We report the case of a patient with tetralogy of Fallot with absent pulmonary valve and familial Alagille syndrome who successfully underwent cardiac repair. The patient’s sister had liver and congenital heart disease. The father had undergone liver transplantation but showed no significant cardiac abnormalities. A yet-unknown mutation of the JAG1 gene was discovered in this family with variable expression of Alagille syndrome.

Management of patients with Eisenmenger syndrome with pulmonary atresia is challenging because of the complexity of the structure–function relationship of the components of the syndrome. Multi-modality imaging including cardiac magnetic resonance (CMR) 4D Flow offers unprecedented opportunities to unravel, at least in part, some of these components, and thus help in the management of these patients. In this study, we describe the use of these integrated methods with particular reference to CMR 4D Flow in a patient with Eisenmenger syndrome and pulmonary atresia and outline both the utility and the limitations. A comprehensive cardiac magnetic resonance (CMR) 4D Flow analysis was performed preoperatively and postoperatively, during peak systole, late systole, early diastole, and late diastole. The focus of the present study was to investigate the pattern of flow and dynamic changes at different levels of the aorta, as well as in the duct and the pulmonary arteries. Preoperatively, a right-handed helix and a vortex were observed in the dilated ascending aorta, and the duct flow was mainly dependent on reverse, upstream flow from the descending aorta, distal to the duct, during diastole, denoting low pulmonary vascular capacitance. Following repair, the flow in the ascending aorta and the descending aorta changed markedly. These changes included both timing and intensity of the right-handed helix, as well as the vortex in the ascending aorta. The significance of the observed changes in flow pattern and their influence on wall structure and function are discussed. Our study demonstrates the extremely powerful potential of CMR 4D Flow in the management of complex congenital anomalies.

It has been reported that the presence of intermittent pre-excitation indicates low risk of rapid conduction via the accessory pathway in atrial fibrillation. We report a case of a 10-year-old boy with a history of intermittent pre-excitation who presented with atrial fibrillation with very rapid conduction.

In this study, three patients presenting with early or late postoperative Fontan complications were identified to suffer from restriction of the native atrial septum. This caused significant obstruction to pulmonary venous return and elevated systemic venous pressure. Dobutamine stress testing was used in one patient to identify this lesion. Transcatheter stenting was performed in the other two patients. Patients improved after relief of the obstruction.

We report the case of a neonate with pulmonary atresia with intact ventricular septum and coronary cameral fistulae despite having a subsystemic right ventricle. We review the literature on coronary cameral fistulae in this disease and right ventricle-dependent coronary circulation. We discuss the potential consequences of this physiology, including risk of adverse cardiovascular events that may impact risk stratification and surgical palliation.

We describe the case of an infant who was a late presenter of transposition of the great arteries where we proceeded with ductal stenting to improve oxygenation and left ventricle training. Stenting improved the infant’s saturation while keeping the left ventricle well trained for 4 months after the procedure. This report demonstrates that intermediate-term left ventricle training can be achieved via ductal stenting.

We present the case report of a war victim child with severe burn scars, orthopnoea, and dyspnoea due to diffuse pulmonary thromboembolism. During ICU stay, a central venous catheter’s 45-mm wire embolised into the heart. The embolised wire was successfully removed via transhepatic access through the creation of an artificial simple snare.

Sewing needles are rare causes of penetrating cardiac injury. Suicidal attempt, accidental penetration, domestic violence, and child abuse are likely causes for such injury.1 Owing to their sharp nature, needles can rapidly migrate through the tissues. Fatalities are due to cardiac tamponade, infection, peripheral embolism, and valve dysfunction.

A neonate with transposition of the great arteries and intact ventricular septum presented without pulmonary over-circulation, and subsequently developed pulmonary haemorrhage after corrective surgery. Postoperative CT revealed an aortopulmonary collateral artery arising from the descending aorta, and we performed successful embolisation on postoperative day 9. Aggressive imaging modalities such as angiography and/or CT imaging with contrast can detect unexpected extra-pulmonary blood supply and guide further management.

We present the case of a 4-year-old girl with idiopathic hypereosinophilia syndrome, endomyocardial fibrosis, and mural thrombus. This condition is rarely seen in children outside the tropics. Myocardial biopsy is historically the standard for diagnosis. Reports in adult literature, however, have shown the utility of cardiac MRI as a non-invasive tool for diagnosis, prognosis, and monitoring. To our knowledge, this is the first reported case with serial cardiac MRI in a child.

Although extrarenal manifestations of haemolytic–uraemic syndrome are not frequent, myocardial dysfunction should be given special consideration because of the importance of proper early haemodynamic management and potential complications. We report the case of a 21-month-old child with haemolytic–uraemic syndrome who developed clinical signs of poor myocardial function with depressed myocardial function noted by bedside echocardiography and significant elevation of biomarkers. Early intervention and supportive treatment for the patient were crucial during the acute phase of cardiac failure, and repeated monitoring of biomarkers and ecocardiography were useful diagnostic tools that provided relevant information throughout the patient’s evolution.

We present a case of circumferential fracture of aortic coarctation stent with severe re-stentosis presenting 16 years after initial stent implantation with end-stage renal disease. The patient was treated with a covered stent using the stent-in-stent technique. The use of an ultra-high-pressure balloon was proved necessary to overcome the tight, non-compliant stenosis.

We describe the case of a 7-year-old girl with a single coronary artery. The coronary artery passed between the ascending aorta and pulmonary artery, and an aberrant vessel ran anterior to the latter.

The objectives of this review were to discuss the potential impact of Big Data analytics in paediatric cardiovascular disease and its potential to address the challenges of transparency in delivery of care to this unique population.

Although the spectrum of physiology seen in infants and children with double-outlet right ventricle is broad, this anatomy in combination with an aortopulmonary window is extremely rare. We present an interesting case of an infant prenatally diagnosed with this rare CHD. To our knowledge, this is the first report of complete repair in the neonatal period for such a combination of defects.

CHD is associated with poor growth, delayed motor and language skills development, and increased length of hospital stay; 28.2% of infants were stunted, with z-scores<−2. The severity of surgery score was not associated with an increased length of stay, suggesting that a low weight-for-age z-score at the time of surgery may impact on length of stay.

The Fontan connection, originally described in 1971, is used to provide palliation for patients with many forms of CHDs that cannot support a biventricular circulation. An increasing number of females who have undergone these connections in childhood are now surviving into adulthood and some are becoming pregnant. We report a case of a 29-year-old woman who presented with a twin pregnancy at 33 weeks of gestation. She had significant deterioration of her cardiovascular status before the twin babies were delivered by emergency caesarean section owing to associated obstetric complications. This report also highlights the various maternal and fetal complications occurring in pregnancy of Fontan-palliated patients and suggests the need for meticulous pre-conception counselling and strict perinatal care.