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.

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.

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.

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.

Heart failure is the most common complication of congenital heart disease (CHD), characterized by high morbidity and mortality. Early recognition and intervention are crucial for improving outcomes in pediatric patients with CHD and heart failure. This study aimed to analyze the clinical value of N-terminal pro-brain natriuretic peptide, adrenomedullin, and cardiac troponin I in pediatric patients with CHD and heart failure.

Ninety-eight pediatric patients with CHD complicated by heart failure were included in the Case Group, and 61 pediatric patients with CHD were included in the Control Group. The Case Group was categorized into subgroups based on the cardiac function of patients: grade I (n = 35), grade II (n = 40), and grade III (n = 23). Left ventricular ejection fraction was collected from pediatric patients in the case group. The correlation of the cardiac functional indicators with N-terminal pro-brain natriuretic peptide, adrenomedullin, and cardiac troponin I levels was assessed using Pearson correlation analysis.

The serum levels of N-terminal pro-brain natriuretic peptide, adrenomedullin, and cardiac troponin I in pediatric patients at grade III of cardiac function were significantly elevated compared to those at grade II, and these levels in patients at grade II were higher than those at grade I (P < 0.05). The left ventricular ejection fraction of pediatric patients in the Case Group were markedly negatively correlated with N-terminal pro-brain natriuretic peptide, adrenomedullin, and cardiac troponin I (r = − 0.6807, r = −0.3013, r = −0.5412, P < 0.0001).

N-terminal pro-brain natriuretic peptide, adrenomedullin, and cardiac troponin I have a certain diagnostic value in determining concurrent heart failure in pediatric patients with CHD.

Atrial septal defect is the second most common CHD in adults. This study aimed to evaluate the relationship between age and early postoperative changes in cardiac function—including right ventricular function, tricuspid regurgitation, pulmonary artery pressure, mitral regurgitation, and left ventricular ejection fraction—in adult patients undergoing surgical atrial septal defect repair.

This retrospective study included 214 patients aged 16–65 years who underwent isolated atrial septal defect repair between 2014 and 2024. Patients were divided into five age groups. Echocardiographic parameters were compared at three time points: preoperative, 1-month, and 6-month postoperative.

Of the patients, 60.7% were female. A significant postoperative left ventricular ejection fraction decrease was observed only in the 56–65 age group. Preoperative tricuspid regurgitation severity and pulmonary artery pressure values increased with age, but both showed significant postoperative improvement across all age groups. Postoperative pulmonary artery pressure reduction was most prominent in the youngest and oldest groups. Mitral regurgitation was mildly increased in all groups, without progression to moderate severity. Tricuspid annular plane systolic excursion values, inversely correlated with age, decreased in younger patients postoperatively but improved in older patients, indicating enhanced right ventricular function after volume unloading.

Surgical atrial septal defect repair leads to early improvement in several cardiac parameters. However, the benefit is more pronounced in younger patients. These findings support early surgical intervention to preserve right ventricular function and prevent long-term complications, particularly in asymptomatic adults.

Amiodarone is a frequently used medication in paediatric patients to manage atrial and ventricular arrhythmias, but its acute haemodynamic effects, particularly in children, remain underexplored. This retrospective, single-centre study aimed to characterise the clinical impact of amiodarone intravenous infusions on heart rate, blood pressure, oxygen delivery, and transaminase levels within the first 48 hours of amiodarone initiation in paediatric cardiac ICU patients.

Single-centre, retrospective study of patients receiving amiodarone infusions, with measurements taken at baseline and at various intervals up to 48 hours after initiation. The primary outcome was the effect on heart rate, while secondary outcomes included blood pressure, arterial saturation, near-infrared spectroscopy values, central venous pressure, and transaminase levels. Several types of analysis models were employed to assess the results.

Data were collected from 87 paediatric patients. No significant changes in liver enzymes, blood pressure, or renal oxygen extraction were observed. These findings provide novel insights into the acute haemodynamic effects of amiodarone infusions in paediatric patients, suggesting that while amiodarone significantly lowers heart rate, it does not substantially affect oxygen delivery or necessitate increased vasoactive support.

