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  • New
  • Research Article
  • 10.1017/s1047951126111950
The road to innovation: Collaborative enhancement of clinical prediction-A 9th World Congress of Pediatric Cardiology and Cardiac Surgery closing plenary lecture.
  • Apr 7, 2026
  • Cardiology in the young
  • Shelby Kutty

Innovation in paediatric and adult congenital cardiology increasingly depends on collaboration among academia, industry, and professional communities. From this perspective, the author argues that clinical prediction represents a natural convergence point for these stakeholders, aligning safe, personalised care with economic incentives. The author discusses emerging evidence highlighting the promise of artificial intelligence-driven prediction across various cardiovascular domains, while highlighting current limitations related to narrow scope, static design, and weak integration into clinical decision-making. Medicine-based evidence and a high-quality, inclusive data infrastructure may help address these gaps. Together, these approaches, along with stakeholders upholding their responsibilities, define a path towards predictive innovation.

  • New
  • Research Article
  • 10.1017/s1047951126111962
The state of the unit: variable care models in paediatric acute care cardiology units documented by the fourth iteration of the Paediatric Acute Care Cardiology Collaborative (PAC3) Hospital Survey.
  • Apr 7, 2026
  • Cardiology in the young
  • Megan E Rodts + 9 more

Pediatric acute care cardiology is a distinct subspecialty field within paediatric cardiology that has grown rapidly in recognition, with previously documented heterogeneity in its practice across 31 centres surveyed in 2017. Unit composition and care delivery across centres participating in the Paediatric Acute Care Cardiology Collaborative (PAC3) have not been formally reassessed and shared, despite significant growth in the field. A 214-stem question Hospital Survey was created with 454 total response fields across eight domains important to paediatric acute care cardiology such a demographics, staffing, resources and therapies, and standard practices. PAC3 centres were surveyed in September 2023 via REDCap. Descriptive statistics were performed. Surveys were completed by 100% (47/47) of PAC3 centers. Diverse unit composition exists with 37% of centres utilising a single, dedicated acute care cardiology unit, 28% using mixed-specialty acute care units, and 19% using acuity adaptable units, housing critical and acute care patients in one physical space. Since 2017, acute care cardiology-dedicated multidisciplinary staff has increased (physical therapy (PT): 0 to 4; occupational therapy (OT): 1 to 5; speech-language pathology (SLP): 0 to 4; PharmD: 7 to 26). There is heterogeneity in utilisation of many of the resources and therapies used in acute care cardiology, and use of ventricular assist devices on the acute care cardiology unit has increased. Significant variability exists in unit structure and care delivery models across a diverse group of centres providing acute care cardiology services. The Hospital Survey may assist in identifying best practices for similar centres across PAC3.

  • New
  • Research Article
  • 10.1017/s1047951126111925
Severe pulmonary hypertension as a diagnostic challenge: a case of congenitally corrected transposition of the great arteries with dextrocardia in an elderly patient.
  • Apr 7, 2026
  • Cardiology in the young
  • Meta Novak + 3 more

Congenitally corrected transposition of the great arteries is a rare congenital heart defect that may remain undiagnosed well into adulthood. We present the case of a 71-year-old male with dextrocardia and exertional dyspnoea, initially diagnosed with severe pulmonary hypertension. Further evaluation revealed an underlying congenitally corrected transposition of the great arteries. This case highlights the importance of considering CHD in older adults with unusual cardiac findings.

  • New
  • Open Access Icon
  • Research Article
  • 10.1017/s1047951126112104
Leadless pacing in pre-adolescent patients weighing 30kg or less: case report and systematic review
  • Apr 6, 2026
  • Cardiology in the Young
  • Pipin Kojodjojo + 1 more

Abstract Transvenous and epicardial pacing in children carries significant risks of lead failure and infection. Leadless pacing is an alternative with lower risks of infection, lead failure, and longer battery longevity. A retrievable leadless pacemaker was implanted in an 8-year-old, 24 kg patient with heart block. A systematic review evaluated procedural success and the safety of leadless pacemakers in preadolescents weighing less than 30 kg.

