Circular shunt is a very rare, albeit critical, condition. We present a case of a post-surgical circular shunt that resulted after correction of an anomalous total pulmonary venous connection to the coronary sinus with a persistent left superior vena cava. A left cavo-pulmonary anastomosis was performed; however, she developed heart failure during follow-up. The anastomosis was closed by interventional cardiac catheterisation without complications that led to resolution of heart failure symptoms.

Three-dimensional computed tomography provides an accurate interpretation of anomalous coronary arteries and related anatomy, which helps determine the ideal surgical approach in rare cases.

This case describes a 32-year-old adult male who was incidentally diagnosed with isolated dextrocardia, Double inlet left ventricle (l-looped), l-transposed great arteries with subpulmonary stenosis during evaluation for an orbital abscess. This case highlights protective factors that enabled this patient’s survival into adulthood without cardiac surgeries or medications despite single ventricle physiology, namely his “self-banded” pulmonary flow.

Bodily isomerism, often referred to as “Heterotaxy,” is the clinical entity in which the thoracic organs are themselves mirror-imaged in the same individual. The thoracic isomerism can be right or left. The left thoracic isomerism always has both atria with their atrial appendages of left morphology and frequently a mixed atrioventricular connection. Usually, there are two well-developed ventricles; however, in some cases, there may be univentricular physiology. In the set of left thoracic isomerism, no case of imperforate tricuspid valve with severe hypoplasia associated with a vascular ring has been reported in the literature.

Dextro-transposition of the great arteries is a critical CHD traditionally considered sporadic, with low familial recurrence. Emerging evidence suggests a genetic component in select cases, particularly with rare familial clustering. We report concordant d-TGA in monochorionic diamniotic twins, a highly unusual occurrence, strengthening the argument for a heritable predisposition.

Wolff-Parkinson-White syndrome, characterised by accessory pathways, is rarely seen with dextrocardia. We present a case of situs inversus-dextrocardia with Wolff-Parkinson-White syndrome successfully treated via catheter ablation using 3D mapping (EnSite Precision®). Adjustments included reversed electrocardiogram (ECG) electrode placement, EnSite patch positioning, and fluoroscopic views. Coronary sinus access required counterclockwise manoeuvers. Mapping identified a left-sided accessory pathway, necessitating transseptal puncture with mirror-image adjustments. Ablation at the optimal site resulted in success. This case highlights the feasibility of catheter ablation in dextrocardia with tailored procedural modifications.

We report the case of a 15-year-old male patient with severe pulmonary regurgitation who successfully underwent Alterra Adaptive Prestent implantation through the right jugular vein because of bilateral femoral vein occlusion.

We described a 14-year-old boy who underwent catheter ablation for atrial tachycardia that had difficulty in creating the whole circuit of 3D map due to widely spread scar after repeated surgery for multivalvular heart disease. The classical atrial entrainment method was very effective in planning the catheter ablation for the invisible circuit of the atrial tachycardia.

Pulmonary valve or main pulmonary artery infective endocarditis is rare in children and is associated with high morbidity and mortality. Fungal infective endocarditis, most commonly caused by Candida species, is particularly aggressive and often requires a combination of antifungal therapy and surgical intervention. We report two infants who developed Candida endocarditis following pulmonary artery banding.

In the first case, a female infant developed persistent candidemia after pulmonary artery banding, with echocardiography revealing a mobile mass at the pulmonary bifurcation and computed tomography angiography demonstrating a mycotic pseudoaneurysm. Blood cultures confirmed Candida albicans. She underwent debanding and main pulmonary artery reconstruction, later requiring pacemaker implantation, and recovered without relapse. In the second case, a female infant presented with fever and candidaemia after pulmonary artery banding. Echocardiography identified a distal main pulmonary artery vegetation, and cultures grew Candida parapsilosis. She received amphotericin B–based induction therapy followed by fluconazole step-down after surgical source control, achieving clinical cure at follow-up.

These cases highlight the diagnostic and therapeutic challenges of Candida endocarditis after right-sided palliation. Early multimodality imaging, species-directed antifungal therapy, and timely surgery are critical to optimise outcomes in this rare but life-threatening complication.

Neonatal truncal valve insufficiency carries high postoperative mortality. Mechanical replacement is challenging, and leaflet-sparing repair alone may be insufficient due to fragile tissue and annular dilatation. We report a successful case using geometry-based root reconstruction guided by body-weight–adjusted reference values. This native-tissue approach restored physiologic root geometry, preserved the valve, and enabled growth potential, demonstrating a feasible strategy for durable neonatal repair.

