Archives of Cardiovascular Diseases最新文献

{"title":"Innovative approaches to organ preservation in heart transplantation: A comprehensive review by the French Society of Thoracic and Cardiovascular Surgery","authors":"Julien Guihaire ,&nbsp;Guillaume Guimbretière ,&nbsp;Guillaume Lebreton ,&nbsp;Géraldine Allain ,&nbsp;Charles-Henri David ,&nbsp;Matteo Pozzi ,&nbsp;Marylou Para ,&nbsp;Erwan Flecher ,&nbsp;Olivier Bouchot ,&nbsp;Pascal Leprince ,&nbsp;André Vincentelli ,&nbsp;for the French Society for Thoracic, Cardiovascular Surgery Heart Transplantation Work Force","doi":"10.1016/j.acvd.2025.03.119","DOIUrl":"10.1016/j.acvd.2025.03.119","url":null,"abstract":"<div><div>Improved approaches for organ preservation have been recently applied in heart transplantation to prevent the risk of primary graft dysfunction. To review heart-graft preservation systems and to identify criteria for using innovative devices in each specific situation. A working group of the French Society of Thoracic and Cardiovascular Surgery performed a literature review focusing on organ preservation and post-transplant outcomes. Static cold storage is the most widely used method but involves cold ischaemia and is therefore limited for prolonged preservation. Optimizing this method by ensuring uniform and stable cooling (SherpaPak™) seems to be associated with favourable results, even with expanded-criteria grafts. Continuous normothermic organ perfusion (Organ Care System) shortens the cold ischaemia time, thus maintaining heart-graft viability despite long transportation times or long waits to achieve complex recipient-heart explantation. Moreover, this method can rehabilitate Maastricht III heart grafts. Continuous hypothermic oxygenated perfusion (XVivo™, not yet approved by regulatory authorities) has recently been associated with favourable outcomes, even in case of extended out-of-body preservation<!--> <!-->&gt;<!--> <!-->8<!--> <!-->hours. The new devices for heart preservation can be expected to allow successful transplantation despite long transport times, lengthy explantation procedures and the use of grafts from expanded-criteria donors, including donors after controlled circulatory arrest. Further studies are needed to assess patient and graft outcomes, determine the optimal device for each situation and evaluate the cost-benefit ratio.</div></div>","PeriodicalId":55472,"journal":{"name":"Archives of Cardiovascular Diseases","volume":"118 8","pages":"Pages 489-496"},"PeriodicalIF":2.2,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144029687","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":"Inferior vena cava agenesis revealed by neonatal hypertension","authors":"Lahlou Haddad,&nbsp;Ourida Gacem","doi":"10.1016/j.acvd.2025.06.037","DOIUrl":"10.1016/j.acvd.2025.06.037","url":null,"abstract":"<div><h3>Introduction</h3><div>Most often, interruption of the inferior vena cava results from insufficient development of the anastomosis between the subcardinal vein and the vitelline vein.</div></div><div><h3>Method</h3><div>Clinical case: A young male infant, aged 49 days, with a gestational age of 35 weeks and 4 days. Birth weight: 3<!--> <!-->kg. The infant presents with moderate respiratory distress due to a well-evolved early neonatal infection, under antibiotic treatment. On day 13 of life (corrected age: 37 weeks), the infant develops edema of the lower limbs and hypertension (120/50<!--> <!-->mmHg). Clinical examination: Respiratory rate: 40 cycles/min, SaO2: 94%, heart rate: 120 bpm, regular rhythm, heart sounds well audible, and all pulses palpable. No lumbar tenderness or abdominal mass. Neurological examination is normal.</div></div><div><h3>Results</h3><div>Abdominal echo-Doppler showed a thrombus measuring 22<!--> <!-->×<!--> <!-->6<!--> <!-->mm in the IVC, which was confirmed by an angioscanner revealing a thrombus in the IVC and the right renal vein, along with dilation of the compensatory azygos vein and the presence of a collateral network, without impact on the right kidney. Cardiac echodoppler: normal. Echodoppler of both lower limbs: absence of signs of deep vein thrombosis.</div><div>Blood tests: Renal function normal (no proteinuria, no hematuria, urea: 0.05<!--> <!-->g/l and creatinine: 3<!--> <!-->mg/l). Hemostasis assessment: normal, Antithrombin III: 51% (N: 41 to 93%), Protein C: 24% (N: 20 to 64%) and Protein S: 76% (N: 22 to 78%).</div><div>Mangement: anticoagulant treatment (Lovenox) 150 UI/kg/12<!