Cardiology in the Young最新文献

{"title":"The economic burden of complex CHD in the United States.","authors":"Diane M Pickles, Kirstie Keller","doi":"10.1017/S1047951125109256","DOIUrl":"10.1017/S1047951125109256","url":null,"abstract":"<p><p>In the United States, about 1 in 100 children are born with a CHD, with complex cases requiring intensive, lifelong care. Despite medical severity, little data exist on economic burden, driving low impact scores in federal research funding applications, a lack of specific funding appropriations, and minimal research investment. Here, the financial and economic impact was quantified by identifying direct, indirect, and mortality costs of six complex CHDs and compared to two common cardiovascular diseases: coronary heart disease and congestive heart failure. Despite lower prevalence, complex CHDs exert disproportionate, often invisible financial burdens on families and the healthcare system. Annually, per patient, direct and indirect costs of complex CHDs are four times higher than coronary heart disease and eight times higher than congestive heart failure. The average lifetime direct and indirect cost for complex CHDs is $2.1 million per patient-nearly 44 times that of congestive heart failure. 31% of lifetime costs for complex CHDs are incurred in the first five years of life with families paying an average of $190,000 out-of-pocket. Many caregivers are forced to reduce employment hours, change jobs, or completely withdraw from the labour force. The annual national cost of complex CHDs is $74 billion, and the total lifetime economic impact exceeds $3.35 trillion. Systemic under-recognition has led to chronic underinvestment in research and stagnant patient outcomes. The biomedical community can catalyse an interdisciplinary response from research institutions, policymakers, and clinical systems to alleviate the economic and human toll of complex CHDs.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1751-1758"},"PeriodicalIF":0.7,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144999785","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":"Neonatal transposition of the great arteries associated with abnormal right pulmonary artery.","authors":"Xinrui Deng, Lingyi Wen, Chuan Fu","doi":"10.1017/S1047951125109384","DOIUrl":"10.1017/S1047951125109384","url":null,"abstract":"<p><p>An anomalous origin of the right pulmonary artery is a rare phenomenon in neonates with transposition of the great arteries. We report a case of dextro-transposition of the great arteries with an anomalous right pulmonary artery originating from the innominate artery, diagnosed by cardiac CT angiography and the cinematic rendering technique. Although surgical correction was possible, it was deferred due to complex anatomy and parental preference. This case highlights the diagnostic value and role of advanced imaging in complex congenital heart disease.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1923-1925"},"PeriodicalIF":0.7,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145112021","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 closure of ventricular septal defect with aortic cusp prolapse with or without mild aortic regurgitation: experience from tertiary care referral hospital from Eastern India.","authors":"Mahua Roy, Sucheta Barman, Debasree Gangopadhyay, Somrita Laha, Shantanu Jain, Priyantha Goyal, Joyeeta Dutta","doi":"10.1017/S1047951125100966","DOIUrl":"10.1017/S1047951125100966","url":null,"abstract":"<p><strong>Objective: </strong>To evaluate feasibility, safety, and short-term outcome of transcatheter closure of ventricular septal defect with aortic cusp prolapse with or without mild aortic regurgitation.</p><p><strong>Methods: </strong>All data were collected prospectively for all ventricular septal defect with aortic cusp prolapse with or without mild aortic regurgitation who were attempted for transcatheter device closure between January 2018 and December 2023.</p><p><strong>Results: </strong>The device closure was successful and not associated with appearance of new-onset aortic regurgitation or aggravation of existing trivial to mild aortic regurgitation in 92.6% cases. In 2 patients (2.9%), device appeared to be touching the aortic valve and aggravating aortic regurgitation even after repositioning and re-deploying the device and ultimately that devices were taken out and sent for surgical closure. In 1 patient, device position appeared to be perfect on table before release, aortic regurgitation was same as before, and aortic root angiogram was also satisfactory. But re-evaluation on the next day by echocardiography revealed aggravating aortic regurgitation. The patient was sent for removal of device and surgical ventricular septal defect closure. In 2 patients (2.9%), device was embolised few hours after release, and the patients were sent for surgical closure. All patients were followed up for minimum of 6 months, and no case was found with new-onset aortic regurgitation or aggravation of existing aortic regurgitation.