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.

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.

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.

Risk stratification is recommended for patients with ventricular pre-excitation, particularly when sports eligibility is required. Few studies have examined the changes in the electrophysiological properties of the accessory pathway during growth. This study investigates the evolution of electrophysiological properties of the ventricular pre-excitation in young athletes referred for sports eligibility.

Between January 2011 and July 2022, 44 paediatric patients (32 males; mean age, 10 ± 2.42) with ventricular pre-excitation underwent an electrophysiological study, both at rest and during adrenergic stress at two different times (T0 and T1) within a minimal interval of 2 years. Transcatheter ablation was not performed between the two electrophysiological studies. Electrophysiological data were collected and compared.

Electrophysiological study under basal conditions showed a significant decrease in the anterograde accessory pathway effective refractory period and 1:1 conduction over the accessory pathway from T0 to T1. The shortest pre-excited R-R interval during atrial fibrillation did not significantly change at the basal condition; however, it decreased during the stress test. Furthermore, six patients (13.6%) changed the risk profile of their accessory pathway: two “high-risk” patients at T0 became “low-risk” and four “low-risk” patients became “high-risk” at T1. Atrioventricular re-entry tachycardia inducibility did not differ significantly between the two electrophysiological studies.

This study highlights the importance of repeating electrophysiological study (transesophageal or intracardiac) in paediatric athletes with ventricular pre-excitation because significant and clinically relevant changes in the conduction and refractoriness of accessory pathway can occur. This could influence risk stratification for sports eligibility and the correct indication and timing for accessory pathway ablation.

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.

Cases of Fontan failure with normal Fontan pressure have been reported. This study aimed to identify catheterisation-derived haemodynamic predictors of heart transplantation/death, other than Fontan pressure, in late post-Fontan patients.

This retrospective study evaluated post-Fontan patients who underwent cardiac catheterisation at age ≥10 years between 1993 and 2018. The predictive effect of cardiac index-systemic vascular resistance index plot and perfusion pressure on freedom from the primary outcome (heart transplantation/death) was evaluated. Patients were categorised into haemodynamic categories A (cardiac index ≥ 3, systemic vascular resistance index ≥ 13), B (cardiac index < 3, systemic vascular resistance index ≥ 13), C (cardiac index ≥ 3, systemic vascular resistance index < 13), and D (cardiac index < 3, systemic vascular resistance index < 13).

In total, 79 patients (median age: 15.7 [range: 10.1–50.2] years) were included; of them, the primary endpoint occurred in 10 (13%; median follow-up: 1.9 [range: 0.1–18.8] years). Category C patients had significantly shorter freedom from the endpoint than categories A and B patients. Univariate analysis identified significant haemodynamic predictors, including Fontan pressure, pulmonary/systemic vascular resistance index, pulmonary/systemic flow, systemic arterial oxygen saturation, systemic venous oxygen saturation, systemic vascular resistance index, perfusion pressure, perfusion pressure < 53 mmHg, and category C. In multivariable analysis, perfusion pressure < 53 mmHg and category C emerged as predictors of heart transplantation/death alongside Fontan pressure.

Haemodynamic profiling of late post-Fontan patients using the cardiac index-systemic vascular resistance index plot can aid to comprehend the post-Fontan status and predict clinical prognosis.

Northwest axis on an electrocardiogram is conventionally thought to be associated with CHD, but there is a paucity of data regarding the types and incidence of CHD associated with this finding. The purpose of this study was to report the types and incidence of CHD found at our institution to determine the efficacy of electrocardiograms as a screening test in infants.

Retrospective, single-centre study of all infants ≤ 60 days of age who underwent a first-time electrocardiogram from 2015 to 2021. Our inclusion criteria included those found to have a northwest axis on electrocardiogram and who also had an echocardiogram performed. Data were described using standard summary statistics.

Two hundred sixty-eight infants met inclusion criteria (median age 4 days, interquartile range 2–24). The most common reason for an electrocardiogram to be obtained was the presence of a heart murmur (n = 102, 38%). One hundred thirty-one infants (49%) were found to have significant CHD. Cardiac defects included ventricular septal defect (n = 36, 28%), severe pulmonary valve stenosis (n = 17, 13%), atrioventricular canal defect (n = 16, 12%), and single ventricle disease (n = 16, 12%).

In this study, nearly half (49%) of these infants with northwest axis on electrocardiogram were found to have significant CHD. Electrocardiograms remain an important screening tool for infants with findings associated with potential cardiac disease and echocardiogram is warranted for infants found to have northwest axis on a first-time electrocardiogram.

Dysphagia is common in infants born with critical CHD. Thickened liquids are often used to treat dysphagia, but associated risks limit widespread use among feeding specialists. This survey aims to assess dysphagia treatment patterns and thickened liquid use across paediatric cardiac surgical centres.

