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.

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.

Variations in venous circulation can affect either the superior or inferior vena cava, with diagnoses often being incidental and presenting with highly variable symptoms. This rare case discusses the detection and investigation of bilateral superior vena cava agenesis in an asymptomatic patient.

Transvenous and epicardial pacing in children carries significant risks of lead failure and infection. Leadless pacing is an alternative with lower risks of infection, lead failure, and longer battery longevity. A retrievable leadless pacemaker was implanted in an 8-year-old, 24 kg patient with heart block. A systematic review evaluated procedural success and the safety of leadless pacemakers in preadolescents weighing less than 30 kg.

Partial anomalous left pulmonary artery sling is an exceptionally rare congenital anomaly, and its coexistence with ventricular septal defect and right lung agenesis has not been reported. We describe a neonate presenting with right lung agenesis, a large ventricular septal defect, partial anomalous left pulmonary artery sling, and congenital tracheal stenosis. Following surgical repair of the ventricular septal defect at 73 days of age, the patient showed marked improvement in respiratory status and was discharged home without respiratory support.

Left atrial appendage thrombosis is exceedingly rare in neonates and may present with nonspecific findings. We report a term neonate presenting with poor feeding and a gallop rhythm, who was found to have a large left atrial appendage thrombus with ventricular dysfunction. Surgical excision resulted in rapid recovery, highlighting the importance of early echocardiographic evaluation in subtle neonatal heart failure.

Fontan-associated lymphatic failure may result in severe complications such as plastic bronchitis. We report a 6-year-old Fontan patient with refractory plastic bronchitis despite catheter-based interventions. Surgical thoracic duct decompression via innominate vein rerouting to the right atrium resulted in marked symptomatic improvement and resolution of bronchial cast expectoration, highlighting its role in refractory Fontan-related lymphatic disease.

Middle aortic syndrome is a rare vascular disorder characterised by segmental narrowing of the distal thoracic aorta and/or proximal abdominal aorta, often accompanied by renal artery involvement. The condition may arise from genetic abnormalities, acquired factors, or idiopathic causes. In this study, we report the case of a 9-year-old boy who was found to have hypertension during a routine school health examination. Computed tomography angiography confirmed the diagnosis of middle aortic syndrome, revealing long-segment stenosis of the distal thoracic aorta. Following a comprehensive assessment, the patient underwent descending aortic reconstruction with a 14 mm Intergard knitted vascular graft. At the 6-month follow-up, imaging demonstrated satisfactory graft patency, and the patient’s blood pressure remained well-controlled without the need for antihypertensive medications.

We report an extremely low birth weight infant (494 g, 23 weeks + 1 day) with transient right ventricular hypertrophy, initially suspected as pulmonary atresia with ductus-dependent circulation. Patent ductus arteriosus management was complicated, requiring low-dose PGE1 and eventual surgical ligation. Serial echocardiography revealed progressive right ventricular dilation and normalisation of function, confirming transient hypertrophy. This case highlights diagnostic challenges and the importance of repeated cardiac assessment in extremely preterm infants.

Marfan syndrome (MFS, OMIM #154700) is an autosomal dominant disorder that presents a challenging diagnosis due to its wide clinical variability. The prenatal diagnosis of the syndrome is rare and often associated with unfavourable prognoses, such as early mortality. This case report, written by scientist parents, aims to document the clinical journey, management, and development of a paediatric patient diagnosed with Marfan syndrome at 24 weeks of gestation. In the immediate postpartum period, the patient did not show any severe warning signs, such as tachypnoea or prostration. The initial findings included mild dilation of the aortic and pulmonary roots, funnel chest, downward-slanting palpebral fissures, and elongated fingers. Subsequent multidisciplinary follow-up revealed motor delays, hypotonia, and orofacial alterations. Despite the repercussions of Marfan syndrome, the patient’s overall health is positive, and their development is consistent with what is expected for childhood. This case report offers a humanised and multidisciplinary perspective on the patient’s journey, highlighting the importance of early diagnosis, access to healthcare services, and information to redefine the course of clinical histories, offering hope to other families.

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 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.

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.

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.

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.

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.