Archives of Cardiovascular Diseases最新文献

{"title":"Mot de bienvenue","authors":"Le Comité d’organisation local","doi":"10.1016/j.acvd.2026.06.004","DOIUrl":"10.1016/j.acvd.2026.06.004","url":null,"abstract":"","PeriodicalId":55472,"journal":{"name":"Archives of Cardiovascular Diseases","volume":"119 8","pages":"Page S239"},"PeriodicalIF":2.9,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148815860","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":"State of the art-review and guidelines for management of Homozygous Familial Hypercholesterolemia in Children","authors":"Sophie Quennelle, Caroline Ovaert, Bertrand Cariou, Alain Carrié, Sybil Charrière, Mathilde Di Filippo, Jean Ferrières, Antonio Gallo, Julie Lemale, Philippe Moulin, Noel Peretti, Poullin Pascale, Jean Pierre Rabès, Samir Saheb, Tim Ulinski, Bruno Vergès, Yelnik Cécile, Sophie Beliard","doi":"10.1016/j.acvd.2026.06.037","DOIUrl":"10.1016/j.acvd.2026.06.037","url":null,"abstract":"<div><h3>Introduction</h3><div>Homozygous familial hypercholesterolemia is a rare genetic disorder characterized by extremely elevated low-density lipoprotein cholesterol from birth, leading to life-threatening atherosclerotic cardiovascular disease from childhood. Early recognition and appropriate management can significantly improve survival and long-term outcomes. Major progress has been achieved in recent years, including refined diagnostic criteria, systematic use of genetic testing, and the availability of novel lipid-lowering therapies such as PCSK9 inhibitors and ANGPTL3 inhibition, in addition to LDL apheresis. No updated guidelines for management during childhood is currently available.</div></div><div>Homozygous familial hypercholesterolemia is a rare genetic disorder characterized by extremely elevated low-density lipoprotein cholesterol from birth, leading to life-threatening atherosclerotic cardiovascular disease from childhood. Early recognition and appropriate management can significantly improve survival and long-term outcomes. Major progress has been achieved in recent years, including refined diagnostic criteria, systematic use of genetic testing, and the availability of novel lipid-lowering therapies such as PCSK9 inhibitors and ANGPTL3 inhibition, in addition to LDL apheresis. No updated guidelines for management during childhood is currently available.</div><div><h3>Methods</h3><div>These guidelines were developed by a French multidisciplinary panel of 20 experts in genetics, lipid disorders, pediatrics, and cardiology. Each section was prepared by a minimum of three experts. A comprehensive literature review was performed by each group and was supplemented by the experts’ own clinical expertise and experience.</div></div><div>These guidelines were developed by a French multidisciplinary panel of 20 experts in genetics, lipid disorders, pediatrics, and cardiology. Each section was prepared by a minimum of three experts. A comprehensive literature review was performed by each group and was supplemented by the experts’ own clinical expertise and experience.</div><div><h3>Results</h3><div>Early identification, prompt referral to expert multidisciplinary teams, early detection of cardiovascular disease and aggressive lipid-lowering strategies are essential to improve outcomes.</div></div><div>Early identification, prompt referral to expert multidisciplinary teams, early detection of cardiovascular disease and aggressive lipid-lowering strategies are essential to improve outcomes.</div><div><h3>Conclusion</h3><div>Our state-of-the-art review include guidelines, based on current evidence and clinical expertise provides a practical framework for pediatricians involved in the care of children with suspected or confirmed homozygous familial hypercholesterolemia.</div></div><div>Our state-of-the-art review include guidelines, based on current evidence and clinical expertise provides a practical framework for pediatricians involved in ","PeriodicalId":55472,"journal":{"name":"Archives of Cardiovascular Diseases","volume":"119 8","pages":"Page S256"},"PeriodicalIF":2.9,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148815866","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":"Long-term survival after late complete repair of tetralogy of Fallot in children from developing countries","authors":"Noor Gignoux, Marielle Gouton, Francine Leca","doi":"10.1016/j.acvd.2026.06.029","DOIUrl":"10.1016/j.acvd.2026.06.029","url":null,"abstract":"<div><h3>Introduction</h3><div>Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease. While outcomes after surgical repair are excellent in developed countries, access to early diagnosis and corrective surgery remains limited in many developing regions. Humanitarian programs such as Mécénat Chirurgie Cardiaque (MCC) aim to address these disparities by providing access to complete surgical repair for children from disadvantaged countries. Data on long-term outcomes in this specific population remain limited.