Pulmonary atresia with ventricular septal defect (PA/VSD) is a complex cyanotic CHD that requires an early diagnosis for optimal management and outcomes. The purpose of this study is to evaluate the effectiveness of an inter-hospital management protocol utilising the prenatal CHD diagnosis for achieving favourable postnatal outcomes in PA/VSD patients in Vietnam.

We described the protocol implemented between two tertiary medical settings in Vietnam for the prenatal diagnosis and postnatal management of PA/VSD infants. All PA/VSD patients with prenatal diagnosis between January 2016 and December 2022 were retrospectively reviewed. The primary outcome was postnatal survival, and the secondary outcome was the presence of major morbidities such as bleeding or the need for Extra Corporeal Membrane Oxygenation (ECMO) after total repair.

During the study period, 35 PA/VSD patients were identified including 29 infants who underwent surgical correction utilising a valved conduit and 6 infants who are still waiting for the next evaluation after the palliative surgery. No death prior to the surgery occurred. For 29 patients, one hospital death happened, two patients required ECMO initially in their postoperative course but both survived, one late mortality due to pneumonia, and three reoperations were due to conduit failure. In the mean follow-up time of 2.92 (0.51–7.92) years, all survivors had completed follow-up.

Our protocol including a multidisciplinary management and a close follow-up has shown promising short-term results in achieving favourable postnatal outcomes for PA/VSD patients.

Extubation failure after neonatal cardiac surgery is associated with increased intensive care unit length of stay, morbidity, and mortality. We performed a quality improvement project to create and implement a peri-extubation bundle, including extubation readiness testing, spontaneous breathing trial, and high-risk criteria identification, using best practices at high-performing centers to decrease neonatal and infant extubation failure by 20% from a baseline of 15.7% to 12.6% over a 2-year period.

Utilising the transparency of the Pediatric Cardiac Critical Care Consortium database, five centres were identified as high performers, having better-than-expected neonatal extubation success rates with the balancing metric of as-expected or better-than-expected mechanical ventilation duration. Structured interviews were conducted with cardiac intensive care unit physician leadership at the identified centers to determine centre-specific extubation practices. Data from those interviews underwent qualitative content analysis which was used to develop a peri-extubation bundle. The bundle was implemented at a single-centre 17-bed cardiac intensive care unit. Extubation failure, defined as reintubation within 48 hours of extubation for anything other than a procedure, ventilator days and bundle compliance was tracked.

There was a 41.4% decrease in extubation failure following bundle implementation (12 failures of 76 extubations pre-implantation; 6 failures of 65 extubations post-implementation). Bundle compliance was 95.4%. There was no difference in ventilator days (p = 0.079) between groups.

Implementation of a peri-extubation bundle created from best practices at high-performing centres reduced extubation failure by 41.4% in neonates and infants undergoing congenital heart surgery.

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.

To evaluate the motor proficiency, identify risk factors for abnormal motor scores, and examine the relationship between motor proficiency and health-related quality of life in school-aged patients with CHD.

Patients ≥ 4 years old referred to the cardiac neurodevelopmental program between June 2017 and April 2020 were included. Motor skills were evaluated by therapist-administered Bruininks-Oseretsky Test of Motor Proficiency Second-Edition Short Form and parent-reported Adaptive Behavior Assessment System and Patient-Reported Outcomes Measurement Inventory System Physical Functioning questionnaires. Neuropsychological status and health-related quality of life were assessed using a battery of validated questionnaires. Demographic, clinical, and educational variables were collected from electronic medical records. General linear modelling was used for multivariable analysis.

The median motor proficiency score was the 10th percentile, and the cohort (n = 272; mean age: 9.1 years) scored well below normative values on all administered neuropsychological questionnaires. In the final multivariable model, worse motor proficiency score was associated with family income, presence of a genetic syndrome, developmental delay recognised in infancy, abnormal neuroimaging, history of heart transplant, and executive dysfunction, and presence of an individualised education plan (p < 0.03 for all predictors). Worse motor proficiency correlated with reduced health-related quality of life. Parent-reported adaptive behaviour (p < 0.001) and physical functioning (p < 0.001) had a strong association with motor proficiency scores.

This study highlights the need for continued motor screening for school-aged patients with CHD. Clinical factors, neuropsychological screening results, and health-related quality of life were associated with worse motor proficiency.

