Kawasaki disease is a leading cause of acquired heart disease in children in the developed world, characterised by acute systemic vasculitis, with a complex aetiology that remains poorly understood. Recent studies have highlighted the potential anti-inflammatory effects of Interleukin-35 in various proinflammatory and cardiovascular conditions. However, the relationship between Interleukin-35 gene polymorphisms and Kawasaki disease susceptibility, particularly in Chinese children, has not been well-explored.

In this study, we investigated the association between five Interleukin-35 single-nucleotide polymorphisms—rs2243115, rs2243123, rs583911, rs353698, and rs2302164—and Kawasaki disease in a cohort of Chinese children. A total of ninety-four Kawasaki disease patients and one hundred healthy controls were enrolled, with the Kawasaki disease patients further divided into subgroups based on the presence or absence of coronary artery lesions and incomplete or complete Kawasaki disease. Genotyping of Interleukin-35 polymorphisms was performed using the MassARRAY system.

The results showed the GT + GG genotypes and G allele of rs2243115 (T > G) were significantly more prevalent in Kawasaki disease patients with coronary artery lesions than in those without coronary artery lesions, suggesting a possible association with the development of coronary artery lesions. Additionally, the G allele of rs353698 (A > G) was more frequently observed in the incomplete Kawasaki disease group compared to the complete Kawasaki disease group, suggesting a possible association with the risk of incomplete Kawasaki disease.

Patent ductus arteriosus is a common condition in preterm neonates, often necessitating medical or surgical intervention. This report presents a case of a preterm neonate born at 27 gestational weeks and who experienced patent ductus arteriosus rupture during a device closure procedure. Patent ductus arteriosus rupturing during device closure is rare and life threatening but can be successfully managed with prompt recognition and intervention.

This is a unique case of microbubbles incidentally found on echocardiogram in a child with unpalliated CHD. The microbubbles were found in both the venous and arterial circulations and spontaneously resolved. A detailed evaluation was done to identify the source of air in the circulation. The child did have concurrent necrotising enterocolitis, and it was felt that the bubbles were secondary to this and intestinal translocation of air.

The latent transforming growth factor-beta-binding protein 3 (LTBP3), which encodes one of the extracellular matrix proteins, plays an essential role in skeletal formation through both stimulatory and inhibitory effects on the growth of different cell types, as well as on the production and degradation of the extracellular matrix. Pathogenic variants in LTBP3 have been associated with genetic skeletal disorders that exhibit various cardiovascular features, including aortic root dilatation, aneurysm or dissection of the ascending and descending aorta, and mitral valve prolapse). Aortic root dilatation, an aortopathy that may have lifethreatening consequences, is among the clinical findings in various connective tissue disorders, including Marfan syndrome, Ehlers-Danlos syndrome, and Loeys-Dietz syndrome. Aortic root dilatation, aneurysm, and aortic dissection should be carefully investigated by cardiologists. In this study, we describe three siblings with short stature and dental anomalies. A homozygous, novel, c.2726-1G > A pathogenic variant in LTBP3 was identified through exome sequencing. After the detection of the LTBP3 variant, the patients were evaluated for possible cardiac findings, which revealed mitral valve prolapse and aortic root dilatation despite the absence of clinical symptoms. In this study, we aimed to shed light on the diagnosis of dental anomalies and short tature syndrome in three siblings with a novel LTBP3 pathogenic variant revealed through exome sequencing. Additionally, we emphasise the importance of searching for cardiac findings, even in the absence of clinical symptoms. We highly suggest that cardiologists take note of cardiac findings in patients with dental anomalies and short stature syndrome.

A case of an adolescent male with hypoplastic coronary arteries and myocardial bridging who had a sudden cardiac death event playing soccer. This rare anomaly is not easily identified and may be missed during routine work-up. Treatment options are limited and there is a need for discussion of additional treatment considerations that may prevent sudden cardiac death in this population.

