Arthur Gavotto, Ines Liard, Helena Huguet, Marie-Christine Picot, Sophie Guillaumont, Stefan Matecki, Illies Ouhab, Pascal Amedro
{"title":"Which classification best predicts functional status in children with CHD?","authors":"Arthur Gavotto, Ines Liard, Helena Huguet, Marie-Christine Picot, Sophie Guillaumont, Stefan Matecki, Illies Ouhab, Pascal Amedro","doi":"10.1017/S1047951125001799","DOIUrl":"https://doi.org/10.1017/S1047951125001799","url":null,"abstract":"<p><strong>Background: </strong>Medical advances in the management of CHD have shifted the focus from childhood mortality to life-long morbidity; therefore, clinical research in paediatric cardiology tends to replace mortality outcomes with functional and patient-reported outcomes. Despite these advances, the stratification of disease severity using a simple and reproducible CHD classification has not been established. The aim was to determine which classification best predicts functional status in children with CHD, in terms of cardiopulmonary fitness.</p><p><strong>Method: </strong>This retrospective cohort study was assessed from a cohort study of 296 children (mean age 11.3 ± 3.1 years, 129 female), who underwent a baseline and final cardiopulmonary exercise tests with a mean follow-up of 4.1 ± 1.6 years.</p><p><strong>Results: </strong>Seven CHD classifications were identified, assessing anatomical, physiological, therapeutic, or functional parameters in foetal, paediatric, or adult CHD populations. The four-stage paediatric CHD disease severity classification established by Uzark et al. best predicted functional status at the final cardiopulmonary exercise test assessment, with an area under the receiver operating characteristics curves of 0.62 (0.55-0.69) for impaired cardiopulmonary fitness (maximum oxygen uptake (VO<sub>2max</sub>) or ventilatory anaerobic threshold < -1.64 Z-score). The best inter-judge classification agreement was also observed for Uzark classification, with a Kappa coefficient of 0.88 (0.78-0.98).</p><p><strong>Conclusion: </strong>The CHD classification by Uzark is reliable in terms of functional status prediction and reproducibility in children with CHD. This simple classification may contribute to identifying children with CHD most at risk of cardiopulmonary fitness impairment and initiating early preventive cardiovascular interventions in paediatric cardiology.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-7"},"PeriodicalIF":0.9,"publicationDate":"2025-05-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144092898","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Special type B interrupted aortic arch diagnosis by angiography in an adult patient: a case report.","authors":"Si-Yi Chen, Lei Fang, Jia-Bing Huang","doi":"10.1017/S1047951125001805","DOIUrl":"https://doi.org/10.1017/S1047951125001805","url":null,"abstract":"<p><p>Interrupted aortic arch is an uncommon cardiac anomaly characterised by a lack of continuity between the ascending and descending aorta. The presence of interrupted aortic arch in adults is extremely rare, and there is limited documentation of such cases in the literature. In this article, we present a unique case of interrupted aortic arch in an adult diagnosed through angiography. This case falls under the anatomical classification of type B interruption, although the blood supply to the left subclavian artery originates from the ascending aorta. Its haemodynamic characteristics are completely different from those of the classical type B interruption.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-4"},"PeriodicalIF":0.9,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143977087","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Mohammad Ryan Abumehdi, Matthew Jones, Naychi Lwin, Sophia Yong, Eric Rosenthal, Shakeel Qureshi
