H. Lucron , M. Brard , J. D’orazio , L. Long , A. Le Harrivel De Gonneville , S. Tuttle , N. Elenga , J. Inamo , D. Bonnet , R. Banydeen
{"title":"Infant congenital cardiac mortality and univentricular hearts in Latin America and Caribbean: A population-based study in French Guiana","authors":"H. Lucron , M. Brard , J. D’orazio , L. Long , A. Le Harrivel De Gonneville , S. Tuttle , N. Elenga , J. Inamo , D. Bonnet , R. Banydeen","doi":"10.1016/j.acvd.2024.07.047","DOIUrl":"10.1016/j.acvd.2024.07.047","url":null,"abstract":"<div><h3>Introduction</h3><p>Except for projections from the Global Burden of Disease (GBD) study, congenital heart disease (CHD) burden remains undetermined in Latin America and Caribbean (LAC), especially for complex types such as functionally univentricular hearts (FUH) responsible for higher disease morbidity and mortality.</p></div><div><h3>Objective</h3><p>To describe CHD prevalence, characteristics and related infant mortality in French Guiana, a LAC territory.</p></div><div><h3>Methods</h3><p>Population-based registry analysis of all fetal and live birth (LB) CHD cases from January 2012 to December 2016.</p></div><div><h3>Results</h3><p>Overall, 231 CHD were diagnosed, among which 56 fetal diagnoses and 215 resulting LB. Among all CHD (<em>n</em> <!-->=<!--> <!-->231), there were 18.6% of chromosomal or genetic anomalies, and 6.5% of termination of pregnancy for fetal anomaly. Total and LB CHD prevalence were respectively 68.4 [95% CI: 67.9–68.8] and 65.2 [95% CI: 64.7–65.7] per 10,000, similar to GBD projections for TLA. The total infant mortality in the study sample was 9.4/10,000 LB [95% CI: 9.1–9.7]. Children with FUH presented the highest mortality rates. When compared to mainland France, total FUH prevalence for French Guiana was significantly higher (<em>P</em> <!-->=<!--> <!-->0.03), as were LB FUH prevalence and related infant mortality respectively two to three-fold higher (<em>P</em> <!--><<!--> <!-->0.01). However, no significant difference was observed for FUH prevalence when compared to GBD projections for TLA. FUH infant mortality could not be contrasted due to the lack of GBD data.</p></div><div><h3>Conclusion</h3><p>This first population-based study ever performed in this part of the world suggests a distinct profile for CHD in the LAC region, with elevated prevalence and mortality rates linked to FUH. Another potential determinant of the excess mortality risk might be the frequent presence of chromosomal or genetic anomalies. This constitutes a stepping-stone towards better understanding CHD burden and underlying specificities while providing future keys towards reducing infant mortality in French Guiana and others surrounding countries.</p></div>","PeriodicalId":55472,"journal":{"name":"Archives of Cardiovascular Diseases","volume":"117 8","pages":"Pages S241-S242"},"PeriodicalIF":2.3,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142271173","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}
J. Grynblat , H.J. Bogaard , M. Eyries , O. Meyrignac , L. Savale , M.R. Ghigna , L. Celant , A. Houweling , M. Levy , F. Antigny , A. Chaouat , V. Cottin , D. Bonnet , M. Humbert , D. Montani
{"title":"Pulmonary vascular phenotype identified in patients with GDF2 (BMP9) or BMP10 variants: An international multicentre study","authors":"J. Grynblat , H.J. Bogaard , M. Eyries , O. Meyrignac , L. Savale , M.R. Ghigna , L. Celant , A. Houweling , M. Levy , F. Antigny , A. Chaouat , V. Cottin , D. Bonnet , M. Humbert , D. Montani","doi":"10.1016/j.acvd.2024.07.032","DOIUrl":"10.1016/j.acvd.2024.07.032","url":null,"abstract":"<div><h3>Introduction</h3><p>Bone morphogenetic proteins 9 and 10 (BMP9 and BMP10), encoded by GDF2 and BMP10, respectively, play a pivotal role in pulmonary vascular regulation. GDF2 variants have been reported in pulmonary arterial hypertension (PAH) and hereditary haemorrhagic telangiectasia (HHT). However, the phenotype of GDF2 and BMP10 carriers remains largely unexplored.