Cardiology in the YoungPub Date : 2025-05-01Epub Date: 2025-04-21DOI: 10.1017/S1047951125001453
Line Høffner, Anna Maria Dehn, Sofie Dannesbo, Elisabeth Blixenkrone-Møller, Louise Lind, Anna Axelsson Raja, Anne-Sophie Sillesen, Christian Pihl, Marie Maagaard, Niels Grove Vejlstrup, Kasper Iversen, Henning Bundgaard, Vibeke Hjortdal
{"title":"Head circumference in neonates with septal defects.","authors":"Line Høffner, Anna Maria Dehn, Sofie Dannesbo, Elisabeth Blixenkrone-Møller, Louise Lind, Anna Axelsson Raja, Anne-Sophie Sillesen, Christian Pihl, Marie Maagaard, Niels Grove Vejlstrup, Kasper Iversen, Henning Bundgaard, Vibeke Hjortdal","doi":"10.1017/S1047951125001453","DOIUrl":"10.1017/S1047951125001453","url":null,"abstract":"<p><strong>Background: </strong>Neurodevelopmental disorders occur in up to 50% of children with CHD. Small head circumference at birth has been associated with impaired neurodevelopment in patients with complex CHD. It is unknown if patients with simple CHD such as septal defects have smaller head circumferences. The objective of this study was to investigate the head circumference at birth in neonates with either an atrial or a ventricular septal defect.</p><p><strong>Methods: </strong>This study is part of the Copenhagen Baby Heart Study; a prospective, population-based cohort study of more than 25,000 neonates. The neonates were examined with a comprehensive transthoracic echocardiography within the first 30 days of birth including assessment for atrial or ventricular septal defects. The head circumference at birth in term neonates with septal defects was compared to the head circumference in matched controls, term neonates without septal defects from the same birth cohort.</p><p><strong>Results: </strong>Neonates with septal defects (<i>n</i> = 1,030; 45.2% male; mean birthweight 3,534g ± 483g) had a mean head circumference of 34.8 cm (95% confidence interval 34.7-34.9 cm), compared to neonates without septal defects (<i>n</i> = 5,150; 45.6% male; mean birthweight 3,546g ± 476g) of 34.7 cm (95% confidence interval 34.7-34.8 cm); <i>p</i>-value 0.07. Mean calculated <i>z</i>-score of head circumferences was 0.05 for neonates with septal defects and -0.01 for neonates without septal defects, <i>p</i> = 0.07. Dividing cases into neonates with atrial septal defects, ventricular septal defects, and those without septal defects did not show differences between groups, <i>p</i> = 0.14.</p><p><strong>Conclusion: </strong>The head circumference in term neonates with septal defects did not differ from matched controls without septal defects.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"915-920"},"PeriodicalIF":0.9,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143984380","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: 2025-05-19DOI: 10.1017/S1047951125001660
Chiara Di Padua, Teresa Lemmen, Jonas Palm, Muneaki Matsubara, Thibault Schaeffer, Nicole Piber, Andrea Amici, Paul Philipp Heinisch, Stanimir Georgiev, Alfred Hager, Peter Ewert, Jürgen Hörer, Masamichi Ono
{"title":"Impact of pulmonary artery size on early haemodynamic and laboratory variables following total cavopulmonary connection.","authors":"Chiara Di Padua, Teresa Lemmen, Jonas Palm, Muneaki Matsubara, Thibault Schaeffer, Nicole Piber, Andrea Amici, Paul Philipp Heinisch, Stanimir Georgiev, Alfred Hager, Peter Ewert, Jürgen Hörer, Masamichi Ono","doi":"10.1017/S1047951125001660","DOIUrl":"10.1017/S1047951125001660","url":null,"abstract":"<p><strong>Objective: </strong>Current research suggests that a small pulmonary artery can cause adverse events and reduce exercise capacity after the Fontan procedure. This study aimed to evaluate the impact of pulmonary artery size on early haemodynamic and laboratory variables after total cavopulmonary connection.</p><p><strong>Methods: </strong>We reviewed all patients who underwent staged Fontan between 2012 and 2022. Pulmonary artery index before bidirectional cavopulmonary shunt and before total cavopulmonary connection was calculated according to Nakata and colleagues. We sought to analyse the impact of the pulmonary artery index on early haemodynamic and laboratory variables, including pulmonary artery pressure and mean arterial pressure 12 hours after extubation and lactate levels 6 hours after extubation.