->=<!--> <!-->0.01), and reading comprehension (97.2<!--> <!-->±<!--> <!-->10.8, <em>P</em> <!-->=<!--> <!-->0.02). Adults who had been randomly assigned to predominant DHCA vs. LFBP during the neonatal ASO had lower scores on working memory (8.7<!--> <!-->±<!--> <!-->2.6 vs. 9.8<!--> <!-->±<!--> <!-->2.6, ","PeriodicalId":55472,"journal":{"name":"Archives of Cardiovascular Diseases","volume":"119 8","pages":"Pages S241-S242"},"PeriodicalIF":2.9,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148816086","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Pregnancy outcomes in women with grown-up congenital heart: A 10-year cohort study","authors":"Luca Campagni, Marie Christophe, Sylvie Schouvey, Anne Claire Casalta, Caroline Ovaert, Florence Bretelle, Julie Blanc","doi":"10.1016/j.acvd.2026.06.019","DOIUrl":"10.1016/j.acvd.2026.06.019","url":null,"abstract":"<div><h3>Introduction</h3><div>Cardiovascular diseases are the leading cause of maternal mortality within 42 days postpartum in France, representing a significant public health concern. Medical and surgical advancements now enable women with grown-up congenital heart (GUCH) to carry a pregnancy to term. However, recent data on maternal and neonatal morbidity and mortality in this population remain limited. This issue is of particular concern given that nearly two-thirds of cardiovascular-related maternal deaths are deemed preventable. This retrospective multicentric study aims to describe maternal and neonatal outcomes in pregnant women with GUCH.</div></div><div>Cardiovascular diseases are the leading cause of maternal mortality within 42 days postpartum in France, representing a significant public health concern. Medical and surgical advancements now enable women with grown-up congenital heart (GUCH) to carry a pregnancy to term. However, recent data on maternal and neonatal morbidity and mortality in this population remain limited. This issue is of particular concern given that nearly two-thirds of cardiovascular-related maternal deaths are deemed preventable. This retrospective multicentric study aims to describe maternal and neonatal outcomes in pregnant women with GUCH.</div><div><h3>Methods</h3><div>A multicenter, retrospective cohort study included women with GUCH who underwent at least one pregnancy between 2015 and 2024 was conducted in three maternity centers in Marseille, France.</div></div><div>A multicenter, retrospective cohort study included women with GUCH who underwent at least one pregnancy between 2015 and 2024 was conducted in three maternity centers in Marseille, France.</div><div><h3>Results</h3><div>Ninety pregnant women were included yielding data for 129 pregnancies. The most common GUCH were tetralogy of Fallot (18.6%), ventricular septal defect (17.8%), and aortic coarctation (10.9%). No maternal deaths were observed. Only 6 (4,7%) pregnancies were planned. Twenty-three patients (17.8%) experienced at least one cardiovascular event during the pregnancy or within the year following delivery, with heart failure (8.5%) and arrhythmias (8.5%) being the most frequent. Twenty-one (16.3%) cesarean delivery for cardiac indication were performed, including 12 (9.3%) elective procedures. Preterm delivery (<<!--> <!-->37 weeks of gestation) occurred in 23 pregnancies (17.8%), of which nine (7%) induced for maternal cardiac indication.</div></div><div>Ninety pregnant women were included yielding data for 129 pregnancies. The most common GUCH were tetralogy of Fallot (18.6%), ventricular septal defect (17.8%), and aortic coarctation (10.9%). No maternal deaths were observed. Only 6 (4,7%) pregnancies were planned. Twenty-three patients (17.8%) experienced at least one cardiovascular event during the pregnancy or within the year following delivery, with heart failure (8.5%) and arrhythmias (8.5%) being the most frequent. Twenty-o","PeriodicalId":55472,"journal":{"name":"Archives of Cardiovascular Diseases","volume":"119 8","pages":"Pages S247-S248"},"PeriodicalIF":2.9,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148816087","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}