{"title":"Percutaneous VenusP-valve implantation in the paediatric population.","authors":"Mohammad Ryan Abumehdi, Matthew Jones, Naychi Lwin, Sophia Yong, Eric Rosenthal, Shakeel Qureshi","doi":"10.1017/S1047951125001763","DOIUrl":"https://doi.org/10.1017/S1047951125001763","url":null,"abstract":"<p><strong>Objectives: </strong>To assess short- and medium-term outcomes of VenusP-valve implantation in the pulmonary position in the paediatric population.</p><p><strong>Background: </strong>Percutaneous pulmonary valve implantation is now an established alternative to surgical pulmonary valve replacement, especially in those with conduits in the right ventricular outflow tract. The VenusP-valve™ (Venus Medtech, Shanghai, China) has demonstrated early efficacy in the adult population with larger conduit-free right ventricular outflow tracts. However, its use in children has not been well described.</p><p><strong>Methods: </strong>Retrospective review of patients under 18 years of age undergoing VenusP-valve implantation at a single institution between June 2015 and February 2023.</p><p><strong>Results: </strong>Fifteen patients under the age of 18 years underwent VenusP-valve™ implantation. All had severe pulmonary regurgitation and fulfilled accepted criteria for pulmonary valve implantation. Mean age at valve implantation was 14.1 (range 9.8-17.9) years, and mean weight was 54.9 (34.0-98.5) kg. The valve was deployed successfully in all the patients. The valve diameter and length ranged between 28-36 mm and 25-35 mm, respectively. Mean follow-up was 3.4 (0.5-8.1) years. At follow-up, twelve patients have undergone magnetic resonance imaging MRI as part of the regular surveillance. Indexed right ventricular end-diastolic volume improved from 157.8 (140.0-197.0) ml/m<sup>2</sup> to 117.6 (91.0-152.0) ml/m<sup>2</sup> (<i>p</i> = 0.004). Pulmonary regurgitation fraction had reduced from a mean of 44.3 (31.0-60.0) % to 3.6 (0.0-15.0) % (<i>p</i> = 0.003).</p><p><strong>Conclusion: </strong>This study demonstrates the safety and feasibility of the VenusP-valve implantation in children. Medium-term follow-up suggests that valve implantation is associated with a reduction in the degree of pulmonary regurgitation and right ventricular end-diastolic volume.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-9"},"PeriodicalIF":0.9,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143981354","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Biagio Castaldi, Alice Pozza, Roberta Biffanti, Jolanda Sabatino, Irene Cattapan, Jennifer Fumanelli, Giovanni Di Salvo
{"title":"Left ventricular longitudinal strain in the follow-up of arterial switch operation: a fingerprint of the patient's history.","authors":"Biagio Castaldi, Alice Pozza, Roberta Biffanti, Jolanda Sabatino, Irene Cattapan, Jennifer Fumanelli, Giovanni Di Salvo","doi":"10.1017/S1047951125001696","DOIUrl":"https://doi.org/10.1017/S1047951125001696","url":null,"abstract":"<p><strong>Background: </strong>Left ventricular function after arterial switch operation for d-transposition of the great arteries is notoriously compromised because of abnormal coronary artery anatomy or altered loading conditions. We sought to longitudinally investigate the performance of the left ventricle in a cohort of d-transposition of the great artery patients after arterial switch operation, by using advanced echocardiographic deformation imaging and grouping patients according to pre- and post-surgery variables, labelled as risk factors.</p><p><strong>Methods: </strong>Longitudinal single-centre study involving 53 d-transposition of the great artery patients (81.1% male) after arterial switch operation, the latter being performed as unique surgical procedure in 39 patients (76.5%). Median follow-up was 59 months [23.5-72].</p><p><strong>Results: </strong>Selected patients were split into two groups according to risk factors. Fifteen patients (30.6%) were grouped into high-risk class (<3 risk factors). Echocardiographic variables such as tricuspid annular plane systolic excursion, ejection fraction, and global longitudinal strain were compared between the two groups. Only global longitudinal strain reached statistical significance (-17.56 ± 2.26 versus -19.82 ± 1.97 %; <i>p</i> < 0.001). To discriminate high- versus low-risk patients, a receiver operating characteristic (ROC) curve identified a global longitudinal strain cut-off value of -17.75% (sensitivity 57.1%, specificity 97%, AUC 80%).</p><p><strong>Conclusions: </strong>Several neonatal and post-surgical variables might conditionate long-term follow-up of d-transposition of the great artery patients after arterial switch operation, and global longitudinal strain best conveys the overall risk profile of these patients.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-7"},"PeriodicalIF":0.9,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143964282","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Alessia Callegari, Damien Bonnet, Sophie Malekzadeh-Milani
