Qiu Y, Liu F, Duan H
… +3 more, Zhou K, Hua Y, Li Y
Cardiol Young
· 2026 Jun · PMID 42318680
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INTRODUCTION: Atrial standstill represents a rare cardiac arrhythmia characterised by the complete absence of atrial electrical and mechanical activity. Early diagnostic recognition coupled with comprehensive genetic cou...INTRODUCTION: Atrial standstill represents a rare cardiac arrhythmia characterised by the complete absence of atrial electrical and mechanical activity. Early diagnostic recognition coupled with comprehensive genetic counselling assumes paramount importance. Herein, we report a rare case of a patient who presented with bradycardia at an early age and demonstrated aggressive atrial standstill during a pacemaker upgrade, which challenged the therapeutic strategy. CASE PRESENTATION: A 5-year-old girl was initially diagnosed with bradycardia. Holter monitoring confirmed severe bradycardia with prolonged sinus arrest episodes. Subsequently, the patient underwent single-chamber pacemaker implantation. During a planned dual-chamber upgrade at age 11, despite systematic exploration of multiple anatomical sites within the right atrium, adequate atrial capture could not be achieved. Intracardiac electrophysiological assessment demonstrated a complete absence of electrical activity in the bi-atrium. While speckle-tracking echocardiography revealed mildly reduced global longitudinal strain with impairment noted in the lateral myocardial segments, indicating potential injuries from ventricular demand pacing. Genetic test identified a compound heterozygous variant of c.2431C>T and c.2893C>T. The protein structure of SCN5A has been built and named AF-P21333-F1, and the molecular function of the variant site has been annotated. Additionally, murine scRNA-seq data (GSE132658) revealed cardiac expression is confined to the conductive bundles and fibres rather than cardiomyocytes, and the loss-of-function caused aggressive atrial standstill. CONCLUSION: This case provides valuable insights into genotype-phenotype correlations in -associated atrial standstill, emphasises the importance of comprehensive electrophysiological assessment during device implantation, and underscores considerations for physiological pacing strategies in paediatric patients requiring lifelong device dependency.
Cesna S, Bielinis A, Zvirblis T
… +2 more, Miglinas M, Tarutis V
Cardiol Young
· 2026 Jun · PMID 42318671
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OBJECTIVES: Despite the effectiveness of the surgical or percutaneous treatment of coarctation of the aorta, the presence of hypertension in these patients remains a common and concerning issue. This study was undertaken...OBJECTIVES: Despite the effectiveness of the surgical or percutaneous treatment of coarctation of the aorta, the presence of hypertension in these patients remains a common and concerning issue. This study was undertaken to explore the relation between the severity of coarctation of the aorta and renal perfusion and to evaluate the role of renal scintigraphy as a predictive tool for clinical outcomes after percutaneous treatment of coarctation of the aorta. METHODS: Adult patients with coarctation of the aorta and concomitant arterial hypertension were enrolled in the study. Ambulatory 24-hour blood pressure monitoring, CT angiography, and a renal perfusion scan were performed, along with invasive pressure gradient measurements. Patients with invasive peak gradients ≥20mmHg and/or a difference of ≥50% in diameter between the isthmus and the descending aorta at the diaphragmatic level were selected for percutaneous stent implantation. RESULTS: The mean time to peak change in renal scintigraphy after captopril did not significantly differ between the Medical group (-1.01 ± 2.526 min. for the left kidney and -0.19 ± 1.46 min. for the right kidney) and the Stent group (-0.59 ± 1.188 min. for the left kidney and -0.17 ± 1.461 min. for the right kidney) (-values > 0.05). No statistically significant changes in renal perfusion were observed between pre-and post-stent implantation measurements. CONCLUSION: In this pilot study, no clear association was found between the degree of aortic narrowing and renal perfusion, and no measurable reduction in renal perfusion was detected. Stent implantation did not result in significant short-term changes in blood pressure. These findings are exploratory and require confirmation in larger studies.