</p><p><strong>Results: </strong>A total of 263 patients were included. Median age and weight at total cavopulmonary connection were 2.2 (interquartile ranges: 1.8-2.7) years and 11.7 (interquartile range: 10.7-13.3) kg, respectively. Before that, all patients underwent bidirectional cavopulmonary shunt at a median age of 4.1 (interquartile range: 3.2-5.8) months. In the multivariable analysis, pre-bidirectional cavopulmonary shunt pulmonary artery index (<i>p</i> = 0.016, odds ratio 0.993), with a cut-off value of 154 mm<sup>2</sup>/m<sup>2</sup> was an independent risk factor for a higher pulmonary artery pressure (> 17 mmHg). No variable was identified as a significant risk factor for lower mean arterial pressure (< 57 mmHg). Regarding lactate levels (> 4.5 mg/dl), pre-bidirectional cavopulmonary shunt right pulmonary artery index (<i>p</i> < 0.001, odds ratio 0.983), with a cut-off value of 70 mm<sup>2</sup>/m<sup>2</sup> was identified as an independent risk factor.</p><p><strong>Conclusions: </strong>In patients with staged Fontan palliation, a small pulmonary artery size before bidirectional cavopulmonary shunt and total cavopulmonary connection was a determinant factor associated with unfavourable early postoperative haemodynamics after total cavopulmonary connection.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1002-1010"},"PeriodicalIF":0.9,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144092890","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: 2025-03-17DOI: 10.1017/S1047951125001209
Lisette M Harteveld, Nico A Blom, J Gert van Dijk, Robert H A M Reijntjes, Paul J van Someren, Fabian I Kerkhof, Irene M Kuipers, Lukas A J Rammeloo, Arend D J Ten Harkel
{"title":"Fluid responsiveness of ambulatory paediatric patients with a Fontan circulation by passive leg raising.","authors":"Lisette M Harteveld, Nico A Blom, J Gert van Dijk, Robert H A M Reijntjes, Paul J van Someren, Fabian I Kerkhof, Irene M Kuipers, Lukas A J Rammeloo, Arend D J Ten Harkel","doi":"10.1017/S1047951125001209","DOIUrl":"10.1017/S1047951125001209","url":null,"abstract":"<p><strong>Background: </strong>Passive leg raising is used to predict who will benefit from fluid therapy in critically ill patients, including children. Patients with a Fontan circulation may have a different haemodynamic response to a fluid challenge by passive leg raising.</p><p><strong>Methods: </strong>The haemodynamic response of 31 paediatric patients with a Fontan circulation from the outpatient clinic (median age 14.0 years) and 35 healthy controls (median age 12.8 years) to passive leg raising was evaluated non-invasively by echocardiography for the assessment of, e.g., velocity time integral across the (neo)aortic valve, blood pressure measurements, and respiration. Participants were considered responders when the velocity time integral increased ≥ 10.0%.</p><p><strong>Results: </strong>Overall, patients and controls did not differ in the haemodynamic response. Twelve patients (38.7%) and 8 controls (22.9%) were responders, which was not statistically different (<i>P</i> = 0.22). Responders in the patient and control group also had a similar echo-estimated velocity time integral increase of + 18.9% and + 15.2%, respectively (<i>P</i> = 0.91). There was no difference in echo-estimated velocity time integral change between patient and control non-responders with a decrease of -1.4% and -6.4%, respectively (<i>P</i> = 0.70) and no difference in the amount of patients who were negatively affected by passive leg raising, ith a decrease of ≤-10.0% in 7 patients (22.6%) and 9 controls (25.7%)(<i>P</i> = 0.77).</p><p><strong>Conclusion: </strong>The haemodynamic response of ambulatory paediatric patients with a Fontan circulation to passive leg raising is like that of healthy controls. Patients who did not respond were similarly affected as healthy controls. Whether the haemodynamic response is different in critically ill patients warrants further investigation.</p><p><strong>Trail registration: </strong>The Netherlands National Trial Register (NTR), Trial: NL6415; date of registration 20-07-2017; Trial information: https://www.trialregister.nl/trial/6415.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"938-943"},"PeriodicalIF":0.9,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143647359","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: 2025-03-26DOI: 10.1017/S1047951124026301
Alexa C Escapita, Anna Caroline Gregg, Kelsey Lambou, Heather Watson, Lawrence Greiten, Brian Reemtsen, Elijah Bolin, Dala Zakaria, Tara L Johnson
