There is geographic disparity in the provision of Pediatric and Congenital Heart Disease (PCHD) services; Africa accounts for only 1% of global cardiothoracic surgical capacity. Methods: We conducted a survey of PCHD services in Africa, to investigate institution and national-level resources for pediatric cardiology and cardiothoracic surgery. Results were compared with international guidelines for PCHD services and institutions were ranked by a composite score for low- and middle-income PCHD services. Results: There were 124 respondents from 96 institutions in 45 countries. Eighteen (40%) countries provided a full PCHD service including interventional cardiology and cardiopulmonary bypass (CPB) cardiac surgery. Ten countries (22%) provided cardiac surgery services but no interventional cardiology service, 4 of which did not have CPB facilities. One provided interventional cardiology services but no cardiac surgery service. Ten countries (22%) had no PCHD service. There were 0.04 (interquartile range [IQR]: 0.00-0.13) pediatric cardiothoracic surgeons and 0.17 (IQR: 0.02-0.35) pediatric cardiologists per million population. No institution met all criteria for level 5 PCHD national referral centers, and 8/87 (9.2%) met the criteria for level 4 regional referral centers. Thirteen (29%) countries report both pediatric cardiology and cardiothoracic surgery fellowship training programs. Conclusions: Only 18 (40%) countries provided full PCHD services. The number of pediatric cardiologists and cardiothoracic surgeons is below international recommendations. Only Libya and Mauritius have the recommended 2 pediatric cardiologists per million population, and no country meets the recommended 1.25 cardiothoracic surgeons per million. There is a significant shortage of fellowship training programs which must be addressed if PCHD capacity is to be increased.

Pulmonary regurgitation leading to right ventricular enlargement may occur after repaired tetralogy of Fallot (rTOF) or balloon dilation for pulmonary valve stenosis. Cardiac magnetic resonance imaging (CMR) guidelines to identify the timing of valve replacement in rTOF are not necessarily applicable to isolated pulmonary regurgitation. This study aims to compare deformation parameters of isolated pulmonary regurgitation and rTOF at comparable right ventricular volume loads.

Adopting a quantitative retrospective analytic framework, CMR was performed in 44 patients (0–30 years), 22 in each of the isolated pulmonary regurgitation and rTOF study arms, matched for age (±12 months), and Right ventricular end-diastolic volume z-score (±1). Right ventricular longitudinal strain/strain rate and circumferential strain/strain rate were measured. Comparisons between groups were analysed using two-tailed T-tests and one-way ANOVA.

Both groups showed predominance of longitudinal over circumferential strain. Circumferential strain was significantly greater in rTOF compared to isolated pulmonary regurgitation (–26.5% versus –22.3%, p < 0.05). Longitudinal strain did not differ between groups. The longitudinal:circumferential strain ratio was significantly lower in rTOF compared to isolated pulmonary regurgitation (1.24 versus 1.53, p = 0.05). Circumferential and longitudinal strain rates did not differ between groups.

The right ventricles in rTOF demonstrate greater reliance on circumferential strain in response to increased volumes. The decrease in longitudinal:circumferential strain ratio suggests rTOF right ventricles display a greater adaptive response to the volume load than isolated pulmonary regurgitation, highlighting the importance of the relative contributions of both circumferential and longitudinal strain in order to understand the mechanisms of right ventricular dysfunction in pulmonary regurgitation.

Cardiomyopathy is the leading cause of death in patients with Duchenne muscular dystrophy. The relationship between cardiac and skeletal muscle progression is unclear. The objective of this study was to evaluate the correlation between muscle activity and cardiomyopathy. We hypothesised that cardiomyopathy and skeletal muscle activity are directly related.

Physical activity was assessed with accelerometers worn for 7 days. Average activity (vector magnitude/min) and percentage of time in different activities were reported. Cardiac MRI was used to assess left ventricular ejection fraction, global circumferential strain (Ecc), late gadolinium enhancement, and cardiac index. Associations were assessed between physical activity and cardiac variables using a Spearman correlation.