A 24-question, cross-sectional survey. Convenience and snowball sampling methods were used to engage 52 paediatric cardiac surgical centres affiliated with the Cardiac Newborn Neuroprotective Network. Descriptive statistics were used to analyse and compare responses.

Twenty-six individual respondents represented 21 unique paediatric cardiac surgical centres. Most responses were from experienced, speech–language pathologists (78%) at medium size centres (88%). Ninety-three percent of responding centres used thickened liquids to treat dysphagia and 81% only after formal instrumental assessment of swallowing. Thickened oral feeding was used for single-ventricle patients by 85% versus 69% for two-ventricle patients. Barriers to recommending thickened oral feedings included the cost of thickening agents, parental non-adherence, and gastrointestinal concerns.

This is the first survey to report multi-institutional dysphagia treatment practice variation at United States congenital cardiac surgical centres. Thickened oral feedings are frequently used across centres in high-risk critical CHD patients but treatment benefit remains unclear. This survey highlights a broad scientific community poised to direct dysphagia research in critical CHD to address practice variation, short- and long-term impact of thickened oral feeding on feeding outcomes, and barriers to use and access of thickening agents.

Paediatric patients with heart failure requiring ventricular assist devices are at heightened risk of neurologic injury and psychosocial adjustment challenges, resulting in a need for neurodevelopmental and psychosocial support following device placement. Through a descriptive survey developed in collaboration by the Advanced Cardiac Therapies Improving Outcomes Network and the Cardiac Neurodevelopmental Outcome Collaborative, the present study aimed to characterise current neurodevelopmental and psychosocial care practices for paediatric patients with ventricular assist devices.

Members of both learning networks developed a 25-item electronic survey assessing neurodevelopmental and psychosocial care practices specific to paediatric ventricular assist device patients. The survey was sent to Advanced Cardiac Therapies Improving Outcomes Network site primary investigators and co-primary investigators via email.

Of the 63 eligible sites contacted, responses were received from 24 unique North and South American cardiology centres. Access to neurodevelopmental providers, referral practices, and family neurodevelopmental education varied across sites. Inpatient neurodevelopmental care consults were available at many centres, as were inpatient family support services. Over half of heart centres had outpatient neurodevelopmental testing and individual psychotherapy services available to patients with ventricular assist devices, though few centres had outpatient group psychotherapy (12.5%) or parent support groups (16.7%) available. Barriers to inpatient and outpatient neurodevelopmental care included limited access to neurodevelopmental providers and parent/provider focus on the child’s medical status.

Paediatric patients with ventricular assist devices often have access to neurodevelopmental providers in the inpatient setting, though supports vary by centre. Strengthening family neurodevelopmental education, referral processes, and family-centred psychosocial services may improve current neurodevelopmental/psychosocial care for paediatric ventricular assist device patients.

This study aimed to evaluate veno-venous collaterals between bidirectional cavopulmonary shunt and total cavopulmonary connection.

Patients who underwent staged total cavopulmonary connection between 1995 and 2022 were reviewed. Veno-venous collaterals between bidirectional cavopulmonary shunt and total cavopulmonary connection were depicted using angiograms. The prevalence of veno-venous collaterals, the risks for the development of veno-venous collaterals, and the impact of veno-venous collaterals on outcomes were analysed.

In total, 586 patients were included. Veno-venous collaterals were found in 72 (12.3%) patients. Majority of veno-venous collaterals originated from the superior caval vein and drained into the inferior caval vein. Before bidirectional cavopulmonary shunt, mean pulmonary artery pressure (16.3 vs. 14.5 mmHg, p = 0.018), and trans-pulmonary gradient (9.5 vs. 8.0 mmHg, p = 0.030) were higher in patients with veno-venous collaterals compared to those without. Veno-venous collaterals intervention was performed in 32 (5.5%) patients, in a median of 29 (16–152) days after bidirectional cavopulmonary shunt. Before total cavopulmonary connection, pulmonary artery pressure (10.3 vs. 9.4 mmHg, p = 0.015) and ventricular end-diastolic pressure (8.4 vs. 7.6 mmHg, p = 0.035) were higher, and arterial oxygen saturation (SaO2, 80.6 vs. 82.6 %, p = 0.018) was lower in patients with veno-venous collaterals compared to those without. More palliations before total cavopulmonary connection (p < 0.001, odds ratio: 1.689) were an independent risk for the development of veno-venous collaterals. Veno-venous collaterals did not affect survival after total cavopulmonary connection (92.8 vs. 92.7% at 10 years, p = 0.600).