</p></div><div><h3>Objective</h3><p>Describe the features of <em>GDF2</em> and <em>BMP10</em> carriers with PAH.</p></div><div><h3>Methods</h3><p>We report the characteristics and outcomes of PAH patients in GDF2 and BMP10 carriers from the French and Dutch pulmonary hypertension registries. A literature review explored the phenotypic spectrum of these patients.</p></div><div><h3>Results</h3><p>Twenty-six PAH patients were identified: 20 harbouring heterozygous GDF2 variants, one homozygous GDF2 variant, four heterozygous BMP10 variants, and one with both GDF2 and BMP10 variants. The prevalence of GDF2 and BMP10 variants was 1.3% and 0.4%, respectively. Median age at PAH diagnosis was 30 years, with a female/male ratio of 1.9. Congenital heart disease (CHD) was present in 15.4% of the patients. At diagnosis, most of the patients (61.5%) were in New York Heart Association Functional Class III or IV with severe haemodynamic compromise [median (range) pulmonary vascular resistance 9.0 (3.3–40.6) WU]. Haemoptysis was reported in four patients; none met the HHT criteria. Two patients carrying BMP10 variants underwent lung transplantation, revealing typical PAH histopathology. The literature analysis showed that 7.6% of GDF2 carriers developed isolated HHT, and identified cardiomyopathy and developmental disorders in BMP10 carriers (<span><span>Fig. 1</span></span>).</p></div><div><h3>Conclusion</h3><p>GDF2 and BMP10 pathogenic variants are rare among PAH patients, and occasionally associated with CHD. HHT cases among GDF2 carriers are limited according to the literature. BMP10 full phenotypic ramifications warrant further investigation.</p></div>","PeriodicalId":55472,"journal":{"name":"Archives of Cardiovascular Diseases","volume":"117 8","pages":"Page S234"},"PeriodicalIF":2.3,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142271228","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}
A. Moiroux-Sahraoui, N. Derridj, R. Gaudin, M. Pontailler, P. Vouhe, D. Bonnet, O. Raisky
{"title":"Restrictive LV-PA conduit in ccTGA with VSD and LVOTO","authors":"A. Moiroux-Sahraoui, N. Derridj, R. Gaudin, M. Pontailler, P. Vouhe, D. Bonnet, O. Raisky","doi":"10.1016/j.acvd.2024.07.015","DOIUrl":"10.1016/j.acvd.2024.07.015","url":null,"abstract":"<div><h3>Introduction</h3><p>Congenitally corrected transposition of the great arteries (ccTGA) is a rare, complex and challenging structural heart disease. Therapeutic management remains controversial, particularly in patients with left ventricle outflow tract obstruction (LVOTO). As the natural history of ccTGA with LVOTO is overall very satisfactory and interventions that increase sub-pulmonary left ventricle (LV) pressure load are associated with a reduction of tricuspid regurgitation (TR) and systemic right ventricle (RV) dysfunction, we began to use restrictive left ventricle-to-pulmonary artery (LV-PA) conduit without ventricular septal defect (VSD) closure as part of the physiological repair of ccTGA/VSD/LVOTO.</p></div><div><h3>Objective</h3><p>To report on the outcome of ccTGA/VSD/LVOTO patients treated with restrictive LV-PA conduit without VSD closure.</p></div><div><h3>Methods</h3><p>Between 1979 and 2024, 9 consecutive patients with ccTGA/non-restrictive VSD/LVOTO underwent restrictive LV-PA conduit interposition without VSD closure at Necker Sick Children's Hospitals (Paris, France).</p></div><div><h3>Results</h3><p>Six (66.7%) patients had pulmonary stenosis and 3 (33.3%) patients had pulmonary atresia. Median age and weight at surgery were 5 (IQR: 16.6) years and 18 (IQR: 36.5) kgs. Five (55.6%) patients had previously undergone surgical palliation. No patient died after surgery with a median follow-up of 9.8 (IQR: 13.4) years. No patient required permanent pacemaker implantation for complete AV block. Freedom from reintervention was 62.5% (95% CI: 22.9–86.1) at 10<!