{"title":"Mitochondrial cardiomyopathy: a puzzle for the final diagnosis - CORRIGENDUM.","authors":"Andreia Duarte Constante, Susana Martins Abreu, Conceição Trigo, Susana Lemos Ferreira","doi":"10.1017/S1047951125100498","DOIUrl":"10.1017/S1047951125100498","url":null,"abstract":"","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1967"},"PeriodicalIF":0.7,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145112013","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}
Cardiology in the YoungPub Date : 2025-09-01Epub Date: 2025-09-22DOI: 10.1017/S1047951125100577
James Andrew Bishara, Patrick Evers
{"title":"Pre-existing pulmonary arterial hypertension decompensation associated with e-cigarettes in an adolescent.","authors":"James Andrew Bishara, Patrick Evers","doi":"10.1017/S1047951125100577","DOIUrl":"10.1017/S1047951125100577","url":null,"abstract":"<p><p>An adolescent girl with a long-standing history of pulmonary arterial hypertension experienced severe clinical decompensation after starting to use e-cigarettes. A combination of e-cigarette cessation, atrial septostomy, increasing treprostinil, and initiation of sotatercept led to clinical improvement. Her new baseline was improved over her pre-e-cigarette baseline.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1959-1961"},"PeriodicalIF":0.7,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145111947","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}
Cardiology in the YoungPub Date : 2025-09-01Epub Date: 2025-09-22DOI: 10.1017/S1047951125109244
Karthik Gopinath, Gayathri Bhuvaneswaran Kartha, Ravi Sankar Tulluru, Oommen K George
{"title":"Hypercholesterolemic valvulopathy and severe atherosclerosis in paediatric patients.","authors":"Karthik Gopinath, Gayathri Bhuvaneswaran Kartha, Ravi Sankar Tulluru, Oommen K George","doi":"10.1017/S1047951125109244","DOIUrl":"10.1017/S1047951125109244","url":null,"abstract":"<p><strong>Background: </strong>Atherosclerosis is the leading cause of vascular disease worldwide, and traditionally it has been considered a disease of older individuals. However, this atherosclerotic process begins early in childhood, and when exposed to critically high levels of atherogenic risk factors, coronary artery disease may develop even during childhood. There are very few reports of coronary artery disease in young children, and most are linked to Kawasaki disease and congenital coronary abnormalities. Involvement of the mitral valve due to hypercholesterolaemia is rare and under-reported.</p><p><strong>Methods: </strong>We did a retrospective audit of all children (age <14 years) who underwent coronary angiogram between January 2005 and July 2024 in our tertiary care hospital. Only those children with atherosclerotic coronary artery disease were included.</p><p><strong>Results: </strong>We studied four paediatric cases of atherosclerotic coronary artery disease with concomitant valvular involvement despite ongoing lipid-lowering therapy. We highlight the mechanisms of valvular involvement and the challenges to the diagnosis and treatment of familial hypercholesterolaemia.</p><p><strong>Conclusions: </strong>These cases highlight the cardiovascular changes associated with this \"malignant\" atherosclerosis and emphasise the need for early recognition and prompt initiation of aggressive lipid-lowering therapy at diagnosis.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1917-1922"},"PeriodicalIF":0.7,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145112018","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}
Cardiology in the YoungPub Date : 2025-08-01Epub Date: 2025-08-11DOI: 10.1017/S1047951125101194
Daniela Barisano, Gwen Nance, Michelle Gleason, Paul Chai, Michael P Fundora, Gary Woods, Joshua M Rosenblum, Joshua W Branstetter
