Mohd Rizal Mohd Zain, Saravanan Krishinan, Anis Munirah Mohd Kori
{"title":"Resolution of late-onset bigeminy following percutaneous transcatheter balloon valvuloplasty of the aortic valve stenosis in paediatric.","authors":"Mohd Rizal Mohd Zain, Saravanan Krishinan, Anis Munirah Mohd Kori","doi":"10.1017/S1047951125109876","DOIUrl":"https://doi.org/10.1017/S1047951125109876","url":null,"abstract":"<p><p>A four-month-old infant with severe congenital aortic stenosis underwent successful percutaneous transcatheter balloon valvuloplasty. While initially stable, ventricular bigeminy was detected at six-week follow-up, likely due to post-procedural myocardial inflammation. The arrhythmia resolved spontaneously by 12 weeks. This case highlights the need for vigilant post-percutaneous transcatheter balloon valvuloplasty monitoring to detect and manage late-onset arrhythmic complications.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-3"},"PeriodicalIF":0.7,"publicationDate":"2025-10-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145231542","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}
Adam Dziacky, John Morrison, Jamie Fierstein, Joyce Johnson
{"title":"Trends in procedure choice, cost, and health outcomes in infants undergoing Stage 1 palliation for hypoplastic left heart syndrome: a retrospective analysis of the Paediatric Health Information System.","authors":"Adam Dziacky, John Morrison, Jamie Fierstein, Joyce Johnson","doi":"10.1017/S1047951125109992","DOIUrl":"https://doi.org/10.1017/S1047951125109992","url":null,"abstract":"<p><p>Management of infants with hypoplastic left heart syndrome is resource-intensive. Trends in initial Stage 1 palliation choice and associated hospital cost and outcomes over time are unclear.Using a retrospective cohort of infants <30 days of age (2004-22) from the Paediatric Health Information Systems database, we analysed the annual prevalence of Stage 1 palliation choice, as well as the association between palliation choice and outcomes and resource use. Prevalence of palliation choice was calculated, and Mann-Kendall tests evaluated linear trends. Study outcomes were pooled across years and compared by palliation choice. Associations over time between palliation choice and outcomes and resource use were evaluated with generalised linear mixed models.Of 7701 patients, 67.45% (<i>n</i> = 5194) underwent a Norwood with modified Blalock-Taussig shunt, (NmBT) 22.06% (<i>n</i> = 1699) underwent a Norwood with right ventricle to pulmonary artery conduit (NRVPA), and 10.49% (<i>n</i> = 808) underwent a hybrid procedure. The annual prevalence of NRVPA surpassed that of NBT in 2017. In the pooled analysis, infants undergoing NRV-PA had the lowest in-hospital mortality (11.2%, <i>P</i> < 0.0001) and lowest cost at $335,406 (IQR: $208,624 to $583,322 (<i>P</i> = 0.001). A trend for increased median estimated hospitalisation cost was observed across time for all procedure choices (<i>P</i> for trend <0.0001 for all).These data suggest that the NRV-PA is the preferred palliation choice, has the lowest in-hospital mortality, and is the most cost-effective option. Our findings suggest that all Stage 1 palliation options have become more expensive with no observed change in mortality.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-7"},"PeriodicalIF":0.7,"publicationDate":"2025-10-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145231530","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 case of congenital dilated cardiomyopathy, cholestatic liver injury, and hyperferritinemia with <i>FHL2</i> variant: a case report suggesting possible novel phenotypes.","authors":"Hidenori Yamamoto, Naoki Ohashi, Taichi Kato","doi":"10.1017/S1047951125109682","DOIUrl":"https://doi.org/10.1017/S1047951125109682","url":null,"abstract":"<p><p>A male infant with fetal hydrops was diagnosed with congenital dilated cardiomyopathy and died at 2 months despite intensive treatment. Trio-exome sequencing identified as de novo <i>FHL2</i> variant (p.Arg131Cys). He also presented with non-cardiogenic cholestatic liver injury and hyperferritinemia, which may represent novel <i>FHL2</i>-related phenotypes based on supporting evidence from experimental models.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-3"},"PeriodicalIF":0.7,"publicationDate":"2025-10-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145211845","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":"Challenges in ablating ventricular arrhythmias originating from the left ventricular summit region.","authors":"Musa Öztürk, İlker Ertuğrul, Tevfik Karagöz","doi":"10.1017/S1047951125109864","DOIUrl":"https://doi.org/10.1017/S1047951125109864","url":null,"abstract":"<p><strong>Background: </strong>Radiofrequency catheter ablation of ventricular arrhythmias, originating from the left ventricular summit, is challenging due to epicardial localisation of the substrate, surrounded by coronary arteries. This paper highlights the successful elimination of LVOT summit ventricular arrhythmia which was ablated from the aortic cusp and aortic mitral continuity in two paediatric patients.