{"title":"Developmental language trajectories in children with critical CHD.","authors":"Alexa C Escapita, Anna Caroline Gregg, Kelsey Lambou, Heather Watson, Lawrence Greiten, Brian Reemtsen, Elijah Bolin, Dala Zakaria, Tara L Johnson","doi":"10.1017/S1047951124026301","DOIUrl":"10.1017/S1047951124026301","url":null,"abstract":"<p><strong>Introduction: </strong>Medical and surgical advancements have enabled a 95% survival rate for children with CHD. However, these survivors are disproportionately affected by neurodevelopmental disabilities. In particular, they have behavioural problems in toddlerhood. Because there is a known relationship between behavioural problems and early language delay, we hypothesise that children with critical CHD have early detectable language deficits. To test our hypothesis, we performed a retrospective study on a cohort of children with critical CHD to visualise their early language developmental trajectories.</p><p><strong>Methods: </strong>We identified a cohort of 27 children with two diagnoses: single ventricle physiology (19) and transposition of the great arteries (8). As part of their routine clinical care, all of these children had serial developmental evaluations with the language subsection of the Capute Scales. We visualised their developmental language trajectories as a function of chronologic age, and we used a univariate linear regression model to calculate diagnosis-specific expected developmental age equivalents.</p><p><strong>Results: </strong>In each group, language development is age-appropriate in infancy. Deviation from age-appropriate development is apparent by 18 months. This results in borderline-mild language delay by 30 months.</p><p><strong>Discussion: </strong>Using the Capute Scales, our team quantified early language development in infants and toddlers with critical CHD. Our identification of deceleration in skill acquisition reinforces the call for ongoing neurodevelopmental surveillance in these children. Understanding early language development will help clinicians provide informed anticipatory guidance to families of children with critical CHD.</p><p><strong>Social media synopsis: </strong>Children with single ventricle physiology and transposition of the great arteries have measurable early language delays.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"964-970"},"PeriodicalIF":0.9,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143708700","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: 2025-03-28DOI: 10.1017/S1047951125001234
Kari A Phillips, August Brennan, Michael Profsky, Gregory T Hadfield, Brandon W Kirkland, Caroline Heyrend, Eric R Griffiths, Lindsay J May, Michelle S Ploutz
{"title":"Adverse events and ICU readmission rates after floor transfer for paediatric patients on ventricular assist device support.","authors":"Kari A Phillips, August Brennan, Michael Profsky, Gregory T Hadfield, Brandon W Kirkland, Caroline Heyrend, Eric R Griffiths, Lindsay J May, Michelle S Ploutz","doi":"10.1017/S1047951125001234","DOIUrl":"10.1017/S1047951125001234","url":null,"abstract":"<p><p>Paediatric ventricular assist device patients, including those with single ventricle anatomy, are increasingly managed outside of the ICU. We used retrospective chart review of our single centre experience to quantify adverse event rates and ICU readmissions for 22 complex paediatric patients on ventricular assist device support (15 two ventricles, 7 single ventricle) after floor transfer. The median age was 1.65 years. The majority utilised the Berlin EXCOR (17, 77.3%). There were 9 ICU readmissions with median length of stay of 2 days. Adverse events were noted in 9 patients (41%), with infection being most common (1.8 events per patient year). There were no deaths. Single ventricle patients had a higher proportion of ICU readmission and adverse events. ICU readmission rates were low, and adverse event rates were comparable to published rates suggesting ventricular assist device patients can be safely managed on the floor.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"926-930"},"PeriodicalIF":0.9,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143728709","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":"Investigating the possible connection between cystic hygroma, nuchal translucency, and the genetic link underlying cardiac lymphatic abnormalities and congenital heart disease: is there an association?","authors":"Marios Loukas, Ishank Gupta, Brenna Wilson, Shamfa Joseph, Rachel Levene, Michael Montalbano","doi":"10.1017/S1047951125001404","DOIUrl":"10.1017/S1047951125001404","url":null,"abstract":"<p><p>Although a direct link between cardiac lymphatics and congenital heart disease (CHD) has not been established, research indicates that abnormalities in the cardiac lymphatic system are involved in several congenital disorders that present with cardiac defects. However, this contribution remains poorly understood and is still an emerging area of study. One theory proposes that abnormal lymphatic development, which can present as cystic hygroma or increased nuchal translucency, may give rise to heart defects such as coarctation of the aorta, hypoplastic left heart syndrome, or bicuspid aortic valves. The genetic pathways for the development of the cardiac lymphatic system and the heart's major vessels may overlap; therefore, mutations in these genes could result in simultaneous defects in both systems. The close anatomical proximity between cardiac lymphatics and the great vessels of the heart suggests a \"cause and effect\" relationship, where an abnormality in one could affect the other and lead to congenital defects. Given that congenital heart disease is the most common birth defect in the United States, this systematic review, following Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, seeks to explore the potential link between cardiac lymphatics and CHD. Understanding this connection could have significant clinical implications by paving the way for new diagnostic approaches and therapeutic strategies for CHDs.