{"title":"Safety of direct oral anticoagulants in surgical bioprosthetic heart valves: a paediatric institution's experience.","authors":"Daniela Barisano, Gwen Nance, Michelle Gleason, Paul Chai, Michael P Fundora, Gary Woods, Joshua M Rosenblum, Joshua W Branstetter","doi":"10.1017/S1047951125101194","DOIUrl":"10.1017/S1047951125101194","url":null,"abstract":"<p><strong>Introduction: </strong>Surgical pulmonary valve replacement is commonly required to palliate patients with CHD affecting the right ventricular outflow tract; however, concerns remain about mid- and long-term durability. Post-operative short-term anticoagulation has been hypothesised to improve valve durability.</p><p><strong>Methods: </strong>This is a single-centre, retrospective study of paediatric patients who underwent surgical pulmonary valve replacement and received a direct oral anticoagulant in addition to aspirin post heart valve insertion. The primary objective was a composite safety score consisting of clinically relevant non-major bleeding, major bleeding, bleeding-related readmission, and medication discontinuation.</p><p><strong>Results: </strong>The study analysed 34 patients with a median age 14 years (Interquartile range (IQR): 11, 15) and weight 45 kg (IQR: 35, 55). Ten patients met the composite endpoint (10/34, 29%), with 4 patients experiencing major bleeding (4/34, 12%), 6 experiencing clinically relevant non-major bleeding (6/34, 18%), and 3 patients being readmitted within 90 days of surgical pulmonary valve replacement for bleeding (3/29, 8.8%) resulting in 10 patients discontinuing medication early (10/34, 29%). Lower weight was identified as a significant risk factor for adverse event development (<i>p</i> = 0.04).</p><p><strong>Conclusion: </strong>We observed a higher overall bleeding rate, driven predominately by clinically relevant non-major bleeding events, than other studies using short-term anticoagulation after surgical pulmonary valve replacement. Additional studies should be aimed at evaluating the dosing and safety of direct oral anticoagulants in children in the post-operative period.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1631-1636"},"PeriodicalIF":0.7,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144815800","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}
Cardiology in the YoungPub Date : 2025-08-01Epub Date: 2025-08-20DOI: 10.1017/S1047951125101509
Adeola O Awujoola, Piers C Barker, Michael G W Camitta
{"title":"Recurrence of large cardiac rhabdomyoma and pre-excitation syndrome in an infant after sirolimus therapy.","authors":"Adeola O Awujoola, Piers C Barker, Michael G W Camitta","doi":"10.1017/S1047951125101509","DOIUrl":"10.1017/S1047951125101509","url":null,"abstract":"<p><strong>Conclusions: </strong>This finding underscores the need for risk stratification among patients with cardiac rhabdomyomas to identify those that need more prolonged medical treatment or closer monitoring.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1746-1748"},"PeriodicalIF":0.7,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144943260","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}
Cardiology in the YoungPub Date : 2025-08-01Epub Date: 2025-08-22DOI: 10.1017/S1047951125100991
Satoshi Marutani, Yuhei Konishi, Noboru Inamura
{"title":"Successful percutaneous removal of a huge vegetation in the right atrium of an extremely low-birth-weight infant.","authors":"Satoshi Marutani, Yuhei Konishi, Noboru Inamura","doi":"10.1017/S1047951125100991","DOIUrl":"10.1017/S1047951125100991","url":null,"abstract":"<p><p>The patient, the first of twins, weighed 714 g at birth. On day 14, ultrasound revealed a large methicillin-resistant <i>Staphylococcus aureus</i>-induced atrial vegetation; the tumour grew to 6 mm by day 19. Percutaneous removal was selected due to the risk of pulmonary embolism. We used a three-loop EN Snare system. The procedure was safe and successful.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1711-1712"},"PeriodicalIF":0.7,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144943812","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}
Cardiology in the YoungPub Date : 2025-08-01Epub Date: 2025-08-27DOI: 10.1017/S1047951125101571
Thomas Agostini, Eyal Sagiv, Juanita K Hodax, Andrew R Pistner, Jason F Deen