Duchenne muscular dystrophy subjects (n = 46) with an average age of 13 ± 4 years had a mean left ventricular ejection fraction of 57 ± 8%. All physical activity measures showed significant correlations with left ventricular ejection fraction (rho = 0.38, p = 0.01) and left ventricular cardiac index (rho = 0.51, p < 0.001). Less active subjects had lower left ventricular ejection fraction (p = 0.10) and left ventricular cardiac index (p < 0.01). Non-ambulatory patients (n = 29) demonstrated a stronger association between physical activity and left ventricular ejection fraction (rho = 0.40, p = 0.03) while ambulatory patients demonstrated a stronger association between physical activity and left ventricular cardiac index (rho = 0.53, p = 0.03). Ecc did not associate with physical activity in either cohort.

Physical activity correlates with left ventricular ejection fraction and left ventricular cardiac index and is modified by ambulation. Future analysis should assess the temporal relationship between physical activity and cardiomyopathy.

We sought to assess the degree to which environmental risk factors affect CHD prevalence using a case–control study.

A hospital-based study was conducted by collecting data from outpatients between January 2016 and January 2021, which included 31 CHD cases and 72 controls from eastern China. Risk ratios were estimated using univariate and multivariate logistic regression models and mediating effect analysis.

Residential characteristics (usage of cement flooring, odds ratio = 17.04[1.954–148.574], P = 0.01; musty smell, odds ratio = 3.105[1.198–8.051], P = 0.02) and indoor total volatile organic compound levels of participants’ room (odds ratio = 31.846[8.187–123.872, P < 0.001), benzene level (odds ratio = 7.370[2.289–23.726], P = 0.001) increased the risk of CHDs in offspring. And folic acid plays a masking effect, which mitigates the affection of the total volatile organic compound (indirect effect = -0.072[−0.138,-0.033]) and formaldehyde (indirect effect = −0.109[-0.381,-0.006]) levels on the incidence of CHDs. While food intake including milk (odds ratio = 0.396[0.16–0.977], P = 0.044), sea fish (odds ratio = 0.273[0.086–0.867], P = 0.028), and wheat (odds ratio = 0.390[0.154–0.990], P = 0.048) were all protective factors for the occurrence of CHDs. Factors including women reproductive history (history of conception control, odds ratio = 2.648[1.062–6.603], P = 0.037; history of threatened abortion, odds ratio = 2.632[1.005–6.894], P = 0.049; history of dysmenorrhoea (odds ratio = 2.720[1.075–6.878], P = 0.035); sleep status (napping habit during daytime, odds ratio = 0.856[0.355–2.063], P = 0.047; poor sleep quality, odds ratio = 3.180[1.037–9.754], P = 0.043); and work status (working time > 40h weekly, odds ratio = 2.882[1.172–7.086], P = 0.021) also influenced the CHDs incidence to differing degrees.

Diet habits, nutrients intake, psychological status of pregnant women, and residential air quality were associated with fetal CHDs. Indoor total volatile organic compound content was significantly correlated with CHDs risk, and folic acid may serve as a masking factor that reduce the harmful effects of air pollutants.

The Wolff–Parkinson–White pattern is a delta wave frequently detected in school-based cardiovascular screening programs in Japan. Although most children with Wolff–Parkinson–White pattern are asymptomatic, initial symptoms may include syncope or sudden death, necessitating accurate diagnosis and management. Delta waves can also indicate a fasciculoventricular pathway, which poses no risk and does not require management.

We reviewed the medical records of patients referred to our hospital between April 2008 and March 2022 to evaluate the electrocardiographic signs of the Wolff–Parkinson–White pattern. The existence of Wolff–Parkinson–White syndrome and fasciculoventricular pathway were determined based on atrioventricular block and QRS waveform changes after adenosine administration during sinus rhythm.

