Pulmonary arteriovenous malformations (PAVMs) are a recognised cause of progressive cyanosis following bidirectional superior cavopulmonary anastomosis (BDG). The role of antegrade pulmonary blood flow (APBF) in preventing PAVM formation remains controversial. While preserved APBF may improve oxygen saturation and pulmonary artery growth, its protective effect against PAVMs is uncertain. Increasing evidence suggests that exclusion of hepatic venous blood from the pulmonary circulation, rather than absence of pulsatile flow, may be the primary mechanism underlying PAVM development. Therefore, management of APBF after BDG should be individualized.

This retrospective observational study included children with univentricular physiology who underwent BDG before 4 years of age with a minimum follow-up of two years. Patients were divided into two groups based on the presence or interruption of APBF. Clinical data, oxygen saturation, echocardiographic findings, pulmonary artery dimensions, and imaging evidence of PAVMs were analysed using computed tomography angiography and/or cardiac catheterisation. Outcomes were compared between groups.

Sixty patients were included. PAVMs occurred in 26.7% of patients with interrupted APBF and 23.3% with preserved APBF (p = 0.766). Oxygen saturation was higher in the preserved APBF group but not statistically significant. Pulmonary artery dimensions and pressures were comparable, and all patients remained suitable for Fontan completion. Cyanosis was more frequent in the APBF-interrupted group.

Preserved APBF improves oxygen saturation but does not reduce PAVM incidence. Hepatic venous exclusion appears to be the primary determinant of PAVM development, supporting individualized surgical decision-making.

Complex CHDs may impair organ development. One proposed mechanism is an altered relationship between blood flow, oxygen delivery, and subsequent organ growth. In this study, we examined whether fetal lung, intracranial, liver, and kidney volumes differ among fetuses with transposition of the great arteries with an intact ventricular septum, transposition of the great arteries with a ventricular septal defect, and healthy controls.

Eleven fetuses with transposition of the great arteries (6 with a ventricular septal defect and 5 with an intact ventricular septum) and 22 healthy controls were scanned between 1 and 3 times at gestational age 27–38 weeks, using fetal MRI. We measured lung, total intracranial, liver, and kidney volumes and compared fetuses with and without transposition of the great arteries while subsequently correcting for ventricular septal defect/intact ventricular septum status, estimated fetal weight, and gestational age, using mixed effects regression analysis.

Fetuses with transposition of the great arteries+intact ventricular septum had significantly larger lung volumes compared to controls. After adjusting for estimated fetal weight and gestational age, median lung volume ratio (transposition of the great arteries+intact ventricular septum vs. controls) was 1.30 (95% CI: 1.08–1.57; p = 0.005). No difference was found in lung volume between fetuses with transposition of the great arteries+ventricular septal defect and controls. No significant differences in total intracranial, liver, and kidney volumes were found between transposition of the great arteries+ventricular septal defect, transposition of the great arteries+intact ventricular septum, and controls.

In this preliminary study, late-gestation fetuses with transposition of the great arteries-intact ventricular septum had a 30% larger lung volume compared with both transposition of the great arteries-ventricular septal defect and healthy controls. Together with existing evidence of higher fetal pulmonary blood flow and increased oxygen saturation in transposition of the great arteries-intact ventricular septum, these findings support a potential link between blood flow, oxygen delivery, and organ growth.

Acute kidney injury after infant congenital cardiac surgery is common and worsens outcomes. Renal resistive index may aid early postoperative risk stratification.

To evaluate a single six-hour postoperative renal resistive index as an early acute kidney injury marker and its association with postoperative haemodynamic support.

A prospective single-centre cohort of neonates and infants < 12 months (n = 41) was classified as acute kidney injury (n = 19) or non-acute kidney injury (n = 22). Renal resistive index was measured at six hours (primary), 48 hr, and discharge. Postoperative acute kidney injury was diagnosed using Kidney Disease: Improving Global Outcomes serum creatinine and urine output criteria over 48 hr. Vasoactive–inotropic score was recorded, and discrimination was assessed using receiver operating characteristic analysis.

At six hours, renal resistive index was higher in acute kidney injury vs. non-acute kidney injury (0.84 ± 0.07 vs. 0.74 ± 0.08; p = 0.0002) with good unadjusted discrimination (area under the curve 0.83; 95% CI 0.68–0.97). Surgical complexity was higher in the acute kidney injury group (median STAT 4 vs. 3). At 48 hr, renal resistive index remained higher (p = 0.017; area under the curve 0.70), while discharge values were similar (p = 0.18; area under the curve 0.62). A six-hour renal resistive index < 0.80 excluded moderate-to-severe acute kidney injury, whereas ≥ 0.86 identified a higher risk. Vasoactive–inotropic score at 48 hr was higher in acute kidney injury (7.2 ± 8.1 vs. 1.9 ± 3.4; p = 0.009), and six-hour renal resistive index correlated modestly with inotropic burden (r = 0.56; p < 0.001). Three acute kidney injury infants required peritoneal dialysis, all with six-hour renal resistive index ≥ 0.90.