--> <!-->years. Late reintervention consisted in LV-PA conduit replacement (<em>n</em> <!-->=<!--> <!-->3), PA stenting (<em>n</em> <!-->=<!--> <!-->1) and one and half ventricle repair (hemi-Senning, Glenn, Rastelli, <em>n</em> <!-->=<!--> <!-->1). At last follow-up, only one patient was significantly limited in physical activity (NYHA class III). No patient developed atrial or ventricular arrythmia. No patients developed moderate or severe TR and only 2 (22.2%) patients had decreased RV function and dilatation.</p></div><div><h3>Conclusion</h3><p>We believe that restrictive LV-PA conduit placement without VSD closure represents a worthwhile biventricular alternative strategy to anatomic repair when it is deemed too risky and/or impractical.</p></div>","PeriodicalId":55472,"journal":{"name":"Archives of Cardiovascular Diseases","volume":"117 8","pages":"Page S227"},"PeriodicalIF":2.3,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142271097","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}
H. Lucron , M. Brard , V. Atallah , J. D’orazio , S. Tuttle , J. Yssap , C. Isetta , F. Lion , A. Le Harrivel De Gonneville , Y. Hatchuel , J. Inamo , F. Barbotin-Larrieu
{"title":"Characteristics and results of the ongoing French and Caribbean congenital cardiac catheterization program","authors":"H. Lucron , M. Brard , V. Atallah , J. D’orazio , S. Tuttle , J. Yssap , C. Isetta , F. Lion , A. Le Harrivel De Gonneville , Y. Hatchuel , J. Inamo , F. Barbotin-Larrieu","doi":"10.1016/j.acvd.2024.07.045","DOIUrl":"10.1016/j.acvd.2024.07.045","url":null,"abstract":"<div><h3>Introduction</h3><p>Disparities persist regarding the access to pediatric and congenital cardiac catheterization procedures (PCCC). Developing capacities in French overseas territories located far away from mainland France remains essential to reducing inequalities and mortality.</p></div><div><h3>Objective</h3><p>To describe the characteristics of patients, procedures type risk categories (PTRC) and outcomes for all PCCC performed on the French island of Martinique (Caribbean).</p></div><div><h3>Methods</h3><p>Single center observational study. We recorded prospectively all consecutives PCCC performed in the catheter laboratory of the university Hospital of Martinique from January 2007 to December 2023. Demographics, procedures characteristics, PTRC, adverse events occurrence, as well as 24-hours mortality were documented. All results were retrospectively analysed.</p></div><div><h3>Results</h3><p>We performed 869 PCCC in 770 consecutives patients (31.3%<!--> <!--><<!--> <!-->1-year-old; 9.4% aged<!--> <!--><<!--> <!-->1<!--> <!-->month) living in French territories (90%) of Martinique, Guadeloupe, French Guiana, Saint-Martin, or natives from others Caribbean islands (10%). The median age and weight (IQR) were 4<!--> <!-->years (0.6–13) and 16<!--> <!-->kg (6.4–4.6). There were 126 urgent and 43 (4.9%) rescue procedures. PCCC were predominantly interventional (68.7%; success rate: 95.1%) covering all types of procedures, except revalvulation. The overall median PTRC was 2 (2–3) with a 24-h mortality of 1.1%. Complications occurred in 35 cases (4%), of which 18 were major, either procedure and anaesthesia-related (40%) or likely linked (40%) to the critical condition on admission at the cathlab. Overtime, the case-mix complexity continued to increase (PTRC<!--> <!-->><!--> <!-->3 in 25% of patients, with a median age and weight of 2.6<!--> <!-->years and 6.5<!--> <!-->kg, within the past 5<!--> <!-->years).</p></div><div><h3>Conclusion</h3><p>Developing permanent PCCC capabilities in French Martinique is efficient and safe. This ongoing international program is sustainable with the inclusion of patients coming from all French territories and neighbouring Caribbean islands and the continuous support of French Health Authorities.</p></div>","PeriodicalId":55472,"journal":{"name":"Archives of Cardiovascular Diseases","volume":"117 8","pages":"Page S241"},"PeriodicalIF":2.3,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142271171","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}