</div></div><div>Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease. While outcomes after surgical repair are excellent in developed countries, access to early diagnosis and corrective surgery remains limited in many developing regions. Humanitarian programs such as Mécénat Chirurgie Cardiaque (MCC) aim to address these disparities by providing access to complete surgical repair for children from disadvantaged countries. Data on long-term outcomes in this specific population remain limited.</div><div><h3>Methods</h3><div>We conducted a retrospective cohort study including patients who underwent complete surgical repair of TOF through the MCC program in France between 1996 and 2010. All patients originated from countries without access to full corrective congenital cardiac surgery. The primary outcome was long-term overall survival after repair. Secondary outcomes included early postoperative mortality, major postoperative complications, and identification of predictive factors for mortality and complications. Survival was analyzed using the Kaplan–Meier method and Cox proportional hazards models. Logistic regression was used to assess predictors of major early postoperative complications.</div></div><div>We conducted a retrospective cohort study including patients who underwent complete surgical repair of TOF through the MCC program in France between 1996 and 2010. All patients originated from countries without access to full corrective congenital cardiac surgery. The primary outcome was long-term overall survival after repair. Secondary outcomes included early postoperative mortality, major postoperative complications, and identification of predictive factors for mortality and complications. Survival was analyzed using the Kaplan–Meier method and Cox proportional hazards models. Logistic regression was used to assess predictors of major early postoperative complications.</div><div><h3>Results</h3><div>A total of 314 patients from 37 countries were included. Median age at surgery was 4.48 years, 70.71% of patients had a weight below the 10th percentile and the median preoperative oxygen saturation was 78%. Overall mortality occurred in 12 patients (3.84%). The median follow-up duration was 6.45 years. Event-free survival was 95.8% at 10 years and 92.3% at 20 years. In univariable analysis, transannular incision and longer cardiopulmonary bypass duration were associated with mortality, but no ","PeriodicalId":55472,"journal":{"name":"Archives of Cardiovascular Diseases","volume":"119 8","pages":"Pages S252-S253"},"PeriodicalIF":2.9,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148815914","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":"Validation and diagnostic performance of point-of-care NT-proBNP testing using the LumiraDx® instrument in a paediatric population","authors":"Louise Canovi, Alexandre Delarue","doi":"10.1016/j.acvd.2026.06.038","DOIUrl":"10.1016/j.acvd.2026.06.038","url":null,"abstract":"<div><h3>Introduction</h3><div>NT-proBNP is a key biomarker for the diagnosis, monitoring, and prognostic assessment of heart failure. While point-of-care NT-proBNP analysers have been validated in adults, correlation data in paediatric populations are limited.</div></div><div>NT-proBNP is a key biomarker for the diagnosis, monitoring, and prognostic assessment of heart failure. While point-of-care NT-proBNP analysers have been validated in adults, correlation data in paediatric populations are limited.</div><div><h3>Objectives</h3><div>To assess the correlation, kinetics, diagnostic performance, and turnaround time of a point-of-care NT-proBNP analyser compared with reference laboratory measurements in paediatric patients.</div></div><div>To assess the correlation, kinetics, diagnostic performance, and turnaround time of a point-of-care NT-proBNP analyser compared with reference laboratory measurements in paediatric patients.</div><div><h3>Methods</h3><div>We conducted a single-centre prospective study including patients younger than 18 years hospitalised in a paediatric cardiology department who required NT-proBNP measurement for routine clinical management. NT-proBNP values obtained using a point-of-care analyser (LumiraDx®) were compared with reference laboratory measurements. Correlation was assessed using Spearman's coefficient. Diagnostic performance was evaluated using age-adjusted NT-proBNP reference values. Turnaround times and NT-proBNP kinetics were analysed in patients with repeated measurements.