  • New
  • Research Article
  • 10.1017/s1047951126112049
Right ventricular function after repair of tetralogy of Fallot: an echocardiographic and clinical evaluation.
  • Apr 1, 2026
  • Cardiology in the young
  • Onur Benli + 2 more

Right ventricular dysfunction is a major determinant of long-term outcomes after tetralogy of Fallot repair, particularly in the presence of chronic pulmonary regurgitation. Strain analysis may detect early right ventricular dysfunction more sensitively than conventional echocardiographic parameters. This retrospective single-centre study included 63 patients after tetralogy of Fallot repair who underwent postoperative echocardiographic evaluation during mid-term follow-up. Right ventricular function was assessed using tricuspid annular plane systolic excursion, right ventricular systolic velocity, fractional area change, and right ventricular free-wall longitudinal strain. Pulmonary regurgitation severity was evaluated by colour Doppler, with pulmonary regurgitation jet width expressed as a ratio relative to the pulmonary annulus diameter. Associations between pulmonary regurgitation severity and right ventricular function were analysed using correlation and multivariable linear regression. Conventional right ventricular systolic parameters were largely preserved, whereas right ventricular free-wall longitudinal strain worsened with increasing pulmonary regurgitation severity (p = 0.036). Pulmonary regurgitation jet width emerged as the sole independently associated determinant of impaired right ventricular strain (β = 0.132, p = 0.038). QRS duration was not independently associated. Pulmonary regurgitation jet width showed moderate discriminatory ability for impaired right ventricular strain (area under the curve = 0.67). Right ventricular free-wall longitudinal strain is a sensitive marker of pulmonary regurgitation-related right ventricular dysfunction after tetralogy of Fallot repair. Pulmonary regurgitation jet width, expressed relative to the pulmonary annulus, appears to be the dominant determinant of right ventricular mechanical impairment and may complement conventional assessment during follow-up.

  • New
  • Research Article
  • 10.1017/s1047951126111883
A novel homozygous PPP1R13L frameshift variant in a child with syndromic dilated cardiomyopathy and fatal arrhythmia.
  • Apr 1, 2026
  • Cardiology in the young
  • Hakan Kurt + 5 more

Dilated cardiomyopathy is a rare but severe myocardial disease in the paediatric population, often leading to heart failure, heart transplantation, or sudden cardiac death. Genetic factors are a major contributor to childhood dilated cardiomyopathy. Recently, biallelic variants in the PPP1R13L gene have been implicated in a novel syndromic form of early-onset dilated cardiomyopathy, characterised by cardiac dysfunction alongside variable ectodermal features. We report a 4-year-old boy who presented with decompensated heart failure and echocardiographic findings consistent with dilated cardiomyopathy. Syndromic features included sparse, dry hair, high anterior hairline, broad nasal bridge, and pointed teeth. Genetic analysis revealed a novel homozygous frameshift variant in the PPP1R13L gene (c.2368_2375dup; p. Pro793Glyfs*32), classified as pathogenic. The clinical course was complicated by recurrent ventricular arrhythmias and ultimately sudden cardiac death. PPP1R13L-related cardiomyopathy should be considered in children with early-onset dilated cardiomyopathy and syndromic features. Early diagnosis is critical for clinical management, arrhythmia surveillance, and appropriate family counselling.

  • New
  • Research Article
  • 10.1017/s1047951126111949
Turner syndrome and acute type A aortic dissection: a national inpatient analysis of risk and outcomes.
  • Apr 1, 2026
  • Cardiology in the young
  • Yesh Datar + 2 more

