Cardiology in the Young最新文献

{"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":"https://doi.org/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":"1-9"},"PeriodicalIF":0.9,"publicationDate":"2025-03-26","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}

{"title":"Microbubbles in the venous and arterial circulations due to intestinal pathology in a child with unrepaired CHD: is all gas bad?","authors":"Lisha E Thomas, Rohit S Loomba","doi":"10.1017/S104795112500143X","DOIUrl":"https://doi.org/10.1017/S104795112500143X","url":null,"abstract":"<p><p>This is a unique case of microbubbles incidentally found on echocardiogram in a child with unpalliated CHD. The microbubbles were found in both the venous and arterial circulations and spontaneously resolved. A detailed evaluation was done to identify the source of air in the circulation. The child did have concurrent necrotising enterocolitis, and it was felt that the bubbles were secondary to this and intestinal translocation of air.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-3"},"PeriodicalIF":0.9,"publicationDate":"2025-03-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143708701","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":"Transcatheter patent ductus arteriosus closure in a syndromic patient with interrupted inferior vena cava: overcoming anatomical challenges.","authors":"Ilker Ufuk Sayici, Pelin Altinbezer, Utku Arman Örün","doi":"10.1017/S1047951125001416","DOIUrl":"https://doi.org/10.1017/S1047951125001416","url":null,"abstract":"<p><p>This case report discusses a 1-year-old female with severe growth retardation and multiple congenital anomalies, including a large patent ductus arteriosus and interrupted inferior vena cava. Successful percutaneous patent ductus arteriosus closure was achieved via the azygos venous route, overcoming anatomical challenges and highlighting the importance of individualized procedural strategies in complex cases.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-3"},"PeriodicalIF":0.9,"publicationDate":"2025-03-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143673372","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":"Extracorporeal membrane oxygenation: a bridge to palliation in single ventricle physiology.","authors":"Ivana Capin, George Ofori-Amanfo, Maria Esperanza, Raghav Murthy, Gary Oldenburg, Jacqueline M Lamour, Kristi Glotzbach, Scott I Aydin","doi":"10.1017/S1047951125001386","DOIUrl":"https://doi.org/10.1017/S1047951125001386","url":null,"abstract":"<p><strong>Objective: </strong>To evaluate the outcomes of patients with single ventricle physiology supported with extracorporeal membrane oxygenation as a bridge to first-stage palliation.</p><p><strong>Methods: </strong>This was a retrospective registry-based study. Data from the Extracorporeal Life Support Organization registry were used to identify single ventricle physiology patients supported with extracorporeal membrane oxygenation prior to palliation from 2016 to 2021. Descriptive statistics and multivariate analyses for associations with mortality were conducted.</p><p><strong>Results: </strong>Primary outcome was death before hospital discharge. Patient characteristics including demographics and associated complications were evaluated as secondary outcomes. Sixty-five patients met inclusion criteria. Survival to discharge was 42%. Twenty-four (37%) patients died while on extracorporeal membrane oxygenation. There was no significant difference in demographics between survivors and non-survivors. Non-survivors had a significantly longer median duration on extracorporeal membrane oxygenation compared to survivors, 99-hrs [IQR (Interquartile Range), 160, 300] vs. 59-hrs [43, 124] (<i>p</i><0.001). Multivariate analysis demonstrated extracorporeal membrane oxygenation duration (adjusted-OR [Odds Ratio] 1.01, 95% CI [Confidence Interval] 0.98, 0.99; <i>p = 0.03</i>) and requiring renal replacement therapy (42% vs. 19%; <i>p = 0.04</i>) were associated with mortality prior to discharge.</p><p><strong>Conclusions: </strong>Clinicians managing decompensated patients with single ventricle physiology may consider extracorporeal membrane oxygenation as a bridge to palliation. Survival to discharge was 42%. Evidence of renal injury and longer extracorporeal membrane oxygenation durations were associated with mortality. These data may be used to guide providers and to counsel families. However, more data are needed to refine indications and assess associations related to outcomes and decision-making.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-6"},"PeriodicalIF":0.9,"publicationDate":"2025-03-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143669208","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 \"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":"https://doi.org/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":"1-4"},"PeriodicalIF":0.9,"publicationDate":"2025-03-21","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":"The pulmonary valve in tetralogy of Fallot: insights from a necroscopy series.","authors":"Rohit S Loomba, Justin T Tretter, Robert H Anderson, Diane E Spicer","doi":"10.1017/S1047951125001398","DOIUrl":"https://doi.org/10.1017/S1047951125001398","url":null,"abstract":"<p><strong>Background: </strong>Tetralogy of Fallot is the most common cyanotic congenital malformation of the heart. The right ventricular outflow tract is of great interest in this setting, but most of the focus on this feature has been on the size of the so-called pulmonary valvar \"annulus\". We aimed to characterise other aspects of the morphology of the pulmonary root in heart specimens with tetralogy of Fallot.</p><p><strong>Methods: </strong>We reviewed archived hearts with tetralogy of Fallot from four registries. The pulmonary root was examined with specific attention to the number of sinuses, the number of leaflets, presence of any fusion of leaflets, and the direction of the zone of apposition between the leaflets. Cluster analyses were then conducted to see if the features permitted segregation into groups.</p><p><strong>Results: </strong>We examined a total of 155 hearts. The pulmonary valve had two leaflets in 62%, three leaflets in 34%, and one leaflet in 3%. Irrespective of leaflet morphology, most hearts had two sinuses. Cluster analysis permitted segregation into three groups, with the direction of the zone of apposition being the most important feature for segregation.</p><p><strong>Conclusion: </strong>In two-thirds of our hearts with tetralogy of Fallot, the pulmonary valve had two leaflets. Most frequently there were three sinuses. In the setting of a valve with two sinuses, the zone of apposition between the leaflets pointing towards the aorta. Cluster analysis permitted statistically sound segregation of the heart and highlights the importance of delineating these features, specifically the leaflet and sinus morphology, with clinical imaging.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-7"},"PeriodicalIF":0.9,"publicationDate":"2025-03-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143662547","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":"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":"https://doi.org/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":"1-6"},"PeriodicalIF":0.9,"publicationDate":"2025-03-17","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}

