Anomalous aortic origin of the right coronary artery poses serious risks when involving high-risk features such as a long intramural course or a slit-like orifice, with demand ischaemia as the likely pathophysiology of sudden cardiac arrest. This case links sudden cardiac arrest to demand ischaemia, confirmed by cardiac MRI showing a transmural infarct in the right coronary artery territory. This is a rare case demonstrating transmural infarction in a patient with an anomalous aortic origin of the right coronary artery, as demonstrated by cardiac MRI findings.

Hearts with a double-inlet and double-outlet right ventricle are infrequent. Due to their infrequency, it is unclear whether there are natural patterns in hearts like this. A systematic review was performed to identify published cases of double-inlet double-outlet right ventricle. Characteristics for individual reported hearts were collated and entered into a cluster analysis. Hearts with double-inlet, double-outlet right ventricles tended to fall into two clusters largely based on aortic atresia and systemic venous connections.

We present the case of an 8-year-old male with frequent premature ventricular contractions and mild left ventricular dysfunction, who was found to carry heterozygous variants in both FLNC and CTNNA3. Medical therapy resulted in improved systolic function and reduced arrhythmia burden. This rare combination suggests a potential genotype–phenotype correlation in paediatric arrhythmogenic cardiomyopathy.

This study examined the incidence, type of electrocardiogram abnormalities, and associated risk factors in healthy paediatric athletes. A single-centre retrospective cohort study was conducted on a local male youth competitive soccer programme, ages 11–18. The primary outcome was the incidence of electrocardiogram abnormalities. The secondary outcomes were recurrence and the type of electrocardiogram abnormalities. Covariables were age, race, family history, and current medications. Statistical analysis program was used for analysis. There was a total of 368 electrocardiograms reviewed from 2021–2024, of which 68 (18.5%) had at least one abnormality. 182 unique athletes were screened. A higher proportion of multi-racial and African American athletes had electrocardiogram abnormalities (p-value 0.02). In our sample of healthy paediatric athletes, electrocardiogram abnormality incidence was similar to that noted in other studies. There were no significant cardiac findings noted after further evaluation on any of the athletes with abnormal electrocardiogram findings. Our data support that universal electrocardiogram screening of healthy paediatric athletes is not beneficial.

We report a 13-year follow-up of an infant with severe Kawasaki disease complicated by bilateral axillary artery aneurysms. Right-sided occlusion led to upper limb hypoplasia, while progressive changes in the contralateral aneurysm produced unilateral digital clubbing. This unique combination of contralateral limb hypoplasia and unilateral clubbing, documented through serial imaging, morphometric measurements, and microvascular assessment, highlights how long-term arterial flow disturbances can result in asymmetric peripheral outcomes. This case underscores the need for extended vascular screening in severe Kawasaki disease.

A congenital left atrial appendage aneurysm represents a very rare entity. CT angiography proved to be a valuable diagnostic tool, allowing for effective diagnosis and precise visualisation of spatial relationships.

We present a 14-year-old female with a history of ventricular septal defect repair who presented with a striking complaint of a heartbeat sensation in the neck and was subsequently diagnosed with a rare cervical aortic arch, classified as Zhong’s B2 and Haughton’s B type, associated with a Kommerell diverticulum. Comprehensive cross-sectional imaging and conventional angiography excluded true and pseudo-coarctation, highlighting the importance of meticulous anatomical assessment in directing treatment and preventing unnecessary interventions in asymptomatic or non-obstructive cases.

The ductus venosus plays a critical role in fetal circulation. Postnatal persistence of the ductus venosus (PDV) is associated with secondary portal vein hypoplasia and results in a porto-systemic shunt. In this extremely rare case, we present a 6-year-old male diagnosed with PDV, who had a history of elevated liver enzymes and mild hepatic dysfunction first detected at 11 months of age. Due to the presence of PDV, endovascular closure was planned. Portal venous pressure was measured as 10 mmHg both before and after balloon occlusion. Percutaneous occlusion was successfully performed using a vascular plug. Post-intervention ultrasonography confirmed complete occlusion of the ductus venosus and increased portal vein flow. During follow-up, liver function tests returned to normal; a slight elevation in transaminases persists. PDV is a rare congenital vascular anomaly that may present with a wide spectrum of clinical symptoms. While some patients may be diagnosed during early infancy due to cholestatic jaundice, hepatic dysfunction, or hyperammonaemia, others may remain asymptomatic and undiagnosed for years. Endovascular closure represents a minimally invasive treatment option. A balloon occlusion test to measure portal venous pressure is recommended before and after the procedure to evaluate the safety of shunt closure.