{"title":"Simplified approach to Venus-P pulmonary valve implantation in a patient with complex cardiac anatomy: a simple solution for a difficult anatomy.","authors":"Alessia Callegari, Damien Bonnet, Sophie Malekzadeh-Milani","doi":"10.1017/S1047951125001817","DOIUrl":"https://doi.org/10.1017/S1047951125001817","url":null,"abstract":"<p><p>This report describes a 41-year-old female with left isomerism, interrupted inferior caval vein with azygos continuation, dextrocardia, and repaired tetralogy of Fallot, who underwent percutaneous pulmonary valve implantation using the Venus P-valve system. Due to anatomical constraints, left jugular venous access was utilised. A Venus P-valve (30 by 25 mm) was successfully implanted in the right ventricular outflow tract using a simplified, one-curve trajectory directly on its delivery system without a delivery sheath. Contrast injections during valve implantation were not possible, and the pre-implanted duct occluder was our anatomical landmarks. This case highlights the adaptability of the Venus P-valve and the importance of individualised procedural strategies in addressing anatomical challenges and achieving optimal outcomes.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-3"},"PeriodicalIF":0.9,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143974158","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Nelly Fabiani, Alexandra Heath-Freudenthal, Inge Von Alvensleben, Gabriel Echazú, Franz Freudenthal, Abraham Rothman
{"title":"Use of a ductal occluder device as a test and then permanent closure of a large ductus arteriosus with increased pulmonary vascular resistance: a case report.","authors":"Nelly Fabiani, Alexandra Heath-Freudenthal, Inge Von Alvensleben, Gabriel Echazú, Franz Freudenthal, Abraham Rothman","doi":"10.1017/S1047951125001726","DOIUrl":"https://doi.org/10.1017/S1047951125001726","url":null,"abstract":"<p><p>A 3-year-old girl with a large ductus arteriosus had increased pulmonary vascular resistance at cardiac catheterisation. Test occlusion of the ductus arteriosus with a Nit-Occlud PDA-R device and hyperoxia decreased the pulmonary arterial pressure to < 50% of systemic level. The ductus was closed with the same device. Two years later, an echocardiogram showed normal pulmonary arterial pressure.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-4"},"PeriodicalIF":0.9,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143972746","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"A predictive index for the need for neonatal surgical intervention in severe Ebstein's disease.","authors":"Shinichiro Sakaki, Shin Ono, Naka Saito, Fumiya Inoue, Tomoaki Taruya, Daichi Hosokawa, Takeshi Ikegawa, Takuya Wakamiya, Sadamitsu Yanagi, Hideaki Ueda","doi":"10.1017/S1047951125001787","DOIUrl":"https://doi.org/10.1017/S1047951125001787","url":null,"abstract":"<p><p>Severe Ebstein's disease often necessitates early neonatal surgical intervention, although, in some patients, surgery may be delayed until infancy or later, and medical management may be undertaken instead. Various indicators, such as tricuspid regurgitation flow velocity, have been studied to predict single or biventricular circulation; however, indicators predicting the need for neonatal surgery have not been addressed. We aimed to identify predictive indices for neonatal surgical necessity by analysing the clinical characteristics of those requiring and not requiring neonatal surgery. We enrolled eight patients with severe Ebstein's disease who were born at our hospital from 2018 to 2023. Four patients underwent neonatal surgery (Group S), whereas four did not (Group F). We compared pregnancy, delivery, and postnatal clinical courses and laboratory indices before and after birth between the groups. The systolic/diastolic time ratio, an index of ventricular function calculated from the TR waveform of echocardiography at birth, was significantly prolonged in Group S [2.18 ± 0.31 vs. 1.72 ± 0.20 in Group F (<i>p</i> = 0.043)]. The area under the receiver operating characteristic curve was 0.94, with a threshold systolic/diastolic time ratio value of 1.99 using Youden's method. This ratio predicted neonatal surgery necessity with 100% sensitivity and 75% specificity. Despite the small sample size, we demonstrated that the systolic/diastolic time ratio is a valuable predictive index of ventricular function and prognosis in severe Ebstein's disease patients. A systolic/diastolic time ratio <2, determined from the tricuspid regurgitation waveform on echocardiography at birth, supports patient monitoring without surgical intervention through the newborn period and beyond.