--> <!-->h, extended for 6 months (anti-Xa activity: 0.54 UI/l)<!--> <!-->+<!--> <!-->antihypertensive medication with an ACE inhibitor (captopril) 0.5<!--> <!-->mg/kg/day in 2 doses (the blood pressure normalized within a few days).</div><div>Six months later: Abdominal echodoppler shows the inferior vena cava below the liver at the limit of visibility, non-vascularized in Doppler mode. Thoraco-abdominal angioscanner reveals the absence of opacification of the inferior vena cava. Renal and sub-renal venous drainage occurs at the level of the right hemi-azygos vein, which drains into the superior vena cava and then into the right atrium (RA). The hepatic veins drain directly into the RA.</div></div><div><h3>Conclusion</h3><div>Inferior vena cava agenesis is an extremely rare pathological entity. This case highlights the importance of considering this malformation in a newborn presenting with lower limb edema and hypertension due to thrombosis of the inferior vena cava and the right renal vein. Consequently, further radiological investigations should be conducted to detect it.</div></div>","PeriodicalId":55472,"journal":{"name":"Archives of Cardiovascular Diseases","volume":"118 8","pages":"Page S267"},"PeriodicalIF":2.2,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144934297","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":"Genetic and Clinical Diversity in Generalized Arterial Calcification of Infancy: Insights from Two Families","authors":"Fabien Labombarda ,&nbsp;Isabelle Durand ,&nbsp;Mireille Castanet ,&nbsp;Alexandra Desdoit ,&nbsp;David Brossier ,&nbsp;Pascale Maragnes ,&nbsp;Cynthia Cousergue ,&nbsp;Simon Anquetil ,&nbsp;Caroline Parrod ,&nbsp;Elise Barre","doi":"10.1016/j.acvd.2025.06.032","DOIUrl":"10.1016/j.acvd.2025.06.032","url":null,"abstract":"<div><h3>Introduction</h3><div>Generalized arterial calcification of infancy (GACI) is a rare genetic disorder characterized by arterial calcifications, stenoses, and hypertension, often leading to intrauterine or early infancy death.</div></div><div><h3>Method</h3><div>We report two recently diagnosed families in Normandy, each presenting with distinct clinical features linked to different genetic mutations.</div></div><div><h3>Results</h3><div>Family 1: The index patient presented at 10 days of life with heart failure, severe hypertension, myocardial infarction, and vascular calcifications, leading to a diagnosis of GACI type 1 (ENPP1, two pathogenic mutations). Treatment with bisphosphonate infusions over 16 months, combined with antihypertensive therapy, resulted in clinical improvement and complete regression of tissue calcifications (<span><span>Figure 1</span></span>). This diagnosis also led to the identification of an older sibling with a milder phenotype, characterized by periarticular and aortic valve calcifications. Family 2: The index patient was diagnosed at 2 months of age, before the onset of clinical manifestations, during a routine echocardiographic evaluation for the follow-up of in utero left ventricular hypertrophy (LVH) in the context of maternal diabetes. Echocardiography revealed LV dysfunction, LV dilation and hypertrophy, a mildly reduced ejection fraction (50%), and repolarization abnormalities on ECG. The patient later developed vascular calcifications, leading to a diagnosis of GACI type 2 (ABCC6, pathogenic mutation). His condition improved with cardiovascular treatment, and compassionate use of specific enzyme replacement therapy was considered. These cases illustrate the phenotypic variability of GACI and highlight key considerations: 1- the critical importance of early neonatal recognition for timely diagnosis; 2- Contrasting clinical presentations of ENPP1- and ABCC6-related GACI, with ENPP1 mutations generally associated with more severe disease; 3- Current therapeutic strategies, including non-specific cardiac treatments, bisphosphonate infusion, and emerging enzyme replacement therapy (ERT), which remains under evaluation.</div></div><div><h3>Conclusion</h3><div>These cases emphasize the importance of early diagnosis, genetic characterization, and evolving treatment strategies in GACI. Further research is needed to refine genotype-phenotype correlations and assess the long-term benefits of ERT.