</p><p><strong>Conclusion: </strong>Transcatheter closure of ventricular septal defect with aortic cusp prolapse with or without mild aortic regurgitation in selected patients is technically feasible and safe with high procedural success rate.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1889-1898"},"PeriodicalIF":0.7,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144815801","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":"Echocardiography versus cardiac magnetic resonance imaging in evaluating right ventricle systolic function in paediatric patients with repaired tetralogy of Fallot.","authors":"Hossam Elmontaser, Hala Elmarsafawy, Ahmed Shabana, Rehab Elnagar, Doaa El Amrousy","doi":"10.1017/S1047951125101649","DOIUrl":"10.1017/S1047951125101649","url":null,"abstract":"<p><strong>Background: </strong>Right ventricular dysfunction is a common complication after surgical correction of tetralogy of Fallot. The assessment of right ventricular systolic function using cardiac magnetic resonance is the gold method. Our study aimed to compare the diagnostic accuracy of echocardiographic parameters in identifying right ventricular systolic dysfunction in comparison to cardiac magnetic resonance in children with repaired tetralogy of Fallot.</p><p><strong>Patients and methods: </strong>Fifty-six patients with corrected tetralogy of Fallot were included in the study. Right ventricular fractional area change, Tricuspid annular plane systolic excursion, right ventricular peak systolic tricuspid annular velocity, two-dimensional right ventricular global longitudinal strain, and two-dimensional right ventricular free wall strain were measured using echocardiography. Right ventricular ejection fraction and pulmonary regurgitant fraction were assessed using cardiac magnetic resonance.</p><p><strong>Results: </strong>Twenty (35.7%) patients showed right ventricular systolic dysfunction using cardiac magnetic resonance-derived right ventricular ejection fraction%, while 36 patients had normal right ventricular systolic function. Patients with right ventricular systolic dysfunction had significantly lower right ventricular fractional area change, tricuspid annular plane systolic excursion, right ventricular S', 2D-right ventricular global longitudinal strain, 2D-right ventricular free wall strain compared to those with normal right ventricular systolic function. Right ventricular ejection fraction% was directly correlated to tricuspid annular plane systolic excursion, right ventricular fractional area change, right ventricular S', 2D-right ventricular global longitudinal strain, and 2D-right ventricular free wall strain. Receiver operating characteristic curve revealed that 2D-right ventricular free wall strain and 2D-right ventricular global longitudinal strain had 70% sensitivity, 94.4% specificity, and 85% predictive accuracy, while right ventricular S' and RV fractional area change had 100 % sensitivity in detection of right ventricular dysfunction, but they showed lower specificity, 30.6% and 52.8% respectively.</p><p><strong>Conclusion: </strong>2D-right ventricular global longitudinal strain and 2D-right ventricular FWL have good diagnostic value for right ventricular systolic dysfunction comparable to cardiac magnetic resonance in children with repaired tetralogy of Fallot.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1899-1905"},"PeriodicalIF":0.7,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144871613","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":"<i>LAMP2</i> variants in four Chinese children with Danon disease: clinical and molecular analysis in a monocentric cohort.","authors":"Jie Lu, Yuying Qi, Mei Chen, Chunli Wang, Jie Yin, Shi Wei Yang","doi":"10.1017/S1047951125101546","DOIUrl":"10.1017/S1047951125101546","url":null,"abstract":"<p><strong>Background: </strong>Danon disease is an X-linked disorder caused by variants in the lysosome-associated membrane protein-2 (<i>LAMP2</i>) gene located on Xq24. Due to its inheritance in an X-chromosome dominant manner, males typically experience more severe manifestations than females.