Cardiology in the Young最新文献

{"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}

{"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}

{"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}

{"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":"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":"1076-1078"},"PeriodicalIF":0.9,"publicationDate":"2025-05-01","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":"Aortic stiffness assessed by blood pressure and echocardiography in young and normotensive patients with isolated aortic coarctation versus those with aortic coarctation and ventricular septal defect.","authors":"Sophie Duignan, Michaela Pentony, Kevin Patrick Walsh, Colin Joseph McMahon, Pier Paolo Bassareo","doi":"10.1017/S1047951125001362","DOIUrl":"10.1017/S1047951125001362","url":null,"abstract":"<p><strong>Background: </strong>Aortic coarctation can occur isolated or associated with ventricular septal defect. This study evaluated aortic stiffness in normotensive patients surgically treated for aortic coarctation and ventricular septal defect and in those who underwent simple aortic coarctation repair. Both groups were compared with healthy controls. Again, the two pathological groups were compared with each other regarding aortic stiffness and left ventricular diastolic function. A possible relationship between aortic stiffness and left ventricular diastolic function was investigated.</p><p><strong>Methods: </strong>Twenty-two isolated aortic coarctation patients and 17 aortic coarctation and ventricular septal defect patients were enrolled. Aortic root distensibility and aortic stiffness index were calculated from echocardiography and blood pressure. E wave to A wave (E/A) ratio was measured from mitral valve inflow profile.</p><p><strong>Results: </strong>Aortic root distensibility and aortic stiffness index in simple aortic coarctation vs healthy controls: both <i>p</i> < 0.0001. Aortic root distensibility and aortic stiffness index in aortic coarctation/ventricular septal defect vs healthy controls: both <i>p</i> < 0.0001. Aortic root distensibility and aortic stiffness index were similar in the two pathological groups (both <i>p</i> = ns). No statistically significant difference was detected in relation to left ventricular diastolic function (<i>p</i> = ns). No correlation was detected between aortic stiffness and diastolic function in simple aortic coarctation and aortic coarctation/ventricular septal defect groups (both <i>p</i> = ns).</p><p><strong>Conclusions: </strong>In both normotensive isolated aortic coarctation and aortic coarctation/ventricular septal defects subgroups, aortic stiffness is increased in a similar way in comparison with controls. Diastolic function was normal and similar in both groups. Aortic stiffness was not related to left ventricular diastolic function in this specific setting.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"985-989"},"PeriodicalIF":0.9,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143708549","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":"Implementation of a multiphase, multi-institutional QI initiative to optimise sedation practices in cardiac ICU patients.","authors":"Richard U Garcia, Kimberly DiMaria, Jamie Penk, Sherrill Caprarola, Amy Romer, Michael P Fundora, Deborah U Frank, Barbara-Jo Achuff","doi":"10.1017/S104795112500160X","DOIUrl":"10.1017/S104795112500160X","url":null,"abstract":"<p><strong>Introduction: </strong>There is wide variation in institutional sedation strategies in paediatric cardiac ICU. Validated tools such as State Behavioral Scale and Richmond Agitation Sedation Scale were created to help standardise sedation practices.</p><p><strong>Methods: </strong>This is a multi-phase, multicentre, prospective project with the goal of optimising safety and comfort for paediatric cardiac ICU patients. Phase one consisted of an educational intervention with a self-paced, web-based video module on optimal sedation practices using validated sedation screening tools. Participant knowledge was assessed via a de-identified, unmatched pre- and post-test survey. Survey scores were reported as an aggregate average score and compared using a t-test.</p><p><strong>Results: </strong>There were 259 pre-tests, and 142 post-tests collected during the video-assisted educational intervention. There was a significant increase in mean score on the post-test compared to the pre-test for both instruments: from 4 to 4.8/10 for State Behavioral Scale (<i>p</i> = 0.01) and from 4.5 to 4.9 for Richmond Agitation Sedation Scale (<i>p</i> = 0.04). 81% of respondents who completed the Richmond Agitation Sedation Scale post-test and 88.1% of those who completed the State Behavioral Scale post-test said their practice would change based on the new knowledge acquired.</p><p><strong>Conclusion: </strong>We report that our newly developed learning module intervention was effective in increasing short-term knowledge about optimal sedation and sedation scoring. Ongoing phase two efforts include evaluation of long-term compliance of validated sedation screening tools and developing an objective score to measure individual cumulative opioid dosing in the cardiac critical care unit.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"900-902"},"PeriodicalIF":0.9,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143977746","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":"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":"990-995"},"PeriodicalIF":0.9,"publicationDate":"2025-05-01","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":"Global incidence of Kawasaki disease: a systematic review.","authors":"Cho Ryok Kang, Jue Seong Lee, Young June Choe","doi":"10.1017/S104795112500191X","DOIUrl":"10.1017/S104795112500191X","url":null,"abstract":"<p><strong>Background: </strong>Kawasaki disease is a systemic vasculitis that primarily affects young children and represents a major cause of acquired heart disease in children in developed countries. The incidence of Kawasaki disease exhibits significant global variation, and the worldwide burden remains limited.</p><p><strong>Methods: </strong>A systematic review was conducted to investigate the global incidence of Kawasaki disease in children under 5 years of age. A comprehensive literature search was performed in PubMed, Embase, and KoreaMed up to July 15, 2024. Studies reporting population-level Kawasaki disease incidence were included. Data extraction and quality assessment were performed independently by two reviewers.</p><p><strong>Results: </strong>The search yielded 3,197 articles, of which 105 met the inclusion criteria. These studies examined Kawasaki disease incidence in children under 5 years of age across 34 countries, with the majority focusing on the Western Pacific Region and the Region of the Americas. The results demonstrated a wide range of Kawasaki disease incidence globally, with significant geographic variations. The highest incidence rates were observed in Japan, Korea, and Taiwan, with a trend of gradual increase over time.</p><p><strong>Conclusions: </strong>This study represents the most comprehensive review of global Kawasaki disease incidence to date. The substantial variation in incidence underscores the need to understand the factors influencing regional differences.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1028-1039"},"PeriodicalIF":0.9,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143983275","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":"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}

{"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 mm²/m² 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 mm²/m² 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}