Cardiol Young
· 2026 Jun · PMID 42318670
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A child with a pathogenic MYBPC3 gene mutation progressed from normal to severe obstructive hypertrophic cardiomyopathy with dynamic outflow obstruction from septal hypertrophy and abnormal papillary muscle insertion, hi...A child with a pathogenic MYBPC3 gene mutation progressed from normal to severe obstructive hypertrophic cardiomyopathy with dynamic outflow obstruction from septal hypertrophy and abnormal papillary muscle insertion, highlighting careful subvalvular assessment for surgical planning.
Elhedai H, Shebani S, Kalantre A
… +3 more, Skinner G, Fraisse A, Kantzis M
Cardiol Young
· 2026 Jun · PMID 42318665
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INTRODUCTION: The superior sinus venosus atrial septal defect is situated immediately inferior to the junction of the superior caval vein (SCV) and the right atrium. It is usually associated with partial anomalous pulmon...INTRODUCTION: The superior sinus venosus atrial septal defect is situated immediately inferior to the junction of the superior caval vein (SCV) and the right atrium. It is usually associated with partial anomalous pulmonary venous drainage. Surgical correction used to be the only treatment option, but the transcatheter approach using a covered stent has recently evolved substantially. PATIENTS AND METHODS: This is a single-centre retrospective analysis of all patients who underwent transcatheter correction of sinus venosus atrial septal defect in our institution between January 2024 and January 2026. Data was collected from the Adult CHD multidisciplinary meeting records and patients' medical records. The hospital records, including case notes and other electronic data, were reviewed. RESULTS: Five patients were treated with transcatheter correction of sinus venosus atrial septal defect and partial anomalous pulmonary venous drainage. All patients were adults with an age range of 30-61 years. Three were males. One patient received a 100-mm Optimus-CVS polytetrafluoroethylene (PTFE)-Covered Stent XXL, whereas all remaining 4 patients received an 80-mm stent. One patient received a second stent for a residual shunt. There were no mortalities or immediate adverse events. The follow-up period at the time of manuscript submission so far is 3-24 months, and no late complications have been observed. CONCLUSIONS: Transcatheter closure of sinus venosus atrial septal defect is a feasible and safe procedure in selected patients with good results. The learning curve is still to be completed, and surgical options are not to be completely abolished.
Pahsini K, Marinschek S, Beckenbach E
… +7 more, Reininghaus E, Dalkner N, Russell M, Russell AN, Stoesslein S, Scheer P, Dunitz-Scheer M
Cardiol Young
· 2026 Jun · PMID 42312590
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BACKGROUND: Feeding difficulties and prolonged reliance on enteral nutrition are common in children with CHD, often leading to tube dependency. Despite its clinical relevance, evidence-based recommendations for tube wean...BACKGROUND: Feeding difficulties and prolonged reliance on enteral nutrition are common in children with CHD, often leading to tube dependency. Despite its clinical relevance, evidence-based recommendations for tube weaning remain limited. METHODS: This study prospectively evaluated outcomes of the "Graz Model of Tube Weaning" in 70 tube-dependent children with CHD (median age 1.67 years) treated between 2016 and 2024. Children participated either in a two-week on-site programme ("Eating School") or a telemedical home-based programme ("Netcoaching"). Outcomes included weaning success, changes in growth parameters (weight-for-age -score, height-for-age -score, standards-z-values for body mass index), treatment duration, feeding-related adverse effects, and caregiver burden. RESULTS: A total of 98.6% of children ( = 69) transitioned to full oral feeding. Both programmes were effective. Younger children more often enrolled in Netcoaching and required longer treatment durations (median 2.23 vs. 1.48 months, < 0.05). Adverse effects from tube feeding were prevalent in 97.1% of cases, with oral aversion and vomiting being most common. Temporary but significant decreases in growth parameters were observed in both groups; infants under one year showed greater height reductions in the Eating School cohort. Caregiver burden remained substantial, particularly among mothers. Average treatment duration until tube feeding cessation was 29 days. CONCLUSIONS: Both treatments offer safe, effective methods to help children with CHD transition from enteral to oral feeding. Early intervention with an interdisciplinary, hunger-based approach emphasising autonomy appears advantageous. Further research is needed on long-term growth, nutritional development, motor skills, social interactions, and speech after tube weaning.