A six-hour renal resistive index may help early acute kidney injury risk assessment after infant cardiac surgery. External validation is warranted.

Cardiac CT angiography has seen a steady increase in use for the evaluation of CHD in children. While its primary role is to assess cardiovascular structures, CT angiography also captures extracardiac regions, often revealing previously unsuspected abnormalities. This study sought to determine the prevalence, classification, and clinical relevance of extracardiac abnormalities detected in paediatric patients undergoing CT angiography for CHD evaluation.

A retrospective review was conducted on CT angiography examinations from 1336 patients, aged from newborn to 17 years, at a single tertiary care institution. Extracardiac findings within the thoracic and abdominal regions were systematically identified, categorised, and analysed statistically.

Extracardiac abnormalities were common, predominantly affecting the respiratory system, including pneumonia (14.5%), atelectasis (13.3%), and pulmonary oedema (11.5%). Airway changes included bronchial malposition (23.3%) and peribronchial thickening (8.3%). Abdominal anomalies were also detected, the most common being hepatomegaly (13.1%), splenomegaly (6.9%), and horseshoe kidney (5.7%). Several of these findings were clinically important and had the potential to affect patient management.

Extracardiac anomalies are a common finding in paediatric CT angiography performed for CHD evaluation and can have significant clinical consequences. Radiologists and clinicians should follow a systematic approach that evaluates both cardiovascular and extracardiac structures to improve diagnostic accuracy and optimise patient care. In addition to common thoracic and abdominal abnormalities, CT angiography enabled the identification of rare and complex extracardiac anatomical patterns, underscoring its value as a comprehensive imaging modality in paediatric CHD.

Electronic medical records (EMRs) have become foundational to healthcare, improving communication, data access, and patient outcomes. However, increasing reliance on EMR’s has increased vulnerabitlity during downtime. In paediatric cardiac care, where patients require highly specialized, multidisciplinary treatment, the absence of a functional EMR significantly disrupts documentation workflows and threatens the accuracy of data submitted to national cardiac registries including the Paediatric Cardiac Critical Care Consortium and the Society of Thoracic Surgeons.

This article examines the impact of an unexpected 27-day complete EMR downtime followed by a 10-day partial downtime, where a data abstraction team manually managed data for 123 unique cardiac patient encounters totalling 762 patient days at a paediatric heart centre and the response of the cardiac data abstraction team. We describe how the team adapted its abstraction process during the downtime, used collaborative strategies, enhanced paper tracking, and proactively communicated to maintain data integrity. Efforts were grounded in a deep understanding of paediatric registry metrics and submission requirements.

Despite significant workflow disruptions, the team was able to preserve data accuracy and meet registry deadlines by identifying documentation gaps, supplementing data from paper records, and coordinating with frontline providers. The event revealed key vulnerabilities in downtime preparedness but also demonstrated the value of dedicated data abstractors in ensuring continuity of quality reporting.

Downtime events highlight the critical role of data abstractors and the need for institutional planning and registry-level guidance. Developing robust downtime protocols and embedding abstraction-aware workflows can mitigate documentation risks and protect data quality, ultimately supporting improved outcomes for paediatric cardiac patients.

Ventricular fibrillation in paediatric recipients of left ventricular assist devices is rare but potentially fatal; however, because systemic perfusion may be temporarily maintained by the mechanical support, haemodynamic deterioration may be delayed, leading to underrecognition and presentation in the outpatient setting rather than as an immediate in-hospital emergency.

We report three paediatric left ventricular assist device recipients who developed outpatient malignant ventricular arrhythmias, each representing a distinct mechanistic category: structural, mechanical, and endocrine. Clinical presentation, diagnostic findings, and therapeutic responses were reviewed to highlight pathophysiologic heterogeneity and inform individualised management.

The first patient, with biopsy-proven arrhythmogenic right ventricular dysplasia, experienced recurrent ventricular fibrillation refractory to multiple antiarrhythmic agents, ultimately requiring heart transplantation. The second patient presented with ventricular fibrillation secondary to mechanical suction and interventricular septal contact; defibrillation and left ventricular assist device speed reduction restored a stable rhythm with no recurrence. The third patient developed polymorphic ventricular arrhythmias during amiodarone-induced thyrotoxicosis and succumbed to refractory electrical storm and multi-organ failure despite intensive endocrine and antiarrhythmic therapy.