The Takeuchi procedure remains an important surgical option for treating anomalous origin of the left coronary artery from the pulmonary artery, particularly in cases where direct coronary reimplantation is not feasible. However, long-term outcome data in paediatric patients are limited.

We retrospectively reviewed 18 paediatric patients who underwent Takeuchi repair between 2007 and 2023. Clinical characteristics, echocardiographic data, and outcomes—including survival, ventricular function, mitral regurgitation, and reintervention—were analysed. Kaplan–Meier analysis was used to assess survival and freedom from reintervention, and paired comparisons were evaluated using the Wilcoxon signed-rank test.

The median age at surgery was 6 months (range: 25 days to 12 years). Preoperative left ventricular ejection fraction was significantly depressed (median 23.5%), and mitral regurgitation was present in all patients. There were two early deaths (11.1%), both in patients with severe heart failure. No late mortality was observed during a maximum follow-up of 10.9 years. All survivors achieved New York Heart Association class I status. Left ventricular ejection fraction improved significantly postoperatively (p < 0.0001), and mitral regurgitation grade also decreased significantly (p < 0.001), with 94.4% showing only mild residual mitral regurgitation. Reintervention occurred in three patients (16.7%) for pulmonary artery stenosis or baffle leak. Freedom from reintervention at 10.9 years was 66.7%.

The Takeuchi procedure offers excellent survival and functional recovery in paediatric anomalous origin of the left coronary artery from the pulmonary artery patients when coronary translocation is not feasible. Although late complications such as pulmonary artery stenosis or baffle leak can occur, outcomes remain favourable with appropriate follow-up.

To evaluate the right ventricle function by conventional echocardiographic methods and strain analysis in the long term after balloon pulmonary valvuloplasty. In addition, we investigated the relationship between pulmonary regurgitation, demographic data at the time of the procedure, and right ventricle dysfunction in late follow-up.

The records of patients submitted to balloon pulmonary valvuloplasty from 2001 to 2015 in a single centre were reviewed. From that sample, a revised cohort was formed, and the patients were submitted to clinical and echocardiographic evaluations.

The retrospective and the revised cohort analyses included 73 and 18 patients, respectively. In the follow-up, pulmonary regurgitation was observed in all patients, and there was a significant worsening of its magnitude over time (p < 0.001); the severity of pulmonary regurgitation was associated with balloon pulmonary valvuloplasty performed in patients with weight < 3 kg (p < 0.03), body surface area < 0.3 m2 (p < 0.04), and < 1 year of age (p < 0.006). Global longitudinal systolic strain of the right ventricle was abnormal in 8 of 18 patients, and conventional methods were abnormal in 2 of 18 patients (p = 0.001). There was a significant relationship between severe pulmonary regurgitation and right ventricle dysfunction detected only by strain evaluation (p = 0.01).

The severity of pulmonary regurgitation was related to the impairment of right ventricle function detected by strain. The predictors of pulmonary regurgitation severity in late follow-up were age < 1 year, weight < 3 kg, and body surface area < 0.3 m2.

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.

Contemporary management of aortic coarctation in adults is primarily by percutaneous intervention; however, this is not always possible. Intrapericardial ascending-to-descending aortic grafting is an alternative approach in adults with complex aortic coarctation. It can be performed off-pump and allows for a simultaneous treatment of concomitant cardiac lesions. Our aim was to examine the indications and the clinical long-term outcomes of intrapericardial ascending-to-descending aortic grafting in adults with aortic coarctation.

We retrospectively reviewed demographic, clinical, surgical, and follow-up data of patients who underwent intrapericardial ascending-to-descending aortic grafting at a single tertiary centre between September 1994 and November 2016.

Ten patients were identified. Indications were primary intervention (n = 4), re-coarctation (n = 5), and iatrogenic coarctation of aorta after stent grafting (n = 1). There were no peri-operative deaths. During a mean follow-up of 13.6 ± 9.4 years, one patient died 11 years after surgery, unrelated to intrapericardial ascending-to-descending aortic grafting. All grafts remained patent with no anastomotic pseudoaneurysms at latest follow-up.

The mean systolic blood pressure decreased from 154 ± 24 mmHg at baseline to 134 ± 20 mmHg at latest follow-up (p = 0.05), with the mean number of prescribed antihypertensive medications decreasing from 2.8 ± 1.5 preoperatively to 1.6 ± 0.5 (p = 0.11).

Intrapericardial ascending-to-descending aortic grafting is a safe and effective approach for adults with complex coarctation of aorta or re-coarctation not suitable for a percutaneous or conventional surgical approach. Improved blood pressure control and reduced anti-hypertensive use were observed long-term.

