This December Issue of Cardiology in the Young represents the tenth annual publication generated from the two meetings that compose “HeartWeek in Florida”. “HeartWeek in Florida”, the joint collaborative project sponsored by the Cardiac Center at the Children's Hospital of Philadelphia, together with All Children's Hospital of Saint Petersburg and Johns Hopkins Medicine, averages over 1000 attendees every year and is now recognized as one of the major planks of continuing medical and nursing education for those working in the fields of diagnosis and treatment of cardiac disease in the fetus, neonate, infant, child, and adult. “HeartWeek in Florida” combines the International Symposium on Congenital Heart Disease, organised by All Children's Hospital and Johns Hopkins Medicine and entering its 13th year, with the Annual Postgraduate Course in Pediatric Cardiovascular Disease, organised by The Children's Hospital of Philadelphia and entering its 17th year. The theme of this supplement generated from the 2012 HeartWeek in Florida is “A Lifelong Interdisciplinary Approach to Common Arterial Trunk, Transposition of the Great Arteries, and Other Evolving Challenges in Paediatric and Congenital Cardiac Disease”. We would like to acknowledge the tremendous contributions made to medicine by Richard Jonas; and therefore, we dedicate this HeartWeek 2012 Supplement to him.

The first World Congress of Paediatric Cardiology was held in London, United Kingdom, in 1980, organised by Dr Jane Somerville and Prof. Fergus Macartney. The idea was that of Jane Somerville, who worked with enormous energy and enthusiasm to bring together paediatric cardiologists and surgeons from around the world. The 2nd World Congress of Paediatric Cardiology took place in New York in 1985, organised by Bill Rashkind, Mary Ellen Engle, and Eugene Doyle. The 3rd World Congress of Paediatric Cardiology was held in Bangkok, Thailand, in 1989, organised by Chompol Vongraprateep. Although cardiac surgeons were heavily involved in these early meetings, a separate World Congress of Paediatric Cardiac Surgery was held in Bergamo, Italy, in 1988, organised by Lucio Parenzan. Thereafter, it was recognised that surgeons and cardiologists working on the same problems and driven by a desire to help children should really rather meet together. A momentous decision was taken to initiate a Joint World Congress of Paediatric Cardiology and Cardiac Surgery. A steering committee was established with membership comprising the main organisers of the four separate previous Congresses, and additional members were recruited in an effort to achieve numerical equality of cardiologists and surgeons and a broad geographical representation. The historic 1st “World Congress of Paediatric Cardiology and Cardiac Surgery” took place in Paris in June, 1993, organised by Jean Kachaner. The next was to be held in Japan, but the catastrophic Kobe earthquake in 1995 forced relocation to Hawaii in 1997. Then followed Toronto, Canada (2001, organised by Bill Williams and Lee Benson), Buenos Aires, Argentina (2005, organised by Horatio Capelli and Guillermo Kreutzer), and most recently Cairns, Australia (2009, organised by Jim Wilkinson). Having visited Europe (1993), Asia-Pacific (1997), North America (2001), South America (2005), and Australia (2009), and reflecting the “African Renaissance”, the World Congress is coming to Africa. The 6th World Congress of Paediatric Cardiology and Cardiac Surgery will be held in Cape Town, South Africa, from 17 to 22 February, 2013. Cape Town is a sublime and compelling African destination with irresistible appeal. The South African Heart Association is the host. Information about The 6th World Congress of Paediatric Cardiology and Cardiac Surgery can be found at http://www.pccs2013.co.za.

The World Congress of Paediatric Cardiology and Cardiac Surgery has survived with minimal assets and simple organisation. Each congress is special, taking on the humour, flavour, and culture of the organising country. It is hard work for a few organisers and money is hard to raise. The steering committee works closely, fairly, and successfully, and even though accused of being secretive and effete that does not matter. It is efficient and produces successful, happy world congresses, where all involved with the speciality are welcome. With so many “grown-ups” with congenital heart disease, it is no longer just a paediatric problem – maybe the name of this congress must change again. Regardless, the flag must fly on.

Hearts having a common arterial trunk belong to a family of congenital cardiac malformations for which traditional systems of classification and nomenclature are plagued by internal paradoxes, incompatibility between systems due to the lack of potential for identification of synonyms, or irreconcilable inconsistencies with our current knowledge of cardiac development and morphology. A simplified categorisation that classifies these hearts on the basis of pulmonary or aortic dominance reconciles the existing disparate categorisations, is in keeping with recent findings concerning cardiac development, and emphasises the principal morphologic determinant of surgical outcome.

