A foetal echocardiogram, in a 27-week foetus referred for cardiomegaly, demonstrated dextrocardia, absence of the ductus venosus, and an unrestricted unusual umbilical venous drainage to a left posterior intercostal vein, which continued to left hemiazygos vein and drained into the coronary sinus. Progressive cardiomegaly led to early delivery. To the best of our knowledge, no case with similar umbilical venous drainage has been previously reported.

Coronary artery fistulas have been described in patients after heart transplantation more often than in the normal population. We reviewed our centre’s database between 2008 and 2020. Thirty patients had coronary angiography and 13 showed non-cameral coronary artery fistulas in their first coronary angiography. Distribution, degree and evolution of the fistulas and characteristics of transplant procedure, patient and immunosuppressive treatments were analysed.

We report a case of a patient with an anomalous left coronary artery originating from the opposite coronary sinus with evidence of intramural course at the level of the septal commissure and no slit-like deformation of the coronary ostium, acute take-off, or proximal vessel narrowing. According to previous publications, patients with anomalous coronary artery and intramural segments identified at surgery had coronary CT findings of acute take-off angle or proximal vessel narrowing; slit-like orifice; and elliptical cross-sectional shape. Although further investigation is required, we suggest that the intramural course may not be ruled out based on the absence of slit-like ostium, acute take-off, or proximal vessel narrowing.

Accessory tricuspid valve is rare congenital abnormality. We describe a case of cryptogenic stroke in teenager boy caused by patent foramen ovale with thrombophilia and accessory tricuspid valve.

In the December 2021 issue of Cardiology in the Young, Hubrechts and colleagues, from Brussels and Leuven in Belgium, describe their experience in which the pulmonary veins were normally connected to the morphologically left atrium. By virtue of the presence of a shelf dividing the morphologically left atrium, however, the venous return was to the morphologically right atrium, with no evidence of formation of the superior interatrial fold, meaning that there was no obstruction of flow into the systemic venous circulation. The question posed by the Belgian authors is whether the shelf dividing the morphologically left atrium is a deviated primary atrial septum, as the arrangement has previously been interpreted. As they discuss, it is currently impossible to arbitrate this conundrum. In our commentary, we discuss the background to the dilemma. We point out that, as yet, it is not possible to code accurately this congenital cardiac malformation within The International Paediatric and Congenital Cardiac Code (IPCCC), nor within the newly produced 11th Revision of the International Classification of Diseases (ICD-11).

Congenital absence of the aortic valve is characterised by the absence of aortic valve and severe regurgitation. The rest of the reported cases were mostly diagnosed either on postnatal echocardiography or autopsy. Here, we present a foetal case with the absence of the aortic valve and “inverse circulatory shunt”.

In a 6-year-old child patient, transthoracic echocardiography revealed a large saccular structure (with anechogenic content) in the left atrium, near the fossa ovalis, and diagnosis of the left atrial septal pouch was made. The left atrial septal pouch is a kangaroo pouch-like structure on the left side of the interatrial septum, opened into the left atrial cavity without a connection between the left and right atria. It occurs when the foramen ovale is absent but the septum primum and septum secundum are only partially fused. The left atrial septal pouch is believed to be present in 47% of population. In many cases, the pocket on the atrial septum is small and it could not be detected by transthoracic echocardiography. Our description is uncommon because we diagnosed a very large septal pouch. Based on our knowledge, this is the youngest reported case of the left atrial septal pouch and the longest follow-up described in this type of congenital heart malformation.

A maternally inherited novel pathogenic non-POU domain-containing octamer-binding gene variant c.767G>T, p.R256I [NM_001145408], manifested in a male infant as dilated cardiomyopathy with severe left ventricular dysfunction and dilation, biventricular non-compaction, tricuspid hypoplasia, and hydrocephaly. To the best of our knowledge, no previous non-POU domain-containing octamer-binding gene variants with biventricular non-compaction have been associated with tricuspid valve hypoplasia. Hence, this case introduces a new pathogenic variant observed in the non-POU domain-containing octamer-binding gene and adds to the range of cardiac phenotypes identified in non-POU domain-containing octamer-binding gene variants.

We present a case of a patient with dextro-transposition of the great arteries palliated with a Senning procedure and a long-term arrhythmic complication that required an intervention, with an Implantable Cardioverter Defibrillator (ICD) implantation in the sub-pulmonary ventricle (morphologically left). This case highlights the need to perform off-label procedures to deal with the long-term complications of these complex patients.

We present an uncommon challenging case of spontaneous thrombosis of the arterial duct and with alloimmune thrombocytopaenia in a full-term newborn who presented with respiratory distress, hypoglycaemia dispersed petechiae on the trunk, and significant haemorrhage of the umbilical venous catheter.

Cardiac fibromas are the second most common benign primary tumour of the heart in the children; the clinical features include chest pain, arrhythmia, low cardiac output due to outflow tract obstruction, and sudden cardiac death. Sports are associated with an increased risk for sudden death in athletes who are affected by cardiovascular conditions predisposing to life-threatening arrhythmias. We present a case report of 10-year-old asymptomatic boy who was referred to the paediatric cardiology department by his general practitioner for cardiac examination before participation in competitive sports. The electrocardiogram showed premature ventricular contractions originated from inferior of left ventricle. A mass was detected by 2D transthoracic echocardiography, and it was found to be compatible with fibroma on MRI. In some cases, cardiac tumours are asymptomatic as in our patient. Electrocardiogram abnormalities require detailed cardiac imaging with echocardiogram, and if necessary CT/MRI. In this article, we emphasise that detailed cardiac examination of individuals before participating in competitive sports is vital.

The congenital left ventricular diverticulum is a rare cardiac malformation, and it may associate with Cantrell’s Pentalogy with other cardiac defects. However, isolated ventricular diverticulum without any other cardiac defect in complete Cantrell’s syndrome is very rare. We describe a 6-year-old male patient with a complete Cantrell’s syndrome with isolated left ventricular diverticulum.

Chronic thromboembolic pulmonary hypertension is an uncommon condition in the children. It almost always accompanies a hypercoagulable state. We described a rare case of Behçet’s disease presenting with chronic thromboembolic pulmonary hypertension and initially misdiagnosed as coronavirus disease 2019 pneumonia.

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.

Double drainage of the confluence of all four pulmonary veins is extremely rare. We present the image findings in a child with double drainage of the pulmonary venous confluence into the coronary sinus and left superior caval vein with co-existent right superior caval venous stenosis.

We report a series of four patients with CHD who tested positive for COVID-19, got treated, and underwent cardiac surgery in the same sitting. All had an uneventful perioperative course similar to COVID-19-negative patients. We conclude that children with asymptomatic or mild COVID-19 disease may be subjected to surgery as early as within 1 week since COVID-19 negative.

The condition of partial anomalous origin of a branch pulmonary artery from the descending aorta could be found in several diseases and should be carefully differentiated. We report an unusual case of anomalous systemic arterial supply to normal basal segments of the left lower lung and another case of intralobar pulmonary sequestration. These two cases were treated successfully by transarterial embolisation using the Amplatzer Vascular Plug. We also set up a diagnostic algorithm to differentiate these diseases from anomalous systemic arterial supply to the pulmonary region. It is possible to make the correct diagnosis using the step-by-step diagnostic algorithm and careful interpretation of chest computed tomography angiography.

A young child presented with hepatomegaly, ascites and bradycardia in the setting of COVID-19. Permanent complete atrioventricular block and severe right heart failure were diagnosed. He was treated with surgical epicardial pacemaker implantation. This report is the first description of COVID-19-induced permanent complete atrioventricular block in a child.