To assess the prevalence of obesity and investigate any changes in body mass index in children with CHD compared to age-matched healthy controls, in Southwestern Ontario.

The body mass index z-scores of 1259 children (aged 2–18) with CHD were compared with 2037 healthy controls. The body mass index z-scores of children who presented to our paediatric cardiology outpatient clinic from 2018 to 2021 were compared with previously collected data from 2008 to 2010. A longitudinal analysis of patients with data in both cohorts was also completed.

In total, 21.4% of patients with CHD and 26.6% of healthy controls were found to be overweight or obese (p < 0.001). The 2018–2021 cohort of CHD patients and controls had significantly higher body mass index z-scores compared to the 2008–2010 cohort (p < 0.001). Longitudinal analysis showed that body mass index z-scores significantly increased over time for CHD patients with data in both cohorts (2018–2021: M = 0.59, SD = 1.26; 2008–2010: M = −0.04, SD = 1.05; p < 0.001).

The prevalence of obesity in all children, irrespective of CHD, is rising. The coexistence of obesity and CHD may pose additional cardiovascular risks and complications.

The functional roles of ventricular dominance and additional ventricular chamber after Fontan operation are still uncertain. We aim to assess and correlate such anatomical features to late clinical outcomes.

Fontan patients undergoing cardiac MRI and cardiopulmonary exercise test between January 2020 and December 2022 were retrospectively reviewed. Clinical, cardiac MRI, and cardiopulmonary exercise test data from the last follow-up were analysed.

Fifty patients were analysed: left dominance was present in 29 patients (58%, median age 20 years, interquartile range:16–26). At a median follow-up after the Fontan operation was 16 years (interquartile range: 4–42), NYHA classes III and IV was present in 3 patients (6%), 4 (8%) underwent Fontan conversion, 2 (4%) were listed for heart transplantation, and 2 (4%) died. Statistical analysis showed that the additional ventricular chamber was larger (>20 mL/m2) in patients with a right dominant ventricle (p = 0.01), and right dominance was associated with a higher incidence of post-operative low-cardiac output syndrome (p = 0.043). Left ventricular dominance was associated with a better ejection fraction (p = 0.04), less extent of late gadolinium enhancement (p = 0.022), higher metabolic equivalents (p = 0.01), and higher peak oxygen consumption (p = 0.033). A larger additional ventricular chamber was associated with a higher need for post-operative extracorporeal membrane oxygenation support (p = 0.007), but it did not influence functional parameters on cardiac MRI or cardiopulmonary exercise test.

In Fontan patients, left ventricular dominance correlated to better functional outcomes. Conversely, a larger additional ventricular chamber is more frequent in right ventricular dominance and can negatively affect the early post-Fontan course.

To examine the added value of an elevated calculated peak right ventricular outflow tract pressure gradient as a surrogate for infective endocarditis diagnosis.

A cohort study included patients admitted between 2003 and 2020 at a tertiary children’s medical centre. Patients with surgically inserted exogenous right ventricular outflow tract conduits and infective endocarditis were included. Infective endocarditis was diagnosed using the revised Duke criteria (2023). Controls had right ventricular outflow tract conduits and febrile illness from other causes. Clinical, laboratory, and echocardiographic findings, including calculated peak right ventricular outflow tract pressure gradient, were collected.

Among 26 febrile episodes (11 with infective endocarditis, 15 controls), the infective endocarditis group had a higher peak right ventricular outflow tract pressure gradient during acute illness (70 vs. 23 mmHg, p < 0.05). On admission, 18% of infective endocarditis patients had a definite diagnosis by Duke’s criteria, 45% had a probable diagnosis, and 36% lacked confirmation. Including peak right ventricular outflow tract gradient as a major criterion would yield a 90% diagnosis rate upon admission (45% definite, 45% possible).

Increased right ventricular outflow tract pressure gradient in febrile patients with exogenous conduit in the right ventricular outflow tract is a potential marker for infective endocarditis. Including this gradient as a major Duke criterion enables earlier and more definitive diagnosis in debatable cases.

Atrial septal defect is commonly considered a minor CHD, but morbidity and mortality are higher compared to the background population. Maternal pre-eclampsia is associated with CHD in the offspring in large registry-based studies. However, the association between pre-eclampsia and atrial septal defects might be subject to detection bias, as many atrial septal defects are asymptomatic or might remain undiagnosed until late in life.