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-5"},"PeriodicalIF":0.9,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143977744","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Cardiology in the YoungPub Date : 2025-05-01Epub Date: 2025-04-02DOI: 10.1017/S1047951125001441
Martina Larsson, Mats Synnergren, Thushara Rodrigo
{"title":"Case report: morphological challenges to surgical repair in complete atrioventricular septal defect with isolated ventricular component.","authors":"Martina Larsson, Mats Synnergren, Thushara Rodrigo","doi":"10.1017/S1047951125001441","DOIUrl":"10.1017/S1047951125001441","url":null,"abstract":"<p><p>We describe a trisomy 21 patient with postnatal diagnosis of atrioventricular septal defect with isolated ventricular component who had a complicated post-surgical course following complete repair. Clinical outcomes included moderate-severe residual atrioventricular valve regurgitation needing re-operation, complete heart block leading to pacemaker insertion, seizures secondary to subdural haemorrhages, and chylothorax. We describe the surgical considerations specific to this cardiac morphology.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1085-1087"},"PeriodicalIF":0.9,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143762899","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Cardiology in the YoungPub Date : 2025-05-01Epub Date: 2025-05-07DOI: 10.1017/S1047951125001593
Cheul Lee, Won Young Lee, Ju Ae Shin, Jae Young Lee
{"title":"Tricuspid atresia with absent pulmonary valve and intact ventricular septum: Fontan operation after complete exclusion of the right ventricle.","authors":"Cheul Lee, Won Young Lee, Ju Ae Shin, Jae Young Lee","doi":"10.1017/S1047951125001593","DOIUrl":"10.1017/S1047951125001593","url":null,"abstract":"<p><p>Tricuspid atresia with absent pulmonary valve and intact ventricular septum is an exceedingly rare CHD. Only few cases with completed single ventricle palliation have been reported in the literature. We present the follow-up case of a 4-year-old child with this cardiac anomaly who underwent Fontan operation after complete exclusion of the right ventricle.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1079-1081"},"PeriodicalIF":0.9,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143960608","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Cardiology in the YoungPub Date : 2025-05-01Epub Date: 2025-04-14DOI: 10.1017/S1047951125001507
Ross Foley, Kevin Walsh, Jonathan J Rome, Jonathan McGuinness, Liam Morris, Eoin O'Cearbhaill, Colin J McMahon
{"title":"3D printed heart models assist in pre-procedural planning of the innominate vein to pulmonary venous atrium redirection (Hraska / Rome procedures).","authors":"Ross Foley, Kevin Walsh, Jonathan J Rome, Jonathan McGuinness, Liam Morris, Eoin O'Cearbhaill, Colin J McMahon","doi":"10.1017/S1047951125001507","DOIUrl":"10.1017/S1047951125001507","url":null,"abstract":"<p><p>Innominate vein redirection to the pulmonary venous atrium has been used in single ventricle patients in order to relieve lymphatic complications resulting from systemic venous hypertension. This has been achieved both surgically, known as the Hraska procedure and via transcatheter approach, known as the Rome procedure. Determining the pathway from the innominate vein to the pulmonary venous atrium is challenging with important intra- and extra-cardiac structures close-by. We present two cases; one surgical and one transcatheter approach, where 3D-printed cardiac models were used to assist in the pre-procedural planning of this relatively novel and challenging strategy.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1066-1069"},"PeriodicalIF":0.9,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143962000","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}