Restrictive cardiomyopathy is a rare paediatric myocardial disorder causing diastolic dysfunction with preserved systolic function. We report a 14-year-old girl with idiopathic restrictive cardiomyopathy and recurrent systemic thromboemboli (stroke, limb ischaemia, renal infarction, and intracardiac thrombi) despite sinus rhythm. Anticoagulation resolved thrombi. She is currently awaiting heart transplantation. Early recognition, prompt anticoagulation, genetic evaluation, and consideration for transplantation are essential to optimise outcomes.

We report successful staged palliation of a rare cardiac anomaly: double outlet right ventricle with intact ventricular septum. Management included neonatal atrial septostomy, pulmonary artery banding, septectomy, lobectomy for congenital emphysema, and left ventricular exclusion to decompress the left ventricle. The patient progressed to a bidirectional Glenn and to a Fontan circulation, demonstrating a rare favourable outcome.

Stressful encounters within the neonatal or early infant period are harmful both acutely and longitudinally. Prior research on stress exposure in hospitalised infants excludes infants with CHD, limiting our understanding of stress exposure in this uniquely vulnerable population. This study aimed to identify and describe sources of stress, stress cues, stress responses, and clinical implications in neonates and infants undergoing cardiac surgery. Conducted at tertiary cardiac centres, 17 expert clinicians in nursing, anaesthesia, surgery, and intensive care medicine were included. Participants represented two care areas (cardiac intensive care unit, operating room) and three phases of care (preoperative, operative, postoperative). Using individual semi-structured interviews informed by Selye’s General Adaptation Syndrome, clinicians were asked about their perceptions of sources of infant stress within each phase of care and signs or cues of overwhelming stress or of stress tolerance. Utilising semantic content analysis, responses were analysed thematically and by frequency. Seven themes were identified involving sources of infant stress (Clinical Environment, Operative Stress, Disrupted Bonding, Cardiac Physiology), and clinician recognition and response to infant stress (Cognitive Integration, Infant Protection, Balance of Care). Perceived sources of infant stress were identified and together describe infant stress burden related to cardiac surgery. This study highlights the concept of infant stress specific to cardiac surgical intervention and offers a foundation to recognise and address infant stress as part of comprehensive cardiac care. The results may inform future research evaluating stress exposure and determining whether stress reduction strategies can improve outcomes in this high-risk population.

Takotsubo cardiomyopathy can be triggered by stress. We report a 2-year-old boy with a single right ventricle after bidirectional Glenn surgery who developed Takotsubo cardiomyopathy following coil embolisation of collateral arteries and subsequently underwent successful Fontan completion.

Echocardiography and computed tomographic angiography in a three-month-old boy confirmed a right coronary artery fistula and a right coronary artery aneurysm. The patient was successfully operated via right axillary thoracotomy approach.

Acute myocardial infarction is rare in paediatric patients, with limited published data on incidence, diagnosis, management strategies, and outcomes. A systematic retrospective review was conducted on patients aged 1 day to 21 years who presented with concerns for ST-elevation myocardial infarction at a paediatric institution and an affiliated adult hospital (1/2013–12/2023). Out of 965 screened patients, 13 met the inclusion criteria. The cohort had a mean age of 15 years (standard deviation ± 4 years), with the youngest patient being 11 months old. The study population consisted of 12 males (92%) and 1 female (8%), with ethnic distribution as follows: Caucasian (69%), African American (15%), and Hispanic (15%). An electrocardiogram with ST-segment changes was observed in all patients. Echocardiography revealed a normal ejection fraction in 54% of patients. Most patients (54%) had CHD, with three patients (23%) being undiagnosed at the time of the initial presentation. Seven patients (54%) underwent cardiac catheterization at the adult hospital, while 2 patients (15%) had coronary evaluation at the paediatric facility. One percutaneous catheter intervention was performed in a patient with known coronary stenosis at the paediatric facility. Three patients (23%), all with congenital heart disease, had cardiac arrest during cardiac catheterisation, resulting in extracorporeal membrane oxygenation cannulation. These findings suggest evaluation of pediatric patients presenting with ST-segment elevation can be performed at a pediatric center to rule out congenital heart disease, especially prior to referral to an adult facility for coronary intervention.