We investigated the association between maternal pre-eclampsia and the risk of atrial septal defects in a population-based cohort of neonates examined with echocardiography.

Neonates included in the Copenhagen Baby Heart Study, who were examined using transthoracic echocardiography within 30 days of birth, were systematically assessed for atrial septal defects and patent foramen ovale using a standardised algorithm. Using log-linear binomial regression and polytomous logistic regression, we compared the risk of atrial septal defects in neonates exposed to maternal pre-eclampsia with the risk in neonates not exposed to pre-eclampsia.

Our study cohort included 12,354 neonates (mean age, 11 days), including 462 exposed to maternal pre-eclampsia. Atrial septal defect was found in 5.9% (n = 732) of the study cohort and compared with unexposed neonates, neonates exposed to maternal pre-eclampsia had a modestly increased risk of atrial septal defects (adjusted risk ratio 1.19, 95% confidence interval 0.83, 1.64). Estimates were robust to various exclusions in sensitivity analyses.

There appears to be an association between maternal pre-eclampsia and atrial septal defect in the neonate in a population-based cohort of neonates.

To evaluate the outcomes of patients with single ventricle physiology supported with extracorporeal membrane oxygenation as a bridge to first-stage palliation.

This was a retrospective registry-based study. Data from the Extracorporeal Life Support Organization registry were used to identify single ventricle physiology patients supported with extracorporeal membrane oxygenation prior to palliation from 2016 to 2021. Descriptive statistics and multivariate analyses for associations with mortality were conducted.

Primary outcome was death before hospital discharge. Patient characteristics including demographics and associated complications were evaluated as secondary outcomes. Sixty-five patients met inclusion criteria. Survival to discharge was 42%. Twenty-four (37%) patients died while on extracorporeal membrane oxygenation. There was no significant difference in demographics between survivors and non-survivors. Non-survivors had a significantly longer median duration on extracorporeal membrane oxygenation compared to survivors, 99-hrs [IQR (Interquartile Range), 160, 300] vs. 59-hrs [43, 124] (p<0.001). Multivariate analysis demonstrated extracorporeal membrane oxygenation duration (adjusted-OR [Odds Ratio] 1.01, 95% CI [Confidence Interval] 0.98, 0.99; p = 0.03) and requiring renal replacement therapy (42% vs. 19%; p = 0.04) were associated with mortality prior to discharge.

Clinicians managing decompensated patients with single ventricle physiology may consider extracorporeal membrane oxygenation as a bridge to palliation. Survival to discharge was 42%. Evidence of renal injury and longer extracorporeal membrane oxygenation durations were associated with mortality. These data may be used to guide providers and to counsel families. However, more data are needed to refine indications and assess associations related to outcomes and decision-making.

Poor weight gain in infants with single ventricle cardiac physiology between stage 1 and stage 2 palliative surgeries is associated with worse outcomes. The growth of infants with single ventricle physiology, enrolled in home monitoring programmes in the United Kingdom, has not been widely described.

To explore the growth of infants with single ventricle physiology supported by a home monitoring programme, at a tertiary centre in the South of England.

A retrospective review of two cohorts, comparing weight gain amongst infants with single ventricle physiology, before and following the implementation of a home monitoring programme. Inclusion was dependent on a diagnosis compatible with single ventricle physiology during the interstage.

Enrolment into a home monitoring programme (cohort 2) was associated with 55% more infants being discharged home during the interstage period (p < 0.05). Interstage mortality did not differ between cohorts. There were no differences in interstage growth velocity between cohorts (cohort 1 23.98 ± 11.7 g/day and cohort 2 23.82 ± 8.3 g/day); however, infants in cohort 2 experienced less growth deceleration early in life, and achieved catch-up growth at 12-23 months. Interstage nasogastric feeding, regardless of the cohort, was associated with worse growth outcomes.

A home monitoring programme for infants with single ventricle physiology provides the opportunity for infants to be safely discharged home to their families and cared for at home during the interstage. Infants in the home monitoring programme experienced better growth, achieving weight restoration at 12–23 months.

This study aims to evaluate patient outcomes related to mitral valve disease (stenosis, regurgitation, or mixed) who benefited from mitral repair or replacement under one year of age.

Monocentric retrospective study including all children with mitral valve repair or replacement under 1 year of age over a period of 22 years (2001–2023).

early mortality (at 30 days), late mortality, and need for re-intervention.