{"title":"Patent ductus arteriosus rupture during percutaneous device closure: a case report.","authors":"Hyun Ho Kim, Jihye You","doi":"10.1017/S1047951125001313","DOIUrl":"https://doi.org/10.1017/S1047951125001313","url":null,"abstract":"<p><p>Patent ductus arteriosus is a common condition in preterm neonates, often necessitating medical or surgical intervention. This report presents a case of a preterm neonate born at 27 gestational weeks and who experienced patent ductus arteriosus rupture during a device closure procedure. Patent ductus arteriosus rupturing during device closure is rare and life threatening but can be successfully managed with prompt recognition and intervention.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-3"},"PeriodicalIF":0.9,"publicationDate":"2025-03-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143647369","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":"Pulmonary vasodilator-induced haemodynamic decline and recovery post-discontinuation in two single-ventricle cases.","authors":"Yuichi Ishikawa, Yuki Iwaya, Ayako Kuraoka","doi":"10.1017/S1047951125000538","DOIUrl":"https://doi.org/10.1017/S1047951125000538","url":null,"abstract":"<p><p>Pulmonary vasodilators increased the systemic-to-pulmonary collateral flow in two single-ventricle cases, causing haemodynamic decline. Discontinuation reversed the collateral flow and improved heart failure, highlighting the need for careful monitoring. Serial cardiac MRI, as a non-invasive tool, may be essential to detect changes in collateral flow and guide vasodilator use for optimal management in this population.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-3"},"PeriodicalIF":0.9,"publicationDate":"2025-03-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143584763","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":"Extremely rare association: Desbuquois dysplasia type 1 with coronary-cameral fistula.","authors":"Musa Öztürk, Merve Tanrısever Türk, Pelin Özlem Şimşek Kiper, Hayrettin Hakan Aykan","doi":"10.1017/S1047951125000551","DOIUrl":"https://doi.org/10.1017/S1047951125000551","url":null,"abstract":"<p><strong>Purpose: </strong>Desbuquois dysplasia type 1 is a rare autosomal recessive chondrodysplasia characterised by distinct skeletal abnormalities and multisystem involvement, including pulmonary, renal, and ocular abnormalities, has also been reported. Cardiac complications, although infrequently discussed in the literature, include aortopathy and atrioventricular valve prolapse, potentially due to defective proteoglycan production.</p><p><strong>Case report: </strong>This case report details a 7-year-old male diagnosed with Desbuquois dysplasia type 1 and a coronary-cameral fistula, both of which are exceedingly rare conditions. Genetic analysis revealed a previously reported homozygous pathogenic variant in the calcium-activated nucleotidase 1 gene, ENST00000c.898C>T; p.Arg300Cys. Echocardiographic findings indicated significant cardiac enlargement, mitral valve prolapse, coronary-cameral fistula, pulmonary hypertension, advanced aortic root enlargement and aneurysmatic ascending aorta, and atrial septal defect, necessitating careful clinical management.</p><p><strong>Conclusion: </strong>This case underscores the complexity of Desbuquois dysplasia and its associated cardiac anomalies, highlighting the need for further research into the systemic implications of this disorder. To the best of our knowledge, this case has importance as it is the first of its kind in the literature.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-3"},"PeriodicalIF":0.9,"publicationDate":"2025-02-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143482262","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}