Claire Bouleti , Benjamin Alos , Damien Legallois , Romain Eschalier , Jerome Costa , Victoria Tea , Jean-Noel Trochu , Guillaume Turlotte , Julie Perrin-Faurie , Teodora Dutoiu , François Picard , Gregory Ducrocq , Pascal de Groote , Thierry Laperche , Clement Delmas , Ariel Cohen , Maxime Doublet , Damien Logeart
{"title":"Rationale and design of the French Observatory of Acute Heart Failure (OFICA2)","authors":"Claire Bouleti , Benjamin Alos , Damien Legallois , Romain Eschalier , Jerome Costa , Victoria Tea , Jean-Noel Trochu , Guillaume Turlotte , Julie Perrin-Faurie , Teodora Dutoiu , François Picard , Gregory Ducrocq , Pascal de Groote , Thierry Laperche , Clement Delmas , Ariel Cohen , Maxime Doublet , Damien Logeart","doi":"10.1016/j.acvd.2024.05.120","DOIUrl":"10.1016/j.acvd.2024.05.120","url":null,"abstract":"<div><h3>Background</h3><div>Acute heart failure (AHF) is a leading cause of hospitalization and mortality – especially in patients aged<!--> <!-->≥<!--> <!-->65 years in high-income countries – and represents a high healthcare burden. In the past decade, the epidemiology and management of heart failure (HF) has changed, with the emergence of new medical and interventional therapeutics, but up-to-date real-life data are scarce.</div></div><div><h3>Aims</h3><div>The main objectives are to describe baseline characteristics (with an emphasis on lifestyle, cognitive status, HF knowledge and treatment adherence), management, and in-hospital and mid-term outcomes of AHF patients in France. Secondary objectives are to investigate determinants of prognosis, modalities of treatment and follow-up, and identify gaps between guidelines and real-life management.</div></div><div><h3>Methods</h3><div>OFICA2 is a prospective multicentre observational survey that enrolled 1513 patients hospitalized for AHF in 80 participating centres in France during March and April 2021. The diagnosis of AHF was made according to the European Society of Cardiology guidelines definition. Inclusion criteria were age<!--> <!-->≥<!--> <!-->18<!--> <!-->years, health coverage and consent to participate. Detailed information was collected prospectively starting at admission. Thanks to direct linking with the French National Health Database, the anteriority up to 2<!--> <!-->years before inclusion, as well as a 3-year follow-up is specified for each patient and includes individual information on death, hospital admissions, major clinical events, drug delivery and use of reimbursed health resources.</div></div><div><h3>Conclusion</h3><div>This cohort provides a representative snapshot on contemporary AHF, with a particular focus on self-care determinants, and will improve knowledge about AHF presentation, management and outcomes.</div></div>","PeriodicalId":55472,"journal":{"name":"Archives of Cardiovascular Diseases","volume":"117 8","pages":"Pages 514-520"},"PeriodicalIF":2.3,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141691647","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}
Ingrid Allagbé , Marianne Zeller , Daniel Thomas , Guillaume Airagnes , Frédéric Limosin , Abdelali Boussadi , Frédéric Chagué , Anne-Laurence Le Faou