The prevalence of veno-venous collaterals between bidirectional cavopulmonary shunt and total cavopulmonary connection was 12%. Veno-venous collaterals may be induced by the elevated pulmonary artery pressure and trans-pulmonary gradient, and also by more previous palliations. However, they had no impact on clinical outcomes following total cavopulmonary connection.

To retrospectively assess the suitability of pulmonary artery banding as a treatment strategy for dilated cardiomyopathy and left ventricular non-compaction cardiomyopathy with depressed left ventricular ejection fraction.

The study was retrospective and included consecutive patients who met the inclusion criteria: diagnosed with dilated cardiomyopathy or left ventricular non-compaction cardiomyopathy and left ventricular ejection fraction less than 35%. Cardiac indices were documented, and clinical outcomes were followed for 5 years.

This study included 21 patients with depressed left ventricular ejection fraction due to dilated cardiomyopathy (n = 11) or left ventricular non-compaction cardiomyopathy (n = 10), treated either with anti-congestion medication alone or in combination with pulmonary artery banding. The groups treated with pulmonary artery banding showed significant improvement in left ventricular ejection fraction compared to controls (ANOVA, p = 0.0002), with no major adverse events. In the subgroup with left ventricular non-compaction, pulmonary artery banding led to significant improvement of the left ventricular ejection fraction (p = 0.00002) and significant reductions in the Z scores of left ventricular end-diastolic diameter (p = 0.0002) and of end-diastolic volume (p = 0.004).

Pulmonary artery banding appears to be a viable strategy for improving heart function in patients with non-compaction and dilated cardiomyopathy and depressed left ventricular ejection fraction. While pulmonary artery banding demonstrated more pronounced benefits in the subgroup with non-compaction cardiomyopathy, significantly enhancing cardiac restoration indices throughout the follow-up period, warranting further investigation in larger studies.

This research seeks to ascertain the prevalence and determinants of mirror-image dextrocardia in fetuses

With December 2022 as the reference point, we compiled colleted data on pregnant women who carried fetuses with mirror-image dextrocardia in Xi’an, Shaanxi Province: September–October 2022, November 2022, and December 2022–January 2023. An online questionnaire was distributed to 209 pregnant across China who had contracted COVID-19. The case group comprised women whose final menstrual cycle occurred in November 2022 and who had a fetus with mirror-image dextrocardia. Women with a November 2022 final menstrual period and a fetus without this condition made up the control group. To identify the risk factors associated with fetal mirror-image dextrocardia, both univariate and multivariate logistic regression analyses were employed.

A significant difference was noted in the gestational age at COVID-19 infection women with a September to October 2022 and December 2022 to January 2023 final menstrual period who did not bear a fetus with mirror-image dextrocardia, and those with a November 2022 final menstrual period whose fetus exhibited this condition. The univariate and multivariate analyses conducted on pregnant women with a final menstrual period in November 2022 who had contracted COVID-19 revealed significant differences in the presence and duration of fever between those bearing fetuses with mirror-image dextrocardia and those without (P = 0.000).

The findings suggest two critical factors to the increased prevalence of fetal mirror-image dextrocardia: 1) the infection timing which occurs between the 4th and 6th week of pregnancy and 2) the presence of fever and its prolonged duration.

To examine feasibility, acceptability, and preliminary effectiveness of a novel group-based telemedicine psychoeducation programme aimed at supporting psychological well-being among adolescents with Fontan-palliated CHD.

A 5-week telemedicine psychoeducation group-based programme (WE BEAT) was developed for adolescents (N = 20; 13–18 years) with Fontan-palliated CHD aimed at improving resiliency and psychological well-being. Outcome measures included surveys of resilience (Connor–Davidson Resilience Scale), benefit finding (Benefit/Burden Scale for Children), depression, anxiety, peer relationships, and life satisfaction (National Institutes of Health Patient-Reported Outcomes Measurement Information System scales). Within-subject changes in these outcomes were compared pre- to post-intervention using Cohen’s d effect size. In addition, acceptability in the form of satisfaction measures and qualitative feedback was assessed.

Among eligible patients reached, 68% expressed interest in study participation. Of those consented, 77% have been scheduled for a group programme to date with 87% programme completion. Twenty adolescents (mean age 16.1 ± SD 1.6 years) participated across five WE BEAT group cohorts (range: 3–6 participants per group). The majority (80%) attended 4–5 sessions in the 5-session programme, and the median programme rating was a 9 out of 10 (10 = most favourable rating). Following WE BEAT participation, resiliency (d = 0.44) and perceptions of purpose in life increased (d = 0.26), while depressive symptoms reduced (d = 0.36). No other changes in assessed outcome measures were noted.

These findings provide preliminary support that a group-based, telemedicine delivered psychoeducation programme to support psychological well-being among adolescents with CHD is feasible, acceptable, and effective. Future directions include examining intervention effects across diverse centres, populations, and implementation methods.