</div></div>","PeriodicalId":55472,"journal":{"name":"Archives of Cardiovascular Diseases","volume":"118 8","pages":"Page S264"},"PeriodicalIF":2.2,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144934301","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":"Exercise stress echocardiography in repaired coarctation of the aorta.","authors":"Reaksmei Ly, Sébastien Hascoet, Nicolas Combes, Paola Di Marco, Clément Karsenty, Miarisoa Ratsimandresy, Jelena Radojevic-Liegeois","doi":"10.1016/j.acvd.2025.06.076","DOIUrl":"https://doi.org/10.1016/j.acvd.2025.06.076","url":null,"abstract":"<p><strong>Background: </strong>Exercise stress echocardiography is a helpful tool for haemodynamic evaluation and follow-up of patients with repaired coarctation of the aorta.</p><p><strong>Aim: </strong>To determine if exercise stress echocardiography variables are predictive of reintervention (angioplasty or surgery).</p><p><strong>Methods: </strong>We retrospectively reviewed 81 exercise stress echocardiograms performed in children (n=9) and adults with repaired coarctation of the aorta in three centres in France.</p><p><strong>Results: </strong>The median age was 28.2 years (range 12-72 years). Twelve patients had a reintervention (14.8%). Forty-five patients (55.6%) had simple coarctation of the aorta, and 36 (44.4%) had complex anatomy. More than one third of patients had hypertension. Poor left ventricular adaptation to effort was present in 13 patients (16.1%). The mean peak isthmus systolic gradient was 48.4±19.7mmHg (range 15-124mmHg). Poor left ventricular adaptation was associated with more interventions (log-rank P=1×10^-5). On univariate analysis, the presence of antihypertensive drugs (hazard ratio 3.98, 95% confidence interval 1.15-13.82; P=0.030), previously stented coarctation of the aorta (hazard ratio 7.87, 95% confidence interval 2.19-28.31; P=0.002), lower exercise power (in Watts) (hazard ratio 0.98, 95% confidence interval 0.97-0.99; P=0.016) and peak isthmus systolic gradient at rest and on effort (hazard ratio 1.05, 95% confidence interval 1.01-1.09 [P=0.016] and hazard ratio 1.04, 95% confidence interval 1.01-1.06 [P=0.004], respectively) were significantly predictive of intervention. On multivariable analysis, lower left ventricular adaptation and peak isthmus systolic gradient at effort were associated with outcome (hazard ratio 10.73, 95% confidence interval 2.83-40.70 [P=0.000488] and hazard ratio 1.04, 95% confidence interval 1.01-1.07 [P=0.00835], respectively).</p><p><strong>Conclusions: </strong>Exercise stress echocardiography can be useful for haemodynamic evaluation of patients with repaired coarctation of the aorta. Patients with less adaptation of the left ventricle to exercise and higher peak systolic gradient at isthmus may be candidates for reintervention.</p>","PeriodicalId":55472,"journal":{"name":"Archives of Cardiovascular Diseases","volume":" ","pages":""},"PeriodicalIF":2.2,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145088194","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":"Unusual Genetic Variant in Familial Dilated Cardiomyopathy Identified Through Pediatric Case Investigation","authors":"Balsam Mlik ,&nbsp;Rania Gargouri","doi":"10.1016/j.acvd.2025.06.035","DOIUrl":"10.1016/j.acvd.2025.06.035","url":null,"abstract":"<div><h3>Introduction</h3><div>Dilated cardiomyopathy (DCM) is a rare but serious cause of heart failure in children. In idiopathic cases, especially when familial, a genetic etiology is often implicated.</div></div><div><h3>Method</h3><div>We report the case of a 2-year-old boy admitted for generalized edema and signs of heart failure. Echocardiography revealed DCM with markedly reduced left ventricular ejection fraction. His paternal aunt had been previously diagnosed with DCM in adulthood. A full etiological assessment, including whole exome sequencing (WES), was performed.</div></div><div><h3>Results</h3><div>WES identified a heterozygous pathogenic variant in the TTN gene (OMIM 188840), associated with autosomal dominant isolated familial DCM (OMIM 604145) and familial hypertrophic cardiomyopathy 9 (OMIM 613765). Additionally, a heterozygous likely pathogenic variant was found in the CTNNA3 gene, associated with autosomal dominant arrhythmogenic right ventricular dysplasia type 13 (OMIM 615616). Both variants are rare and scarcely reported in the literature. These findings supported a genetic diagnosis and led to extended family screening and early clinical monitoring.</div></div><div><h3>Conclusion</h3><div>This case underscores the value of whole exome sequencing in pediatric DCM, enabling the identification of rare genetic variants with major implications for diagnosis, prognosis, and family management.