{"title":"Sex-specific predictive factors of smoking cessation in subjects at high cardiovascular risk","authors":"Ingrid Allagbé , Marianne Zeller , Daniel Thomas , Guillaume Airagnes , Frédéric Limosin , Abdelali Boussadi , Frédéric Chagué , Anne-Laurence Le Faou","doi":"10.1016/j.acvd.2024.06.001","DOIUrl":"10.1016/j.acvd.2024.06.001","url":null,"abstract":"<div><div>Smoking is a major risk factor for cardiovascular diseases (CVD), in particular in women, but smoking cessation (SC) reduces or even cancels the risk for both sexes. Using a nationwide SC services database, we aimed to determine the predictive factors of SC in men and women smokers with CV risk factors (CVRF) or CVD. A retrospective study from the French CDTnet database was conducted. Inclusion criteria were age ≥<!--> <!-->18<!--> <!-->years, and<!--> <!-->≥<!--> <!-->1 CVRF (Body Mass Index ≥<!--> <!-->25<!--> <!-->kg/m<sup>2</sup>, hypertension, diabetes, hypercholesterolemia) or CVD (myocardial infarction (MI) or angina pectoris, stroke, peripheral arterial disease [PAD]). Self-reported smoking abstinence (≥<!--> <!-->28 consecutive days) was confirmed by exhaled carbon monoxide<!--> <!--><<!--> <!-->10<!--> <!-->ppm. Logistic regression analysis assessed the association between SC and sociodemographic, medical characteristics, and smoking profile. Among the 36,864 smokers at high CV risk, abstinence rate was slightly lower in women than in men, (52.6% [<em>n</em> <!-->=<!--> <!-->8,102] vs 55.0% [<em>n</em> <!-->=<!--> <!-->11,848], <em>P</em> <!--><<!--> <!-->0.001). For both sexes, factors associated with the lowest abstinence rates were diabetes, respiratory and psychiatric diseases, anxiolytic/antidepressant use, and cannabis consumption. In women, the factors associated with smoking abstinence were suffering from MI or angina and taking contraceptive pill and the factors associated with persistent smoking were alcohol disorder and high cigarette consumption. In men, there was a positive relationship between overweight and abstinence while being dual users of cigarettes and electronic cigarettes at first visit, having tobacco-related diseases (cancer and PAD) and taking opioid substitution treatment were associated with persistent smoking. Finally, in both sexes, the factors associated with abstinence were: age<!--> <!-->≥<!--> <!-->65<!--> <!-->years, having a diploma, being employed, self-referred or encouraged by entourage, ≥<!--> <!-->1 previous quit attempt, ≤<!--> <!-->20 cigarettes per day consumption, benefiting from SC medication prescription and ≥<!--> <!-->4 follow-up visits. In conclusion, our results suggest the relevance of SC intensive management in smokers at high CV risk, based on sociodemographic, medical, and smoking behaviour characteristics, as well as a gender-specific SC approach.</div></div>","PeriodicalId":55472,"journal":{"name":"Archives of Cardiovascular Diseases","volume":"117 8","pages":"Pages 480-489"},"PeriodicalIF":2.3,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141697491","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}
P. David , A.S. Chaussade , L. Iserin , S. Malekzadeh-Milani , F. Bajolle , D. Bonnet , D. Khraiche , A. Legendre
{"title":"The pre-Fontan cyanosis period: Key factor in post-Fontan exercise cardiac output in young patients","authors":"P. David , A.S. Chaussade , L. Iserin , S. Malekzadeh-Milani , F. Bajolle , D. Bonnet , D. Khraiche , A. Legendre","doi":"10.1016/j.acvd.2024.07.008","DOIUrl":"10.1016/j.acvd.2024.07.008","url":null,"abstract":"<div><h3>Background</h3><p>Most patients with Fontan circulation struggle to increase cardiac output during exercise affecting aerobic capacity, response to training and quality of life. This poor cardiac performance affects their aerobic capacity, their response to training and their quality of life. We sought to identify the pre-Fontan and managerial factors that influence their exercise cardiac performance.