Cardiol Young
· 2026 Jun · PMID 42312579
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Infradiaphragmatic total anomalous pulmonary venous return is a rare CHD often requiring emergent cardiac surgery. Options are limited for premature infants. We describe successful transcatheter palliation of a premature...Infradiaphragmatic total anomalous pulmonary venous return is a rare CHD often requiring emergent cardiac surgery. Options are limited for premature infants. We describe successful transcatheter palliation of a premature infant utilising stenting of the ductus venosus followed by transhepatic portal venous access for stent placement in an obstructed vertical vein with hepatic tract closure using the VASCADE Vascular Closure System (Cardiva Medical Inc., Santa Barbara, CA).
Lim HM, Blume B, Dubin A
… +7 more, Rossano J, Kim JJ, Penny D, Chen S, Lee KJ, Balasubramanian S, Pasquali S
Cardiol Young
· 2026 Jun · PMID 42312456
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INTRODUCTION: Developing the next generation of leaders in congenital cardiology is critical to advancing the field. Increasingly, it is recognised that earlier opportunities to establish professional networks, gain uniq...INTRODUCTION: Developing the next generation of leaders in congenital cardiology is critical to advancing the field. Increasingly, it is recognised that earlier opportunities to establish professional networks, gain unique skills, and disseminate scientific work can be highly beneficial. While cross-institutional collaboration has been successful in supporting quality improvement, it has not been widely used in other areas. We describe our initial experience with a multi-centre congenital heart early-career exchange programme. METHODS: Faculty (<10 years from training) among five participating congenital heart centres were eligible to apply. Applications were reviewed and matched with host sites. Visits (typically 3-5 days) included topics spanning research and collaboration, unique clinical programmes/skills, networking, and presenting a formal lecture. All participants were invited to complete an anonymous post-visit survey, with descriptive results reported. RESULTS: Overall, 20/28 (71%) applicants over the initial two years of the programme were selected for participation. The majority were assistant professors; 75% were <5 years from training. There was an equal distribution of males and females and a diverse range of clinical specialties/areas of interest. Post-visit survey responses (75% response rate) were highly favourable, with 92% indicating "strongly agree" regarding the value of the programme across domains and highlighting valuable exposure to unique clinical and research opportunities, networking, and mentorship. CONCLUSIONS: Initial results from the congenital heart early-career exchange programme highlight the feasibility and positive impact of a collaborative approach to career development. Further efforts should be prioritised to foster broader engagement and impact.
Nikolaou A, Kostoulas C, Hatzidaki E
… +7 more, Apostolou P, Papasotiriou I, Kolios NG, Serbis A, Barkas F, Vlahos A, Milionis H
Cardiol Young
· 2026 Jun · PMID 42298842
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Familial hypercholesterolaemia remains substantially underdiagnosed worldwide despite effective preventive therapies. Evidence increasingly supports paediatric-centred detection combined with cascade and reverse cascade...Familial hypercholesterolaemia remains substantially underdiagnosed worldwide despite effective preventive therapies. Evidence increasingly supports paediatric-centred detection combined with cascade and reverse cascade strategies to improve population-level identification and reduce premature atherosclerotic cardiovascular disease. We summarise contemporary familial hypercholesterolaemia screening models with emphasis on paediatric approaches, implementation barriers, and health system requirements, and we present the EPIRUS Familial Hypercholesterolaemia Reverse Cascade Project as a pragmatic regional model in Greece. A literature search (December 2025 to January 2026) was performed in PubMed, Scopus, and Google Scholar using terms related to "familial hypercholesterolemia," "familial hypercholesterolaemia," "screening," "cascade," "reverse cascade," and "population programme." Eligible publications included human studies and evidence syntheses evaluating universal, opportunistic, cascade, or paediatric screening strategies, and implementation or economic analyses. Evidence consistently indicates that opportunistic detection and conventional cascade screening alone cannot close the diagnostic gap because of low index-case ascertainment and fragmented pathways. Universal or structured paediatric programmes demonstrate feasibility, high diagnostic yield after infancy, and capacity to trigger family-based case-finding. Recent data show low-density lipoprotein cholesterol discrimination is limited in newborns but becomes reliable from age ≥1 year, supporting childhood screening. We describe EPIRUS Familial Hypercholesterolaemia, a targeted paediatric programme (primarily ages 4-16 years) using lipid thresholds and family history triggers, systematic phenotyping, and next-generation sequencing to confirm diagnosis and enable cascade and reverse cascade screening. EPIRUS Familial Hypercholesterolaemia illustrates how paediatric entry points can operationalise family-based detection within routine clinical care in a decentralised setting and can generate real-world implementation evidence to inform scalable national strategies.