Transcatheter closure of large and complex atrial septal defect can pose challenges and complications during device placement. To improve stability, several assistive techniques have been developed.

This retrospective study evaluated the efficacy of the device-assisted device closure technique for large secundum atrial septal defects. Patients who underwent device-assisted device closure of atrial septal defect between December 2023 and August 2024 were analysed.

Twenty patients (mean age 38.69 years) underwent device closure of large secundum atrial septal defect with device-assisted device closure technique. The mean atrial septal defect diameter was 31.9 mm. The average thick-to-thick measurement was 38.3 mm, which determined the device size. The majority (18 cases) had thin, floppy margins and two had deficient inferior rim. Successful closure was achieved in 18 patients (90%), while two patients (10%) required other methods of assistance. Based on fluoroscopic guidance, patients were divided into two groups: Group A (8 patients) used anteroposterior projection, and Group B (12 patients) used left anterior oblique-cranial view. After initial two failures with anteroposterior view, all cases were successfully closed using left anterior oblique-cranial projection. Device sizes ranged from 36 to 50 mm (median 40 mm). Cocoon devices were used for sizes up to 42 mm, and Occlunix for larger devices. No significant procedural complications occurred, although two patients had minor post-procedural events.

Device-assisted device closure technique offers a promising and safe dynamic assistance approach for transcatheter closure of large and challenging atrial septal defects. The left anterior oblique-cranial view showed promising results, though without statistical significance. While results are encouraging, larger prospective studies are needed to validate its effectiveness.

This study aims to examine the surgical outcome of Kabuki syndrome patients after neonatal congenital heart surgery.

This was a single-centre retrospective study of Kabuki syndrome patients undergoing neonatal congenital heart surgery from 2018 to 2023. Primary outcome was survival to discharge after index surgery. Secondary outcomes were morbidities and complications. Survival and hospital length of stay were compared to neonates with non-Kabuki genetic anomalies undergoing congenital heart surgery in the same time period.

A total of seven patients were reviewed. All Kabuki syndrome patients had left-sided lesions including three with hypoplastic left heart syndrome, three with aortic stenosis and/or aortic arch hypoplasia, and one with an isolated coarctation of aorta. Hospital survival was 5/7 (71% compared to 88% for neonates with non-Kabuki genetic anomalies). To date, four remain alive, including one with hypoplastic left heart syndrome. A higher percentage of Kabuki syndrome patients had unplanned interventions (43% vs 15% in non-Kabuki), abnormal brain imaging (29% vs 5%), and bacteremia (29% vs 9%). Median total ventilator days for Kabuki patients were also longer (16 days vs 6 days in non-Kabuki) as was hospital length of stay (66 days vs 41 days).

Despite survival to discharge after index operation, Kabuki syndrome patients with single ventricle physiology remain at high risk of mortality and morbidity after cardiac surgery. However, they may be discharged without ventilator dependency and survive to toddler years.

Alternate electrocardiogram acquisition with fewer leads lacks systematic evaluation in children. This study aims to determine if electrocardiograms with fewer leads maintain diagnostic accuracy in paediatrics.

This is a single-centre review of 200 randomly selected standard 12-lead electrocardiograms from our hospital database (2017–2020) for patients aged 2 weeks to 21 years. An overlay technique generated 8-lead (limb + V1/V6) and 6-lead (limb only) variations of the 12-lead tracings, resulting in a total of 600 electrocardiograms, which were then interpreted by two independent paediatric electrophysiologists.

In total, 18% (35/200) of the baseline electrocardiograms were abnormal. Intervals were measured in lead II for all electrocardiograms. Comparing 12-lead to 6- and 8-lead electrocardiograms, there was almost perfect agreement for specific rhythm identification (97.5–100%, κ 0.85-1). The 8-lead showed substantial agreement with 12-lead electrocardiograms when identifying specific electrocardiogram patterns (97.5–100%, κ 0.66–1). A similar degree of agreement was not demonstrated with the 6-lead variant. Utilising the 12-lead electrocardiogram as the gold standard, sensitivity and specificity of the 8- and 6-lead electrocardiogram were > 89% for specific rhythm identification. Specificity for specific pattern recognition was > 99% while sensitivity was < 90% for certain variables for both 6- and 8-lead electrocardiogram, likely due to smaller sample size and fewer abnormal electrocardiograms. There was high percent reader agreement (92.5–100%).

8-lead electrocardiograms provide comparable diagnostic accuracy to 12-lead electrocardiograms for children. This information holds potential for future technological advancements in electrocardiogram acquisition tailored specifically for paediatrics. Additional studies are required to further refine conventional electrocardiogram acquisition.