</p></div><div><h3>Methods</h3><p>We retrospectively collected pre and post-Fontan anatomical, anamnestic and hemodynamic data in 57 consecutive young Fontan patients (median age 14.7 years) who underwent cardiopulmonary exercise test. We studied their exercise cardiac performance using a thoracic bioelectrical impedance device that allows measurement of the peak cardiac index (pCI).</p></div><div><h3>Results</h3><p>The median age at Fontan was 6.4<!--> <!-->±<!--> <!-->3 years. In multivariable models, pCI was negatively influenced by cyanosis duration (B<!--> <!-->=<!--> <!-->−0.240; <em>P</em> <!-->=<!--> <!-->0.0002). Peak indexed stroke volume (pSVi) was negatively influenced by Norwood procedure (B<!--> <!-->=<!--> <!-->-8.415; <em>P</em> <!-->=<!--> <!-->0.005) and cyanosis duration (B<!--> <!-->=<!--> <!-->−1.591; <em>P</em> <!-->=<!--> <!-->0.0002) and peak heart rate (pHR) by pre-Fontan peripheral oxygen saturation (B<!--> <!-->=<!--> <!-->0.602 <em>P</em> <!-->=<!--> <!-->0.009). Cyanosis duration<!--> <!-->≤<!--> <!-->6.9 years predicted a pSVi<!--> <!-->≥<!--> <!-->45 mL/m2 (AUC<!--> <!-->=<!--> <!-->0.747; <em>P</em> <!-->=<!--> <!-->0.001). Furthermore, pSVi and pHR were correlated with post-Fontan ventricular filling pressure (post-VFP) (respectively rp<!--> <!-->=<!--> <!-->−0.539, p<!--> <!-->=<!--> <!-->0.012 and rp<!--> <!-->=<!--> <!-->−0.552; <em>P</em> <!-->=<!--> <!-->0.010) and post-VFP was correlated with bidirectional cavopulmonary shunt duration (rs<!--> <!-->=<!--> <!-->0.498, <em>P</em> <!-->=<!--> <!-->0.023).</p></div><div><h3>Conclusion</h3><p>In young Fontan patients, the duration of cyanosis and low peripheral oxygen saturation prior to the Fontan procedure affect both components of cardiac exercise performance probably through increased ventricular filling pressure. Shortening the duration of cyanosis could help preserve cardiac performance in the long term.</p></div>","PeriodicalId":55472,"journal":{"name":"Archives of Cardiovascular Diseases","volume":"117 8","pages":"Pages S222-S223"},"PeriodicalIF":2.3,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142271091","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}
A. Gavotto , P. Amedro , I. Ouhab , S. Guillaumont , I. Liard , H. Huguet , M.C. Picot
{"title":"Which classification best predicts functional prognosis in children with congenital heart disease?","authors":"A. Gavotto , P. Amedro , I. Ouhab , S. Guillaumont , I. Liard , H. Huguet , M.C. Picot","doi":"10.1016/j.acvd.2024.07.010","DOIUrl":"10.1016/j.acvd.2024.07.010","url":null,"abstract":"<div><h3>Introduction</h3><p>Despite these advances in paediatric cardiology, the stratification of CHD severity using a simple and reproducible classification has not been established, as can be the NYHA functional class in adult heart failure. Various CHD classifications have been used in CHD, focusing on anatomical lesions, complexity of care, or physiological status, but their prognostic value has not been determined.</p></div><div><h3>Objective</h3><p>We aimed to compare the accuracy of the main existing CHD classifications (Uzark, Stout and Bethesda classifications), in the prediction of functional status in children with CHD, as determined by cardiopulmonary fitness.</p></div><div><h3>Methods</h3><p>Longitudinal cohort study.</p></div><div><h3>Results</h3><p>The CHD population having had 2 CPET included 296 subjects (<em>n</em> <!-->=<!--> <!-->129 female). The time between the first (T1) and second CPET (T2) assessments was 4.1<!--> <!-->±<!--> <!-->1.6<!--> <!-->years. The performance of classifications according to VO<sub>2</sub>max at T1 was better for Uzark classification. The VO<sub>2</sub>max Z-score decreased significantly according to the severity group (groups 1 and 2<!--> <!-->><!--> <!-->group 3<!--> <!-->><!--> <!-->group 4) and group 4 had a significant VO<sub>2</sub>max decrease of −6.68 [−10.69; −2.67] mL/kg/min compared to group 1. The prediction of classifications at T2 according to VO<sub>2</sub>max was better for Uzark classification with AUC values of 0.62 [0.55–0.69], compared to 0.59 [0.51–0.66] for Stout and 0.55 [0.48–0.62] for Bethesda (<span><span>Fig. 1</span></span>).