The study cohort included 127 children (65 males; median age: 12.8 years, resting heart rate: 75 beats/min, PR interval: 109 ms, and QRS duration: 101 ms). The adenosine administration test revealed a fasciculoventricular pathway, Wolff–Parkinson–White syndrome, and indeterminate findings in 64, 54, and 9 children, respectively. More than 60% of children with a QRS duration ≤ 120 ms had a fasciculoventricular pathway. Age ≤ 12 years, QRS duration >120 ms, and type A category (children with R/S ratios >1 in lead V1) were identified as independent risk factors for Wolff–Parkinson–White syndrome. No adverse events were observed in any child.

The adenosine administration test is safe and feasible for differentiating Wolff–Parkinson–White syndrome from fasciculoventricular pathways and can reduce the unnecessary management of children with fasciculoventricular pathways.

Neurodevelopmental disorders occur in up to 50% of children with CHD. Small head circumference at birth has been associated with impaired neurodevelopment in patients with complex CHD. It is unknown if patients with simple CHD such as septal defects have smaller head circumferences. The objective of this study was to investigate the head circumference at birth in neonates with either an atrial or a ventricular septal defect.

This study is part of the Copenhagen Baby Heart Study; a prospective, population-based cohort study of more than 25,000 neonates. The neonates were examined with a comprehensive transthoracic echocardiography within the first 30 days of birth including assessment for atrial or ventricular septal defects. The head circumference at birth in term neonates with septal defects was compared to the head circumference in matched controls, term neonates without septal defects from the same birth cohort.

Neonates with septal defects (n = 1,030; 45.2% male; mean birthweight 3,534g ± 483g) had a mean head circumference of 34.8 cm (95% confidence interval 34.7–34.9 cm), compared to neonates without septal defects (n = 5,150; 45.6% male; mean birthweight 3,546g ± 476g) of 34.7 cm (95% confidence interval 34.7–34.8 cm); p-value 0.07. Mean calculated z-score of head circumferences was 0.05 for neonates with septal defects and –0.01 for neonates without septal defects, p = 0.07. Dividing cases into neonates with atrial septal defects, ventricular septal defects, and those without septal defects did not show differences between groups, p = 0.14.

The head circumference in term neonates with septal defects did not differ from matched controls without septal defects.

We sought to examine the relative importance of surgical lesion complexity versus the presence of genetic/syndromic/extracardiac anomalies (GSAs) in determining survival, morbidity or need for reinterventions following repair for aortic arch hypoplasia.

A single-centre, retrospective cohort study of infants undergoing biventricular aortic arch repair via sternotomy from 2010 to 2021 was conducted. Survival analysis was performed using Kaplan–Meier methods, with additional Bayesian survival modelling for subgroups. Composite morbidity comprised respiratory, renal, neurologic, or sepsis-related complications.

Of 83 included infants, n = 13/83 (15.7%) had complex repairs; 27/83 (32.5%) were GSA+. Operative mortality was significantly higher in GSA+ versus GSA− patients (18.5% vs. 1.8%; p = 0.01), though not for complex versus non-complex repairs. Overall 10-year Kaplan–Meier survival was 86.7%. Bayesian modelling suggested equivalent post-discharge attrition in non-complex/GSA+ and complex/GSA− patients, with the poorest outcomes in complex/GSA+ patients; non-complex/GSA− patients had 100% survival. GSA+ patients exhibited higher composite morbidity (44.4% vs. 7.1% in GSA− p < 0.001), with their mode of death seemingly related to a high incidence of respiratory and neurological morbidity, notably in Dandy–Walker syndrome. The 10-year freedom from arch reinterventions was 87.7%; neither complexity, GSA status, nor post-repair peak arch velocity predicted the need for arch reinterventions.

Whilst anatomic complexity may have been somewhat neutralised as a risk factor for operative mortality, in contrast to GSA+ status, there is further post-discharge attrition attributable to complexity or GSA+ status, with additive risk effects. Morbidity directly related to certain syndromes underlies some of this risk. Non-anatomic substrates represent a persistent limitation to outcomes of surgical aortic arch repair in infants.

The interstage period is a critical phase for single ventricle infants due to their fragile cardiovascular state. Infants often experience medical and feeding challenges during this period, resulting in caregiver stress. We completed a quality improvement project at Children’s Healthcare of Atlanta to understand these challenges to inform targeted interventions.