Amiodarone infusions are associated with a significant decrease in heart rate without greatly impacting oxygen delivery or requiring increased vasoactive support. Heart rate decreases most until a cumulative dose of 30,000 mcg/kg, and heart rate decrease is most pronounced in those with ventricular tachycardia.

Physiologic changes in the peripartum period put women with CHD at increased risk for morbidity. This study examines factors associated with peripartum complications and length of stay compared to patients without CHD.

This single-institution retrospective case-control study included women with CHD (2000–2017) and a control population without CHD. A review of clinical and echocardiographic data was used to assign baseline characteristics, disease severity, and adverse outcomes. Primary outcomes were composite variables of cardiac and obstetric adverse events, along with peripartum length of stay. The relationship between maternal CHD, baseline characteristics, and peripartum adverse events was evaluated by multivariable regression.

The cohort and control groups included 162 deliveries among 113 women and 321 deliveries among 321 women, respectively. Cardiac complications, including arrhythmia, heart failure, pulmonary oedema, and thromboembolic events, occurred in 8.6% of the cohort (RR 2.52, 95% CI 1.17–5.42), with the most common event being arrhythmia. Obstetric events, such as caesarean delivery, assisted vaginal delivery, preterm birth, and pre-eclampsia, occurred in 67.9% versus 56.1% in the control group (RR 1.21, 95% CI 1.05–1.40). In multivariable models, increasing age was associated with increased composite cardiac events. Length of stay was longer in the cohort group (p < 0.001) and significantly associated with modified World Health Organization classification (p = 0.016).

Women with CHD experience increased cardiac and obstetric morbidity compared to controls during peripartum admission. Those with CHD have longer hospital stays around delivery, which is associated with disease severity.

Children with heart conditions, particularly CHDs, may experience adverse neurodevelopmental and psychosocial outcomes. Our study aimed to: (1) compare national prevalence of mental, behavioural, and developmental disorders among children by heart condition status and (2) identify associated characteristics among children with heart conditions.

Nationally representative data from the National Survey of Children’s Health (2016–2021) on U.S. children aged 6–17 years without Down syndrome were analysed. Caregivers reported whether a healthcare provider told them their child has ever had a heart condition or currently has depression, anxiety, ADHD, behavioural, or conduct problems, Tourette syndrome, autism spectrum disorder, developmental delay, intellectual disability, learning disability, or a speech or other language disorder. Logistic regression analysis compared disorder prevalence by heart condition status and, among children with heart conditions, assessed whether disorders were associated with demographic and contextual characteristics.

Among 3,440 children with heart conditions, 42% had an examined disorder, compared to 23% of 133,280 children without heart conditions (adjusted prevalence ratio = 1.8; 95% confidence interval: 1.7, 2.0). Each disorder was more prevalent among children with versus without heart conditions (adjusted prevalence ratio range: 1.9 to 5.1), with anxiety (22.1%), ADHD (20.4%), and learning disabilities (19.6%) most common. Among children with heart conditions, disorders were consistently associated with an increased number of adverse childhood experiences.

These findings support clinical guidelines recommending neurodevelopmental and mental health screening and interventions for children with heart conditions and can be used as a national baseline to gauge progress of guideline implementation.

Cardiac rhabdomyomas are the most common benign paediatric cardiac tumours. Arrhythmias and cardiac conduction abnormalities have both been described with these tumours and resolved with reduction in size of the tumours. Here, we present a case of a child who was prenatally diagnosed with multiple cardiac rhabdomyomas and cardiac arrhythmias and found to have ventricular pre-excitation after birth, in whom the tumours regressed, and pre-excitation resolved with 12 weeks of sirolimus and propranolol therapy. However, 8 weeks after cessation of sirolimus and propranolol therapy, tumour size increased, and manifest ventricular pre-excitation recurred and progressed to ventricular tachycardia. Subsequent follow-up after restarting sirolimus and propranolol therapy showed a significant reduction in tumour burden and resolution of pre-excitation.

