Aorto–left ventricular tunnel is a rare congenital cardiac anomaly characterised by an extracardiac channel connecting the ascending aorta directly to the left ventricle. Surgical repair in neonates poses significant challenges due to the diminutive size and close proximity of critical structures such as the aortic valve and coronary ostia. We report the case of a neonate with an aorto–left ventricular tunnel, mild left ventricular dysfunction, and mild aortic insufficiency who underwent successful surgical repair on the sixth day of life. Following cardiopulmonary bypass initiation and cardioplegic arrest, the ascending aorta was transected for direct visualisation. A 5-mm 0° rigid endoscope was employed intraoperatively, providing high-resolution magnification of the aortic valve, right coronary artery, and tunnel anatomy. The tunnel was closed using an autologous pericardial patch, ensuring preservation of the coronary ostium and aortic valve integrity. Postoperative echocardiography demonstrated good left ventricular function, trivial aortic insufficiency, and no residual shunt. The use of rigid endoscopy significantly enhanced the safety and precision of the repair by allowing accurate anatomical delineation and facilitating patch placement. This case highlights the importance of detailed visualisation in neonatal aorto–left ventricular tunnel repair and underscores the utility of rigid endoscopic technology in complex congenital heart surgery. Endoscopic assistance not only aids intraoperative decision-making but also serves as a valuable tool for surgical education and postoperative review.

Double aortic arch is an exceedingly rare congenital vascular anomaly, and its association with anomalous origins of the vertebral arteries is even more uncommon. Enhanced computed tomography revealed a double aortic arch with the left common carotid artery, left vertebral artery, and left subclavian artery originating from the left arch, and the right common carotid artery, right vertebral artery, and right subclavian artery arising from the right arch. To our knowledge, this is the first report of a double aortic arch with six distinct vessels originating from both arches. Enhanced CT should be considered in double aortic arch patients to identify such anomalous origins of branch arteries.

We present striking and unique imaging features of a child with scimitar syndrome, demonstrating a rare ‘double sword appearance’ across multiple imaging modalities including chest radiography, angiography and computed tomography.

This report describes a 41-year-old female with left isomerism, interrupted inferior caval vein with azygos continuation, dextrocardia, and repaired tetralogy of Fallot, who underwent percutaneous pulmonary valve implantation using the Venus P-valve system. Due to anatomical constraints, left jugular venous access was utilised. A Venus P-valve (30 by 25 mm) was successfully implanted in the right ventricular outflow tract using a simplified, one-curve trajectory directly on its delivery system without a delivery sheath. Contrast injections during valve implantation were not possible, and the pre-implanted duct occluder was our anatomical landmarks. This case highlights the adaptability of the Venus P-valve and the importance of individualised procedural strategies in addressing anatomical challenges and achieving optimal outcomes.

Interrupted aortic arch is an uncommon cardiac anomaly characterised by a lack of continuity between the ascending and descending aorta. The presence of interrupted aortic arch in adults is extremely rare, and there is limited documentation of such cases in the literature. In this article, we present a unique case of interrupted aortic arch in an adult diagnosed through angiography. This case falls under the anatomical classification of type B interruption, although the blood supply to the left subclavian artery originates from the ascending aorta. Its haemodynamic characteristics are completely different from those of the classical type B interruption.

A neonate with unbalanced right-dominant atrioventricular canal defect with intact atrial septum and abnormal pulmonary venous return developed bradycardic arrest and a subsequent diagnosis of tracheal rings and left lung aplasia. This report details the fatal nature of the triad of hypoplastic left heart, pulmonary venous obstruction, and airway anomalies, along with the role of advanced imaging in prognostication.

Pulmonary veno-occlusive disease has no definitive cure apart from lung transplant. The reverse Potts shunt can be a palliative bridge to transplant. A post-arrest 14-year-old with severe pulmonary veno-occlusive disease on extracorporeal membrane oxygenation support was decannulated after reverse Potts shunt and survived to lung transplant. Reverse Potts shunt should be considered as a rescue in select patients with end-stage pulmonary veno-occlusive disease.

We evaluated quality characteristics of cardiac point-of-care ultrasound performed in our paediatric cardiac ICU. Of 47 included patients, diagnostic imaging was feasible in a high percentage of patients with good image quality. Intensivist interpretations of images were highly specific for dichotomised abnormal findings when compared to interpretation from a cardiologist with expertise in imaging but challenges in accurately assessing depressed ventricular function and the risk of discrepant interpretations exist even amongst frequent users with extensive training and experience. These findings may serve as a template for continued development of cardiac point-of-care ultrasound in a high-impact clinical environment.

Recommendations for immunisation practices in children with single ventricle CHD are lacking. A survey of 53 heart centres received responses from 40 centres (33 complete and 7 partial) revealing variability in immunisation recommendations. Only 11% have a written protocol. Immunisations were delayed before cardiopulmonary bypass in 94% (32/34) and after cardiopulmonary bypass in 97% (30/31), with 34% (13/38) re-dosing some immunisations post cardiopulmonary bypass. Further research is needed to develop guidelines.

