A right aortic arch with an isolated left innominate artery is a rare form of aortic arch anomaly. We present a case of neonatal diagnosis of this anomaly with concerning findings of global cerebral white matter atrophy at 13 months of age.

Transposition of the great arteries combined with totally anomalous pulmonary venous connection is extremely rare outside of heterotaxy syndrome. Most reported cases have been treated by a modified atrial switch operation. We report the successful treatment of a neonate with this rare association, repaired by arterial switch operation and connection of the pulmonary venous return to the left atrium.

We present an unusual case of concordant ventriculoarterial connections, subpulmonary infundibulum, and parallel arterial trunks. This case was complicated by extreme pulmonary artery tortuosity and low arching aorta causing severe tracheal compression. We discuss the difficulty in prenatal diagnosis, necessity for advanced imaging postnatally, and associated airway complications.

This paper reports a case of multiple congenital ventricular diverticula, a rare anomaly that is associated with heart failure.

The Melody valve, designed for implantation into the pulmonary outflow tract, can also be used to treat the pathology of atrioventricular (AV) valves. Increasing gradients are seen as an indication for re-dilating the valve. Our case demonstrates the heart rate dependency of the gradient across a Melody implanted in the left AV valve position in an infant. Beta blockers were used to lower both heart rate and gradient.

We present the surgical implantation of the Edwards Inspiris Resilia® aortic valve in mitral position for mechanical mitral valve failure in a severely ill infant after valve replacement because of anomalous origin of the left coronary artery from the pulmonary artery. The biological valve was chosen because the child could not receive oral anticoagulation and was for several months on heparin infusion. The procedure was safely performed with good haemodynamic result.

A 6-year-old patient was diagnosed with an arteriovenous fistula in the neck region. Non-invasive three-dimensional imaging, including computed tomography and ultrasonography, was used for guidance of percutaneous closure of the fistula and evaluation of the final outcome.

Pheochromocytoma/paraganglioma is an exceedingly rare tumour, thought to share an association with cyanotic CHD. This association is thought to be a result of chronic hypoxaemia (Antonio et al, Revista Española de Cardiología (English Edition) 2017; 70: 673–675; Folger et al, Circulation 1964; 29: 750–757; Opotowsky et al, J Clin Endocrinol Metab 2015; 100: 1325–1334) We report two cases of paraganglioma over a 4-year period in patients with hypoplastic left heart syndrome who had undergone Fontan completion by ages 2 and 4. Based on a very small number of reported cases of CHD, the mechanism of tumourigenesis is unclear. It is imperative that cases associated with CHD continue to be reported so that we may learn more about the pathogenesis and epidemiology of this entity.

Aortic arch anomalies are rare congenital cardiovascular malformations. We present a case of a 9-year-old asymptomatic boy with an unusual, unknown arch anomaly.

Pulmonary artery stenting may not be possible transcutaneously because of anatomic features. Although intraoperative stenting has been well described, we present a case in which stenting of the left pulmonary artery was performed transthoracically in a separate procedure. Unusual anatomic conditions may require a multi-disciplinary hybrid approach to achieve the desired results.

A percutaneous transcatheter balloon dilation of a pulmonary venous pathway obstruction was successfully performed in a 40-year-old patient after a Mustard procedure. During the procedure, real-time three-dimensional trans-oesophageal echocardiography demonstrated the morphology of the obstruction. Our case highlights the usefulness of real-time three-dimensional trans-oesophageal echocardiography as a guide for transcatheter intervention in the increasing number of adults with CHD.

The coexistence of long QT syndrome with 2:1 or complete atrioventricular blocks has been reported in the literature, but, to the best of our knowledge, this is the first pediatric case of long QT syndrome coexisting with first-degree atrioventricular blocks(1–3).

An increased incidence of CHD has been noted in twin gestations and in infants conceived using assisted reproductive technologies. However, CHD in these populations remains understudied and the mechanisms underlying these phenomena remain unclear. We present the case of twins conceived via in vitro fertilisation both with Tetralogy of Fallot and additional cardiac and extracardiac malformations.

The Fontan operation is a palliative surgical procedure for patients whose hearts cannot support a biventricular circulation. The haemodynamic changes that occur in pregnancy are particularly challenging for Fontan patients and the outcomes are variable. We present a case where fetal outcome was particularly poor despite a lack of high risk features pre-pregnancy.

We present a case of a 3-week-old boy with persistent truncus arteriosus associated with interrupted right aortic arch having an anomalous origin of the left vertebral artery from the ascending aorta.

We report a rare association of interstitial deletion of 5p15.2-p13.3 with situs inversus, dextrocardia, L-loop of the ventricles, and transposition of great arteries: [I, L, L] Transposition of Great Arteries. We did not find such an association reported in the medical literature.