This single-center project included a medical chart review and a cross-sectional caregiver survey. Data were collected on patient and caregiver demographics and clinical variables. Feeding outcomes were assessed using the Pediatric Functional Oral Intake Scale. Caregiver impact was measured using the Feeding/Swallowing Impact Survey.

The project included 15 single ventricle patients with a mean (standard deviation) age of 151.73(25.92) days at the time of the second-stage palliation. Forty percent of patients experienced at least one readmission, primarily due to feeding intolerance (20%) and desaturations (26.7%). Milk protein allergy (26.9%) was the most common medical complication, followed by interstage unplanned reinterventions. Pediatric Functional Oral Intake Scale scores demonstrated that 33% consumed minimal volumes or no oral intake at the time of the bidirectional Glenn, and 93.3% of patients did not receive outpatient feeding services during the interstage. Caregiver stress scores resulted in mean scores (standard deviation) of 2.23(1.54), with the highest impact on daily activities. All caregivers affirmed the need for a dedicated multidisciplinary clinic.

The interstage period for single ventricle patients poses significant medical and feeding challenges, resulting in caregiver stress. Comprehensive, multidisciplinary feeding support during the interstage period may improve patient outcomes and alleviate caregiver burden.

To enumerate the experience in different parameters, including demographic, anatomic, procedural, ICU care, and outcome of ductal stenting procedures in duct-dependent pulmonary circulation in a resource-limited setup.

Stenting the arterial duct emerged as an alternative to a variety of surgical interventions in the early 1990s. Ductal stenting in neonates with duct-dependent pulmonary circulation may maintain duct patency reliably for several months as an alternative to systemic-to-pulmonary shunts.

This is a retrospective review from a single centre with a developing paediatric cardiology unit and limited paediatric cardiac surgical scope. Eighty-nine symptomatic patients who needed stable ductal flow for adequate pulmonary circulation underwent the procedure from October 2018 to December 2022.

We had a procedural success rate of 98.8% (one case failed out of 89) and a successful discharge rate of 96.6% (85 discharged out of 88; 3 immediate mortalities). At the six-month follow-up, the success rate was 93% (6 mortalities in 88 patients). No patient underwent a rescue surgical shunt, but an elective Blalock-Taussig shunt was needed in 1 patient (1.1%). In the remaining 82 cases, 65 are now in follow-up, among them 22 already underwent single-ventricle palliation, and 8 underwent biventricular repair successfully.

Ductal stenting is less invasive well-accepted alternative for first-stage palliation. Our focus in this study is to practice this even in resource-limited settings as an alternative to surgical shunts with good short- and mid-term outcomes.

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.

Medical advances in the management of CHD have shifted the focus from childhood mortality to life-long morbidity; therefore, clinical research in paediatric cardiology tends to replace mortality outcomes with functional and patient-reported outcomes. Despite these advances, the stratification of disease severity using a simple and reproducible CHD classification has not been established. The aim was to determine which classification best predicts functional status in children with CHD, in terms of cardiopulmonary fitness.

This retrospective cohort study was assessed from a cohort study of 296 children (mean age 11.3 ± 3.1 years, 129 female), who underwent a baseline and final cardiopulmonary exercise tests with a mean follow-up of 4.1 ± 1.6 years.

Seven CHD classifications were identified, assessing anatomical, physiological, therapeutic, or functional parameters in foetal, paediatric, or adult CHD populations. The four-stage paediatric CHD disease severity classification established by Uzark et al. best predicted functional status at the final cardiopulmonary exercise test assessment, with an area under the receiver operating characteristics curves of 0.62 (0.55–0.69) for impaired cardiopulmonary fitness (maximum oxygen uptake (VO2max) or ventilatory anaerobic threshold < −1.64 Z-score). The best inter-judge classification agreement was also observed for Uzark classification, with a Kappa coefficient of 0.88 (0.78–0.98).