</p><p><strong>Case report: </strong>Ventricular tachycardia arising from the basal region of the left ventricular summit was identified in two male patients aged 9 and 13 years. Electroanatomic mapping of ventricular arrhythmia revealed the earliest ventricular signal within the left coronary artery which was successfully ablated from the left coronary cusp. The second patient with exactly similar ECG of ventricular arrhythmia was treated by delivering energy to the aorta-mitral continuity beneath the aortic valve. No recurrences were observed during the follow-up period of 20 months.</p><p><strong>Conclusion: </strong>Ventricular tachycardia arising from the basal region of the left ventricular summit is very rarely observed in paediatric patients. Utilising radiofrequency catheter ablation in proximity to the source can effectively and safely eliminate tachycardia.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-4"},"PeriodicalIF":0.7,"publicationDate":"2025-10-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145211806","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}
Laia Brunet-Garcia, Flavio Zuccarino, Juan Manuel Carretero Bellon
{"title":"Unilateral absence of the pulmonary artery in a paediatric cohort.","authors":"Laia Brunet-Garcia, Flavio Zuccarino, Juan Manuel Carretero Bellon","doi":"10.1017/S1047951125109979","DOIUrl":"https://doi.org/10.1017/S1047951125109979","url":null,"abstract":"<p><p>We present six paediatric patients with unilateral absence of the pulmonary artery in pursuance of rising the clinician's recognition. Unilateral absence of the pulmonary artery was misdiagnosed in one patient by echocardiography. One patient had corrective surgery of a complete atrioventricular septal defect, and another had a right lobectomy. The remaining patients did not undergo any intervention and were asymptomatic at the end of the study period. Although there is a lack of consensus regarding optimal surgical approach, we opted for a more conservative strategy with good results.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-3"},"PeriodicalIF":0.7,"publicationDate":"2025-10-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145211772","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}
Daniel O'Meara, Michael Wolf, Matthew Eric Ferguson
{"title":"Milestones in paediatric cardiology: how prostaglandins revolutionised the management of CHD.","authors":"Daniel O'Meara, Michael Wolf, Matthew Eric Ferguson","doi":"10.1017/S104795112510975X","DOIUrl":"https://doi.org/10.1017/S104795112510975X","url":null,"abstract":"","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-6"},"PeriodicalIF":0.7,"publicationDate":"2025-10-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145205630","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":"Postnatal short-term outcomes of pulmonary atresia with ventricular septal defect following prenatal diagnosis: a preliminary Vietnamese report.","authors":"Linh Thuy Dinh, Quang-Vinh Tran","doi":"10.1017/S1047951125109645","DOIUrl":"https://doi.org/10.1017/S1047951125109645","url":null,"abstract":"<p><strong>Introduction: </strong>Pulmonary atresia with ventricular septal defect (PA/VSD) is a complex cyanotic CHD that requires an early diagnosis for optimal management and outcomes. The purpose of this study is to evaluate the effectiveness of an inter-hospital management protocol utilising the prenatal CHD diagnosis for achieving favourable postnatal outcomes in PA/VSD patients in Vietnam.</p><p><strong>Methods: </strong>We described the protocol implemented between two tertiary medical settings in Vietnam for the prenatal diagnosis and postnatal management of PA/VSD infants. All PA/VSD patients with prenatal diagnosis between January 2016 and December 2022 were retrospectively reviewed. The primary outcome was postnatal survival, and the secondary outcome was the presence of major morbidities such as bleeding or the need for Extra Corporeal Membrane Oxygenation (ECMO) after total repair.</p><p><strong>Results: </strong>During the study period, 35 PA/VSD patients were identified including 29 infants who underwent surgical correction utilising a valved conduit and 6 infants who are still waiting for the next evaluation after the palliative surgery. No death prior to the surgery occurred. For 29 patients, one hospital death happened, two patients required ECMO initially in their postoperative course but both survived, one late mortality due to pneumonia, and three reoperations were due to conduit failure. In the mean follow-up time of 2.92 (0.51-7.92) years, all survivors had completed follow-up.</p><p><strong>Conclusion: </strong>Our protocol including a multidisciplinary management and a close follow-up has shown promising short-term results in achieving favourable postnatal outcomes for PA/VSD patients.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-5"},"PeriodicalIF":0.7,"publicationDate":"2025-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145190963","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}