A total of 56 patients were identified, with a median age of 147 days and median weight of 5.1 kg. Of these, 39 underwent mitral valve repair and 17 underwent replacement. The median follow-up duration was 2.9 years (interquartile range 0.3–8.1). Patients who underwent replacement had longer ICU stays, hospital stays, and assisted ventilation times (p = 0.005, p = 0.01, p = 0.019), with higher early mortality (12% vs. 0%). Survival was significantly higher in the repair group (p = 0.039). Re-intervention was required in 23 patients (41.1%): 16 had replacement, 6 had re-repair, and 2 needed pacemaker implantation. Seven patients (12.5%) needed more than one re-intervention. Re-intervention-free survival rates after repair were 81%, 65%, and 46% at 1, 5, and 10 years, respectively. After replacement, rates were 74% at 1 year and 55% at 5 and 10 years. Conclusion Mitral valve surgery in infants is particularly high risk and is associated with high rate of re-intervention. While mitral repair demonstrates superior outcomes in mortality, it often delays but does not always prevent the need for valve replacement.

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

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

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

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

Duchenne muscular dystrophy is a devastating neuromuscular disorder characterized by the loss of dystrophin, inevitably leading to cardiomyopathy. Despite publications on prophylaxis and treatment with cardiac medications to mitigate cardiomyopathy progression, gaps remain in the specifics of medication initiation and optimization.

This document is an expert opinion statement, addressing a critical gap in cardiac care for Duchenne muscular dystrophy. It provides thorough recommendations for the initiation and titration of cardiac medications based on disease progression and patient response. Recommendations are derived from the expertise of the Advance Cardiac Therapies Improving Outcomes Network and are informed by established guidelines from the American Heart Association, American College of Cardiology, and Duchenne Muscular Dystrophy Care Considerations. These expert-derived recommendations aim to navigate the complexities of Duchenne muscular dystrophy-related cardiac care.

Comprehensive recommendations for initiation, titration, and optimization of critical cardiac medications are provided to address Duchenne muscular dystrophy-associated cardiomyopathy.

The management of Duchenne muscular dystrophy requires a multidisciplinary approach. However, the diversity of healthcare providers involved in Duchenne muscular dystrophy can result in variations in cardiac care, complicating treatment standardization and patient outcomes. The aim of this report is to provide a roadmap for managing Duchenne muscular dystrophy-associated cardiomyopathy, by elucidating timing and dosage nuances crucial for optimal therapeutic efficacy, ultimately improving cardiac outcomes, and improving the quality of life for individuals with Duchenne muscular dystrophy.

This document seeks to establish a standardized framework for cardiac care in Duchenne muscular dystrophy, aiming to improve cardiac prognosis.

Inherited cardiac arrhythmias are life-threatening conditions associated with a high risk of sudden cardiac death. These diseases impose a substantial psychological burden. Parents experience heightened anxiety due to uncertainty, medical interventions, and risk of adverse events. However, limited research has examined anxiety levels in affected individuals and their families.

This study aimed to assess anxiety levels in children and adolescents diagnosed with inherited cardiac arrhythmias, specifically Long QT Syndrome and Catecholaminergic Polymorphic Ventricular Tachycardia, and to identify factors influencing anxiety in both patients and parents.

A prospective, survey-based cohort study was conducted between June 2023 and June 2024, including 53 patients (0–18 years) diagnosed with inherited arrhythmias. Anxiety was assessed using the State-Trait Anxiety Inventory. Demographic and clinical variables, including disease type, invasive procedures, family history of sudden cardiac death, and parental education, were analysed.

Mothers exhibited the highest anxiety (STAI-T: 46.6 ± 10.6) while fathers had the lowest (37.3 ± 7.0). Catecholaminergic polymorphic ventricular tachycardia patients reported significantly higher anxiety (49.2 ± 7.7) than long QT syndrome patients (38.0 ± 7.0, p < 0.01). Children undergoing invasive procedures, particularly sympathetic denervation, had elevated anxiety (45.1 ± 8.2 vs. 36.5 ± 6.4, p < 0.05). Mothers of male children and those with a family history of sudden cardiac death had significantly higher anxiety (p < 0.01).

Inherited arrhythmias significantly impact psychological well-being, with mothers experiencing the highest anxiety levels. Disease severity, invasive procedures, and family history of sudden cardiac death contribute to increased anxiety, emphasising the need for psychological support in managing these conditions.

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.