</div></div><div>We conducted a single-centre prospective study including patients younger than 18 years hospitalised in a paediatric cardiology department who required NT-proBNP measurement for routine clinical management. NT-proBNP values obtained using a point-of-care analyser (LumiraDx®) were compared with reference laboratory measurements. Correlation was assessed using Spearman's coefficient. Diagnostic performance was evaluated using age-adjusted NT-proBNP reference values. Turnaround times and NT-proBNP kinetics were analysed in patients with repeated measurements.</div><div><h3>Results</h3><div>A total of 79 NT-proBNP measurements were obtained from 59 patients. Correlation between two methods was excellent (Spearman's <em>r</em> <!-->=<!--> <!-->0.985, <em>P</em> <!--><<!--> <!-->0.0001). Diagnostic performance was high, with a sensitivity of 98%, specificity of 100%, positive predictive value of 100%, and negative predictive value of 96%. Agreement was high (<em>κ</em> <!-->=<!--> <!-->0.97). NT-proBNP kinetic trends were concordant within individual patients (<span><span>Fig. 1</span></span>). Median turnaround time was 126<!--> <!-->minutes for the reference laboratory method compared with 12<!--> <!-->minutes for the point-of-care analyser.</div></div><div>A total of 79 NT-proBNP measurements were obtained from 59 patients. Correlation between two methods was excellent (Spearman's <em>r</em> <!-->=<!--> <!-->0.985, <em>P</em>","PeriodicalId":55472,"journal":{"name":"Archives of Cardiovascular Diseases","volume":"119 8","pages":"Pages S256-S257"},"PeriodicalIF":2.9,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148816011","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":"Feasibility and tolerance of systemic sirolimus therapy in the treatment of pulmonary vein stenosis","authors":"Paolo Hollis, Katia Librandi, Samuel Parsons, Andreas Hoschtitzky, Carles Bautista-Rodriguez, Alain Fraisse","doi":"10.1016/j.acvd.2026.06.056","DOIUrl":"10.1016/j.acvd.2026.06.056","url":null,"abstract":"<div><h3>Introduction</h3><div>Pulmonary vein stenosis is a debilitating condition that carries a high mortality despite aggressive surgical and transcatheter intervention strategies. Systemic sirolimus therapy (SST) given alongside conventional interventional management of pulmonary vein stenosis has shown improvement in outcomes and a slower progression of the disease. No data is currently available of the tolerance and feasibility of systemic sirolimus therapy in this fragile patient population.</div></div><div>Pulmonary vein stenosis is a debilitating condition that carries a high mortality despite aggressive surgical and transcatheter intervention strategies. Systemic sirolimus therapy (SST) given alongside conventional interventional management of pulmonary vein stenosis has shown improvement in outcomes and a slower progression of the disease. No data is currently available of the tolerance and feasibility of systemic sirolimus therapy in this fragile patient population.</div><div><h3>Methods</h3><div>In this single-centre study we report our experience from December 2022 to December 2025 looking at primary outcomes of survival, duration of sirolimus therapy, and frequency and duration of sirolimus therapy pauses. Secondary outcome measures included the number of surgical and interventional procedures undertaken, any adverse events related to systemic sirolimus therapy, the most recently available pulmonary artery pressure measurements, and the latest clinical status of the patients.</div></div><div>In this single-centre study we report our experience from December 2022 to December 2025 looking at primary outcomes of survival, duration of sirolimus therapy, and frequency and duration of sirolimus therapy pauses. Secondary outcome measures included the number of surgical and interventional procedures undertaken, any adverse events related to systemic sirolimus therapy, the most recently available pulmonary artery pressure measurements, and the latest clinical status of the patients.</div><div><h3>Results</h3><div>Twelve patients received systemic sirolimus therapy, with one patient undergoing two separate episodes of treatment. All patients were alive at the time of follow-up, and 11 out of 12 patients had no cardiac symptoms at their latest clinical review. 1 patient was removed from analysis as their SST is managed by their local hospital. The total number of days of treatment was 4851 days with a median for each episode of therapy of 373 days (range 74–1102). There were 43 pauses in therapy, with a median number of 3 pauses per episode of therapy (range 0–11). The total number of days paused was 401 (8.3%), and the median number of days paused per episode was 20 days (range 0–158).