This study aimed to address the role of various inflammation-related blood indices for the assessment of in-hospital outcomes in subjects undergoing Glenn procedure. Subjects who underwent the Glenn procedure for hypoplastic left heart syndrome were analysed retrospectively. Subjects were divided into two groups: Group 1 consisted of 78 patients who were discharged, and Group 2 included 12 patients who died after surgery. Post-operative third-day neutrophil count and neutrophil-to-lymphocyte ratio value were significantly higher in the exitus group compared to the discharged group (p = 0.006 and p = 0.003, respectively). Third-day neutrophil-to-lymphocyte ratio was positively correlated with duration of intubation (r = 0.253, p = 0.018), length of stay in ICU (r = 0.296, p = 0.006) and length of hospital stay (r = 0.297, p = 0.005). Multiple logistic regression analysis revealed that patients with high third-day neutrophil-to-lymphocyte ratio (≥6) had 14.227-fold higher risk of death compared to those with lower values. In addition, higher pulmonary arterial pressure was associated with increased risk of death. Receiver operating characteristics analysis revealed that neutrophil-to-lymphocyte ratio had 66.67% sensitivity, 84% specificity, 81.61% accuracy, 40.00% positive predictive value and 94.03% negative predictive value with a cut-off point of ≥6 to predict mortality. Third-day neutrophil-to-lymphocyte ratio and increased post-operative pulmonary arterial pressure are significant predictors for in-hospital mortality in Glenn procedure recipients. A cut-off value of ≥6 for third-day neutrophil-to-lymphocyte ratio predicts mortality with 66.67% sensitivity and 84% specificity. Given its simplicity and availability, post-operative neutrophil-to-lymphocyte ratio should be monitored on a daily basis to identify patients with high risk for mortality after Glenn procedure.

Subcutaneous treprostinil is commonly used to improve idiopathic pulmonary arterial hypertension in children. However, its effectiveness has not been reported in trisomy 21. We report the case of 9-year-old boy in trisomy 21 with CHD-pulmonary artery hypertension after surgical correction of CHD. Haemodynamics and exercise capacity dramatically improved with a transition from oral selexipag to subcutaneous treprostinil.

This article reported two cases of brucellosis in children on the waiting list for a cardiac transplant who have been referred with the diagnosis of Fever of Unknown Origin. The serology was positive and patients were treated with cardiac transplantation in the 6th week. They had no recurrence or rejection. Therefore, brucellosis should be considered in patients on the waiting list for cardiac transplants.

Paediatric coronary artery bypass surgery with internal thoracic artery grafting is the optimal choice for left main coronary artery atresia; we report successful reconstruction in a patient with bypass graft obstruction. The pulmonary trunk was transected to expose the left main coronary stem, which was opened beyond the obstruction and the incision extended through the left main coronary artery to the circumflex bifurcation and left anterior descending artery. A funnel-shaped coronary ostium was created with a glutaraldehyde-treated autologous pericardium onlay patch. Surgical reconstruction is a good alternative for left main coronary artery atresia with graft obstruction and preserves the remaining internal thoracic artery for future use.

Congenital junctional ectopic tachycardia is a rare but serious cardiac arrhythmia seen in neonates and young infants. It is frequently resistant and refractory to first-line treatment options such as cardioversion with adenosine and direct current shock, and it carries a high morbidity and mortality rate. The aim of this article is to present the case of congenital junctional ectopic tachycardia observed in a 14-day-old neonate, highlighting the role of ivabradine in the management, followed by a discussion about current approaches to treatment.

Among 65 neonates with encephalopathy undergoing cooling, 30 (46.1%) received chest compressions during delivery room resuscitation. Despite differences in encephalopathy severity, early (<24 hours) biventricular function on echocardiogram (fractional area change, myocardial performance indices, systolic to diastolic duration ratios, tricuspid annular plane systolic excursion) was comparable between groups with and without chest compressions. Epinephrine receipt was associated with abnormal tricuspid annular plane systolic excursion.

The association of truncus arteriosus communis with interrupted aortic arch and mitral atresia is an exceptionally rare congenital defect. We describe the initial decision-making and management of this lesion, which eventually achieved a Fontan palliation.

An isolated carotid artery is a rare aortic arch anomaly. Instead of connecting to the aorta, the isolated carotid artery connects anomalously to the pulmonary artery. Chronically altered cerebral circulation poses the risk of cerebral hyperaemia following surgical reimplantation. We describe successful reimplantation of the isolated left common carotid artery in a child, highlighting the importance of a multidisciplinary approach for good clinical and neurological outcomes. We also briefly discuss the embryologic basis of this rare arch anomaly.

Tetralogy of Fallot with absent pulmonary valve syndrome is commonly associated with trachea-bronchial anomalies, possibly due to airway compression caused by massively dilated pulmonary arteries secondary to severe pulmonary regurgitation. This airway obstruction may persist post-operatively also. We report a case of an infant who required a series of management strategies including bronchial stenting to manage his obstructive symptoms.

Prematurity and low birthweight are associated with increased mortality in infants undergoing cardiac surgery. Pulmonary atresia with intact ventricular septum and right ventricle-dependent coronary circulation carries one of the highest risks of mortality. We present a patient who was born at 28 weeks of gestation at 1.2 kg, with pulmonary atresia intact ventricular septum, right ventricle-dependent coronary circulation, coronary artery atresia, and discontinuous pulmonary arteries, who successfully underwent palliation with a modified Blalock–Taussig shunt, pulmonary arterioplasty, and subsequently a bidirectional Glenn.

Cardiovascular complications are the most frequent cause of death in patients with the Hutchinson–Gilford progeria syndrome. However, due to its rarity, studying the course of cardiac abnormalities has been a challenge. The cardiovascular phenotype helps to provide greater insight into the natural history of these abnormalities.

Neonatal total anomalous pulmonary venous connection has an incidence of about ∼1–2%.1 The clinical presentation of total anomalous pulmonary venous connection in the neonatal period is dependent on the presence or absence of pulmonary venous obstruction, which is usual when the veins connect to the umbilicovitelline system, as in infradiaphragmatic total anomalous pulmonary venous connection. Obstructed total anomalous pulmonary venous connection presents with severe respiratory distress, metabolic acidosis, and cyanosis and requires urgent surgical intervention. However, critically ill neonates with obstructed total anomalous pulmonary venous connection have a higher surgical morbidity and mortality, and pre-operative stabilisation can improve outcomes in them. We present a case of a septic term neonate with obstructed infradiaphragmatic total anomalous pulmonary venous connection who underwent emergency palliative stent placement for immediate relief of pulmonary venous obstruction.

A 40-year-old Down patient without previous cardiological history was admitted to our institution for dyspnoea after COVID-19 vaccine. CT scan revealed a pulmonary thromboembolism. One week later, he developed neurological impairment and CT scan evidenced a left parietal ischaemic lesion. Concomitantly, he underwent echocardiography showing an atrioventricular septal defect typically associated to Down syndrome and never diagnosed earlier. The diagnosis of paradoxical embolisation was then supposed. Echocardiography also revealed a severe right heart section dilatation, with bidirectional shunt on the septal defects and systemic right heart pressure. Down patients affected by CHD are more prone to develop pulmonary vasculopathy than non-syndromic patients. In this case, the pulmonary vasculopathy was further exacerbated by the pulmonary embolism and by the late diagnosis of CHD. Finally, an appropriate timely diagnosis of atrioventricular septal defect could potentially avoid the neurological complication in this patient.