Mowat–Wilson syndrome is a genetic condition due to a mutation in the ZEB2 gene; it affects many systems including the cardiovascular system. The pulmonary arterial sling originates from a failure of development of the proximal portion of the left sixth aortic arch, resulting in an anomalous left pulmonary artery origin from the posterior wall of the right pulmonary artery and the left pulmonary artery crossing to the left lung between the trachea and the oesophagus. We present a 4-month-old infant with Mowat–Wilson syndrome and left pulmonary arterial sling, and discuss the association of these two rare conditions. Pulmonary arterial sling is significantly more frequent in patients with Mowat–Wilson syndrome than in the general population.

Thromboembolic complications occur frequently in Fontan patients with atrial arrhythmias and are a cause of significant morbidity and all-cause mortality. We report the case of an adult woman with direct atriopulmonary connection and atrial arrhythmia who developed a right atrial thrombus. She was switched to apixaban therapy because of echocardiographic evidence of thrombus progression despite combined therapy with warfarin and aspirin. After 1 year of treatment, there was evidence of complete thrombus resolution, in the absence of bleeding events. Our case shows that direct oral anticoagulants can be effective and safe for the treatment of thrombosis in adult patients with complex CHD.

This presentation will demonstrate the essential features of tetralogy of Fallot in the infant and child before surgery, as well as some noteworthy features in the foetus. The four features, namely, subpulmonary stenosis, ventricular septal defect, aortic override, and right ventricular hypertrophy, can all be easily demonstrated by echocardiography. In addition, morphology of the pulmonary valve and the main and branch pulmonary arteries can be seen. The position of the coronary arteries and the major variants of proximal coronary anatomy can be defined. The arch anatomy and the presence of associated major aortopulmonary collateral arteries can be defined. All these features can be demonstrated in the foetus as well, after the first trimester, and the presence of major aortopulmonary collateral arteries can be seen more clearly because the lungs, being fluid filled, aid in ultrasound and do not provide the barrier that the air-filled lung presents after birth.

As far as we are aware, right-sided bacterial endocarditis has not previously been described as presenting with systemic illness and testicular swelling. We report a teenager who presented with this unusual combination as a consequence of right-sided endocarditis. He presented with high fever, with chills and rigor, along with painful enlargement of the left testicle, a productive cough with progressive breathlessness, and joint pains. His blood culture was positive for Staphylococcus aureus, and a computerised tomographic scan of the chest revealed multiple pulmonary emboluses. Ultrasound of the testicles showed features of inflammation, and an echocardiogram revealed a vegetation on the tricuspid valve.

A vein of Galen malformation – a rare cause of cardiac insufficiency in neonates – is sometimes associated with coarctation of the aorta, two diseases requiring urgent therapy in the neonatal period. We report on a term neonate in whom we first palliated the coarctation by stent implantation, providing time to treat the vein of Galen malformation by endovascular embolisation. Following this, the coarctation was surgically repaired and the stent was explanted.

We describe a rare type of atrioventricular septal defect with common atrioventricular orifice and malaligned atrial septum, the latter feature resulting in severe obstruction of the left atrial egress. Excision of the atrial septum and banding of the pulmonary trunk was performed at the age of thirteen months. Lung biopsy demonstrated marked medial hypertrophy with severe intimal fibrosis in the small pulmonary arteries and mild thickening of the pulmonary veins, considered contraindications for corrective surgery.

We utilised a cast preoperatively prepared on the basis of the patient’s computed tomography data to clarify unusual orientation of the great arteries (transposition of great arteries, coarctation of the aorta, and abnormally branching central pulmonary arteries) in a 2.2-kg neonate. The three-dimensional model was very useful for designing the surgical procedure – being easy to handle for real and precise simulation of the reparative manoeuvres.

We determined the clinical value of routine transthoracic echocardiography performed after catheter ablation of supraventricular tachyarrhythmias in children. Between April 1996 and December 2003, 253 children, of whom 135 male, with the overall group having a median age of 9, ranging from 0.1 to 19 years, underwent 280 uncomplicated radiofrequency catheter ablation procedures for supraventricular tachyarrhythmias at three institutions. In every child, transthoracic Doppler echocardiography was performed before and after the procedure. The pre-ablation transthoracic echocardiograms were normal in all, and this was one of the criterions for inclusion. The post-ablation echocardiogram showed a disorder in four asymptomatic patients. In one patient, with focal atrial tachycardia, ablated via a retrograde aortic approach, there was mild aortic valvar insufficiency. This had resolved 6 months later. Pericardial effusions developed in 3 other children. In 2 the effusions resolved spontaneously but 1 patient required pericardial drainage. This same patient also developed clinically asymptomatic mild aortic insufficiency, which resolved spontaneously within 6 months. Routine echocardiography after uncomplicated catheter ablation procedures is of clinical value, and is especially indicated when a retrograde aortic approach has been used.

