Reports of paediatric cardiac neurodevelopmental programmes are predominately limited to high-volume surgical centres. Regional characterisation of neurodevelopmental care practices across multiple centres both with and without cardiac surgery programmes has not yet been described.

Assess cardiac neurodevelopmental infrastructure and approach across New England to describe regional support for children with CHD.

A 16-item survey assessing inpatient and outpatient neurodevelopmental care practices was sent electronically via REDCap to all member institutions of the New England Congenital Cardiology Association Health Disparities Working Group. Centres were characterised by surgical capability, annual volume, and academic affiliation. Descriptive statistics and Fisher’s exact test were used for comparisons.

The survey response rate was 90% (9/10). Participating centres included non-surgical academic centres (55%), small surgical centres (22%) and medium-large surgical centres (22%). Surgical centres typically refer their patients to internal neurodevelopmental services (100%) and rely on automatic referral processes (100%). Non-surgical centres predominately refer to the institution where cardiac surgery was performed (80%) compared to engaging local teams (20%) and depend on family or physician-initiated referral (100%) to schedule neurodevelopmental services. While none of the non-surgical centres surveyed have cardiac-specific neurodevelopmental programmes, all have other developmental programmes that accommodate children with CHD.

Varying neurodevelopmental infrastructure and resources are reported across New England. Academic, non-surgical centres may have infrastructure for neurodevelopmental follow-up despite not having cardiac neurodevelopmental programmes. Collaboration between surgical and non-surgical cardiac centres in New England may be leveraged to promote neurodevelopmental care closer to home.

Surgical pulmonary valve replacement is commonly required to palliate patients with CHD affecting the right ventricular outflow tract; however, concerns remain about mid- and long-term durability. Post-operative short-term anticoagulation has been hypothesised to improve valve durability.

This is a single-centre, retrospective study of paediatric patients who underwent surgical pulmonary valve replacement and received a direct oral anticoagulant in addition to aspirin post heart valve insertion. The primary objective was a composite safety score consisting of clinically relevant non-major bleeding, major bleeding, bleeding-related readmission, and medication discontinuation.

The study analysed 34 patients with a median age 14 years (Interquartile range (IQR): 11, 15) and weight 45 kg (IQR: 35, 55). Ten patients met the composite endpoint (10/34, 29%), with 4 patients experiencing major bleeding (4/34, 12%), 6 experiencing clinically relevant non-major bleeding (6/34, 18%), and 3 patients being readmitted within 90 days of surgical pulmonary valve replacement for bleeding (3/29, 8.8%) resulting in 10 patients discontinuing medication early (10/34, 29%). Lower weight was identified as a significant risk factor for adverse event development (p = 0.04).

We observed a higher overall bleeding rate, driven predominately by clinically relevant non-major bleeding events, than other studies using short-term anticoagulation after surgical pulmonary valve replacement. Additional studies should be aimed at evaluating the dosing and safety of direct oral anticoagulants in children in the post-operative period.

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.

Integrating psychosocial health services into paediatric surgical specialty care is essential for addressing behavioural and psychological aspects of illness and reducing healthcare disparities. This is crucial for patients facing CHD, who are at higher risk for depression, anxiety, and attention-deficit/hyperactivity disorder, which is significantly influenced by their caregivers’ mental well-being.

The Pediatric Psychosocial Preventative Health Model framework was utilised by a psychosocial team to assess biopsychosocial needs in CHD patients during their first cardiac surgery evaluations. Patient and family needs were categorised into universal, targeted, and clinical tiers, allowing for responsive interdisciplinary services. Screening tools such as the Psychosocial Assessment Tool, Pediatric Quality of Life Inventory, and Depression, Anxiety, and Stress Scales were used during initial consultations to guide appropriate interventions and referrals.

Universal-tier patients received comprehensive support focused on preventive measures, resource access, and education to promote resilience. Targeted-tier care involved intensive, collaborative efforts, providing specialized psychological evaluations, and one-on-one time with experts. Clinical-tier families required specialised, intensive interventions such as advanced cognitive behavioural therapy and medication management. The Pediatric Psychosocial Preventative Health Model framework and psychosocial team workflow allow for individualised management strategies, ensuring that each family received timely and appropriate interventions based on their unique needs.

Integrating psychosocial services into initial surgical evaluations is critical for addressing CHD patients’ psychological and social needs, promoting an interdisciplinary approach that enhances overall family functioning and well-being.

To highlight veno-venous collaterals and portosystemic shunts as possible causes of cyanosis requiring evaluation or treatment below the diaphragm in patients with single-ventricle physiology during staged palliation.

Cyanosis can complicate staged single-ventricle palliation and is often evaluated with imaging isolated to the thorax. As a result, lesions below the diaphragm, such as veno-venous collaterals and portosystemic shunts, may be missed. Identification is important since these lesions can be treated via transcatheter techniques.

