Cardiology in the Young最新文献

{"title":"A fatal triad: a case of obstructed total anomalous pulmonary venous return, single ventricle physiology, and single lung with tracheal rings.","authors":"Megan M McKittrick, Stephanie-Grace A Raymundo, Laura A Maitoza","doi":"10.1017/S1047951125001477","DOIUrl":"https://doi.org/10.1017/S1047951125001477","url":null,"abstract":"<p><p>A neonate with unbalanced right-dominant atrioventricular canal defect with intact atrial septum and abnormal pulmonary venous return developed bradycardic arrest and a subsequent diagnosis of tracheal rings and left lung aplasia. This report details the fatal nature of the triad of hypoplastic left heart, pulmonary venous obstruction, and airway anomalies, along with the role of advanced imaging in prognostication.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-3"},"PeriodicalIF":0.9,"publicationDate":"2025-05-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144118843","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":"Isolated foetal left ventricular apical hypoplasia: would you know how to spot it?","authors":"Beatriz Picazo-Angelin, Gonzalo Cortazar-Rocandio, Almudena Ortiz-Garrido","doi":"10.1017/S1047951125001908","DOIUrl":"https://doi.org/10.1017/S1047951125001908","url":null,"abstract":"<p><p>Isolated left ventricular apical hypoplasia is a rare cardiac malformation. This entity is easily diagnosed if its key features are recognised, but will puzzle the clinician who is not aware of its existence. It is characterised by echocardiographic findings of a spherical left ventricle, an elongated right ventricle wrapping around the deficient left ventricular apex, and abnormal papillary muscles' origin.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-3"},"PeriodicalIF":0.9,"publicationDate":"2025-05-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144118844","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":"Current status and improvement opportunities for onboarding paediatric acute care cardiology advanced practice providers.","authors":"Lyndsey Hunter-Adamson, Alexandra Birely, Ashley E Neal, Sonali S Patel, Alaina K Kipps","doi":"10.1017/S1047951125001866","DOIUrl":"https://doi.org/10.1017/S1047951125001866","url":null,"abstract":"<p><strong>Background: </strong>Specialised training opportunities in paediatric cardiology are rare for advanced practice providers, creating an educational gap for novice practitioners. Standardised curricula have been cited as a beneficial intervention to optimally prepare these providers for highly specialised fields. We sought to understand the current onboarding practices of advanced practice providers in paediatric acute care cardiology to identify opportunities for curricular improvement.</p><p><strong>Materials and methods: </strong>A survey developed by a task force by the Pediatric Acute Care Cardiology Collaborative (PAC³) was distributed across PAC³ programmes in May 2023 to evaluate the onboarding practices of advanced practice providers at paediatric heart centres nationwide.</p><p><strong>Results: </strong>Survey responses reflected orienting practices at 19 paediatric heart centres representing varying programme and team sizes. Of the respondents, 32% felt their current model does not meet all the needs of the new team member. Key successful onboarding elements included a structured curriculum with goals and objectives, dedicated education time and materials, standardised assessments, and individualised learning in the presence of a supportive team. All respondents agreed that an online curriculum would be beneficial.</p><p><strong>Conclusions: </strong>There is no national standardised educational pathway for advanced practice providers entering paediatric acute care cardiology practice. There are opportunities to develop a formalised curriculum with structured learner assessment at a national level, which could be modified at the institution or learner level to enhance current onboarding practices.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-6"},"PeriodicalIF":0.9,"publicationDate":"2025-05-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144109759","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":"Parental and adolescent perceptions of clinical trial participation: the FUEL randomised controlled trial and open-label extension study.","authors":"Linda M Lambert, Felicia Trachtenberg, Russell Gongwer, Thomas Giorgio, Olukayode Garuba, Sandra Mihelic, Kathleen Rathge, Martha Rolland, Cynthia Smith, Mary Stumpf, Stacy Woyciechowski, Frances Woodard, Jennifer S Yauck, Victoria L Pemberton","doi":"10.1017/S1047951125001702","DOIUrl":"https://doi.org/10.1017/S1047951125001702","url":null,"abstract":"<p><strong>Introduction: </strong>Adolescent and parental perceptions of the Fontan Udenafil Exercise Longitudinal Assessment Randomised Controlled Trial (FUEL) and its open-label extension were examined, to identify factors affecting future research participation.