</div></div>","PeriodicalId":55472,"journal":{"name":"Archives of Cardiovascular Diseases","volume":"118 8","pages":"Page S266"},"PeriodicalIF":2.2,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144934168","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":"Welcome message","authors":"","doi":"10.1016/j.acvd.2025.06.005","DOIUrl":"10.1016/j.acvd.2025.06.005","url":null,"abstract":"","PeriodicalId":55472,"journal":{"name":"Archives of Cardiovascular Diseases","volume":"118 8","pages":"Page S249"},"PeriodicalIF":2.2,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144934276","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":"Health-related quality of life in pediatric inherited cardiac disease: The QUALIMYORYTHM multicenter cross-sectional controlled study","authors":"Oscar Werner ,&nbsp;Luc Souilla ,&nbsp;Marie Vincenti ,&nbsp;Jean-Benoît Thambo ,&nbsp;Sophie Guillaumont ,&nbsp;Helena Huguet ,&nbsp;Jean-Luc Pasquié ,&nbsp;Philippe Chevalier ,&nbsp;Constance Beyler ,&nbsp;Grégoire Delavilleon ,&nbsp;Marie-Christine Picot ,&nbsp;Alban-Elouen Baruteau ,&nbsp;Isabelle Denjoy ,&nbsp;Antoine Delinière ,&nbsp;Lauriane Cornuault ,&nbsp;Pascal Amédro","doi":"10.1016/j.acvd.2025.06.020","DOIUrl":"10.1016/j.acvd.2025.06.020","url":null,"abstract":"<div><h3>Introduction</h3><div>Medical advances in the management of children with inherited cardiac disease have shifted the focus from mortality to life-long morbidity, with a stronger attention on patient-reported outcomes. Health-related quality of life (HRQoL) has been increasingly used as a primary outcome in cross-sectional or randomized controlled trials in CHD, however no consistent controlled data is available in children with inherited cardiac arrhythmia or cardiomyopathy. The QUALIMYORYTHM trial aimed to evaluate HRQoL in cohort of children with inherited cardiac disease, in comparison with healthy controls, and to determine their predictors of HRQoL.</div></div><div><h3>Method</h3><div>Children aged–6-17 years, diagnosed with inherited cardiac arrhythmia (long QT syndrome, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia) or inherited cardiomyopathy (hypertrophic, dilated, restrictive, or arrhythmogenic right ventricular cardiomyopathy), and healthy age- and gender-matched controls were prospectively recruited in 7 pediatric tertiary care centers in France. HRQoL was assessed using the self and proxy PedsQL questionnaires. Clinical data, physical activity (accelerometer) and cardiorespiratory fitness (cardiopulmonary exercise testing) were assessed (NCT04712136).</div></div><div><h3>Results</h3><div>A total of 210 children were included (103 patients, mean age 12.7<!--> <!-->±<!--> <!-->3.13 years and 107 controls). HRQoL in children with inherited cardiac disease was good, overall, but remained significantly lower than healthy controls in self-reported (78.8<!--> <!-->±<!--> <!-->13.3 vs. 82.1<!--> <!-->±<!--> <!-->9.6, <em>P</em> <!-->=<!--> <!-->0.04) and proxy-reported (75.1<!--> <!-->±<!--> <!-->15.7 vs. 78.9<!--> <!-->±<!--> <!-->15.2, <em>P</em> <!-->=<!--> <!-->0.05) total scores. HRQoL was not significantly different in disease subgroups. In multivariate analysis, predictors of better HRQoL included higher resting heart rate, postnatal diagnosis, better cardiopulmonary fitness, lower controlled motivation score, and normal school status.</div></div><div><h3>Conclusion</h3><div>Currently, HRQoL in children with inherited cardiac disease is good, but significantly lower than healthy peers, and influenced by clinical, functional, and sociodemographic factors. These findings highlight the need for multidisciplinary interventions focusing on physical fitness, psychosocial support, and educational stability to improve their long-term patient-reported outcomes.