{"title":"Gender-affirming care in adolescents and young adults with cardiovascular conditions: clinical considerations in a limited evidence landscape.","authors":"Thomas Agostini, Eyal Sagiv, Juanita K Hodax, Andrew R Pistner, Jason F Deen","doi":"10.1017/S1047951125101571","DOIUrl":"10.1017/S1047951125101571","url":null,"abstract":"<p><p>Gender-affirming care is a multi-faceted healthcare provided to transgender and gender diverse individuals to support their physical and mental health. As more adolescents and young adults may be interested in various gender care options, cardiologists should have familiarity with the medical and surgical therapies offered and the risk and considerations these may have, particularly for youth with the history of congenital or acquired cardiovascular disease. The existing literature on exogenous hormone therapy, for the general population and transgender individuals specifically, is by large limited to the adult population, with little relevant information for younger individuals. Current evidence indicates several potential cardiovascular implications to consider with gender-affirming hormone therapy without documented morbidity or clear contraindication for its use. These include the risk of thromboembolism, atherosclerotic and coronary artery disease, hypertension, and dyslipidemia among others. This review summarises existing evidence to assist in navigating the risks and therapeutic options so that offering gender-affirming care can be considered and delivered safely for adolescents and young adults with cardiovascular conditions and makes recommendations for future research.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1531-1539"},"PeriodicalIF":0.7,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144943933","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}
Cardiology in the YoungPub Date : 2025-08-01Epub Date: 2025-08-26DOI: 10.1017/S1047951125101595
Hannah L McMullen, Rhea Birusingh, Camilla Rossi, Amer Heider, Lili Miles, Peter D Wearden, Jennifer S Nelson
{"title":"Histological examination of the pulmonary artery and aorta in an adolescent undergoing the Ross procedure.","authors":"Hannah L McMullen, Rhea Birusingh, Camilla Rossi, Amer Heider, Lili Miles, Peter D Wearden, Jennifer S Nelson","doi":"10.1017/S1047951125101595","DOIUrl":"10.1017/S1047951125101595","url":null,"abstract":"<p><strong>Background: </strong>The Ross procedure offers several advantages for adolescents requiring aortic valve replacement, but progressive pulmonary autograft dilation is a well-described risk. To provide novel insight into the pre-Ross histology in an adolescent with bicuspid aortic valve (BAV)-associated aortopathy, we describe the extracellular architecture of the pulmonary artery (PA) compared to the native ascending aorta.</p><p><strong>Methods: </strong>A 15-year-old with BAV, symptomatic moderate aortic insufficiency and aortic stenosis, and ascending aortic dilation underwent Ross. Intraoperative specimens included the main PA and ascending aorta. Tissue specimens were fixed, stained using 1) haematoxylin and eosin, 2) Verhoeff's van Gieson, and 3) trichrome, and compared using light microscopy.</p><p><strong>Results: </strong>Elastin van Gieson stain revealed that the aortic media in the dilated ascending aorta contained a greater concentration of dense elastin weaves and a regular distribution of collagen compared to the PA. In contrast to the dense and organised compaction of elastic fibres in the media of the aortic specimen, the PA, though grossly normal, demonstrated extensive disruption and fragmentation. Trichrome staining revealed minimal fibrosis in both specimens.</p><p><strong>Conclusions: </strong>Notable pre-Ross histological differences include marked disruption of elastin in the PA compared to the aorta. Age-based differences in Ross outcomes suggest that adolescents may experience proportionally more significant autograft dilation over time, so future studies should include prospective collection and histological analysis of specimens across the age spectrum, both pre- and post-Ross, to allow comparison to age-matched controls.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1719-1722"},"PeriodicalIF":0.7,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144943971","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}
Cardiology in the YoungPub Date : 2025-08-01Epub Date: 2025-08-18DOI: 10.1017/S104795112510142X
Anish R Katta, Shivni Patel, Miza S Hammoud, Amol Pande, Andrew Toth, Samuel M Hoenig, Yibei Zhu, Lin Chin, Rashed Mahboubi, Justin A Robinson, Tara Karamlou
