It is an honour to present the Anthony Chang lecture at this 10th International Conference of the Pediatric Cardiac Intensive Care Society. I have had the privilege of knowing Dr Chang for over 20 years, and although we only worked for a short period of time together at the Children’s Hospital, Boston, in the Cardiac Intensive Care Unit, we have remained close colleagues and friends since that time. The contributions of Dr Chang to the development of paediatric cardiac intensive care are very clear, based on his clinical expertise, research and scholarship, and the development of the Pediatric Cardiac Intensive Care Society in its early days. More than this, Dr Chang is an individual with vision; in many respects, he has been ahead of the curve, anticipating and leading the direction of paediatric cardiac intensive care.

We report a rare case of CHD and abdominal aorta interruption. Renal hypertension was the first sign of the abdominal aortic malformation. The aetiology of abdominal aortic interruption remains unclear, it could be congenital or acquired.

No reports on pulmonary atresia with ventricular septal defect with the combination of double aortic arch and interruption between left and right carotid arteries have been published so far.

Common arterial trunk with arch obstruction is a rare conotruncal abnormality. We report, with confirmatory images, a neonate with this anomaly and additional supracardiac partial anomalous pulmonary venous connection, as well as phenylketonuria.

In the spectrum of mitral valve anomalies, unguarded mitral orifice is an exceedingly rare malformation, with only four cases described in the current literature. All previously reported cases have been associated with discordant atrioventricular connections. We describe the first known case of unguarded mitral valve orifice, in the setting of atrioventricular concordance, in a newborn with hypoplastic left heart syndrome.

Anomalous origin of the right coronary artery from the posterior, non-coronary sinus is extremely rare and has never been reported in a paediatric-age competitive athlete. We report this very rare case of the right coronary artery arising from the posterior sinus. In the anomalous right coronary from the posterior sinus, the proximal right coronary artery appears widely patent and has no interarterial course.

Persistent primitive hepatic venous plexus is an anomaly of the systemic venous return characterised by postnatal persistence of the foetal intrahepatic venous drainage. Scimitar syndrome is a condition that consists of partial anomalous pulmonary venous return of the right pulmonary venous drainage into the systemic veins, associated with pulmonary artery hypoplasia with the underdeveloped right lung, pulmonary sequestration, and cardiac malposition. Both conditions are rare and together have been rarely described in the literature. We report the first case of this combination of lesions imaged by cardiac magnetic resonance imaging with a three-dimensional reconstruction and reviewed the literature to characterise this uncommon combination.

We describe two cases of anomalous origin of the left lower-lobe pulmonary artery from the right pulmonary artery. The primary diagnosis was mitral atresia, hypoplastic left ventricle, aortic arch hypoplasia in the first child, and tetralogy of Fallot in the second. In both cases, the pulmonary trunk gave rise to a left pulmonary artery in the normal position. In addition, a second branch of the left pulmonary artery arose from the right pulmonary artery, and passed posterior and inferior to the left main or upper-lobe bronchus to supply the left lower lobe. In this review, we compare our findings with previously reported examples of this extremely rare cardiac malformation, and discuss possible embryological explanations for the lesion.

Oesophageal perforation is a rarely reported complication of transoesophageal echocardiography in infants. This case involves a 3.1-kg neonate with Trisomy 21, atrioventricular septal defect, and hypoplastic aortic arch undergoing aortic arch advancement and pulmonary artery banding. A paediatric transoesophageal echocardiography probe was placed intraoperatively causing a contained false passage from the oesophagus below the cricopharyngeus muscle with extension into the left posterior mediastinum. The perforation healed within 2 weeks without permanent sequelae after conservative medical management.

We report a baby girl with an antenatal diagnosis of biventricular non-compaction and complete heart block detected at 22 weeks’ gestation. Postnatal echocardiography confirmed severe biventricular non-compaction hypertrophic cardiomyopathy, multiple muscular ventricular septal defects, and mild–moderate pulmonary valve stenosis. Skeletal muscle biopsy confirmed complex 1 mitochondrial respiratory chain deficiency. An epicardial VVI pacemaker was implanted on day 3 of life and revised at 7 years of age. She remains stable at 8 years of age following pacing and medical treatment with carvedilol, aspirin, co-enzyme Q10, and carnitine. This represents the first report of biventricular non-compaction hypertrophic phenotype in association with congenital complete heart block and complex 1 mitochondrial respiratory chain deficiency in a child.