</div></div>","PeriodicalId":55472,"journal":{"name":"Archives of Cardiovascular Diseases","volume":"118 8","pages":"Page S258"},"PeriodicalIF":2.2,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144934235","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":"Association between pediatric feeding disorders (PFD) and suboptimal neurodevelopment (SND) in children with congenital heart disease requiring bypass surgery in the first year of life: Results from a single-center prospective observational study","authors":"Julia Chambon ,&nbsp;Olivia Domanski ,&nbsp;Sylvie Nguyen The Tich ,&nbsp;Sylvie Joriot ,&nbsp;Laurence Chaton ,&nbsp;Riyad Hanafi ,&nbsp;Alix Persyn ,&nbsp;François Godart ,&nbsp;Jean Benoit Baudelet ,&nbsp;Francis Juthier ,&nbsp;Gabriella Riccardi ,&nbsp;Martin Dubernet ,&nbsp;Clémence Etienne ,&nbsp;Stephanie Delangue ,&nbsp;Morgan Recher","doi":"10.1016/j.acvd.2025.06.031","DOIUrl":"10.1016/j.acvd.2025.06.031","url":null,"abstract":"<div><h3>Introduction</h3><div>Suboptimal neurodevelopmental issues concern 30 to 60% of infants with congenital heart disease, cardiac surgery before 1 year being a major risk factor. We ought to demonstrate the association between pediatric feeding disorders (PFD) and suboptimal neurodevelopment (SND) in this population.</div></div><div><h3>Method</h3><div>We conducted a prospective study to assess the prevalence of PFD, as defined by Goday et al., diagnosed by a speech therapist, and their correlation with SND at twelve months in children who underwent surgery with cardiopulmonary bypass (CPB) before the age of one. A total of 45 children were included between October 2020 and February 2024 in a systematic monitoring protocol of our institution.</div></div><div><h3>Results</h3><div>Preoperatively, 38% of infants had a PFD, compared to 29% at twelve months (<span><span>Figure 1</span></span>a) Additionally, 77% showed suboptimal neurodevelopment (SND) (defined by ASQ<!--> <!-->&lt;<!--> <!-->2SD and/or AIMS<!--> <!-->&lt;5<!--> ^th percentile) at 12 months (<span><span>Figure 1</span></span>b and c). No significant association was found between PFD and neurodevelopment at twelve months, but preoperative PFD was linked to SND at twelve months (<em>p</em> <!-->=<!--> <!-->0,03). Infants with preoperative PFD were more cyanotic before surgery (<em>p</em> <!-->=<!--> <!-->0.017), underwent surgery at an older age (<em>p</em> <!-->=<!--> <!-->0.013), and had a smaller head circumference at twelve months (<em>p</em> <!-->=<!--> <!-->0.006) compared to those without feeding disorders. The presence of a nasogastric tube (NGT) was associated with preoperative PFD (<em>p</em> <!-->=<!--> <!-->0.01), and with 12 months PFD (<em>p</em> <!-->=<!--> <!-->0.01) but positive predictive value was low (VPP respectively 61% and 50%). NGT pre or postoperatively was not significantly associated with neurodevelopment outcome at twelve months. But 12 months PFD was associated with lower ASQ communication result (<em>p</em> <!-->=<!--> <!-->0.04).</div></div><div><h3>Conclusion</h3><div>Feeding difficulties diagnosed by speech therapists (and not only the presence of a feeding tube) appearing before surgery could serve as an early warning sign to predict neurodevelopmental issues in this population and help identify prematurely children that will need dedicated support.</div></div>","PeriodicalId":55472,"journal":{"name":"Archives of Cardiovascular Diseases","volume":"118 8","pages":"Pages S263-S264"},"PeriodicalIF":2.2,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144934283","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":"Improving therapeutic education with 3D printing models of congenital heart disease","authors":"Phuong Hong Do ,&nbsp;Jean Marc El Arid ,&nbsp;Paul Neville ,&nbsp;Bruno Lefort","doi":"10.1016/j.acvd.2025.06.055","DOIUrl":"10.1016/j.acvd.2025.06.055","url":null,"abstract":"<div><h3>Introduction</h3><div>Explaining the anatomy and surgical repear of a congenital heart defect (CHD) during a consultation is a crucial moment for patients and their families. A good understanding of heart disease is essential for adherence to therapy and reducing anxiety. We aim to evaluate the impact of CHD 3D models on improving understanding and reducing anxiety in parents prior to surgery.</div></div><div><h3>Method</h3><div>Parents of children scheduled for cardiac surgery were prospectively included and randomized into 2 groups: a control group who received a conventional explanation using drawing, and a 3D group who additionally received an explanation using a 3D model of the heart defect (<span><span>Figure 1</span></span>). Before and after the surgical consultation, each participant completed the same questionnaire assessing knowledge and understanding of the disease and surgery (5-point Lickert scale), and the same questionnaire assessing anxiety (State-Trait Anxiety Inventory Y-A).</div></div><div><h3>Results</h3><div>22 parents of 15 children were assigned to the “control group” (n<!--> <!-->=<!--> <!-->10) or the “3D group” (n<!--> <!-->=<!--> <!