The CHD classification by Uzark is reliable in terms of functional status prediction and reproducibility in children with CHD. This simple classification may contribute to identifying children with CHD most at risk of cardiopulmonary fitness impairment and initiating early preventive cardiovascular interventions in paediatric cardiology.

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.

Vascular rings cause highly variable clinical presentations. This study assesses the impact of prenatal versus postnatal diagnosis on clinical outcomes.

We conducted a single centre retrospective review of isolated vascular ring patients (without significant CHD) from 2011 to 2022 and compared clinical and operative data between patients with prenatal and postnatal diagnoses.

Of 177 patients, 45% (N = 80) had prenatal diagnosis. Between 2018 and 2022, 78% had prenatal diagnosis compared to 41% from 2013 to 2018 and 4% before 2013 (p < 0.001). 76.3% (N = 135) had a right aortic arch with left ligamentum arteriosum, 22.6% (N = 40) had a double aortic arch, and 1.1% (N = 2) had a left aortic arch with right ligamentum arteriosum. Postnatal diagnosis patients were more likely to have preoperative respiratory symptoms (55.7%), medications (34.0%), or admissions (24.7%) (versus 32.5%, 10.0%, and 11.3% of the prenatal diagnosis patients, p < 0.05) and require surgical repair (68.0% versus 38.8% of prenatal diagnosis patients, p < 0.0001). 54.8% of patients had surgical repair; prenatal diagnosis patients were younger at surgery, 7.5 (3-11) months compared to 16.0 (5-18) months in the postnatal diagnosis patients (p = .0014). Double aortic arch patients were more likely to require surgical repair (90.0%, compared to 44.5% with right aortic arch, p < 1e−4). Postnatal diagnosis patients had more residual postoperative symptoms (40.9% versus 16.1% in prenatal diagnosis patients, p = 0.01).

Prenatal diagnosis of vascular rings improves clinical surveillance, resulting in earlier surgical repair in symptomatic patients and diminished morbidity. Higher risk double aortic arch patients should have a tailored evaluation pathway.

To evaluate the influence of pre-existing maternal diabetes mellitus on fetal myocardial performance index and systolic-to-diastolic duration ratio.

Prospective cohort study included 179 pregnant women between 20 and 36w6d, divided into 3 groups: Group 1 (120, normal), Group 2 (31, type 1 diabetes mellitus), and Group 3 (28, type 2 diabetes mellitus). Systolic-to-diastolic duration ratio was calculated as the sum of isovolumic contraction time and ejection time divided by the sum of isovolumic relaxation time and ventricular filling time. Spectral Doppler was used to assess left ventricle systolic-to-diastolic duration ratio. Tissue Doppler was used to assess right ventricular filling time. Using spectral Doppler, left ventricle myocardial performance index was calculated as the sum of isovolumic contraction time and isovolumic relaxation time divided by ejection time.

Pre-existing maternal diabetes mellitus had a significant influence on fasting glucose levels (p < 0.001), left ventricle isovolumic contraction time (p < 0.001), left ventricle ejection time (p = 0.025), and left ventricle myocardial performance index (p < 0.001). Group 2 had higher left ventricle isovolumic contraction time (0.036 vs. 0.031 sec, p = 0.001) and left ventricle myocardial performance index (0.487 vs. 0.453, p = 0.003) compared with Group 1. Group 3 showed higher left ventricle myocardial performance index (0.492 vs. 0.449, p = 0.006) and lower left ventricle ejection time (0.161 vs. 0.169 sec, p = 0.038) than Group 1. Left ventricle systolic-to-diastolic duration (p = 0.704), right ventricle systolic-to-diastolic duration ratio’ (p = 0.757), left ventricle isovolumic contraction time (p = 0.163), left ventricle ejection time (p = 0.093), and left ventricle myocardial performance index (p = 0.087) were not useful parameters in predicting composite neonatal outcomes.

Pre-existing maternal diabetes mellitus had significant influence on fetal left ventricle myocardial performance index, but no effect on systolic-to-diastolic duration ratio. Systolic-to-diastolic duration ratio was not useful in predicting adverse perinatal outcomes.

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.