Outpatient ventricular fibrillation in paediatric left ventricular assist device recipients may result from diverse mechanisms, including myocardial structural disease, mechanical pump heart interaction, and systemic endocrine disturbances. Mechanism-guided management combining antiarrhythmic, mechanical, and metabolic interventions is essential for optimal outcomes. Continuous rhythm surveillance and readiness for immediate defibrillation are key to improving survival in this vulnerable group.

Early identification of patients at risk for bulboventricular foramen restriction is critical for selecting the most appropriate palliative strategy and preventing late reinterventions. The primary aim of our study was to identify parameters that predict bulboventricular foramen restriction in this patient population.

We retrospectively reviewed 17 patients with double-inlet left ventricle or tricuspid atresia associated with ventriculoarterial discordance who underwent staged palliation at our institution between August 2020 and December 2025. The bulboventricular foramen-to–aortic annulus ratio was measured by echocardiography, and receiver operating characteristic analysis was performed to identify a predictive cut-off.

Bulboventricular foramen restriction developed in 8 patients (47.1%). Bulboventricular foramen restriction occurred more frequently after pulmonary artery banding with concomitant aortic arch reconstruction (PAB + AR) than after other initial strategies (p = 0.041). The pre–bidirectional cavopulmonary shunt bulboventricular foramen/aortic annulus ratio was significantly lower in patients who developed bulboventricular foramen restriction (0.6 [IQR 0.5–0.8] versus 0.8 [IQR 0.67–0,99], p = 0.02). Receiver operating characteristic analysis showed that a pre-bidirectional cavopulmonary shunt bulboventricular foramen/aortic annulus ratio ≤ 0.65 predicted bulboventricular foramen restriction with an aortic annulus ratio of 0.87 (sensitivity 71%, specificity 83%). In multivariable.

Pulmonary artery banding + arch reconstruction and a pre-bidirectional cavopulmonary shunt bulboventricular foramen/aortic annulus ratio ≤ 0.65 are associated with subsequent bulboventricular foramen restriction. In high-risk patients, performing a Damus–Kaye–Stansel anastomosis at the time of bidirectional cavopulmonary shunt may reduce the need for bulboventricular foramen enlargement and its related complications.

To evaluate the prevalence and clinical implications of QT interval prolongation and other electrocardiographic changes in paediatric patients with rheumatic diseases using hydroxychloroquine.

This was a retrospective and prospective, observational, and analytical study conducted at a centre of perinatology and paediatrics in Rio de Janeiro, Brazil. A total of 26 evaluations of patients ≤18 years old on hydroxychloroquine were included, all following paediatric rheumatology and cardiology services. Patients were included if they had been receiving hydroxychloroquine for at least six months and had complete clinical records; those with pre-existing cardiac conditions unrelated to hydroxychloroquine were excluded. Clinical, demographic, and electrocardiographic data were collected from medical records using standardised protocols.

The corrected QT interval was manually measured on 12-lead electrocardiograms and analysed in relation to cumulative drug dose. All electrocardiograms were reviewed independently by two cardiologists to ensure accuracy of corrected QT interval measurements, and discrepancies were resolved by consensus. Most patients were female (76.9%), and systemic lupus erythematosus was the most prevalent diagnosis (88.9%). The cumulative hydroxychloroquine dose ranged from 12 to 447.6 g (mean: 223 g). Corrected QT interval values ranged from 377 to 454 ms (mean: 413 ms). Correlation analysis between cumulative dose and corrected QT interval showed a weak negative association (r = –0.24; p = 0.338), not statistically significant. Simple linear regression confirmed no association between variables (R2 = 5.7%).

In this cohort of paediatric patients with rheumatic diseases, no significant association was observed between cumulative hydroxychloroquine use and QT interval prolongation.

To investigate the effect of maternal gestational diabetes mellitus (GDM) on neonatal cardiac development.

A retrospective analysis of full-term newborns admitted in 2024 was conducted. 100 newborns of mothers with GDM (IADPSG criteria) were the GDM group, and 100 of non-GDM mothers were the control group. We compared their birth parameters,echocardiographic indicators and congenital heart disease (CHD) incidence, and analyzed factors related to neonatal interventricular septal (IVS) hypertrophy.

The GDM group had significantly higher birth weight, length and placental weight (P < 0.05); echocardiography showed larger cardiac chambers, great vessels and thicker IVS (P > 0.05); CHD incidence was 2% (vs. 0% in control, P = 0.047). Maternal glycemic indicators and neonatal birth weight were positively correlated with IVS thickness (P < 0.05).