Varghese P, Elder RW, Vinocur JM
… +5 more, Spatz ES, Shabanova V, McCollum S, Harper M, Pastor T
Cardiol Young
· 2026 Jun · PMID 42290594
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BACKGROUND: Glucagon-like-peptide-1 (GLP-1) agonists are beneficial for glycaemic control, weight loss, and cardiovascular health. Because GLP-1 agonists may be a useful tool in the management of obesity in patients with...BACKGROUND: Glucagon-like-peptide-1 (GLP-1) agonists are beneficial for glycaemic control, weight loss, and cardiovascular health. Because GLP-1 agonists may be a useful tool in the management of obesity in patients with congenital heart disease (CHD), we examined whether GLP-1 agonists are effective and safe for weight loss in adults with congenital heart disease (ACHD) and explored the effects of sex and CHD complexity. METHODS: We conducted a retrospective cohort study of ACHD patients who were prescribed a GLP-1 agonist from 1/1/2022-3/31/2024 at Yale New Haven Hospital. Patients were subdivided by CHD anatomic complexity. The primary outcome was the change in weight over time. Side effects were explored as a secondary outcome. RESULTS: The moderate complexity ACHD group had the fastest weight loss during both the first 6 months and 12 months of treatment, but was comparable to the other two ACHD complexities in the full period of follow-up. Patients in the simple and great complexity groups required 12 months of treatment to reach meaningful weight loss. Females had a faster rate of weight loss compared to males across all ACHD complexity levels. The most common side effects reported were gastrointestinal, such as nausea, acid reflux, and diarrhoea. CONCLUSION: This study provides evidence that GLP-1 agonists are effective for weight loss in ACHD patients, with the greatest weight loss in females and patients with moderate ACHD complexity. Moreover, GLP-1 agonists in the ACHD population have a reasonable side effect profile that is typical for these agents.
Mathews V, Francis J, Ravishankar R
… +5 more, Hussein N, George J, Ismail A, Peng E, Novick W
Cardiol Young
· 2026 Jun · PMID 42290591
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INTRODUCTION: The 2025 Global Peace Index highlights that there are now 59 active state-based conflicts, the highest number since the Second World War, and overall levels of peacefulness are at their lowest since the ind...INTRODUCTION: The 2025 Global Peace Index highlights that there are now 59 active state-based conflicts, the highest number since the Second World War, and overall levels of peacefulness are at their lowest since the index began. For children with CHD, this escalation means that more regions face disruption of surgical services, growing backlogs of untreated cases, and greater risks of displacement and loss to follow-up. METHODS: We aimed to conduct a narrative review mapping existing evidence on the provision, models, outcomes, barriers, and enablers of paediatric cardiac surgery in conflict-affected and disaster settings while identifying gaps to inform future research and practice. RESULTS: Conflicts and disasters amplify the burden of both congenital and acquired paediatric heart disease, worsened by late presentation and progressive loss of operability. The collapse of hospital infrastructure, targeting of health workers, interruption of supply chains, and diversion of resources to trauma care severely limit the feasibility of surgery. Perioperative challenges, including the absence of paediatric intensive care, infection control difficulties, and disrupted follow-up, frequently compromise outcomes. CONCLUSION: Conflict and disaster settings significantly exacerbate existing structural weaknesses in congenital cardiac care and may introduce additional system shocks that disrupt infrastructure, workforce stability, supply chains, and continuity of care. Future humanitarian health frameworks should explicitly include congenital and paediatric cardiac care within non-communicable disease response, supported by pre-positioned critical equipment, telemedicine, and long-term partnerships.
