Robert Anderson has made a huge contribution to almost all aspects of morphology and understanding of congenital cardiac malformations, none more so than the group of anomalies that many of those in the practice of paediatric cardiology and adult congenital heart disease now call ‘Atrioventricular Septal Defect’ (AVSD). In 1982, with Anton Becker working in Amsterdam, their hallmark ‘What’s in a name?’ editorial was published in the Journal of Thoracic and Cardiovascular Surgery. At that time most described the group of lesions as ‘atrioventricular canal malformation’ or ‘endocardial cushion defect’. Perhaps more significantly, the so-called ostium primum defect was thought to represent a partial variant. It was also universally thought, at that time, that the left atrioventricular valve was no more than a mitral valve with a cleft in the aortic leaflet. In addition to this, lesions such as isolated cleft of the mitral valve, large ventricular septal defects opening to the inlet of the right and hearts with straddling or overriding tricuspid valve were variations of the atrioventricular canal malformation. Anderson and Becker emphasised the differences between the atrioventricular junction in the normal heart and those with a common junction for which they recommended the generic name, ‘atrioventricular septal defect’. As I will discuss, over many years, they continued to work with clinical cardiologists and cardiac surgeons to refine diagnostic criteria and transform the classification and understanding of this complex group of anomalies. Their emphasis was always on accurate diagnosis and communication, which is conveyed in this review.
This review, which is intended for both radiologists and clinical cardiologists, highlights the use of cardiac MRI in pediatric and adult patients after completion of the Fontan procedure and provi... Cardiac MRI has become a widely accepted standard for anatomic and functional assessment of complex Fontan physiology, because it is noninvasive and suitable for comprehensive follow-up evaluation ...
Objective To describe the intervention spectrum, complexity, and safety outcomes of catheter-based interventions in a contemporary adult congenital heart disease (ACHD) tertiary cohort. Methods All patients over 16 years who underwent a catheter-based intervention for ACHD in our centre between 2000 and 2016 were included. Baseline demographics, clinical characteristics, indications for and complexity of intervention, procedural complications and early and mid-term mortality were analysed. Results Overall, 1644 catheter-based interventions were performed. Intervention complexity ranged from simple (67.5%) to intermediate (26.4%) and to high (6.1%). Commonly performed procedures were atrial septal defect (33.4%) and patent foramen ovale closure (26.1%) followed by coarctation of the aorta (11.1%) and pulmonary artery interventions (7.0%). Age at index intervention was 40±16 years, 758 (46.1%) patients were male, 73.2% in New York Heart Association (NYHA) class I, 20.2% in NYHA class II, whereas 6.6% in NYHA class III/IV. In-hospital mortality was 0.7%. Median postinterventional length of stay was 1 day. Complications occurred in 129 (7.9%) with major adverse events in 21 (1.3%). One-year postintervention survival rates were 98.7% (95% CI 98.2 to 99.2). Over the study period, there was a notable shift in intervention complexity, with a predominance of simple procedures performed in early years and more complex procedures in later years. Furthermore, the case mix during the study broadened (p<0.001) with new catheter-based interventions and a more individualised approach to therapy. Conclusion This study shows an increasing complexity and expanding variability of ACHD catheter-based interventions, associated with low major complications, short hospital stays and low early and mid-term mortality. Ongoing investment in ACHD catheter interventions is warranted.