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"931-937"},"PeriodicalIF":0.9,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143794608","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/S1047951125001581
Dai Dac Tran, Tien Anh Do, Huy Quang Bui, Thanh Ngoc Le, Long Hoang Vo
{"title":"Protein-losing enteropathy after fontan surgery: insights from Vietnamese clinical experience and the need for global collaboration.","authors":"Dai Dac Tran, Tien Anh Do, Huy Quang Bui, Thanh Ngoc Le, Long Hoang Vo","doi":"10.1017/S1047951125001581","DOIUrl":"10.1017/S1047951125001581","url":null,"abstract":"<p><p>Protein-losing enteropathy remains a formidable and underrecognised complication following Fontan surgery, contributing significantly to morbidity and mortality among affected patients. Despite substantial progress in managing single-ventricle CHDs, protein-losing enteropathy persists as a major clinical challenge, particularly in resource-constrained environments where specialised diagnostic modalities are scarce. This manuscript discusses the prevalence, clinical presentation, diagnostic hurdles, and management of protein-losing enteropathy in post-Fontan patients, drawing from clinical experiences in Vietnam. Among 95 post-Fontan patients reviewed, the prevalence of protein-losing enteropathy was 4.2%, with considerable morbidity necessitating invasive interventions and, in some cases, resulting in mortality. Diagnostic limitations, including restricted access to alpha-1-antitrypsin clearance testing, highlight the urgent need for heightened clinical suspicion and pragmatic diagnostic approaches. We advocate for integrating low-cost, widely accessible screening measures, such as routine serum protein monitoring, into standard post-Fontan care protocols to enhance early detection and management of protein-losing enteropathy. Furthermore, we underscore the critical importance of global collaboration and multicentre research initiatives to address the worldwide burden of protein-losing enteropathy, facilitate resource-sharing, and develop evidence-based interventions. Coordinated international efforts are essential to closing diagnostic and treatment gaps, ultimately improving survival and quality of life for patients living with the Fontan circulation.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1057-1061"},"PeriodicalIF":0.9,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143964242","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: 2025-04-22DOI: 10.1017/S1047951125001532
Demet Tosun, Nihal Akçay, İlyas Bingöl, Damla Gökçeer Akbulut
{"title":"Noncompaction and dilated cardiomyopathy in carvajal syndrome.","authors":"Demet Tosun, Nihal Akçay, İlyas Bingöl, Damla Gökçeer Akbulut","doi":"10.1017/S1047951125001532","DOIUrl":"10.1017/S1047951125001532","url":null,"abstract":"<p><strong>Introduction: </strong>Carvajal syndrome, a rare autosomal recessive disorder caused by mutations in the <i>DSP</i> gene, is characterised by woolly hair, palmoplantar keratoderma, and left ventricular dilated cardiomyopathy. Although less frequently reported, noncompaction cardiomyopathy can co-occur, further complicating the clinical picture. Early diagnosis and management are crucial due to the high risk of progressive heart failure and sudden cardiac death in affected individuals.</p><p><strong>Case report: </strong>A 13-year-old male with autism presented with a 1.5-month history of persistent cough and worsening clinical symptoms, including hepatomegaly and signs of heart failure. Physical examination revealed woolly hair, patchy alopecia, nail anomalies, and ectodermal dysplasia. Echocardiography demonstrated left ventricular dilated cardiomyopathy, noncompaction, and a severely reduced ejection fraction of 23%. Initial management in the paediatric intensive care unit included inotropic support, diuretics, and beta-blockers. Genetic analysis confirmed a homozygous c.7912G > T nonsense variant in the <i>DSP</i> gene, establishing the diagnosis of Carvajal syndrome. The patient was referred to an advanced cardiac centre.