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.

A retrospective analysis of paediatric infective endocarditis characterised causative pathogens, antimicrobial susceptibility patterns, and treatment outcomes to guide clinical decision-making.

The data of patients who received infective endocarditis between 2016 and 2023 were retrospectively collected from the medical records database. The clinical characteristics, treatment plans, and pharmaceutical monitoring characteristics were analysed and summarised.

A total of 12 paediatric infective endocarditis cases were identified. Bacterial isolates included 27 Gram-positive and 1 Gram-negative strains. The most common pathogen was Staphylococcus aureus (n = 13), all methicillin-resistant Staphylococcus aureus (MRSA), followed by Abiotrophia defectiva (n = 6), Streptococcus mitis (n = 5), Streptococcus sanguinis (n = 2), Bacillus cereus (n = 1), and Klebsiella oxytoca (n = 1). Antimicrobial therapy primarily involved linezolid, vancomycin, and cephalosporin/enzyme inhibitor combinations. Cardiac glycosides were used in 10 cases, and all patients received phosphocreatine to support myocardial energy metabolism. Therapeutic drug monitoring for vancomycin was performed in 25% of cases, while no therapeutic drug monitoring was conducted for meropenem or linezolid.

All the causative organisms were predominantly Gram-positive cocci, with MRSA accounting for the largest proportion; different streptococci varied considerably in terms of drug resistance. The antimicrobial drugs used were predominantly linezolid and glycopeptides. The rate of blood concentration monitoring was low.

Right ventricular dysfunction is a common complication after surgical correction of tetralogy of Fallot. The assessment of right ventricular systolic function using cardiac magnetic resonance is the gold method. Our study aimed to compare the diagnostic accuracy of echocardiographic parameters in identifying right ventricular systolic dysfunction in comparison to cardiac magnetic resonance in children with repaired tetralogy of Fallot.

Fifty-six patients with corrected tetralogy of Fallot were included in the study. Right ventricular fractional area change, Tricuspid annular plane systolic excursion, right ventricular peak systolic tricuspid annular velocity, two-dimensional right ventricular global longitudinal strain, and two-dimensional right ventricular free wall strain were measured using echocardiography. Right ventricular ejection fraction and pulmonary regurgitant fraction were assessed using cardiac magnetic resonance.

Twenty (35.7%) patients showed right ventricular systolic dysfunction using cardiac magnetic resonance-derived right ventricular ejection fraction%, while 36 patients had normal right ventricular systolic function. Patients with right ventricular systolic dysfunction had significantly lower right ventricular fractional area change, tricuspid annular plane systolic excursion, right ventricular S’, 2D-right ventricular global longitudinal strain, 2D-right ventricular free wall strain compared to those with normal right ventricular systolic function. Right ventricular ejection fraction% was directly correlated to tricuspid annular plane systolic excursion, right ventricular fractional area change, right ventricular S’, 2D-right ventricular global longitudinal strain, and 2D-right ventricular free wall strain. Receiver operating characteristic curve revealed that 2D-right ventricular free wall strain and 2D-right ventricular global longitudinal strain had 70% sensitivity, 94.4% specificity, and 85% predictive accuracy, while right ventricular S’ and RV fractional area change had 100 % sensitivity in detection of right ventricular dysfunction, but they showed lower specificity, 30.6% and 52.8% respectively.

2D-right ventricular global longitudinal strain and 2D-right ventricular FWL have good diagnostic value for right ventricular systolic dysfunction comparable to cardiac magnetic resonance in children with repaired tetralogy of Fallot.

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.

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.

Anatomic repair had significantly higher PROMIS-10 Physical T-scores compared to physiologic repair (median 50.9 vs 41.6, p = 0.04). MacNew Social scores were significantly higher for medical management compared to physiologic repair (median 6.8 vs 6.0, p = 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.

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.