</div></div><div>Twelve patients received systemic sirolimus therapy, with one patient undergoing two separate episodes of treatment. All patients were alive at the time of follow-up, and 11 out of 12 patients had no cardiac symptoms at their latest clinical re","PeriodicalId":55472,"journal":{"name":"Archives of Cardiovascular Diseases","volume":"119 8","pages":"Page S265"},"PeriodicalIF":2.9,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148816014","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":"Mavacamten therapy in pediatric obstructive hypertrophic cardiomyopathy: Real-world safety and efficacy","authors":"Ramona Ghenghea, Alexandre Delarue, Claire Lucas, Gilbert Habib, Olivia Domanski, Clement Karsenty","doi":"10.1016/j.acvd.2026.06.040","DOIUrl":"10.1016/j.acvd.2026.06.040","url":null,"abstract":"<div><h3>Introduction</h3><div>Mavacamten is the first approved cardiac myosin inhibitor for symptomatic obstructive hypertrophic cardiomyopathy (HOCM), targeting myocardial hypercontractility and reducing left ventricular outflow tract (LVOT) obstruction. While randomized trials have demonstrated its efficacy and safety in adults, data in pediatric patients remain scarce. The ongoing SCOUT-HCM trial is the first phase 3 randomized study evaluating mavacamten in symptomatic adolescents with HOCM. Real-world pediatric experience is currently limited.</div></div><div>Mavacamten is the first approved cardiac myosin inhibitor for symptomatic obstructive hypertrophic cardiomyopathy (HOCM), targeting myocardial hypercontractility and reducing left ventricular outflow tract (LVOT) obstruction. While randomized trials have demonstrated its efficacy and safety in adults, data in pediatric patients remain scarce. The ongoing SCOUT-HCM trial is the first phase 3 randomized study evaluating mavacamten in symptomatic adolescents with HOCM. Real-world pediatric experience is currently limited.</div><div><h3>Methods</h3><div>We report an initial real-world experience with mavacamten in pediatric patients with HOCM in France. Patients had persistent LVOT obstruction despite maximally tolerated beta-blocker therapy. Clinical and echocardiographic parameters were assessed at baseline and after 3 months of follow-up.</div></div><div>We report an initial real-world experience with mavacamten in pediatric patients with HOCM in France. Patients had persistent LVOT obstruction despite maximally tolerated beta-blocker therapy. Clinical and echocardiographic parameters were assessed at baseline and after 3 months of follow-up.</div><div><h3>Results</h3><div>Four pediatric patients aged 15–17 years, all male, with a median weight of 103.4<!--> <!-->kg (range: 45–135) were treated. The median duration of prior beta-blocker therapy was 3.5 months. Genetic analysis showed that two patients carried pathogenic variants (MYH7 and MYBPC3), while two patients were genotype-negative. Mavacamten was initiated at 2.5<!--> <!-->mg in two patients and 5<!--> <!-->mg in two patients, with repeated echocardiographic monitoring. At baseline, the median left ventricular ejection fraction (LVEF) was 65% (range: 64–65). The median LVOT gradient at rest was 23<!--> <!-->mmHg (range: 15–100) and with exercise was 79<!--> <!-->mmHg (range: 55–110) under maximal beta-blocker therapy.</div><div>After 3 months, the median LVOT gradient decreased to 17<!--> <!-->mmHg (range: 14–20) at rest and 48.5<!--> <!-->mmHg (range: 37–60) with exercise. LVEF remained preserved, with a median of 62% (range: 60–65). Treatment was well tolerated, with no reported adverse events.</div></div><div>Four pediatric patients aged 15–17 years, all male, with a median weight of 103.4<!--> <!-->kg (range: 45–135) were treated. The median duration of prior beta-blocker therapy was 3.5 months. Genetic analysis showed that ","PeriodicalId":55472,"journal":{"name":"Archives of Cardiovascular Diseases","volume":"119 8","pages":"Pages S257-S258"},"PeriodicalIF":2.9,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148816048","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":"Thrombophilia testing in venous thromboembolism: When, who and why it matters. A practical review","authors":"Elena-Mihaela Cordeanu MCU-PH,&nbsp;Corina Mirea,&nbsp;Camille Zamperini,&nbsp;Solène Brouder,&nbsp;Dominique Stephan","doi":"10.1016/j.acvd.2026.04.004","DOIUrl":"10.1016/j.acvd.2026.04.004","url":null,"abstract":"<div><div>Thrombophilia encompasses inherited and acquired biological abnormalities that predispose to venous thromboembolism (VTE). Although thrombophilia testing has been available for decades, routine screening of all patients presenting with deep vein thrombosis or pulmonary embolism is neither recommended nor cost-effective. Current international guidelines support testing for clinical scenarios where results may meaningfully inform recurrence risk and thereby influence therapeutic decisions regarding anticoagulation duration, anticoagulant choice, contraceptive counselling or