</p></div><div><h3>Conclusion</h3><p>Among the existing cardiovascular risk classifications for CHD, the Uzark classification appeared to be the most reliable for discriminating the severity of CHD according to exercise capacity and for predicting the VO<sub>2</sub>max impairment than the other classifications tested. This longitudinal study also showed the continued decline in exercise capacity, whatever the CHD, and recalls the interest of regular monitoring to offer care adapted to these patients (such as rehabilitation programs) for primary prevention of the added cardiovascular risks of adulthood.</p></div>","PeriodicalId":55472,"journal":{"name":"Archives of Cardiovascular Diseases","volume":"117 8","pages":"Pages S224-S225"},"PeriodicalIF":2.3,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142271092","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}
D. Madec , S. Bernheim , A. Desgrange , N. Boddaert , O. Raisky
{"title":"A 3D analysis of ventricle position in patients with heterotaxy shows high frequency of ventricle malposition in D-Loop hearts","authors":"D. Madec , S. Bernheim , A. Desgrange , N. Boddaert , O. Raisky","doi":"10.1016/j.acvd.2024.07.014","DOIUrl":"10.1016/j.acvd.2024.07.014","url":null,"abstract":"<div><h3>Introduction</h3><p>Congenital heart defects in the context of heterotaxy are severe, with a complex anatomy. These defects are described using the binary concept of clinical loop (D-Loop or L-Loop), which implies that ventricle position is determined during heart looping. Recent work in the mouse has shown unexpected plasticity of ventricle position after heart looping. Based on a analysis of Nodal mutants with heterotaxy and right bronchus isomerism, 27% of D-Loop mutants at birth underwent leftward embryonic heart looping. These mice with a revertant loop, all display abnormal ventricle position.</p></div><div><h3>Objective</h3><p>Analysis of 3D ventricle position in human patients with heterotaxy and right bronchus isomerism, using the strategy developed in the mouse based on the 3D orientation of the interventricular septum.</p></div><div><h3>Methods</h3><p>Constitution of an heterotaxy cohort from the Necker database. Controls were selected as patients with transposition of the great arteries who had a systematic CT scan at 6 years. Three dimensions reconstructions of CT scans and quantitative analyses were performed using the Imaris software.</p></div><div><h3>Expected results</h3><p>Control patients had their right ventricle-left ventricle axis in the expected anteroposterior and left-right orientation. We collected 506 patients with heterotaxy syndrome, 186 of whom had CT scan. Forty patients had right bronchial isomerism. Among them, we found 29 (72%) patients who had an orientation of the interventricular septal axis similar to control, 7 (18%) patients with abnormal supero-inferior ventricles and 4 (10%) patients with abnormal left-right ventricles (<span><span>Fig. 1</span></span>). In total, 32% of patients in D-Loop have a malpositioned interventricular septum whereas 0 of patients in L-Loop.</p></div><div><h3>Perspectives</h3><p>There is a high proportion of malpositioned ventricles in patients with D-Loop (32%), similar to what is observed in Nodal mouse mutants. This result supports conserved mechanisms in the mouse and human, including plasticity of ventricle position after heart looping.</p></div>","PeriodicalId":55472,"journal":{"name":"Archives of Cardiovascular Diseases","volume":"117 8","pages":"Pages S226-S227"},"PeriodicalIF":2.3,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142271096","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}