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.

Although isolated ductus arteriosus aneurysm has been reported as 0.8% in autopsy series, it has recently been reported as up to 8.8% with increased awareness. Most cases are asymptomatic and regress spontaneously. In this study, we aimed to evaluate the characteristics, clinical manifestations, and outcomes of ductus arteriosus aneurysm in neonates.

A total of 22 newborns with echocardiographic images recorded in the hospital registry system were identified to have a ductus arteriosus aneurysm. The demographic, clinical, and echocardiographic findings of the patients were extracted from the hospital records.

The mean gestational age and weight at birth of the newborns were 38 6/7 weeks (35 3/7 – 40 6/7 weeks) and 3203 g (2445–4080 g), respectively. The maximal length and width of the aneurysm ranged from 6 to 21 mm and 5.3 to 13 mm, respectively. In three of the patients, thrombus in the aneurysm was detected on the third day of life, leading to surgical excision of thrombus and ligation of the aneurysm in two. In the remaining 19 patients, the aneurysm regressed spontaneously and closed in an average of 5.3 days.

With increased awareness and earlier and more frequent application of echocardiography in newborns, the diagnosis of ductus arteriosus aneurysm is being reported more frequently. The most common scenario is spontaneous regression, however, it should not be forgotten that it can lead to serious complications, as reported previously. In selected cases, surgical treatment may be an option to prevent more serious or late complications, though the indications for surgical intervention remain unclear.

Educational experience of children with CHD is often adversely impacted by factors such as medical burden, social and school functioning challenges. It is, therefore, vitally important that adequate support is provided at an early stage in order to facilitate better educational outcomes for this cohort. The role of the teacher is pivotal in supporting the overall healthy development of a child with CHD. Thus, it is important to understand how we can also support teachers to provide optimal support to this cohort. This systematic scoping review aimed to offer a comprehensive understanding of existing research in this area and identify any knowledge gaps.

The methodological framework for scoping reviews developed by Arksey and O’Malley (2005) was employed.

Children with CHD face educational challenges in cognitive, psychomotor, behavioural, and affective domains and also with school attendance. The main challenges for teachers include a lack of information around CHD and how it affects the individual child. Building a strong relationship and having frequent communication between the teacher/ parent/ child were considered key in alleviating anxiety and promoting a supportive environment.

Children with CHD often require additional support from educational professionals in the classroom. Teachers of children with CHD would benefit from condition-specific training, updated on a regular basis.

Outcomes of single ventricle heart defects may be influenced by the morphological type of the hypoplastic ventricle. Recent multi-centre studies have targeted on outcomes of hypoplastic left heart syndrome with limited focus on outcomes of hypoplastic right heart syndrome. We aimed at studying the clinical outcomes of hypoplastic right heart syndrome in the recent era.

We performed a retrospective analysis of all hypoplastic right heart syndrome patients (n = 153) born between January 2010 and January 2023. Five-year transplant-free survival was compared with hypoplastic left heart syndrome patients without heterotaxy (n = 144) born during the same time.

Double-inlet left ventricle was the most common anatomic hypoplastic right heart syndrome subtype (n = 39, 25%). Twenty-six (17%) patients with hypoplastic right heart had associated heterotaxy. Five-year transplant-free survival was high for most groups (double inlet left ventricle: 100%, pulmonary atresia: 94%, Ebstein’s anomaly: 92%, tricuspid atresia: 90% respectively). The heterotaxy group had worse early outcomes with 3 deaths and 3 heart transplants, before Fontan completion. Heterotaxy was a significant risk factor for death/transplant prior to Fontan completion in hypoplastic right heart syndrome patients (p = 0.03). Patients with hypoplastic right heart syndrome with heterotaxy had significantly worse 5-year transplant-free survival (71%) when compared to hypoplastic right heart syndrome without heterotaxy (95%) and hypoplastic left heart syndrome without heterotaxy (75%), p < 0.001.

Hypoplastic right heart syndrome patients have excellent clinical outcomes and better early childhood survival than hypoplastic left heart syndrome in the current era, except when associated with heterotaxy. The survival advantage conferred by a single left ventricle appears to be negated by heterotaxy syndrome and should be strongly considered during caregiver counselling and medical decision-making.

Kartagener’s syndrome is a rare subset of primary ciliary dyskinesia, a genetically heterogeneous disorder characterised by chronic sinusitis, bronchiectasis, and situs inversus. To our knowledge, the association of this syndrome with coarctation of the aorta (CoAo) and pulmonary hypertension (PH) has not been previously reported.

We report the case of a 17-year-old female patient with situs inversus and CoAo surgically corrected at two months of age. At 12 years old, she developed a chronic cough, nasal congestion, and an estimated pulmonary artery systolic pressure (PASP) of 70 mmHg on echocardiography. Cardiac catheterisation revealed a pulmonary artery pressure of 58/10/24 mmHg with no gradient at the aortic isthmus. Thoracic CT scan demonstrated multiple bronchiectasis in the upper lobes bilaterally, despite normal pulmonary spirometry, plethysmography, and 24-hour oximetry. Genetic testing identified a pathogenic variant and a variant of uncertain significance of the DNAH5 gene, associated with primary ciliary dyskinesia. The patient was diagnosed with Kartagener’s syndrome with PH and was started on inhaled glucocorticoids and chest physiotherapy. No episodes of pneumonia or acute bronchiectasis exacerbations were recorded. Annual lung function tests remained normal, and semiannual echocardiograms showed stable findings. A follow-up thoracic CT scan at 16 years of age revealed no progression of lung disease. At 16 years old, the patient developed significant physical activity limitation. Echocardiographic evaluation demonstrated right ventricular dilatation with reduced longitudinal function (TAPSE 15 mm, Z-score 4.4), suprasystemic PASP (120 mmHg), an eccentricity index of 2.3, normal left ventricular function, moderate pericardial effusion, and an NT-ProBNP level of 2292 pg/mL. Combined therapy targeting PH was initiated, including an endothelin receptor antagonist, a phosphodiesterase type 5 inhibitor, diuretics, and supplemental oxygen therapy. Significant clinical improvement was observed (WHO functional class IIIa to I), along with echocardiographic improvement (PASP reduced to 80 mmHg, TAPSE increased to 16 mm, resolution of pericardial effusion), and a marked decrease in NT-proBNP (171 pg/mL).

The rapid progression of PH in this patient, despite normal lung function and unremarkable CT scan findings, is atypical for PH associated with lung disease. Extensive investigation for alternative causes of PH, including genetic testing, yielded negative results. A more aggressive treatment strategy for PH is currently being pursued.