The association of hypoplastic left heart syndrome with pulmonary valvular abnormalities such as dysplasia, stenosis, or insufficiency is rare and not well defined in the literature. We report a rare case of hypoplastic left heart syndrome with a thickened four-leaflet, four-sinus pulmonary valve. After stage I palliation, this neo-aortic valve developed clinically significant insufficiency in the setting of increased pulmonary blood flow.

Kawasaki disease is a self-limiting vasculitis of unknown aetiology, and coronary artery lesions are its most common and serious complication. The Systemic Immune-Inflammation Index is a new biomarker of inflammation that may have prognostic value in Kawasaki disease. This study evaluated patients with Kawasaki disease and the prognostic role of Systemic Immune-Inflammation Index in coronary involvement. A total of 62 children with Kawasaki disease and 49 healthy controls were included. Systemic Immune-Inflammation Index was calculated as: neutrophils × platelets/lymphocytes). Systemic Immune-Inflammation Index was also significantly higher in Kawasaki disease patients than controls (2373 ± 2040 vs. 300 ± 218, p < 0.001). Subgroup analysis of the Kawasaki disease patients showed that Systemic Immune-Inflammation Index was significantly higher in patients with coronary artery lesions than in those without (p < 0.05). In conclusion, Systemic Immune-Inflammation Index is a composite inflammatory biomarker easily obtained from routine blood parameters and may be an independent predictor of coronary artery involvement in Kawasaki disease.

Left atrial appendage aneurysms are uncommon cardiac anomalies often discovered incidentally, with potential to cause arrhythmias or thromboembolic events, prompting surgical correction. Herein, we present the successful surgical treatment of a left atrial appendage aneurysm identified in a 7-year-old asymptomatic patient.

Burkholderia cepacia is a rare cause of prosthetic valve endocarditis. We report an 18-year-old male with Tetralogy of Fallot and two sequential transcatheter pulmonary valves (Melody and Myval), presenting with persistent bacteraemia unresponsive to antibiotics. Surgical explantation of both valves with homograft replacement and tricuspid repair achieved complete recovery, emphasising surgery in refractory infections.

Scimitar syndrome is an uncommon congenital malformation of pulmonary venous drainage to the junction of the right atrium and inferior caval vein. Treatment is usually surgical, depending on the morphological variant. When there is dual drainage of the anomalous veins to the inferior caval vein and the left atrium, a transcatheter procedure may represent an alternative to surgery. We report a series of four patients with the scimitar variant with dual pulmonary venous drainage treated with a transcatheter approach. All four patients (three children, one adult) had dual drainage of right pulmonary veins into the inferior caval vein and to the left atrium via a connecting vein. All patients underwent a successful catheter occlusion of the anomalous connection to the systemic vein without complication. Vascular plug devices were used in two patients: a left atrial appendage closure device in one patient and a ventricular septal defect closure device in one patient. All the procedures resulted in complete occlusion of the anomalous venous drainage to the inferior caval vein and unobstructed drainage to the left atrium.

The genesis and significance of epsilon waves still remains an enigma. In this case report, we document occurrence of epsilon waves in a neonate with left ventricular diverticulum and its disappearance after the surgical division of the diverticulum. We postulate that the presence of myocardial cells in the diverticulum was the cause for epsilon waves.

Background:Single ventricle pulmonary arteriovenous malformations are poorly understood and variably assessed in published literature. To improve our understanding of single ventricle pulmonary arteriovenous malformations and facilitate multi-centre studies, it will be necessary to have uniform clinical practice patterns among paediatric heart institutions.Objectives:The aim of this study was to assess paediatric interventional cardiologists’ clinical perspectives and practice patterns for diagnosing single ventricle pulmonary arteriovenous malformations.Methods:We surveyed paediatric interventional cardiologists using the Congenital Cardiovascular Interventional Consortium listserv. A single survey was distributed electronically with two subsequent reminder emails. Voluntary participants completed the anonymous survey electronically via RedCap.Results:Among 253 Congenital Cardiovascular Interventional Consortium members, a total of 55 (21.7%) paediatric cardiology interventional attending physicians completed the survey. There was near unanimity (98%) that pulmonary arteriovenous malformations develop due to lack of hepatic vein blood flow to the lungs; however, there was wide variation among practice patterns. A minority (20%) of respondents perform bubble contrast echocardiograms (bubble studies) more than half the time pre-Fontan, whereas many (31%) almost never (< 5% of cases) perform bubble studies pre-Fontan. Most respondents reported that they did not perform bubble studies because results do not impact clinical decision making pre-Fontan (56%) or post-Fontan (60%). Many respondents (49%) do not have a typical volume of agitated saline that they inject for bubble studies.Conclusions:Clinical practice patterns vary widely among paediatric cardiology interventionalists. A standardised clinical approach, new diagnostic tools, or both are needed to standardise our field’s approach to diagnosing, studying, and treating single ventricle pulmonary arteriovenous malformations.

In the past few decades, long-term basic research and clinical practice of surgical treatment for tetralogy of Fallot have been carried out at home and abroad, and the treatment effect has been improved, the complications and mortality have been gradually decreased. Pulmonary regurgitation is a common complication after traditional radical repair of tetralogy of Fallot, which is an important factor leading to many adverse outcomes. Valve-sparing repair has gradually become the first choice for the treatment of tetralogy of Fallot, which can effectively prevent the occurrence of postoperative pulmonary regurgitation, maintain right ventricular function, and improve the prognosis of children. However, there are controversies about the application indications and surgical strategies of valve-sparing repair, and a lack of clear clinical guidelines. This article reviews the research progress on the advantages, surgical indications, surgical techniques, prognosis and prospects of valve-sparing repair for tetralogy of Fallot, in order to provide evidence-based medical evidence for when to undergo valve-sparing repair for tetralogy of Fallot patients and to better improve the quality of life of patients.

Balloon-expandable MyVal OCTACOR valves were evaluated in 3D-printed conduits under simulated flow. The 20 mm valve functioned from 16-24 mm, rupturing at 27 mm. The 29 mm expanded to 33 mm with mild insufficiency. Findings suggest OCTACOR valves may support repeated valve-in-valve procedures, with the 20 mm showing greater versatility for small-infant mitral replacements and as a potential alternative to Melody valves.