Cardiology in the Young最新文献

{"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}

{"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":"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":"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":"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":"Association of interleukin-35 polymorphisms with Kawasaki disease in Chinese children.","authors":"Jinhui Wu, Siqi Feng, Ya Su, Qijian Yi","doi":"10.1017/S1047951125001829","DOIUrl":"10.1017/S1047951125001829","url":null,"abstract":"<p><p>Kawasaki disease is a leading cause of acquired heart disease in children in the developed world, characterised by acute systemic vasculitis, with a complex aetiology that remains poorly understood. Recent studies have highlighted the potential anti-inflammatory effects of Interleukin-35 in various proinflammatory and cardiovascular conditions. However, the relationship between Interleukin-35 gene polymorphisms and Kawasaki disease susceptibility, particularly in Chinese children, has not been well-explored.In this study, we investigated the association between five Interleukin-35 single-nucleotide polymorphisms-rs2243115, rs2243123, rs583911, rs353698, and rs2302164-and Kawasaki disease in a cohort of Chinese children. A total of ninety-four Kawasaki disease patients and one hundred healthy controls were enrolled, with the Kawasaki disease patients further divided into subgroups based on the presence or absence of coronary artery lesions and incomplete or complete Kawasaki disease. Genotyping of Interleukin-35 polymorphisms was performed using the MassARRAY system.The results showed the GT + GG genotypes and G allele of rs2243115 (T > G) were significantly more prevalent in Kawasaki disease patients with coronary artery lesions than in those without coronary artery lesions, suggesting a possible association with the development of coronary artery lesions. Additionally, the G allele of rs353698 (A > G) was more frequently observed in the incomplete Kawasaki disease group compared to the complete Kawasaki disease group, suggesting a possible association with the risk of incomplete Kawasaki disease.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1016-1022"},"PeriodicalIF":0.9,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143983171","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}