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.

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.

Physiologic data streaming and aggregation platforms such as Sickbay® and Etiometry are becoming increasingly used in the paediatric acute care setting. As these platforms gain popularity in clinical settings, there has been a parallel growth in scholarly interest. The primary aim of this study is to characterise research productivity utilising high-fidelity physiologic streaming data with Sickbay® or Etiometry in the acute care paediatric setting.

A systematic review of the literature was conducted to identify paediatric publications using data from Sickbay® or Etiometry. The resulting publications were reviewed to characterise them and identify trends in these publications.

A total of 41 papers have been published over 9 years using either platform. This involved 179 authors across 21 institutions. Most studies utilised Sickbay®, involved cardiac patients, were single-centre, and did not utilise machine learning or artificial intelligence methods. The number of publications has been significantly increasing over the past 9 years, and the average number of citations for each publication was 7.9.

A total of 41 papers have been published over 9 years using Sickbay® or Etiometry data in the paediatric setting. Although the majority of these are single-centre and pertain to cardiac patients, growth in publication volume suggests growing utilisation of high-fidelity physiologic data beyond clinical applications. Multicentre efforts may help increase the number of centres that can do such work and help drive improvements in clinical care.

Implantable haemodynamic monitors allow remote monitoring of Fontan circulation. We report unique opportunities and challenges related to device use in rural, high-altitude regions.

Assess the performance of implantable haemodynamic monitor in Fontan circulation and identify potential sources of measurement discrepancy defined as non-physiological, negative, or significantly lower reading than baseline.

We performed a retrospective review of patients who underwent implantable haemodynamic monitor implantation from September 2021 to April 2024 (n = 17) at our centre (∼1,000 feet above sea level; ASL) and identified those with sensor discrepancies.

During a mean follow-up duration of 26 months (range 13–44 months), there were no procedure-related complications, thromboembolism, or device displacement. Ten patients lived in rural, higher-altitude regions (average altitude 5100 feet above sea level, average distance from centre ∼160 miles, range = 100–400 miles). Challenges in remote monitoring included unreliable home-internet connection, non-compliance, and difficulty performing device recalibration at patient’s home altitude. Sensor discrepancies were noted in 7 patients (41%), of whom 6 (86%) lived remotely. Manual review of the waveforms identified sources of discrepancy, including misinterpretation of the non-pulsatile pressure waveform (n = 3), offset due to change in hospital-interrogation unit (n = 4), and sensor drift (n = 1). Altitude change did not directly affect sensor performance. We were able to apply corrective interventions in 4/7 sensors, including Fontan-specific settings (overriding pulsatility), and back-end recalibration, which were effective in improving device accuracy.

Implantable haemodynamic monitors are a promising tool for monitoring Fontan circulation but may require modified settings and careful attention to potential interpretation errors. Home monitoring remains challenging for rural, high-altitude residents with limited resources.

Chronic pulmonary regurgitation following tetralogy of Fallot repair burdens the right ventricle. This study evaluated early outcomes of pulmonary valve reconstruction using right atrial tissue versus standard transannular patch repair.

A retrospective analysis of 412 tetralogy of Fallot patients (2014–2024) was conducted: Atrial Valve Group (n = 205) underwent valve reconstruction; No-Valve Group (n = 207) received standard repair. Patients were followed for 1 year with echocardiographic assessments. Outcomes included right ventricular insufficiency, ventilation duration, and ICU stay.

Atrial Valve Group had lower right ventricular insufficiency at 12 months (9.3% vs. 19.8%, p = 0.004, OR = 2.39, 95% CI: 1.32–4.33), shorter ventilation times (6.1 vs. 18.0 hours, p < 0.001, Cohen’s d = 3.54), and reduced ICU stays (3.0 vs. 5.7 days, p < 0.001, Cohen’s d = 1.87), despite longer CPB durations (47.1 vs. 40.5 minutes, p = 0.02).

Right atrial tissue reconstruction reduces early and intermediate-term right ventricular dysfunction post-tetralogy of Fallot repair. Long-term studies are needed.

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.

Danon disease is an X-linked disorder caused by variants in the lysosome-associated membrane protein-2 (LAMP2) gene located on Xq24. Due to its inheritance in an X-chromosome dominant manner, males typically experience more severe manifestations than females.

The whole exome sequencing was conducted on a cohort of 218 children diagnosed with hypertrophic cardiomyopathy; four children with hypertrophic cardiomyopathy carrying the LAMP2 variants were diagnosed at our centre. Variants in the LAMP2 gene were summarised, and their pathogenicity and conservation were analysed using bioinformatics methods. A retrospective analysis of genotype-phenotype associations was also conducted in conjunction with previously reported cases.