Both mitral annular disjunction and coronary artery anomalies are associated with ventricular arrhythmia, though data on their co-occurrence are limited. We herein present two patients with anomalous aortic origin of a coronary artery and mitral annular disjunction who had ventricular arrhythmia. Mitral annular disjunction should be considered as a potential cause of ventricular arrhythmia when stress tests rule out ischaemia.

Protein-losing enteropathy remains a formidable and underrecognised complication following Fontan surgery, contributing significantly to morbidity and mortality among affected patients. Despite substantial progress in managing single-ventricle CHDs, protein-losing enteropathy persists as a major clinical challenge, particularly in resource-constrained environments where specialised diagnostic modalities are scarce. This manuscript discusses the prevalence, clinical presentation, diagnostic hurdles, and management of protein-losing enteropathy in post-Fontan patients, drawing from clinical experiences in Vietnam. Among 95 post-Fontan patients reviewed, the prevalence of protein-losing enteropathy was 4.2%, with considerable morbidity necessitating invasive interventions and, in some cases, resulting in mortality. Diagnostic limitations, including restricted access to alpha-1-antitrypsin clearance testing, highlight the urgent need for heightened clinical suspicion and pragmatic diagnostic approaches. We advocate for integrating low-cost, widely accessible screening measures, such as routine serum protein monitoring, into standard post-Fontan care protocols to enhance early detection and management of protein-losing enteropathy. Furthermore, we underscore the critical importance of global collaboration and multicentre research initiatives to address the worldwide burden of protein-losing enteropathy, facilitate resource-sharing, and develop evidence-based interventions. Coordinated international efforts are essential to closing diagnostic and treatment gaps, ultimately improving survival and quality of life for patients living with the Fontan circulation.

Total anomalous pulmonary venous return is a rare CHD that may present in the neonatal period with signs of congestive cardiac failure. First-line treatment is corrective surgery in the first months of life. The authors report the case of a 2-month-old infant with non-obstructive total anomalous pulmonary venous return submitted to a two-stage hybrid repair, involving cardiac surgery and interventional cardiology.

Innominate vein redirection to the pulmonary venous atrium has been used in single ventricle patients in order to relieve lymphatic complications resulting from systemic venous hypertension. This has been achieved both surgically, known as the Hraska procedure and via transcatheter approach, known as the Rome procedure. Determining the pathway from the innominate vein to the pulmonary venous atrium is challenging with important intra- and extra-cardiac structures close-by. We present two cases; one surgical and one transcatheter approach, where 3D-printed cardiac models were used to assist in the pre-procedural planning of this relatively novel and challenging strategy.

We report the case of a 25 kg male with aortic isthmus atresia and small femoral arterial access. A BeGraft covered stent was percutaneously implanted after perforating the atretic segment. This case demonstrates the feasibility and safety of transcatheter treatment using the Bentley stent, which enables smaller femoral access compared to other balloon-expandable stent platforms.

This case report discusses a 1-year-old female with severe growth retardation and multiple congenital anomalies, including a large patent ductus arteriosus and interrupted inferior vena cava. Successful percutaneous patent ductus arteriosus closure was achieved via the azygos venous route, overcoming anatomical challenges and highlighting the importance of individualized procedural strategies in complex cases.

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.

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.

We describe a trisomy 21 patient with postnatal diagnosis of atrioventricular septal defect with isolated ventricular component who had a complicated post-surgical course following complete repair. Clinical outcomes included moderate-severe residual atrioventricular valve regurgitation needing re-operation, complete heart block leading to pacemaker insertion, seizures secondary to subdural haemorrhages, and chylothorax. We describe the surgical considerations specific to this cardiac morphology.

Atrial myxoma in children is rare, with vision loss and cerebral metastasis seldom reported. We present the case of a 12-year-old girl who experienced sudden vision loss and acute infarction, with a left atrial myxoma identified and surgically resected. One year later, she was readmitted with severe neurological symptoms. Imaging revealed multiple hypometabolic brain lesions, which were confirmed as metastatic myxoma through surgical resection and pathology. This case underscores the critical need for long-term follow-up in patients with cardiac myxoma to monitor potential delayed neurological complications.