</p><p><strong>Methods: </strong>A validated survey was administered at two time points to adolescents (12-19 years) and their parents to assess likes/dislikes of study participation, research team, study burden and benefits. A 5-point Likert scale (strongly disagree [-2] to strongly agree [ + 2]) was used, and scores were averaged. Regression models explored potential predictors. Open-ended questions queried the most/least appealing aspects of participation and considerations for future research.</p><p><strong>Results: </strong>Among 250 FUEL participants at 14 centres, 179 adolescent and 183 parent surveys were completed at T1 (6 months after randomisation). Perceptions of research participation were generally positive: 1.35 ± 0.45 for adolescents; 1.56 ± 0.38 (<i>p</i> < 0.001) for parents. There were no significant differences between females vs. males. Themes from open-ended responses included liking to help others and themselves, liking the study team, and disliking study burden. Adolescents liked the compensation and disliked study-related testing. At T2 (end of open-label extension study), 121 adolescents and 114 parents responded. Perception scores remained high at 1.39 ± 0.51 for adolescents and 1.58 ± 0.37 for parents (<i>p</i> = 0.001). There were no significant gender differences in perceptions between adolescents, but mothers had slightly better perceptions than fathers (<i>p</i> = 0.004).</p><p><strong>Conclusions: </strong>Perceptions of research were positive and slightly better for parents. Study teams and compensation were key contributors to positive perceptions. Study burden and testing were viewed less favourably. Future studies should consider families' preferences and potential barriers to participation.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-7"},"PeriodicalIF":0.9,"publicationDate":"2025-05-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144109762","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":"Severe hyperlactatemia from Warburg effect due to post-transplant lymphoproliferative disorder after paediatric heart transplantation with resolution following thiamine administration.","authors":"Michael J Martinez, Hannah St Francis, Gloria Contreras Yametti, Rana Biary, Rakesh K Singh","doi":"10.1017/S1047951125001830","DOIUrl":"https://doi.org/10.1017/S1047951125001830","url":null,"abstract":"<p><p>Hyperlactatemia is a common and concerning finding in the paediatric cardiac ICU as it may signify tissue hypoperfusion and/or hypoxia. However, it is important to include other aetiologies for an elevated lactate in the differential, especially when the lactate is significantly elevated (> 8 mmol/L). We present the case of metabolic acidosis with severe hyperlactatemia secondary to Warburg effect and presumed thiamine deficiency in a paediatric heart transplant patient with post-transplant lymphoproliferative disorder.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-3"},"PeriodicalIF":0.9,"publicationDate":"2025-05-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144109768","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":"Cardiac point-of-care ultrasound in the paediatric cardiac ICU: assessment of imaging quality and interpretation.","authors":"Hunter C Wilson, Kevin Maher, Ritu Sachdeva, Marcos Mills","doi":"10.1017/S104795112500157X","DOIUrl":"https://doi.org/10.1017/S104795112500157X","url":null,"abstract":"<p><p>We evaluated quality characteristics of cardiac point-of-care ultrasound performed in our paediatric cardiac ICU. Of 47 included patients, diagnostic imaging was feasible in a high percentage of patients with good image quality. Intensivist interpretations of images were highly specific for dichotomised abnormal findings when compared to interpretation from a cardiologist with expertise in imaging but challenges in accurately assessing depressed ventricular function and the risk of discrepant interpretations exist even amongst frequent users with extensive training and experience. These findings may serve as a template for continued development of cardiac point-of-care ultrasound in a high-impact clinical environment.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-3"},"PeriodicalIF":0.9,"publicationDate":"2025-05-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144092787","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":"Subvalvular membranous aortic stenosis in Swedish children, a retrospective cohort study between 1994 and 2019.","authors":"Maria Sandstedt, Mikael Sandstedt, Janus Gudnason, Mats Börjesson, Frida Dangardt, Jan Sunnegårdh","doi":"10.1017/S1047951125001854","DOIUrl":"https://doi.org/10.1017/S1047951125001854","url":null,"abstract":"<p><strong>Background and aim: </strong>Membranous subaortic stenosis is a CHD with high recurrence-rate despite surgical treatment. This study investigated the outcome of operated patients and possible predictors for recurrence.