Nicoletta Cantarutti, Maria C Casarini, Fabrizio Drago
{"title":"Atypical clinical features in a young boy with LMNA mutation: expanding the spectrum of laminopathies.","authors":"Nicoletta Cantarutti, Maria C Casarini, Fabrizio Drago","doi":"10.1017/S1047951125109311","DOIUrl":"https://doi.org/10.1017/S1047951125109311","url":null,"abstract":"<p><p>Laminopathies represent a group of inherited disorders, with emerging novel and atypical manifestations. We present the case of a 17-year-old boy with LMNA mutation, showing dilated cardiomyopathy, aortic root dilatation, pontine cavernous angiomas, sensorineural hearing loss, and osteogenic sarcoma. These findings expand the known phenotypic spectrum of laminopathies and highlight the need for multidisciplinary evaluation from a young age.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-2"},"PeriodicalIF":0.7,"publicationDate":"2025-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145190898","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":"Double-orifice mitral valve causing left ventricular outflow tract obstruction: a hitherto unreported entity.","authors":"Ishita Garg, Sreeja Pavithran, Sivakumar Kothandam","doi":"10.1017/S1047951125109591","DOIUrl":"https://doi.org/10.1017/S1047951125109591","url":null,"abstract":"<p><p>We report the first documented case of isolated double-orifice mitral valve causing significant left ventricular outflow tract obstruction in an infant. Echocardiography revealed a complete bridge-type double-orifice mitral valve with aberrant chordal insertion into the interventricular septum, producing severe left ventricular outflow tract obstruction. This rare presentation underscores the importance of meticulous echocardiographic assessment for accurate diagnosis and surgical planning.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-3"},"PeriodicalIF":0.7,"publicationDate":"2025-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145190968","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}
Austin Adair, Maria Elisa Hoyos, Monica Sofia Ponce-Rivera, Stephen Deputy, Chelsey Ortman, Qingqing Wang, Hamidreza Saber, Charles D Fraser, Jeremy T Affolter
{"title":"Cerebral endovascular mechanical thrombectomy in CHD - a literature review and case series.","authors":"Austin Adair, Maria Elisa Hoyos, Monica Sofia Ponce-Rivera, Stephen Deputy, Chelsey Ortman, Qingqing Wang, Hamidreza Saber, Charles D Fraser, Jeremy T Affolter","doi":"10.1017/S1047951125109499","DOIUrl":"https://doi.org/10.1017/S1047951125109499","url":null,"abstract":"<p><strong>Background: </strong>CHD is a major risk factor for acute ischaemic stroke in paediatric patients due to endothelial changes from surgically manipulated vessels, prosthetic material, flow stasis in variable circulations, and hypercoagulability from chronic cyanosis. Stroke recognition in critically or chronically ill patients is challenging, yet rapid identification allows for mechanical thrombectomy to restore cerebral blood flow, particularly in those ineligible for thrombolysis or beyond its therapeutic window. We present a case series highlighting the importance of prompt stroke diagnosis and the role of mechanical thrombectomy in paediatric CHD patients, including children as young as four.</p><p><strong>Methods: </strong>We conducted a single-centre retrospective chart review of paediatric CHD patients who experienced thromboembolic stroke and underwent mechanical thrombectomy from July 2018 to March 2024. Data collected included age, stroke territory, maximum Paediatric NIH Stroke Scale (PedNIHSS) score, pre-thrombectomy neurological deficits, and post-thrombectomy outcomes using thrombolysis in cerebral infarction (TICI) scores.</p><p><strong>Results: </strong>Four CHD patients underwent mechanical thrombectomy for thromboembolic stroke (Table ). They exhibited diverse cardiac anatomies, including two-ventricle and single-ventricle physiology, with a wide age range at presentation.</p><p><strong>Conclusion: </strong>Stroke presentation in CHD patients is variable, necessitating a high index of suspicion. Mechanical thrombectomy is safe and effective in patients as young as four, with no haemorrhagic complications in this series. Further research is needed to develop tailored stroke management guidelines for paediatric CHD patients, particularly younger children and those ineligible for thrombolysis.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-6"},"PeriodicalIF":0.7,"publicationDate":"2025-09-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145184641","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}