This study is a retrospective, single-centre case series of six patients.

Two patients following stage 2 palliation had multiple small veno-venous collaterals connecting to a reconstituted azygos with retrograde flow to below the diaphragm. Accessing veno-venous collaterals closer to their entry points below the diaphragm was more optimal for occlusion. Two patients following stage 3 palliation had veno-venous collaterals originating below the diaphragm and connecting to the pulmonary veins. Both patients required infrarenal inferior vena cava contrast injection to identify the veno-venous collateral. The final two patients had heterotaxy with either stage 2 or 3 palliation and were found to have bilateral pulmonary arteriovenous malformations secondary to a portosystemic shunt diverting hepatic factor away from the lungs. All patients had successful device closure of their veno-venous collaterals or portosystemic shunts.

The six patients discussed demonstrate the importance of evaluating below the diaphragm in single-ventricle physiology patients presenting with cyanosis.

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.

Tacrolimus is the standard immunosuppressant used in paediatric orthotopic heart transplantation, but it can be associated with rare and life-threatening haemolysis.

Retrospective chart review was used for this case report.

We present the case of a 6-year-old heart transplant recipient who developed life-threatening haemolysis in the setting of mycoplasma infection while on tacrolimus immunosuppression that was treated successfully with eculizumab.

Coronary artery abnormalities in children that require bypass grafting are infrequent but represent a well-recognised entity with a broad spectrum of indications beyond Kawasaki disease. Although myocardial revascularisation in children is uncommon, studies have shown that it can yield favourable short- and long-term outcomes, allowing affected children to regain health and grow up to live normal lives.

Myocardial revascularisation in children is an extremely rare intervention in Western countries, accounting for less than 1% of all paediatric cardiac surgeries in this region. It is a highly technically demanding procedure that opens a new arena in cardiac surgery, for which cardiovascular surgeons need to be trained to achieve outcomes as good as those shown in the literature.

We present the experience of paediatric coronary artery bypass grafting in a middle-income country, with a wide range of indications.

A retrospective descriptive study was conducted on paediatric patients (under 18 years of age) who underwent coronary artery bypass grafting between 2004 and 2023 at a cardiovascular centre in Bogotá, Colombia. Data were collected from electronic medical records, including demographics, preoperative diagnoses, surgical details, and outcomes. Follow-up included clinical assessment and imaging with echocardiography. Ethical approval was obtained, and confidentiality was ensured.

Nine paediatric patients (ages 6–17) underwent coronary artery bypass grafting between 2004 and 2023. Kawasaki disease was the most common indication, but there are other aetiologies, including post-arterial switch coronary occlusion, anomalous origin of the LCA from the pulmonary artery, anomalous origin of coronary arteries from the aorta, Takayasu disease, and iatrogenic injury. The internal mammary artery was used in most cases, with successful completion of the planned revascularisation in all patients. There were no perioperative deaths or reinterventions. At a mean follow-up of 5.5 years, all patients showed clinical and biventricular improvement, and all grafts evaluated showed graft patency.

Paediatric coronary artery bypass grafting is a safe and effective treatment for selected congenital and acquired coronary pathologies, even in complex cases. Outcomes are optimised with the use of internal mammary arteries and a multidisciplinary heart team approach. In middle-income settings, favourable short- and mid-term results can be achieved despite follow-up challenges. Paediatric coronary artery bypass grafting should be considered a key component of congenital cardiac surgery training.

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.

We compared the results of conventional electroanatomic mapping and high-density mapping in ablations of the right ventricular outflow tract.

Between 2014 and 2024, a total of 92 patients underwent ablation using an electroanatomic mapping system. A contact force radiofrequency ablation catheter was used in all patients, except for two who underwent cryoablation.

Our study comprised two groups, with a total of 92 patients. Among them, 45 patients underwent conventional three-dimensional mapping, while 47 patients underwent high-density three-dimensional mapping. In both groups, there were three patients with nonsustained ventricular tachycardia and two patients with sustained ventricular tachycardia, and there was no statistically significant difference in the frequency of ventricular premature contractions between the two groups. There were four patients with systolic dysfunction in the high-density mapping group and four in the conventional mapping group. In the high-density mapping group, the procedure time was significantly shorter. There was a notable difference regarding the reduced fluoroscopy time. The total lesion time and number were also significantly lower in the high-density mapping group. The earliest activation time was significantly longer in this group. The procedure’s success rate was 97.9% for the high-density mapping group and 93.4% for the conventional mapping group. Successful ablation was performed on one ventricular extrasystole in the parahisian localisation in each group, while a cooled type radiofrequency catheter was used for all other cases.

In children with right ventricular outflow tract arrhythmias, high-density mapping can be used with high success rates and safety.