A young woman with Epstein’s anomaly had a large endocardial scar in the atrialised ventricular myocardium. In patients with significant preoperative ventricular scarring, more information is needed to help appropriately manage the risk of ventricular arrhythmias and sudden death.

We describe the case of an 8-week-old infant with late presentation of severe refractory atypical Kawasaki disease. In addition to developing giant coronary arterial aneurysms and coronary thrombosis, she formed extensive bilateral arterial aneurysms throughout her systemic circulation.

Rapid prototyping is quickly gaining utility in various complex forms of CHD. In properly selected cases, these printed models provide detailed anatomical understanding that help guide potential surgical and cardiac catheterisation interventions. We present a case of a tunnel-like ventricular septal defect referred for surgical repair, where the decision to obtain a three-dimensional printed model helped in better understanding of the anatomy, leading to delaying, and hopefully avoiding altogether, surgical repair.

A 2-year-old girl with a functionally univentricular heart associated with a pulmonary sequestration underwent right lower lobectomy after which increased lung volume with low mean pulmonary artery pressure and pulmonary vascular resistance was documented. A cardiac catheterisation performed after a subsequent total cavopulmonary connection demonstrated favourable Fontan haemodynamics. Lobectomy may have induced compensatory lung growth, contributing to the maintenance of haemodynamics favourable for the long-term success of the Fontan procedure.

We describe a case of anomalous left coronary artery from the pulmonary artery in association with total anomalous pulmonary venous return. The infant was diagnosed with total anomalous pulmonary venous return at 6 weeks of age and underwent successful surgical repair. On routine follow-up, he was found to have an anomalous left coronary artery from the pulmonary artery without evidence of mitral regurgitation or left ventricular dysfunction. The presence of the left-to-right shunt and secondary elevation in pulmonary artery pressures likely masked the usual findings associated with this coronary anomaly.

We report a case of Uhl’s anomaly imaged at 19 weeks of gestation by fetal echocardiography with pathological confirmation by anatomical gross heart specimen and tissue histology. Uhl’s anomaly of the right ventricle is a rare cardiac disorder with isolated right ventricular enlargement with almost complete absence of the right ventricular myocardium.

Absent pulmonary valve with an intact ventricular septum is a rare malformation. We report a case of absent pulmonary valve and intact ventricular septum with functional near-tricuspid atresia caused by pulmonary regurgitation. Initial palliation with main pulmonary artery ligation and bilateral pulmonary artery banding was performed at 1 day of age. More antegrade flow across the tricuspid valve was recognised postoperatively, resulting in a successful right ventricular outflow tract reconstruction by a hand-sewn bileaflet polytetrafluoroethylene valve and modified Blalock–Taussig shunt at 11 days of age.

Incorrect pacemaker lead placement into the systemic ventricle is a complication that has rarely been described in patients with D-transposition status after atrial baffle palliation. We present a case of ventricular lead misplacement in the systemic right ventricle of a patient with D-transposition of the great arteries after Mustard procedure. This case demonstrates the challenges with proper imaging of lead placement in patients with atrial baffles and long-term management of a lead in the systemic ventricle.

Several surgical approaches for completion of a total cavopulmonary connection have been established for patients with anomalies of systemic venous connection in association with a functionally univentricular heart. We report an alternative technique designed to address the problem of widely separated inferior systemic veins, in which two extracardiac conduits were used. This technique was performed on three patients and yielded excellent short-term results, allowing us to operate on a beating heart with no need for cardioplegic arrest and aortic cross-clamping.

Mitral-aortic intervalvular fibrosa aneurysm is a rare disease with a few cases documented in children. We present the case of a 12-year-old boy, without a history of infection or previous surgery, diagnosed with MRI.

We present an interesting and rare case of traumatic Gerbode ventricular septal defect and complete heart block. The multimodality images illustrate the diagnosis well. This case is an excellent demonstration of the diagnostic utility of multimodality imaging.

In this study, we describe the case of a 36-year-old woman who was diagnosed with hepatocellular carcinoma on a background of Fontan procedure for tricuspid atresia. She had worsening heart failure in the months before presentation, and early investigations noted derangement in liver enzymes and hepatomegaly. Liver biopsy confirmed a hepatocellular carcinoma. Hepatocellular carcinoma is a rare but recognised consequence of cardiac cirrhosis in Fontan patients.