Isolated left ventricular apical hypoplasia is a rare cardiac malformation. This entity is easily diagnosed if its key features are recognised, but will puzzle the clinician who is not aware of its existence. It is characterised by echocardiographic findings of a spherical left ventricle, an elongated right ventricle wrapping around the deficient left ventricular apex, and abnormal papillary muscles’ origin.

Ivabradine has been shown to be safe and effective in treating congenital and postoperative junctional ectopic tachycardia in children; however, in recent years, case reports have suggested that it may also be used to treat automaticity-related ectopic atrial tachycardia and focal atrial tachycardia. Here, we discussed a case in which we used ivabradine to induce sinus rhythm in a 38-day-old baby who had resistant supraventricular tachycardia and investigated potential accessory pathway-related tachycardia.

Noonan syndrome is associated with lymphatic system structural abnormalities and may present with potentially fatal refractory chylothorax. We report a 2-year-old boy with Noonan syndrome with non-traumatic chylothorax who was refractory to dietary therapy with medium-chain triglyceride milk, octreotide, prednisolone, lymphatic embolisation, and lymphatico-venous anastomosis but improved with etilefrine administration. Etilefrine may be a treatment option for paediatric chylothorax, regardless of the aetiology.

Heart failure in children is a clinical and pathophysiological syndrome arising from ventricular dysfunction and pressure or volume overload of the circulatory system. Features of paediatric heart failure include feeding problems, poor weight gain, exercise intolerance, or dyspnoea. The aetiology of heart failure in children is complex, with the primary causes being CHD and cardiomyopathies. Cardiomyopathies occur at an incidence of 1.13–1.24 cases per 100,000 children. The prevalence of cardiomyopathy is estimated to be 7.8–8.3 cases per 100,000 infants, particularly common in patients under one year of age presenting with severe heart failure symptoms. Mitral valve insufficiency is a significant source of morbidity in children with dilated cardiomyopathy. Severe mitral insufficiency can lead to a decrease in cardiac output, independent of the left ventricular ejection fraction, exacerbating the clinical course of heart failure in patients with dilated cardiomyopathy. As ventricular systolic function deteriorates, the options for treating mitral insufficiency decrease, leading to a loss of surgical intervention opportunities and making heart transplantation the only viable option. Close monitoring of mitral valve insufficiency in children with dilated cardiomyopathy is essential, as it may lead to decompensated heart failure. In patients who have lost the chance for valve surgery due to decompensation, the application of left ventricular assist device can help improve the decompensatory state and contribute to the reduction of left ventricular diastolic and systolic dimensions, consequently leading to improvements in the dilation of the mitral annulus and severe mitral insufficiency findings. Further studies are needed to determine the optimal timing for surgery in patients who have not missed the chance for valve surgery due to a decrease in ejection fraction.

We describe caudal analgesia agent, dose, reported adverse events, and outcomes in a single-centre, retrospective cohort study of 200 patients undergoing cardiac surgery from October 2020 to April 2023. Median (interquartile range) doses of clonidine and morphine were 2.7 (2.1–3) mCg/kg and 0.12 (0.1–1.12) mg/kg, respectively. Our findings suggest that a clonidine/morphine caudal was tolerated in cardiac surgical patients.

A 3-year-old girl with a large ductus arteriosus had increased pulmonary vascular resistance at cardiac catheterisation. Test occlusion of the ductus arteriosus with a Nit-Occlud PDA-R device and hyperoxia decreased the pulmonary arterial pressure to < 50% of systemic level. The ductus was closed with the same device. Two years later, an echocardiogram showed normal pulmonary arterial pressure.

Hyperlactatemia is a common and concerning finding in the paediatric cardiac ICU as it may signify tissue hypoperfusion and/or hypoxia. However, it is important to include other aetiologies for an elevated lactate in the differential, especially when the lactate is significantly elevated (> 8 mmol/L). We present the case of metabolic acidosis with severe hyperlactatemia secondary to Warburg effect and presumed thiamine deficiency in a paediatric heart transplant patient with post-transplant lymphoproliferative disorder.

Premature atrial complexes are frequent among patients after heart transplantation. We herein report on the successful use of ivabradine in a 15-year old patient who exhibited marked palpitations caused by atrial premature complexes after orthotopic bicaval heart transplantation.

After right ventricular outflow tract obstruction reconstruction for CHD, surgical pulmonary valve replacement, or transcatheter pulmonary valve replacement may be performed if complicated by moderately severe or severe right ventricular outflow tract dysfunction. However, for patients whose anatomy is not suitable for transcatheter pulmonary valve replacement, surgical pulmonary valve replacement is the only option, but it has a higher rate of perioperative complications and longer hospitalisation for patients. In two cases of severe transcatheter pulmonary valve replacement in which percutaneous pulmonary valve implantation could not be performed directly due to the presence of a large right ventricle, a significant decrease in right ventricular function, and significant widening of the pulmonary arteries, we used a new hybridisation procedure to partially fold and reduce the diameter of the significantly widened pulmonary artery trunk without cardiopulmonary bypass by surgically opening the thorax in a median way, and then successfully implanted a percutaneous self-expanding pulmonary valve valve, with good immediate results in the postoperative period. The postoperative recovery was rapid and the recent results were excellent.