</p><p><strong>Methods: </strong>Retrospective review of all patients (<i>n</i> = 38) ≤ 18 years of age operated for membranous subaortic stenosis between 1994-2019 at Sahlgrenska University Hospital. The primary outcomes were recurrence, reintervention, and mortality. Predictors of recurrence and reintervention were secondary outcomes.</p><p><strong>Results: </strong>Median age (range) at diagnosis, initial intervention, and last follow-up were 2.3 (0.003-17.2), 5.3 (0.03-17.5) and 17.5 (3.6-20.4) years, respectively. Median follow-up time was 9.9 (0.01-19.5) years. 61% were males, and 53% had other associated CHD. 19 patients (56%) developed recurrence and 7 (21%) underwent reintervention. One patient died peri-operatively. Age <u><</u>5 years at first intervention increased the likelihood of reintervention. Postoperative peak/mean gradients were higher in patients with disease recurrence.The median echocardiographic peak-/mean gradients at initial diagnosis, pre-, postoperative, and at last follow-up were 61/36, 83/50, 16/8, and 19/17 mmHg respectively (<i>p</i> < 0.0001 pre/post). Pre-/postoperative peak gradients were linearly correlated, decreasing by 80% pre-/postoperatively (<i>p</i> < 0.01). Presence of symptoms and the preoperative peak gradient were positively associated (<i>p</i> < 0.001) with a peak gradient threshold value of > 90 mmHg. The distance between the subaortic stenosis membrane and the aortic valve was inversely correlated to the preoperative peak-gradient (<i>p</i> < 0.01).</p><p><strong>Conclusions: </strong>Reintervention following surgical intervention of membranous subaortic stenosis is common. A positive correlation exists between high pre- and postoperative peak-gradient. A low postoperative peak gradient may be important in avoiding recurrence.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-11"},"PeriodicalIF":0.9,"publicationDate":"2025-05-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144092897","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":"Ductal stenting in a limited-resource set up: a single naive centre experience in Eastern India of 88 patients.","authors":"Nurul Islam, Siddhartha Saha, Soumyadeep Biswas, Chandrasekhar Dey, Krishnendu Khan","doi":"10.1017/S104795112500174X","DOIUrl":"https://doi.org/10.1017/S104795112500174X","url":null,"abstract":"<p><strong>Objectives: </strong>To enumerate the experience in different parameters, including demographic, anatomic, procedural, ICU care, and outcome of ductal stenting procedures in duct-dependent pulmonary circulation in a resource-limited setup.</p><p><strong>Background: </strong>Stenting the arterial duct emerged as an alternative to a variety of surgical interventions in the early 1990s. Ductal stenting in neonates with duct-dependent pulmonary circulation may maintain duct patency reliably for several months as an alternative to systemic-to-pulmonary shunts.</p><p><strong>Methods: </strong>This is a retrospective review from a single centre with a developing paediatric cardiology unit and limited paediatric cardiac surgical scope. Eighty-nine symptomatic patients who needed stable ductal flow for adequate pulmonary circulation underwent the procedure from October 2018 to December 2022.</p><p><strong>Results: </strong>We had a procedural success rate of 98.8% (one case failed out of 89) and a successful discharge rate of 96.6% (85 discharged out of 88; 3 immediate mortalities). At the six-month follow-up, the success rate was 93% (6 mortalities in 88 patients). No patient underwent a rescue surgical shunt, but an elective Blalock-Taussig shunt was needed in 1 patient (1.1%). In the remaining 82 cases, 65 are now in follow-up, among them 22 already underwent single-ventricle palliation, and 8 underwent biventricular repair successfully.</p><p><strong>Conclusions: </strong>Ductal stenting is less invasive well-accepted alternative for first-stage palliation. Our focus in this study is to practice this even in resource-limited settings as an alternative to surgical shunts with good short- and mid-term outcomes.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-7"},"PeriodicalIF":0.9,"publicationDate":"2025-05-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144092884","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":"Maternal exposure factors in pregnancy that affect fetal CHD risk: a case-control study.","authors":"Jieru Lu, Zhuoyan Li, Yu Wang, Jing Yang, Shujing Li, Xuehua Ruan, Jinping Cheng, Sun Chen, Yurong Wu, Xin Shi, Kun Sun, Jing Sun","doi":"10.1017/S104795112500188X","DOIUrl":"https://doi.org/10.1017/S104795112500188X","url":null,"abstract":"<p><strong>Aim: </strong>We sought to assess the degree to which environmental risk factors affect CHD prevalence using a case-control study.