Hepatoblastoma is the most common malignant liver tumour in early childhood. The metastatic extension of hepatoblastoma into the left atrium via the pulmonary vein is rare. Reported lesions almost always involve a right-sided approach. Here we report the case of a 3-year-old girl with a recurrent hepatoblastoma at multiple sites, including the left atrium, brain, and lung. The patient was treated surgically for the prevention of further embolic complications and cardiac failure.

Heart failure in children can result from a wide range of aetiologies and can manifest in systolic and/or diastolic dysfunction. Echocardiography is the primary test for the diagnosis and follow-up of children with heart failure. In this article, we critically review standard echocardiographic measurements that have been shown to have prognostic importance in children with various types of heart failure. Each of the common forms of cardiomyopathy that is encountered in childhood – dilated, hypertrophic, restrictive, left ventricular non-compaction, and arrhythmogenic right ventricular cardiomyopathy – is discussed separately. Special attention is paid to the failing right ventricle, both in the systemic and in the sub-pulmonary position, to the failing univentricular heart, and to the assessment of diastolic function in children.

To determine whether cardiac catheterisation procedures for low birth weight neonates ≤2.5 kg carries a greater risk of complications compared with neonates >2.5 kg, we conducted a single-centre retrospective case–control study. From 01/03 to 01/09, 46 consecutive neonates <2.5 kg at the time of cardiac catheterisation were identified. For each low birth weight case, three control patients >2.5 kg were randomly selected from our heart centre database during the same time period. Data included demographic characteristics, type of intervention, fluoroscopy time, contrast volume, pre- and post-blood urea nitrogen to creatinine ratio, physician performing procedure, procedural risk category, and all major and minor complications. The overall incidence of complications was higher in neonates ≤2.5 kg compared with neonates >2.5 kg (34.8% versus 17.6%, p = 0.023) because of a greater proportion of minor complications (34.8% versus 16.9%, p = 0.021). When specific minor complications were stratified, there was a greater incidence of hypotension requiring intravenous fluids in neonates ≤2.5 kg (6.5% versus 0%, p = 0.015). After controlling for physician performing procedure and risk category, neonates ≤2.5 kg remained at a higher risk for any complication (adjusted odds ratio = 3.2, 95% confidence interval 1.4–7.2, p = 0.005). The percentage of neonates having at least one major complication was not higher in the ≤2.5-kg group (2.2% versus 2.2%). No procedural deaths occurred in either group.

Primary idiopathic chylopericardium is a rare disease, particularly in children. We report the case of a 5-year-old male child who presented with cough and dyspnoea. Bulging of the chest wall and distant heart sounds suggested the diagnosis of a pericardial effusion, which was confirmed on a chest computed tomography. Pericardial drainage confirmed the diagnosis of a chylopericardium. After the failure of continuous catheter drainage, surgical ligation of the thoracic duct and the creation of a pericardial window were performed under thoracoscopy.

Ascending aortic pseudoaneurysms are a rare complication following cardiac surgery. Their traditional surgical management carries high morbidity and mortality, especially in patients with multiple comorbidities. Transcatheter closure of these pseudoaneurysms using a septal occluder device has been previously reported in adults as an alternative approach with fewer complications. We report the first case of a novel percutaneous femoral arterial-transthoracic approach for exclusion of an ascending aortic pseudoaneurysm with an Amplatzer atrial septal occluder device in a 9-year-old child with Takayasu's arteritis.

We describe a patient in whom we found dual orifices in a straddling and overriding tricuspid valve, with two normally sized ventricles and a cleft in the mitral valve. The patient underwent successful surgical repair. We discuss the concept of “double-orifice right atrium”, as well as the need to differentiate the isolated cleft of the morphologically mitral valve from the zone of apposition between the left ventricular components of the bridging leaflets seen in the setting of atrioventricular septal defect with common atrioventricular junction. We emphasise the unusual association of these abnormalities of the right and left atrioventricular valves in patients with separate atrioventricular junctions.

I am grateful for the opportunity to participate in this debate forum, and for the honor of exchanging concepts with Len Bailey. This debate, fortunately, represents a “win-win” situation. The innovations and evolution of both replacement of the heart and reconstructive surgery for hypoplastic left heart syndrome are stories with lots of winners. The entire field of transplantation biology, the entire scope of management of the newborn with complex congenital heart disease, including both medical and supportive therapy and surgical strategies, has each been advanced tremendously as a consequence of the introduction and refinement of these two very different therapies for hypoplastic left heart syndrome. So, it is a debate that cannot be lost.