</p><p><strong>Discussion: </strong>Carvajal syndrome involves multisystem manifestations, with prominent dermatologic and cardiovascular features. Unlike Naxos disease, which primarily affects the right ventricle, Carvajal syndrome predominantly involves the left ventricle, as observed in this case. Notably, left ventricular noncompaction was a striking feature in our patient, further exacerbating cardiac dysfunction and complicating the clinical course. Although noncompaction cardiomyopathy is less frequently reported in Carvajal syndrome, its pronounced presence in this case underscores the phenotypic variability and severity of myocardial involvement. Intensive care management with a multidisciplinary approach was essential in stabilising this patient. Genetic testing confirmed the diagnosis and highlighted the importance of molecular diagnostics in differentiating cardiocutaneous syndromes.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1073-1075"},"PeriodicalIF":0.9,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143972534","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: 2025-03-21DOI: 10.1017/S1047951125001258
Sofia Rito, Diogo Faim, Patrícia Vaz Silva, Pedro Correia, David La Plaza, António Pires
{"title":"A \"hybrid\" approach to total anomalous pulmonary venous return repair.","authors":"Sofia Rito, Diogo Faim, Patrícia Vaz Silva, Pedro Correia, David La Plaza, António Pires","doi":"10.1017/S1047951125001258","DOIUrl":"10.1017/S1047951125001258","url":null,"abstract":"<p><p>Total anomalous pulmonary venous return is a rare CHD that may present in the neonatal period with signs of congestive cardiac failure. First-line treatment is corrective surgery in the first months of life. The authors report the case of a 2-month-old infant with non-obstructive total anomalous pulmonary venous return submitted to a two-stage hybrid repair, involving cardiac surgery and interventional cardiology.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1062-1065"},"PeriodicalIF":0.9,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143669200","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":"Characteristics and postoperational outcomes in paediatric patients with coronary sinus septal defect: a retrospective cohort study.","authors":"Chengcheng Pang, Huimin Wang, Junjun Shen, Liuqing Yang, Jianglin Li, Hui Liu, Wei Pan, Zhiwei Zhang","doi":"10.1017/S1047951125000101","DOIUrl":"10.1017/S1047951125000101","url":null,"abstract":"<p><strong>Objective: </strong>To summarise the characteristics and postoperative outcomes in paediatric patients with coronary sinus septal defect.</p><p><strong>Method: </strong>This retrospective study recruited paediatric patients diagnosed with coronary sinus septal defect from the Guangdong Cardiovascular Institute between 2011 and 2023. Clinical characteristics, echocardiographic parameters, surgical procedures, and postoperative outcomes were collected from electronic health records.</p><p><strong>Results: </strong>Among the 68 patients, 50% were male, with a median age of 1.0 years. Four cases (5.9%) were diagnosed during the prenatal period. The proportions of patients with type I, II, III, and IV coronary sinus septal defect were 51.5%, 5.9%, 16.1%, and 26.5%, respectively. The most common coexisting cardiac anomalies were persistent left superior caval vein. Twenty-seven cases were either missed or misdiagnosed by echocardiogram, accounting for 39.7% of the overall cases, with type I being the most frequently missed diagnosis. Fifty-four patients underwent surgery, two patients received transcutaneous intervention, while the remaining patients did not undergo any surgery or intervention. At follow-up, two patients with type I coronary sinus septal defect died from multiorgan dysfunction, and one patient underwent reoperation due to narrowing of the extracardiac tunnel. The remaining patients did not experience any major events and recovered well.</p><p><strong>Conclusion: </strong>Paediatric patients with coronary sinus septal defect often do not exhibit specific clinical manifestations. Enhancing our understanding of the anatomic and haemodynamic characteristics of coronary sinus septal defect can improve the diagnostic accuracy of echocardiography. If diagnosis is suspected, confirmation can be obtained by cardiac CT and cardiac magnetic resonance. Accurate preoperative and intraoperative diagnosis of coronary sinus septal defect contributes to high surgical success rates and favourable treatment outcomes.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"944-952"},"PeriodicalIF":0.9,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143708699","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}