</p><p><strong>Methods: </strong>A hospital-based study was conducted by collecting data from outpatients between January 2016 and January 2021, which included 31 CHD cases and 72 controls from eastern China. Risk ratios were estimated using univariate and multivariate logistic regression models and mediating effect analysis.</p><p><strong>Results: </strong>Residential characteristics (usage of cement flooring, odds ratio = 17.04[1.954-148.574], <i>P</i> = 0.01; musty smell, odds ratio = 3.105[1.198-8.051], <i>P</i> = 0.02) and indoor total volatile organic compound levels of participants' room (odds ratio = 31.846[8.187-123.872, <i>P</i> < 0.001), benzene level (odds ratio = 7.370[2.289-23.726], <i>P</i> = 0.001) increased the risk of CHDs in offspring. And folic acid plays a masking effect, which mitigates the affection of the total volatile organic compound (indirect effect = -0.072[-0.138,-0.033]) and formaldehyde (indirect effect = -0.109[-0.381,-0.006]) levels on the incidence of CHDs. While food intake including milk (odds ratio = 0.396[0.16-0.977], <i>P</i> = 0.044), sea fish (odds ratio = 0.273[0.086-0.867], <i>P</i> = 0.028), and wheat (odds ratio = 0.390[0.154-0.990], <i>P</i> = 0.048) were all protective factors for the occurrence of CHDs. Factors including women reproductive history (history of conception control, odds ratio = 2.648[1.062-6.603], <i>P</i> = 0.037; history of threatened abortion, odds ratio = 2.632[1.005-6.894], <i>P</i> = 0.049; history of dysmenorrhoea (odds ratio = 2.720[1.075-6.878], <i>P</i> = 0.035); sleep status (napping habit during daytime, odds ratio = 0.856[0.355-2.063], <i>P</i> = 0.047; poor sleep quality, odds ratio = 3.180[1.037-9.754], <i>P</i> = 0.043); and work status (working time > 40h weekly, odds ratio = 2.882[1.172-7.086], <i>P</i> = 0.021) also influenced the CHDs incidence to differing degrees.</p><p><strong>Conclusion: </strong>Diet habits, nutrients intake, psychological status of pregnant women, and residential air quality were associated with fetal CHDs. Indoor total volatile organic compound content was significantly correlated with CHDs risk, and folic acid may serve as a masking factor that reduce the harmful effects of air pollutants.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-10"},"PeriodicalIF":0.9,"publicationDate":"2025-05-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144092894","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":"Endoscopic visualisation in the surgical repair of an aorto-left ventricular tunnel in a neonate.","authors":"Fumiya Yoneyama, Javier Brenes","doi":"10.1017/S1047951125001982","DOIUrl":"https://doi.org/10.1017/S1047951125001982","url":null,"abstract":"<p><p>Aorto-left ventricular tunnel is a rare congenital cardiac anomaly characterised by an extracardiac channel connecting the ascending aorta directly to the left ventricle. Surgical repair in neonates poses significant challenges due to the diminutive size and close proximity of critical structures such as the aortic valve and coronary ostia. We report the case of a neonate with an aorto-left ventricular tunnel, mild left ventricular dysfunction, and mild aortic insufficiency who underwent successful surgical repair on the sixth day of life. Following cardiopulmonary bypass initiation and cardioplegic arrest, the ascending aorta was transected for direct visualisation. A 5-mm 0° rigid endoscope was employed intraoperatively, providing high-resolution magnification of the aortic valve, right coronary artery, and tunnel anatomy. The tunnel was closed using an autologous pericardial patch, ensuring preservation of the coronary ostium and aortic valve integrity. Postoperative echocardiography demonstrated good left ventricular function, trivial aortic insufficiency, and no residual shunt. The use of rigid endoscopy significantly enhanced the safety and precision of the repair by allowing accurate anatomical delineation and facilitating patch placement. This case highlights the importance of detailed visualisation in neonatal aorto-left ventricular tunnel repair and underscores the utility of rigid endoscopic technology in complex congenital heart surgery. Endoscopic assistance not only aids intraoperative decision-making but also serves as a valuable tool for surgical education and postoperative review.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-2"},"PeriodicalIF":0.9,"publicationDate":"2025-05-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144092886","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}