pregnancy management. This narrative review provides a practical, evidence-based framework for thrombophilia investigation. We address testing indications stratified by clinical context, including unprovoked VTE in young patients, hormone-associated thrombosis and unusual-site thrombosis. Practical considerations for testing timing and anticoagulant interference are detailed, including recent expert guidance on charcoal-based direct oral anticoagulant neutralization strategies. Therapeutic implications are examined across specific populations: women requiring hormonal contraception or menopause hormone therapy, cancer-associated thrombosis and chronic thromboembolic pulmonary hypertension. Thrombophilia results should be interpreted within the broader clinical context. A clinical decision algorithm synthesizing current evidence and recommendations is proposed to guide selective testing.</div></div>","PeriodicalId":55472,"journal":{"name":"Archives of Cardiovascular Diseases","volume":"119 8","pages":"Pages 604-614"},"PeriodicalIF":2.9,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148058219","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"When angiography starts speaking the language of the myocardium","authors":"Ikram El Marzouki,&nbsp;Gilles Barone-Rochette","doi":"10.1016/j.acvd.2026.05.002","DOIUrl":"10.1016/j.acvd.2026.05.002","url":null,"abstract":"","PeriodicalId":55472,"journal":{"name":"Archives of Cardiovascular Diseases","volume":"119 8","pages":"Pages 533-535"},"PeriodicalIF":2.9,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148195155","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Perioperative assessment of myocardial work in children undergoing ventricular septal defects closure","authors":"Ramona Ghenghea,&nbsp;Comme Schmitt,&nbsp;Paul Vignaud,&nbsp;Aitor Guitarte,&nbsp;Yves Dulac,&nbsp;Philippe Acar,&nbsp;Khaled Hadeed,&nbsp;Montserrat Sierra-Colomina,&nbsp;Clement Karsenty","doi":"10.1016/j.acvd.2026.06.041","DOIUrl":"10.1016/j.acvd.2026.06.041","url":null,"abstract":"&lt;div&gt;&lt;h3&gt;Introduction&lt;/h3&gt;&lt;div&gt;Myocardial work (MW) is an emerging echocardiographic parameter that integrates myocardial deformation with afterload, providing a more comprehensive assessment of ventricular performance than traditional load-dependent parameters. While its clinical value has been increasingly investigated in adults, data in the pediatric population remain limited. We aimed to evaluate perioperative changes in left and right ventricular MW and to explore their association with perioperative status.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;Myocardial work (MW) is an emerging echocardiographic parameter that integrates myocardial deformation with afterload, providing a more comprehensive assessment of ventricular performance than traditional load-dependent parameters. While its clinical value has been increasingly investigated in adults, data in the pediatric population remain limited. We aimed to evaluate perioperative changes in left and right ventricular MW and to explore their association with perioperative status.&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Methods&lt;/h3&gt;&lt;div&gt;Eighteen consecutive children undergoing surgical ventricular septal defect (VSD) closure at a single center between January and April 2025 were prospectively evaluated. Echocardiographic assessment was performed preoperatively, within 24&lt;!--&gt; &lt;!--&gt;hours postoperatively, and at hospital discharge.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;Eighteen consecutive children undergoing surgical ventricular septal defect (VSD) closure at a single center between January and April 2025 were prospectively evaluated. Echocardiographic assessment was performed preoperatively, within 24&lt;!--&gt; &lt;!--&gt;hours postoperatively, and at hospital discharge.&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Results&lt;/h3&gt;&lt;div&gt;Median age was 29.9 months (range: 3.3–135.4; IQR: 43.9), and median weight was 8.4&lt;!--&gt; &lt;!--&gt;kg (range: 5.1–26.0; IQR: 4.9).&lt;/div&gt;&lt;div&gt;Left ventricular (LV) global work index (GWI) decreased from 1390.0&lt;!--&gt; &lt;!--&gt;mmHg% (range: 899.0–1837.0; IQR: 479.0) preoperatively to 780.5&lt;!--&gt; &lt;!--&gt;mmHg% (range: 210.0–1983.0; IQR: 297.5) postoperatively and 849.0&lt;!--&gt; &lt;!--&gt;mmHg% (range: 204.0–1423.0; IQR: 250.5) at discharge. Right ventricular (RV) GWI decreased from 531.0&lt;!--&gt; &lt;!--&gt;mmHg% (range: 217.0–1032.0; IQR: 427.5) preoperatively to 216.0&lt;!--&gt; &lt;!--&gt;mmHg% (range: 105.0–555.0; IQR: 103.5) postoperatively and 220.0&lt;!--&gt; &lt;!--&gt;mmHg% (range: 121.0–404.0; IQR: 88.5) at discharge. The decrease was significant for both ventricles compared with baseline (&lt;em&gt;P&lt;/em&gt; &lt;!