{"title":"Health-related quality of life outcomes for congenitally corrected transposition of the great arteries patients across the therapeutic spectrum.","authors":"Anish R Katta, Shivni Patel, Miza S Hammoud, Amol Pande, Andrew Toth, Samuel M Hoenig, Yibei Zhu, Lin Chin, Rashed Mahboubi, Justin A Robinson, Tara Karamlou","doi":"10.1017/S104795112510142X","DOIUrl":"10.1017/S104795112510142X","url":null,"abstract":"<p><strong>Objective: </strong>Congenitally corrected transposition of the great arteries is a rare congenital cardiac condition with varied presentations, complicating treatment decisions. This study evaluates the impact of medical management, physiologic repair, and anatomic repair on health-related quality of life.</p><p><strong>Methods: </strong>A cross-sectional follow-up was conducted on 50 congenitally corrected transposition of the great arteries patients from a cohort of 240 at Cleveland Clinic (1995-2020). Health-related quality of life was assessed using MacNew and PROMIS-10 questionnaires. Echocardiographic data on systemic atrioventricular valvular regurgitation and systemic ventricular dysfunction were analysed. A time-varying coefficient model evaluated these factors' impact on health-related quality of life.</p><p><strong>Results: </strong>Anatomic repair had significantly higher PROMIS-10 Physical T-scores compared to physiologic repair (median 50.9 vs 41.6, <i>p</i> = 0.04). MacNew Social scores were significantly higher for medical management compared to physiologic repair (median 6.8 vs 6.0, <i>p</i> = 0.02). Echocardiographic analysis revealed that systemic ventricular dysfunction had a stronger immediate impact on health-related quality of life. Systemic atrioventricular valve regurgitation showed a delayed negative effect, significant at 6-10 years after echo, although this effect gradually decreased over time. Anatomic repair patients had better systemic ventricular function (84.6% normal), and less systemic atrioventricular valve regurgitation (69.2% had none) compared to medical management and physiologic repair groups.</p><p><strong>Conclusions: </strong>Anatomic repair improves health-related quality of life in congenitally corrected transposition of the great arteries patients, with physiologic repair showing some gains over time. Health-related quality of life data should provide important guidance regarding treatment decisions, especially in well-balanced congenitally corrected transposition of the great arteries patients.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1595-1601"},"PeriodicalIF":0.7,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144871614","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":"Paediatric cardiology-Hall of Fame : Arkalgud Sampath Kumar.","authors":"Aditya Narsipur Doddamane, Smruti Ranjan Mohanty, Krishna Subramony Iyer","doi":"10.1017/S1047951125101042","DOIUrl":"10.1017/S1047951125101042","url":null,"abstract":"<p><p>The oldest civilisation on the face of the Earth can be traced to India, home to the largest democracy of our times. Fast forward five millenia, a young nation at 78 places the aortic cross clamp most of others on a per diem basis. Somewhere in between, Donald Nixon Ross, who has been interred in this great Hall of Fame [1] opined that \"it is rare to know of surgeons who can perform an operation better and often, seamless than its namesake, and among the few there are, Sampath Kumar is one\". It gives us great pleasure to introduce at the time of his induction in the Paediatric Cardiology Hall of Fame, one of finest humans our profession is privileged to have - Professor Arkalgud Sampath Kumar. Kumar has earned the peerage accorded to few surgeons in our milieu for his lifelong commitment to patientcare, teaching, research and innovation - making him the definition of a quadruple threat in academic surgery. Coming from Professor Anderson, we deem it an honor to present this character-sketch of this gentleman surgeon and mentor, who has the rare distinction of expanding the horizons of paediatric cardiovascular surgical care in the public, private and philanthropic institutions, among others through this encomium.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1696-1709"},"PeriodicalIF":0.7,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144943196","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}