GDM adversely affects neonatal cardiac development; routine fetal and postnatal cardiac evaluation is necessary for GDM pregnancies. Further research is needed to clarify mechanisms and establish monitoring strategies.

To investigate the safety and feasibility of deep sedation and general anaesthesia for cardiovascular magnetic resonance imaging in paediatric patients with congenital or acquired cardiac diseases.

This retrospective study included all consecutive patients less than 18 years of age who had deep sedation for cardiovascular magnetic resonance examination at the University Hospital Schleswig-Holstein (Kiel, Germany) between 2010 and 2020 and cardiovascular magnetic resonance examination under general anaesthesia at the Royal Brompton Hospital (London, United Kingdom) between 2013 and 2022.

Five-hundred twenty-two patients were in the deep sedation group and 171 in general anaesthesia group. Most of the patients had CHD (86% in deep sedation and 70% in general anaesthesia group). There were overall 14 adverse events (2%); 8 (1.5%) in the deep sedation group and 6 (3.5%) in the general anaesthesia group. This difference was not statistically significant (p = 0.122). Complications in the deep sedation group included mild anaphylactic reactions in three patients, a severe coughing fit in one patient, increasing cyanosis in three single-ventricle patients, and suspected aspiration in one patient. In the general anaesthesia group, hypotension requiring some intervention was present in three patients (four scans). One patient (0.6%) had inadvertent endobronchial intubation.

Both deep sedation and general anaesthesia can be used for cardiovascular magnetic resonance scans in paediatric patients with a low rate of complications. This, however, requires highly skilled teams who adhere strongly to the safety policies and guidelines set up by each hospital.

Arrhythmic mitral valve prolapse is a rare yet potentially fatal syndrome. Recently, high-risk markers for malignant arrhythmias have been identified in adults with mitral valve prolapse, and a risk stratification scheme has been proposed. Little is known about the prevalence of arrhythmic mitral valve prolapse, the characteristics of the syndrome, and risk markers in children and young adults.

A database of a high-volume, tertiary congenital heart defects department was searched to identify all patients with MVP between 2018 and 2025 (8 years). Patients’ characteristics, including arrhythmic risk factors, AMVP diagnosis, and last survival, were noted.

Of 12,955 hospitalised patients, 52 were diagnosed with mitral valve prolapse (0.4%; median age 14y [IQR 6], 35% male). Arrhythmic mitral valve prolapse was diagnosed in 13 patients (25% of the mitral valve prolapse cohort). Phenotypic risk factors were highly prevalent (88.5%), but in the crosstab analysis, only an enlarged left atrium was associated with high-risk arrhythmic events (p = 0.0432). Furthermore, patients with arrhythmic events were significantly older (median 16y) than those without (median 13y; p = 0.019). One patient (2.7%, with concomitant LQTS2) presented with unstable ventricular tachycardia and received an ICD. During a median follow-up of 3 years (IQR 1–4.5), all 52 patients were alive.

One in 250 patients admitted to a tertiary invasive paediatric cardiology department has mitral valve prolapse. Among those with mitral valve prolapse, 1 in 4 presents with arrhythmic mitral valve prolapse. Although risk factors are common, outcomes among young arrhythmic mitral valve prolapse patients appear good. Overlapping with primary electrical disease may flag a worse prognosis. Identification of new risk factors for children is warranted.

The ryanodine receptor 2 gene mutation associated with catecholaminergic polymorphic ventricular tachycardia is one of the aetiologies of cardiac syncope and has the risk of sudden cardiac death. This study reported two novel ryanodine receptor 2 gene variants.

We described two 9-year-old girls with recurrent syncope during exercise or stress presenting two novel ryanodine receptor 2 gene variants (c.6938T>G/p. Val2313Gly and c.12263A>C/p. His4088Pro) associated with catecholaminergic polymorphic ventricular tachycardia through a comprehensive review of medical history, examination findings and genetic testing. Propranolol was used for treatment, and the two patients didn’t experience episodes of syncope during follow-up for 6 months. Besides, literature associated with catecholaminergic polymorphic ventricular tachycardia and ryanodine receptor 2 mutations was reviewed.

Recurrent syncope during exertion or stress should be focused on catecholaminergic polymorphic ventricular tachycardia caused by ryanodine receptor 2 gene mutations. The genetic testing is a crucial tool in confirming the mutation of catecholaminergic polymorphic ventricular tachycardia. Early recognition of this disease, timely diagnosis of ryanodine receptor 2 gene mutations, and administration of appropriate pharmacological agents or ICD implantation are critical to ensure favourable clinical outcomes.