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 patient with borderline left ventricle successfully underwent biventricular repair following a staged surgical approach to promote left ventricular growth. Despite initial concerns about left ventricle size, apex formation and adequate size of atrioventricular valve indicated potential for future growth. The patient demonstrated significant left ventricular growth, resulting in stable biventricular circulation and a favourable outcome over a three-year postoperative follow-up period.

Clinical Registration Number: Institutional Review Board of Osaka City General Hospital, number 1902139.

Tricuspid atresia with absent pulmonary valve and intact ventricular septum is an exceedingly rare CHD. Only few cases with completed single ventricle palliation have been reported in the literature. We present the follow-up case of a 4-year-old child with this cardiac anomaly who underwent Fontan operation after complete exclusion of the right ventricle.

Primary cardiac tumours are rare. We report a patient with right ventricular cavernous haemangioma, in which non-invasive cardiovascular imaging including echocardiography and MRI helped in the diagnosis with a high pretest probability. The patient also had typical slow-fast atrioventricular nodal reentrant tachycardia, which was an incidental finding and not related to the tumour.

Coronary to pulmonary artery fistula is a rare malformation that can be detected incidentally because most cases are asymptomatic. Here, we present multiple micro coronary to pulmonary artery fistulas in a Fontan physiology adult patient, which leaded dyspnoea due to elevated pulmonary artery pressure and were successfully closed with vascular plugs from the pulmonary side.

Scimitar syndrome is characterised by anomalous right pulmonary venous drainage to the inferior vena cava associated with right pulmonary artery hypoplasia. We present a case of an infant with Scimitar syndrome with a well-formed right pulmonary artery, in high Qp heart failure who was managed with right pulmonary artery banding after thorough haemodynamic evaluation.

Despite advances in the development of systemic anticoagulants, there remain few agents approved for utilisation in patients with mechanical heart valves. Currently, recommendations for periprocedural and long-term anticoagulation in mechanical heart valves include unfractionated heparin or vitamin K antagonists, and there are some reports for off-label use of low-molecular-weight heparins. Emerging data on parenteral direct thrombin inhibitors, such as bivalirudin, have led to increased utilisation in both extracorporeal membrane oxygenation and ventricular assist devices. We present the case of a paediatric patient with rheumatic heart disease who had significant bleeding on unfractionated heparin who successfully received prolonged bivalirudin therapy in the setting of mechanical aortic and mitral heart valves.

The prognosis of neonatal Ebstein’s anomaly requiring early surgery is poor, especially with a circular shunt. We report a critically ill case managed with emergency two-stage Starnes surgery immediately after birth. We hypothesised that the three components of the Starnes procedure, right ventricle exclusion, atrial septal defect creation, and systemic-to-pulmonary shunt, could be organised and reconstructed in a consecutive two-stage strategy to control postoperative circulatory fluctuations.

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.

Biallelic pathogenic variants in the inorganic pyrophosphatase 2 (PPA2) gene are a rare but established cause of sudden infant death, which may be precipitated by a pyrexial or viral illness. It has also been associated with sudden death secondary to alcohol ingestion in young adults. We describe the case of a thirteen-month-old female who presented following out-of- hospital cardiac arrest and was subsequently diagnosed with compound, heterozygous pathogenic variants of PPA2. She survived this episode and made excellent neurological recovery despite her prolonged out-of-hospital resuscitation. This case describes her initial presentation, consideration of PPA2-related cardiac arrest in the differential diagnosis, and the subsequent secondary preventative strategies. These included insertion of an implantable cardiac defibrillator, avoidance of alcohol in her medication preparations and administration of childhood vaccinations in a hospital environment due to risk of pyrexia-induced arrhythmias.

This case underscores an uncommon presentation of Pulmonary artery hypertension which, to our knowledge, has limited neonatal data. Its atypical course reveals diagnostic pitfalls that, if unrecognized, may delay effective therapy. This report highlights options for early recognition and management. It serves as a reminder that clinicians should maintain vigilance for a simple respiratory distress, even in settings where it is not expected. Ultimately, this case adds to the limited literature and could influence practice by expanding differential diagnoses, refining diagnostic algorithms, or prompting guideline modifications in similar clinical scenarios.

Staged Fontan pathway treatment is a recognised surgical approach for managing congenital heart lesions with single ventricle physiology. Some patients necessitate communication between the tunnel and the atrium to maintain circulatory balance. During follow-up, adjustments to fenestration size may be required. While methods for enlarging or completely closing fenestrations are common, partially reducing flow through a fenestration remains challenging. We present an effective technique for partially reducing the size of a large, stented fenestration using a coronary stent and a vascular occluder.

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.

We present the case of a 9-year-old girl with a distal aortopulmonary window (APW) with anomalous origin of right coronary artery from the main pulmonary artery and an isolated left subclavian artery arising from the main pulmonary artery. This report highlights the unusual combination of the three anomalies and late presentation of APW.