Four patients with Danon disease were diagnosed in our single centre by gene sequencing; they all presented with myocardial hypertrophy as the initial manifestation. Both male patients manifested symptoms from infancy, while disease onset in the two female cases occurred below the average age reported for females. Through gene sequencing, a total of four variants were identified in these four patients, including one splicing variant: c.865-1G>C, one loss of heterozygosity variant: loss1 exon:4-9), one frameshift variant: c.973delG(p.(L325Wfs×21)), and one stop codon variant: c.467T>G(p.(L156*)).

This study identified four patients with LAMP2 gene variants, thereby enriching the documented genetic landscape of LAMP2-associated disorders. Bioinformatics analyses corroborated the pathogenicity of these variants. Additionally, we emphasised that women with suspected Danon disease should be thoroughly evaluated, and the possibility of implantable cardioverter defibrillator implantation and heart transplantation should be considered and discussed as early as possible.

Describe the hemodynamic implications of anaesthetic choice among children with heart disease undergoing cardiac catheterisation.

Study 1 was a secondary analysis of data obtained during catheterisation-based hemodynamic assessment of infants with hypoplastic left heart syndrome following Stage 1 palliation, randomised in the Single Ventricle Reconstruction trial. Measured and calculated hemodynamics including pulmonary and systemic vascular resistance indexed to body surface area (PVRi and SVRi respectively) and pulmonary/systemic blood flow (Qp/Qs) were analysed with respect to anaesthetic employed during catheterisation, classified as moderate sedation or general anaesthesia. Study 2 consisted of a single centre, prospective analysis of patients requiring percutaneous closure of a patent ductus arteriosus or endomyocardial biopsy after orthotopic heart transplant. Participants underwent hemodynamic assessment first using inhaled volatile anaesthesia (IA), and then transitioned to total intravenous anaesthesia, comparing hemodynamic measures with respect to anaesthetic approach.

In Study 1, independent of shunt type, PVRi, and patient size, moderate sedation was associated with a greater than two-fold odds of a Qp/Qs >1 (OR 2.12, 95%CI 1.18–3.87, p = 0.013). In Study 2, while PVRi was similar, SVRi was significantly higher using total intravenous anaesthesia. Among the patent ductus arteriosus subgroup, Qp/Qs increased significantly with a total intravenous anaesthesia relative to IA (p = 0.003); additionally, among the orthotopic heart transplant subgroup, left ventricular end diastolic pressure increased following a transition to total intravenous anaesthesia (p = 0.002).

Analyses of hemodynamics during catheterisation support a significant impact of anaesthetic type on hemodynamic values including SVRi, left ventricular end diastolic pressure, and Qp/Qs. Anaesthesia choice and intraprocedural management of SVRi are important considerations when making clinical decisions based on hemodynamic data.

Single ventricle CHD requires lifelong care, yet its broader impact on patients and families remains unclear. Engaging patients in care improvement can strengthen relationships and outcomes.

This study evaluates how individuals with single ventricle CHD prioritise gaps in care based on personal and family impact.

Using Mery et al.’s identified care gaps, a survey was distributed to parents of children with single ventricle CHD and adults with single ventricle CHD in English or Spanish. Participants rated each gap from 1(not important) to 10(extremely important), with a “Not Applicable” option. Responses were analysed using median, weighted, and total rating scores. Sociodemographic data were examined, and univariate analysis and a race/ethnicity and insurance matrix were conducted on parent responses.

Among 36 complete responses, 30(83.3%) were parents and 6(16.7%) patients. Most parents were female(29,96.7%), White non-Hispanic(24,80.0%), with 17(6.7%) having privately insured children. Median child age was 6.5[interquartile range: 3.0–12.8] years, and 55.3% had Hypoplastic Left Heart Syndrome. The highest-rated gap was “Uncertainty of prognosis in adulthood” (9.5[interquartile range: 8.0–10.0]). The lowest was “Pregnancy termination presented repeatedly” (1.0[interquartile range: 1.0–7.0]). Non-White parents rated “Transition to adult healthcare” (p = 0.017) and “Navigating resources” (p = 0.037) higher. Patients (median age 33.0 years) prioritised “Rescheduling surgical procedures” and “Transition to adult healthcare” (both 10.0). “Support in family planning” had the highest total rating score(12). The lowest-rated was “Limited guidance on transition to adolescence” (0.0[interquartile range:0.0–0.0]).

Patients and families prioritise care gaps differently. Aligning their perspectives with clinical expertise can guide tailored solutions to improve outcomes for single ventricle CHD patients.