James R, Ginn LA, Negrin K
… +4 more, Young AC, Pande C, Puri K, Sood E
Cardiol Young
· 2026 Jun · PMID 42290588
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OBJECTIVES: This scoping review evaluates the physiological, neurodevelopmental, and psychosocial effects of reading interventions on critically ill infants and young children. INTRODUCTION: Critically ill children, incl...OBJECTIVES: This scoping review evaluates the physiological, neurodevelopmental, and psychosocial effects of reading interventions on critically ill infants and young children. INTRODUCTION: Critically ill children, including premature infants and those with critical CHDs, are at elevated risk for neurodevelopmental complications that may be exacerbated by noxious stimuli in hospital settings. Given the increased awareness of the significance of developmental care in the critical care environment and the well-established role of reading and literacy interventions in healthy children, a potential method to improve developmental outcomes for critically ill children is early promotion of literacy. We reviewed reading-based interventions in critical care settings using a scoping review framework. METHODS: Searches were performed in clinical literature databases for articles evaluating outcomes consequent to or co-occurring with any neonatal or paediatric intensive care setting intervention involving reading to a child. Data and textual content extracted included measures of child physiological status, length of stay, acuity, stress/pain, health status, quality of life, neurodevelopment, as well as parent psychosocial and emotional functioning. RESULTS: This review identified multiple potential benefits of reading to critically ill children at the bedside, including improved physiological and early literacy outcomes for paediatric patients as well as heightened senses of self-efficacy and psychological well-being for parents. Due to the observed positive impact and lack of negative effects, current literature about reading interventions in critical care settings indicates that such programmes are likely to be a scalable, inexpensive option for facilitating neurodevelopment and bonding for children and their families.
Haddad RN, Albenque G, Batteux C
… +1 more, Fraisse A
Cardiol Young
· 2026 Jun · PMID 42290586
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Balloon-expandable Edwards SAPIEN 3 (23 mm) and MyVal OCTACOR (21.5 mm) valves were tested in 3D-printed silicone tubes using a low-pressure, water-perfused simulator with ultrasound and video monitoring. Balloons were i...Balloon-expandable Edwards SAPIEN 3 (23 mm) and MyVal OCTACOR (21.5 mm) valves were tested in 3D-printed silicone tubes using a low-pressure, water-perfused simulator with ultrasound and video monitoring. Balloons were inflated in 2 mm increments from 18 to 30 mm. Both valves were easily inflated from 18 to 24 mm with 2-3 atm inflation pressures and required 5.5 atm and 7 atm, respectively, to reach an inner diameter of 26 mm at the outflow level for the SAPIEN 3 and OCTACOR valves. Further expansion was not achievable. At 18 mm, leaflet opening was mildly asymmetric, but laminar flow was preserved. Circular geometry improved at 20 mm and normalised at 22 mm for both valves. At 24 mm, SAPIEN 3 showed trivial regurgitation, while OCTACOR exhibited a central coaptation defect with mild-to-moderate regurgitation. At 26 mm, SAPIEN 3 leaflets were maximally stretched yet functional with central regurgitation, whereas OCTACOR failed with leaflet tears at the frame attachment. These results indicate both valves can be implanted at small diameters and stepwise dilated to nominal size, tolerating overexpansion up to 3 mm for SAPIEN 3 (+13% overexpansion) and 4.5 mm for OCTACOR (+20.9% overexpansion) at outflow level, with SAPIEN 3 remaining functional and OCTACOR requiring valve-in-valve replacement. Pending clinical validation, this versatility could support their use for infant mitral and pulmonary replacements as alternatives to the Melody valve; though, under- or overexpansion should be reserved for clinically essential situations.
Stelmaszewski EV, Biancolini J, Gonzalez Ortiz A
… +7 more, Goldsman A, Arevalo Leal O, Maza Caneva O, Prada Casilimas M, Sardella A, Pignatelli R, Working Group on Pediatric and Adolescent Cardio-Oncology of the Pediatric Cardiology and ACHD Council, Inter-American Society of Cardiology (SIAC).