Electrocardiogram interpretation is critical in neonatal care, as artefacts may mimic life-threatening arrhythmias. We report the case of a preterm newborn girl, delivered at 31 weeks of gestation due to acute fetal distress, who developed tachycardia shortly after birth. The initial ECG showed chaotic electrical activity, initially raising suspicion of artifact; however, a subsequent tracing confirmed a life-threatening arrhythmia with wide QRS complexes, prolonged QTc, and torsades de pointes. As the patient remained haemodynamically stable, intravenous lidocaine and magnesium sulphate were administered, achieving pharmacological cardioversion. Propranolol was subsequently initiated, and the lidocaine infusion was tapered and replaced with oral mexiletine. Serial electrocardiograms showed QTc interval improvement, left ventricular function normalised, and the patient was discharged on the 39th postnatal day following stabilisation and supportive care for prematurity. Genetic testing identified a de novo SCN5A c.796C > G (p.L266V) mutation associated with Long QT syndrome type 3. At 1 year of age, the patient remains stable and asymptomatic while receiving combined therapy with propranolol and mexiletine.

Aberrant subclavian artery arising from the pulmonary artery is an extremely rare anomaly of the aortic arch and is often associated with CHD. It can remain asymptomatic or result in subclavian or pulmonary steal syndrome. We present three cases with aberrant subclavian arteries (two left-sided, one right-sided) and reviewed 44 case reports through an extensive PubMed research to contextualise our findings to the existing literature. Fifty-five per cent of patients had a left, 45% had a right aberrant subclavian artery. The majority of patients (86%) had associated CHD. Blood pressure discrepancies and imaging—particularly transthoracic echocardiography, CT, and cardiac catheterisation—were pivotal in diagnosis. Aberrant subclavian arteries are a rare vascular anomaly, understanding of the embryology and anatomy is essential for the understanding of complex congenital heart variations. Early detection and surgical intervention are crucial to prevent complications.

Congenital junctional ectopic tachycardia is a rare arrhythmia that poses significant management challenges. This report presents a case of neonatal-onset congenital junctional ectopic tachycardia treated with cedilanid, amiodarone, and propafenone but persisted in episodes. Sinus rhythm was restored following the initiation of ivabradine therapy. The review of the literature indicates that ivabradine demonstrates efficacy in the treatment of paediatric junctional ectopic tachycardia, particularly in refractory cases, without significant side effects. These findings suggest that ivabradine has broad applications in the treatment of refractory arrhythmias.

Hepatopulmonary fusion is a rare defect describing the physical connection of liver and lung. A neonate with hypoplastic left heart syndrome arrived at the hospital with concern for congenital diaphragmatic hernia, later found to have hepatopulmonary fusion. In the coming months, he underwent a series of operations, including ligation of the hepatopulmonary fusion and the first two stages of surgical palliation of hypoplastic left heart syndrome. He ultimately died of septic shock at 15 months of age.

Pericardial cysts are rare, benign congenital cardiovascular malformations that account for approximately 7% of mediastinal masses. Epicardial cysts attached to the cardiac surface with intimate coronary artery involvement are even rarer and pose significant diagnostic and surgical challenges. This case highlights a giant pericardial cyst with intimate right coronary artery involvement in a 10-month-old infant, where subtotal resection was necessary to preserve coronary integrity. A 10-month-old male infant with a pericardial cyst initially detected at 27 weeks of gestation presented with progressive compression of right heart chambers. Imaging revealed a large multiloculated cystic mass (5.3 × 3.5 × 3.9 cm) compressing the right atrium and right ventricle, with associated pulmonary valve stenosis. Intraoperatively, the cyst was found on the epicardial surface with intimate involvement of the right coronary artery. Complete excision was not feasible due to the risk of coronary injury. The main cystic mass was excised with cavity obliteration, while the portion adjacent to the right coronary artery was intentionally preserved. Concurrent pulmonary valve commissurotomy and pulmonary artery augmentation were performed. Histopathology confirmed a mesothelial-lined pericardial cyst. The patient recovered uneventfully and was discharged. This case underscores the importance of comprehensive preoperative coronary artery assessment in pericardial cysts with atypical locations. When complete excision risks vital structure injury, subtotal resection with cavity obliteration represents a safe alternative strategy.

Persistent ductus venosus is an extremely rare disease that causes liver failure, hypoxaemia, and encephalopathy. We report the successful treatment of our patient with elevated transaminase and ammonia levels due to patent ductus venosus, diagnosed in the neonatal period and treated with a vascular plug device.

Pseudoaneurysm after valve-sparing aortic root replacement is uncommon but potentially life-threatening. We report an unusual case of spontaneous resolution of an aortic root pseudoaneurysm that developed in a 14-year-old boy with Marfan syndrome following valve-sparing aortic root replacement. This case suggests that, in carefully selected situations, non-surgical management with intensive follow-up may be beneficial.

With the passing of Richard Van Praagh in September of 2025, paediatric cardiology has lost one of its giants. With one of his greatest contributions, namely the segmental approach, he revolutionised the practice of paediatric cardiology. He and Stella, his wife and closest collaborator, developed the Cardiac Registry at Boston Children’s Hospital and published more than 300 articles. Together, they trained many fellows, with endless generosity and dedication, and gave conferences all over the world, spreading their ideas and concepts. Richard will be sorely missed, but he has transmitted to us the desire and the tools to continue to build on his firm foundations.