</p><p><strong>Method: </strong>The whole exome sequencing was conducted on a cohort of 218 children diagnosed with hypertrophic cardiomyopathy; four children with hypertrophic cardiomyopathy carrying the <i>LAMP2</i> variants were diagnosed at our centre. Variants in the <i>LAMP2</i> gene were summarised, and their pathogenicity and conservation were analysed using bioinformatics methods. A retrospective analysis of genotype-phenotype associations was also conducted in conjunction with previously reported cases.</p><p><strong>Results: </strong>Four patients with Danon disease were diagnosed in our single centre by gene sequencing; they all presented with myocardial hypertrophy as the initial manifestation. Both male patients manifested symptoms from infancy, while disease onset in the two female cases occurred below the average age reported for females. Through gene sequencing, a total of four variants were identified in these four patients, including one splicing variant: c.865-1G>C, one loss of heterozygosity variant: loss1 exon:4-9), one frameshift variant: c.973delG(p.(L325Wfs×21)), and one stop codon variant: c.467T>G(p.(L156*)).</p><p><strong>Conclusion: </strong>This study identified four patients with <i>LAMP2</i> gene variants, thereby enriching the documented genetic landscape of <i>LAMP2</i>-associated disorders. Bioinformatics analyses corroborated the pathogenicity of these variants. Additionally, we emphasised that women with suspected Danon disease should be thoroughly evaluated, and the possibility of implantable cardioverter defibrillator implantation and heart transplantation should be considered and discussed as early as possible.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1854-1862"},"PeriodicalIF":0.7,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144943893","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":"Application of slide tracheoplasty in children with congenital tracheal stenosis.","authors":"Zhangwei Wang, Honghao Fu, Shoujun Li","doi":"10.1017/S1047951125109189","DOIUrl":"10.1017/S1047951125109189","url":null,"abstract":"<p><p>Over the past two decades, there have been encouraging achievements in the surgical treatment of children with congenital tracheal stenosis. Slide tracheoplasty has become the standard surgical procedure for the correction of long-segment congenital tracheal stenosis with abnormal bronchial morphology in many medical centres around the world. Identification of the shape and degree of tracheal stenosis before operation is helpful to develop a better surgical strategy. Flexible application of slide tracheoplasty can effectively correct different types of congenital tracheal stenosis. Cardiopulmonary bypass and intraoperative fiberoptic bronchoscopy are helpful to improve the efficiency of surgery. Postoperative multidisciplinary cooperative management can improve the prognosis of children. Biodegradable scaffolds, tissue-engineered trachea, and 3D printing technology are based on a completely different perspective from traditional medicine. The initial attempts in the biomedical field provide a new idea for the treatment of congenital tracheal stenosis. This article reviews the classification, past and current situation, advantages, surgical indications, surgical techniques, prognosis, related risk factors, and prospects of slide tracheoplasty in the treatment of congenital tracheal stenosis in children.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1773-1781"},"PeriodicalIF":0.7,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144943924","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 Landscape Analysis of Pediatric and Congenital Heart Disease Services in Africa.","authors":"Thomas Aldersley, Sulafa Ali, Adila Dawood, Frank Edwin, Kathy Jenkins, Alexia Joachim, John Lawrenson, Darshan Reddy, Drissi Boumzebra, James D St Louis, Christo Tchervenkov, Amy Verstappen, Bistra Zheleva, Liesl Zühlke","doi":"10.1017/S1047951125100504","DOIUrl":"10.1017/S1047951125100504","url":null,"abstract":"<p><strong>Background: </strong>There is geographic disparity in the provision of Pediatric and Congenital Heart Disease (PCHD) services; Africa accounts for only 1% of global cardiothoracic surgical capacity. Methods: We conducted a survey of PCHD services in Africa, to investigate institution and national-level resources for pediatric cardiology and cardiothoracic surgery. Results were compared with international guidelines for PCHD services and institutions were ranked by a composite score for low- and middle-income PCHD services. Results: There were 124 respondents from 96 institutions in 45 countries. Eighteen (40%) countries provided a full PCHD service including interventional cardiology and cardiopulmonary bypass (CPB) cardiac surgery. Ten countries (22%) provided cardiac surgery services but no interventional cardiology service, 4 of which did not have CPB facilities. One provided interventional cardiology services but no cardiac surgery service. Ten countries (22%) had no PCHD service. There were 0.04 (interquartile range [IQR]: 0.00-0.13) pediatric cardiothoracic surgeons and 0.17 (IQR: 0.02-0.35) pediatric cardiologists per million population. No institution met all criteria for level 5 PCHD national referral centers, and 8/87 (9.2%) met the criteria for level 4 regional referral centers. Thirteen (29%) countries report both pediatric cardiology and cardiothoracic surgery fellowship training programs. Conclusions: Only 18 (40%) countries provided full PCHD services. The number of pediatric cardiologists and cardiothoracic surgeons is below international recommendations. Only Libya and Mauritius have the recommended 2 pediatric cardiologists per million population, and no country meets the recommended 1.25 cardiothoracic surgeons per million. There is a significant shortage of fellowship training programs which must be addressed if PCHD capacity is to be increased.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1782-1791"},"PeriodicalIF":0.7,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144943964","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 between CHD and cerebral abscess: insights from a national database.","authors":"Keats Ewing, Rohit Seath Loomba, Saul Flores, Enrique G Villarreal, Juan S Farias, Matthew Taylor Coghill","doi":"10.1017/S1047951125109293","DOIUrl":"10.1017/S1047951125109293","url":null,"abstract":"<p><p>CHD has been historically associated with the development of cerebral abscess. This retrospective cohort study examines the association of CHD with the occurrence of cerebral abscess in an inpatient paediatric population. We analysed data from the Pediatric Health Information System database, an administrative database that captures data from participating children's hospitals in the United States. We included all patients admitted to participating paediatric ICUs between 2016 and 2021 for a total of 426,029 admissions. Including all admissions, 1,387 (0.3%) patients experienced a cerebral abscess, and of 80,927 (19%) patients with CHD, only 88 (0.1%) experienced a cerebral abscess (odds ratio 0.29, 0.23-0.36). Patients with seizures, cerebral oedema, stroke, shock, surgical intervention, and older age were associated with increased risk of development of a cerebral abscess. Interestingly, the development of a cerebral abscess was not associated with an increased risk of mortality (<i>p</i> = 0.937). When compared to patients without CHD admitted to the paediatric ICU, those with CHD appear less likely to develop a cerebral abscess. This study reiterates that cerebral abscess is a rare occurrence and does not demonstrate any significant association with CHD in a contemporary, inpatient population in the United States.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1913-1916"},"PeriodicalIF":0.7,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145111949","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":"Letter to the Editor regarding \"Experience with etilefrine in refractory non-traumatic chylothorax: a case report\".","authors":"Ryuzo Nakayoshi, Reiko Tsukuura","doi":"10.1017/S1047951125109426","DOIUrl":"10.1017/S1047951125109426","url":null,"abstract":"","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1965-1966"},"PeriodicalIF":0.7,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145111966","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 valve-in-valve implantation using a Pulsta valve in repaired Tetralogy of Fallot.","authors":"Phuc Nang Vu, Long Pham Hoang Nguyen, Gi Beom Kim","doi":"10.1017/S1047951125109402","DOIUrl":"10.1017/S1047951125109402","url":null,"abstract":"<p><p>We report the first case in Vietnam of transcatheter pulmonary valve-in-valve implantation using the self-expanding Pulsta valve in a young adult with repaired Tetralogy of Fallot. This case demonstrates the feasibility, safety, and early effectiveness of self-expanding valves in complex right ventricular outflow tract anatomies and highlights the importance of multimodal imaging in procedural planning.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1962-1964"},"PeriodicalIF":0.7,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145190916","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}