The limited efficacy of monotherapy and the insufficient clinical experience with triple therapy (levosimendan, dapagliflozin, and sacubitril/valsartan) warrant further investigation. The aim of this study was to evaluate the effects of triple therapy on left ventricular function in children with advanced heart failure whose left ventricular function had not improved despite classical heart failure treatment and who remained dependent on inotropes.

The study included children who were admitted to the hospital with advanced heart failure and who were still inotrope-dependent at a mean of 42 days after the start of classical heart failure treatment and then started triple therapy at our hospital.

The study included 18 patients, 8 (44%) males, with a median age of 4 years (2–7 years). Before and after classical treatment and after triple treatment, statistically significant improvement in two-dimensional left ventricular ejection fraction (%) (median values 30; 38; 55, respectively), left ventricular end-diastolic diameter (median values 44; 45; 40 mm), left ventricular end-systolic diameter (median values 38; 36; 29 mm), left ventricular end-diastolic diameter (z score) (median values 4.2; 3.2; 2.7), left ventricular end-systolic diameter (z score) (median values 5.8; 4.8; 3.2), Simpson left ventricular ejection fraction (%) (median values 29; 36.5; 55), Simpson left ventricular end-diastolic volume (median values 60; 55; 43 ml), left ventricular end-systolic volume (median values 43; 40; 18. 5 ml), left ventricular global longitudinal strain four-chamber (median values -8.1;-10;-19), left ventricular global longitudinal strain three-chamber (median values -5.9;-8.9;-14), and left ventricular global longitudinal strain mean (median values -6.9;-9.7;-19) values was observed (all values p < 0.05).

In children admitted to the hospital with advanced heart failure whose left ventricular function has not improved with classical therapy, it seems likely that both left ventricular systolic and diastolic function will improve, inotrope dependency will resolve, and patients can be discharged with the new triple drug therapy.

To evaluate feasibility, safety, and short-term outcome of transcatheter closure of ventricular septal defect with aortic cusp prolapse with or without mild aortic regurgitation.

All data were collected prospectively for all ventricular septal defect with aortic cusp prolapse with or without mild aortic regurgitation who were attempted for transcatheter device closure between January 2018 and December 2023.

The device closure was successful and not associated with appearance of new-onset aortic regurgitation or aggravation of existing trivial to mild aortic regurgitation in 92.6% cases. In 2 patients (2.9%), device appeared to be touching the aortic valve and aggravating aortic regurgitation even after repositioning and re-deploying the device and ultimately that devices were taken out and sent for surgical closure. In 1 patient, device position appeared to be perfect on table before release, aortic regurgitation was same as before, and aortic root angiogram was also satisfactory. But re-evaluation on the next day by echocardiography revealed aggravating aortic regurgitation. The patient was sent for removal of device and surgical ventricular septal defect closure. In 2 patients (2.9%), device was embolised few hours after release, and the patients were sent for surgical closure. All patients were followed up for minimum of 6 months, and no case was found with new-onset aortic regurgitation or aggravation of existing aortic regurgitation.

Transcatheter closure of ventricular septal defect with aortic cusp prolapse with or without mild aortic regurgitation in selected patients is technically feasible and safe with high procedural success rate.

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.

To evaluate whether landiolol combined with amiodarone improves heart rate and rhythm control compared to amiodarone alone in paediatric patients with postoperative junctional ectopic tachycardia after surgery for congenital heart defect.

We retrospectively identified 24 cases of junctional ectopic tachycardia among 962 children who underwent surgery for congenital heart defects at the German Paediatric Heart Centre between January 2022 and June 2024. Patients received either amiodarone monotherapy or a combination of landiolol and amiodarone. Time to heart rate control and rhythm normalisation, haemodynamic stability, and adverse events were assessed.

Patients who received amiodarone and landiolol achieved faster heart rate control than patients who received amiodarone alone (median 6.7 vs. 14.7 h, p = 0.02, Cohen’s d = 1.05; large effect). Among patients who received landiolol first, control was reached even earlier (2.4 vs. 8 h, p = 0.05, Cohen’s d = 1.49; very large effect). A significant heart rate reduction occurred within 40–120 min after landiolol initiation (mean difference: −23.7 bpm, 95% CI: −45.4 to −1.9, p = 0.04, r = 0.45; medium effect), while no significant effect was observed in patients who received amiodarone alone. Haemodynamic parameters remained stable, although hypotension requiring discontinuation occurred in 11.1% of Landiolol-treated patients.

In this retrospective analysis, combined landiolol and amiodarone therapy demonstrated a shorter time to heart rate control compared to amiodarone alone, especially when landiolol was initiated first. These findings require confirmation in prospective studies.