This case report presents mid- to long-term outcomes of thoracic endovascular aortic repair for high-risk type B aortic dissection in a 15-year-old patient with myosin heavy chain protein 11 genetic mutations. A CT scan showed the primary entry tear located on the lesser curvature near the left subclavian artery, and the dissection originated from the descending aorta to the abdominal aorta, accompanied by 80% narrowing of the true lumen. Following successful endovascular therapy using stent-grafts, the patient recovered well. A four-year follow-up CT scan showed significant thoracic aorta remodelling.

Double aortic arch with bilateral interruptions is a rare abnormality. Indeed, to the best of our knowledge, it has not previously been reported before. Rare variants, such as a double arch with ligamentous atresia of the left component and double arch with bilateral coarctation, have received previous attention. These rare examples can pose significant diagnostic challenges for those relying on transthoracic echocardiography. Use of cardiac CT or MRI is now critical for prompt surgical planning and correction.

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.

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.

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.

We report a case of foetal-onset biventricular noncompaction with an MYH7 variant. Prenatal fetal hydrops and right heart failure resolved after birth, but progressive left heart failure developed at 5 months. This transition reflects how postnatal hemodynamic changes influence the biventricular noncompaction phenotype, necessitating vigilant monitoring for left heart failure in foetal-onset biventricular noncompaction cases with MYH7 variants.

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 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.