Cardiol Young
· 2026 Jun · PMID 42290585
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BACKGROUND: Survivors of childhood cancer face an increased lifetime risk of cardiovascular disease related to cancer therapies. While paediatric and adolescent cancer cardio-oncology programmes have expanded in high-inc...BACKGROUND: Survivors of childhood cancer face an increased lifetime risk of cardiovascular disease related to cancer therapies. While paediatric and adolescent cancer cardio-oncology programmes have expanded in high-income settings, their implementation across Latin America remains poorly characterised. OBJECTIVES: To describe the current landscape of paediatric and adolescent cardio-oncology care in Latin America, focusing on workforce training, clinical infrastructure, diagnostic resources, follow-up strategies, and research capacity. METHODS: We conducted a cross-sectional survey of healthcare professionals involved in the care of paediatric and adolescent oncology patients across Latin America. The questionnaire assessed institutional resources, availability of specialised cardio-oncology services, diagnostic modalities, cardioprotective strategies, survivorship follow-up, and training and research activities. Descriptive analyses were performed. RESULTS: A total of 177 complete surveys were analysed. Most respondents worked in public, tertiary-level institutions. Only 16.4% reported access to cardiologists with formal cardio-oncology training, and 26.0% had a structured cardio-oncology team. While baseline cardiac evaluation was widely performed (77.4%), advanced diagnostic tools such as myocardial strain imaging (35.0%) and cardiac magnetic resonance (22.0%) were infrequently available. Formal long-term cardiovascular follow-up programmes for cancer survivors were present in only 13.6% of institutions, and structured transition to adult cardiology care in 22.0%. Participation in multicentre research and formal training programmes was limited. CONCLUSIONS: Paediatric and adolescent cardio-oncology care in Latin America is characterised by substantial heterogeneity and limited formal infrastructure. These findings highlight critical gaps and underscore the need for regional implementation strategies to improve cardiovascular care across the cancer survivorship continuum.
Cardiol Young
· 2026 Jun · PMID 42290564
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We report a 40-year-old man (167 cm, 69 kg) with a complex surgical history, including repaired coarctation of the aorta and a Bentall procedure with mechanical valve replacement, who presented with recurrent, haemodynam...We report a 40-year-old man (167 cm, 69 kg) with a complex surgical history, including repaired coarctation of the aorta and a Bentall procedure with mechanical valve replacement, who presented with recurrent, haemodynamically significant coarctation of the aorta, severe arterial hypertension, and a challenging 17 mm diameter mismatch between the aortic arch and descending aorta. As surgery was declined, a tailored hybrid solution was adopted: deployment of a custom thoracic endograft to bridge the calibre transition, reinforced with a balloon-expandable stent to optimise luminal gain. The approach achieved complete gradient resolution without complications, allowing for discharge at 48 hours. This case underscores the feasibility of personalised hybrid repair for complex re-coarctation of the aorta.
Cardiol Young
· 2026 Jun · PMID 42272065
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Three infants developed ARDS-like lung injury after Glenn or Fontan procedures, each following infection or inflammation. All showed unusually high elevations of epithelial injury markers such as KL-6 and surfactant prot...Three infants developed ARDS-like lung injury after Glenn or Fontan procedures, each following infection or inflammation. All showed unusually high elevations of epithelial injury markers such as KL-6 and surfactant protein D (SP-D). These findings suggest a distinct post-bypass phenotype in which right-heart circulation may amplify epithelial injury beyond that seen in typical paediatric ARDS.
Bhatia S, Puckett R, McEachern WA
… +3 more, Dennison CB, Slesnick T, Wilson HC
Cardiol Young
· 2026 Jun · PMID 42266108
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Cross-sectional imaging in children on an extracorporeal membranous oxygenator can be challenging due to logistics of transportation and technical aspects of image acquisition. Nevertheless, cardiac CT can be of value in...Cross-sectional imaging in children on an extracorporeal membranous oxygenator can be challenging due to logistics of transportation and technical aspects of image acquisition. Nevertheless, cardiac CT can be of value in answering important clinical questions in these patients. We share our experience while establishing a successful clinical workflow to perform cardiac CT in children with CHD on extracorporeal membranous oxygenator, which has not been previously described.