-->12). The 2 groups had comparable levels of anxiety about surgery and understanding of pathology and treatment before the consultation. With the 3D model, the level of understanding and knowledge improved significantly more than with a conventional explanation (understanding score 27.7<!--> <!-->±<!--> <!-->2.3 <em>vs.</em> 24.8<!--> <!-->±<!--> <!-->3.9; <em>p</em> <!-->=<!--> <!-->0.04). At the same time, the anxiety score decreased more significantly in the 3D group (STAI Y-A score 52.0<!--> <!-->±<!--> <!-->5.8 <em>vs.</em> 46.3<!--> <!-->±<!--> <!-->7.4; <em>p</em> <!-->=<!--> <!-->0.05).</div></div><div><h3>Conclusion</h3><div>Our preliminary results suggest an improvement in parental understanding and a reduction in anxiety prior to surgery, surpassing traditional methods of explanation. They constitute an effective tool for educating parents and managing their preoperative anxiety, thereby improving the therapeutic alliance.</div></div>","PeriodicalId":55472,"journal":{"name":"Archives of Cardiovascular Diseases","volume":"118 8","pages":"Page S277"},"PeriodicalIF":2.2,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144934157","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":"Surgical ventricular septal defect repair: How large is too large?","authors":"Emmanuelle Fournier ,&nbsp;Bastien Provost ,&nbsp;Alice Dirickx ,&nbsp;Estíbaliz Valdeolmillos ,&nbsp;Grégoire Albenque ,&nbsp;Clement Batteux ,&nbsp;Viktoria Weixler ,&nbsp;Sébastien Hascoët ,&nbsp;Belli Emre","doi":"10.1016/j.acvd.2025.06.053","DOIUrl":"10.1016/j.acvd.2025.06.053","url":null,"abstract":"<div><h3>Introduction</h3><div>Ventricular septal defects (VSDs) vary widely in anatomy and size. Surgical treatment of very large VSDs can be challenging as no clear recommendations exist about which of these defects can undergo biventricular repair with septation and which should instead be palliated. While Fontan circulation is known to be associated with long-term morbidity, information is limited about how ventricular septation of large VSDs affects cardiac function.</div></div><div><h3>Method</h3><div>In this descriptive case series, we are presenting three cases in which patients initially deemed candidates for univentricular palliation due to very large VSDs but were ultimately chosen for septation at our institution. The aim was to share our experiences with these complex cases and demonstrate our lessons learned.</div></div><div><h3>Results</h3><div>All patients had a prior pulmonary artery band as the first stage of univentricular palliation. At the time of VSD septation, the median age was 2.5 years [2–2.5], with a median weight of 11<!--> <!-->kg [8.4–12]. All cases presented a large perimembranous inlet-outlet VSD without AV septal malalignment. The median size of the VSDs in our series was 27<!--> <!-->×<!--> <!-->22<!--> <!-->mm and 13.7<!--> <!-->×<!--> <!-->11.2<!--> <!-->mm/m². A fibrous continuity between the tricuspid and the mitral valve with two separate AV junctions were described. No straddling was present and RV/LV ratio was comprised between 0.725 and 0.97 (<span><span>Figure 1</span></span>). Right heart catheterization showed normal pulmonary pressures. After multidisciplinary discussion, ventricular septation was concluded based on the two adequately sized separate AV valves without straddling, the RV/LV ratio, and the VSD which was considered technically feasible for a closure with muscular and fibrous borders. Because of the large VSD-patch, a staged approach was concluded with the pulmonary artery band being removed without enlarging the previously banded area allowing elevated RV pressures of &gt;<!--> <!-->50–60% of the systemic level, to promote positive ventricular–ventricular interactions. Median LVEF was 65% [62–67] at discharge. No heart block was observed.</div></div><div><h3>Conclusion</h3><div>Ventricular septation of three patients with large VSDs<!--> <!-->≤<!--> <!-->15<!--> <!-->mm/m² was successfull with good short-outcomes. A staged approach maintaining elevated right ventricular pressures helps to preserve positive ventricular interactions. Postponing surgical treatment to a later point after infancy seems to be a reasonable approach and allows for biventricular consideration.</div></div>","PeriodicalId":55472,"journal":{"name":"Archives of Cardiovascular Diseases","volume":"118 8","pages":"Page S276"},"PeriodicalIF":2.2,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144934165","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}