Although acute type A aortic dissection (ATAAD) is a recognised complication of Turner syndrome, population-level data describing its prevalence and outcomes remain limited. We queried the National Inpatient Sample (2002-2020) and generated three groups: TS + ATAAD+, TS + ATAAD-, and TS-ATAAD+ in our retrospective observational cohort study. Primary outcomes were ATAAD hospital admission rate and in-hospital mortality; secondary outcomes included length of stay. Sampling weights were applied to all analyses. A total of 51,426 Turner syndrome hospitalisations were identified. Median ages were 40 years (interquartile range 31-50) for TS + ATAAD+ and 32 years (interquartile range 9-49) for TS + ATAAD- (p < 0.001). Overall, 0.27% of Turner syndrome hospitalisations presented with ATAAD. Compared with TS + ATAAD-, those with ATAAD had higher mortality (13.1% vs 2.1%, p < 0.001) and longer length of stay (median 8 vs 3 days, p < 0.05). Rates of bicuspid aortic valve (34.3% vs 3.5%, p < 0.001), hypertension (46.0% vs 25.7%, p < 0.05), and complete heart block (14.6% vs 0.8%, p < 0.001) were also higher. Multivariable analysis showed bicuspid aortic valve (adjusted odds ratio 24.31, 95% CI 8.8-67.3) and age (adjusted odds ratio 1.02, 95% CI 1.01-1.04) predicted ATAAD. Compared with TS-ATAAD+ patients, those withTS + ATAAD+ were younger (40 vs 68 years, p < 0.001), while mortality, length of stay, hypertension, and bleeding rates were similar. ATAAD is highly lethal in patients with and without Turner syndrome. In Turner syndrome, ATAAD is associated with higher mortality, longer hospitalisation, and higher rates of bicuspid valve disease and hypertension.

  • New
  • Research Article
  • 10.1017/s1047951125111220
Unexpected sudden cardiac arrest due to ventricular fibrillation in a 7-year-old boy with Duchenne muscular dystrophy.
  • Apr 1, 2026
  • Cardiology in the young
  • Christiane Mhanna + 2 more

A previously asymptomatic 7-year-old boy with Duchenne muscular dystrophy unexpectedly developed sudden cardiac arrest with no preceding illness. An automated external defibrillator confirmed the presence of ventricular fibrillation. Cardiac MRI showed prominent myocardial fibrosis with no evidence of acute inflammation. Whole exome sequence revealed no associated pathological variance for lethal ventricular arrhythmias. Life-threatening ventricular fibrillation can occur in young patients with Duchenne muscular dystrophy.

  • New
  • Research Article
  • 10.1017/s1047951126111858
Effect of preoperative oral iron supplementation on postoperative outcomes in children with acyanotic congenital heart disease undergoing cardiac surgery: a pragmatic randomised clinical trial.
  • Apr 1, 2026
  • Cardiology in the young
  • Abishek Murugaiyan + 4 more

Studies assessing the effect of preoperative iron supplementation in paediatric cardiac surgery are limited and yield conflicting data. The study aimed to evaluate the effect of preoperative oral iron supplementation on allogeneic blood transfusion after cardiac surgery for acyanotic CHD. This was a prospective, open-label, outcome assessor-blinded, randomised clinical trial performed in a large tertiary care centre in India. Children (haemoglobin <13 gm/dl) with acyanotic CHD were recruited for the study. Children in the intervention arm received colloidal iron (3 mg/kg), folic acid, and cyanocobalamin according to body weight for at least 7 days before cardiac surgery. The primary outcome was the amount of allogeneic blood transfusion, while secondary outcome measures were the duration of mechanical ventilation and ICU stay. A total of eighty-six children (43 in each arm) completed the trials. A total of 43 children received iron supplementation for a minimum of 1 month. The haemoglobin level improved by 1.3 gm/dl immediately before the cardiac surgery in the intervention arm. The allogeneic blood transfusions (ml/kg) median (IQR) were significantly lower in the intervention group (5 (0–8) vs 10 (8–12); p < 0.01). Furthermore, total cumulative allogeneic blood transfusion was also significantly lower in the intervention arm (60 ml; (0–100) vs 100 ml; (70–140); p < 0.001). The duration of mechanical ventilation, ICU stay, and hospital stay was significantly lower in the intervention arm. Preoperative oral iron supplementation significantly reduces the need for allogeneic blood transfusion in children undergoing cardiac surgery for acyanotic heart disease.

  • New
  • Research Article
  • 10.1017/s1047951125100905
Cantrell's pentalogy, complications associated with Glenn surgery, innovation in haemodynamic management.
  • Apr 1, 2026
  • Cardiology in the young
  • Carlos Alberto Guzmán Serrano + 3 more

Cantrell's pentalogy is a rare congenital disorder with high mortality. This report presents an infant with type 1 Cantrell's pentalogy and complex CHD, managed through staged surgeries and hybrid interventions. Genetic studies ruled out hereditary causes. The case highlights the importance of multidisciplinary approaches and further documentation to improve management and outcomes.