--&gt;&lt;&lt;!--&gt; &lt;!--&gt;0.0001), while the difference between post-op and discharge was not statistically significant. Early postoperative RV GWI correlated with cardiopulmonary bypass (CPB) duration (&lt;em&gt;r&lt;/em&gt; &lt;!--&gt;=&lt;!--&gt; &lt;!--&gt;0.53, &lt;em&gt;P&lt;/em&gt; &lt;!--&gt;=&lt;!--&gt; &lt;!--&gt;0.03) and pediatric index of mortality 3 (PIM 3) score (&lt;em&gt;r&lt;/em&gt; &lt;!--&gt;=&lt;!--&gt; &lt;!--&gt;0.63, &lt;em&gt;P&lt;/em&gt; &lt;!--&gt;=&lt;!--&gt; &lt;!--&gt;0.007), whereas LV MW showed no significant associations (&lt;span&gt;&lt;span&gt;Fig. 1&lt;/span&gt;&lt;/span&gt;).&lt;/div&gt;&lt;/div&gt;&lt;div&gt;Median age was 29.9 months (range: 3.3–135.4; IQR: 43.","PeriodicalId":55472,"journal":{"name":"Archives of Cardiovascular Diseases","volume":"119 8","pages":"Page S258"},"PeriodicalIF":2.9,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148816049","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":"PERI-CLOSE: Design and validation of an innovative device for the percutaneous closure of perimembranous ventricular septal defects","authors":"Raymond Haddad,&nbsp;Sebastien Hascoet,&nbsp;Alain Fraisse","doi":"10.1016/j.acvd.2026.06.049","DOIUrl":"10.1016/j.acvd.2026.06.049","url":null,"abstract":"&lt;div&gt;&lt;h3&gt;Introduction&lt;/h3&gt;&lt;div&gt;The PERICLOSE project addresses an unmet medical need: the closure of perimembranous ventricular septal defects (pmVSDs), the most common congenital heart defect in both children and young adults. Currently available treatments, whether surgical (open-heart procedures with associated morbidity) or percutaneous (using devices outside their official regulatory indication), have significant limitations, particularly in terms of postoperative complications.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;The PERICLOSE project addresses an unmet medical need: the closure of perimembranous ventricular septal defects (pmVSDs), the most common congenital heart defect in both children and young adults. Currently available treatments, whether surgical (open-heart procedures with associated morbidity) or percutaneous (using devices outside their official regulatory indication), have significant limitations, particularly in terms of postoperative complications.&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Objective&lt;/h3&gt;&lt;div&gt;This project aims to validate a novel device specifically designed to match the anatomical characteristics of pmVSDs, with a focus on safety, efficacy, and clinical feasibility, while reducing the risks associated with current approaches.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;This project aims to validate a novel device specifically designed to match the anatomical characteristics of pmVSDs, with a focus on safety, efficacy, and clinical feasibility, while reducing the risks associated with current approaches.&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Methods&lt;/h3&gt;&lt;div&gt;The project is based on a collaboration between INSERM UMR_1358, Hôpital Marie Lannelongue, the MALIC, and its strategic academic partners, 3DHeartModeling, and INRIA. The main steps include: (1) Design of a detailed digital model using computer-aided design software; (2) Preparation of documentation for patent filing; (3) Numerical simulations to assess device adaptability; (4) Prototype manufacturing using Nitinol microbraiding; (5) Standardized mechanical testing; (6) Preclinical evaluation using 3D heart models and simulators to assess feasibility and efficacy under controlled conditions.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;The project is based on a collaboration between INSERM UMR_1358, Hôpital Marie Lannelongue, the MALIC, and its strategic academic partners, 3DHeartModeling, and INRIA. The main steps include: (1) Design of a detailed digital model using computer-aided design software; (2) Preparation of documentation for patent filing; (3) Numerical simulations to assess device adaptability; (4) Prototype manufacturing using Nitinol microbraiding; (5) Standardized mechanical testing; (6) Preclinical evaluation using 3D heart models and simulators to assess feasibility and efficacy under controlled conditions.&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Expected results&lt;/h3&gt;&lt;div&gt;PERICLOSE offers a minimally invasive solution for the closure of pmVSDs, thereby reducing procedural complications and improving patients’ quality of life. It involves the development of a patentable device incorporating a","PeriodicalId":55472,"journal":{"name":"Archives of Cardiovascular Diseases","volume":"119 8","pages":"Page S262"},"PeriodicalIF":2.9,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148816080","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}