With the development of three-dimensional techniques for imaging, such as computed tomography and magnetic resonance imaging, it is now possible to demonstrate the precise sinusal origin and epicardial course of the coronary arteries with just as much accuracy as can be achieved by the morphologist holding the heart in his or her hands. At present, however, there is no universally accepted convention for categorising the various patterns found when the heart is congenitally malformed. In this review, we show how, to provide such a convention, it is necessary to take note not only of the sinusal origin of the three major coronary arteries, but also the relationship of the aortic root relative to the cardiac base. We summarise the evidence showing how the proximal portions of the developing coronary arteries grow into the aortic valvar sinuses subsequent to the separation of the aortic root from the subpulmonary infundibulum. We also discuss the evidence showing that the subpulmonary myocardium is impervious to the passage of epicardial coronary arteries, and suggest that the process of septation itself plays an integral role in guiding the arteries into the two aortic sinuses that are adjacent to the pulmonary root. We then show how marriage of convenience between the epicardial coronary arteries and the aortic valvar sinuses provides a good explanation for the known variations found in the setting of transposition. We point out that it is the absence of septation that likely governs the patterns seen in the setting of a common arterial trunk.

The successful diagnosis, surgical planning, and long-term care of children with transposition of the great arteries require high-quality cardiac imaging with echocardiography. Echocardiography must identify the relevant anatomic variants of transposition of the great arteries, such as of ventricular septal defects and aortic arch anomalies. Methodical and detailed imaging of the coronary arteries is particularly important, as translocation of the coronary arteries is a critical component of the arterial switch procedure. Familiarity with the potential coronary artery variants and the ideal imaging planes is essential for an echocardiographer. Knowledge of both the early and late complications following the arterial switch procedure is essential to optimise post-operative echocardiography. These complications can include residual lesions leading to haemodynamic compromise or progressive late phenomena, such as aortic root dilatation and aortic insufficiency. Echocardiography will continue to be the cornerstone to the lifelong management of transposition of the great arteries, and improvements in technology and increased familiarity with modalities such as stress echocardiography will enhance the role of advanced imaging even further.

Advances in foetal echocardiography allow detection of conotruncal anomalies such as transposition of the great arteries and common arterial trunk. Early detection of these anomalies is important for appropriate delivery planning, family counselling, and management. This manuscript will review the anatomic considerations, differential diagnosis, and foetal echocardiographic features of each of these malformations and review the importance of prenatal diagnosis and family counselling.

Cardiac catheterisation continues to play an important role in the long-term management of patients with common arterial trunk and transposition of the great arteries. Although non-invasive imaging has largely eliminated the need for diagnostic catheterisation in newborns with these congenital cardiac lesions, cardiac catheterisation is an important tool for the diagnosis of a variety of problems encountered after surgical intervention, and allows interventions to be performed when feasible. We review the indications for cardiac catheterisation and describe the specifics for various interventional procedures for these patients in this manuscript.

Surgery for common arterial trunk has evolved over the past 30 years. Current management involves total repair during the neonatal period with excellent expected results. The presence of truncal valve insufficiency or interrupted aortic arch may increase the surgical risk for morbidity and mortality. Current therapy and management continues to evolve.

Transcatheter pulmonary valve replacement is fast becoming an accepted alternative to repeat surgical pulmonary valve replacement for selected patients and therefore a complementary strategy in the long-term management of those requiring surgical pulmonary valve replacement. With a combined surgical and percutaneous approach, late morbidity for some of these patients may be diminished. This manuscript will review the current indications for this procedure, its limitations, and its benefits.

Relief of right ventricular outflow tract obstruction in tetralogy of Fallot or similar physiology often results in pulmonary regurgitation. The resultant chronic volume overload can lead to right ventricular dilatation, biventricular dysfunction, heart failure symptoms, arrhythmias, and sudden death. Although pulmonary valve replacement can lead to improvement in functional class and a substantial decrease or normalisation of right ventricular volumes, the optimal timing of pulmonary valve replacement is not well defined. Benefits of pulmonary valve replacement have to be weighed against the risks of this procedure, including subsequent reoperation. This article will review the benefits and risks of pulmonary valve replacement, options for pulmonary valve substitute, and timing of pulmonary valve replacement in patients with chronic pulmonary regurgitation after relief of right ventricular outflow tract obstruction.

Repair of common arterial trunk using an extracardiac right ventricular to pulmonary arterial conduit is the preferred method in most cardiac surgical centres. Reoperation is a fact of life for survivors of common arterial trunk and related cardiac lesions who have undergone such repairs. Long-term survivors may require periodic conduit revisions, with a potentially escalating technical difficulty and risk. Herein we present an analysis of the currently available choices for extracardiac conduits, and outline what we consider to be a safe and reliable surgical strategy for conduit revision.

The arterial switch operation is the extant surgical correction after a long series of palliations attempted and/or successfully achieved for the treatment of discordant ventriculoarterial connections. As early as 1954, pioneers such as Mustard, Bailey, Kay, and Idriss led the way with at first disheartening failures, temporarily leading to abandoning the procedure. The first successful atrial baffle procedure in 1958 established itself as the procedure of choice for treating discordant ventriculoarterial connections, but tenacity, courage, and vision to pursue anatomic correction finally led to the first successful arterial switch in 1975 by Jatene. After a decade to perfect surgical technique and timing indications for the various anatomic subtypes, the new era of the neonatal arterial switch since the late 1980s set the very high standards that we all know and expect today. Despite excellent early and long-term survival, important residual lesions are increasingly being recognised. Expected anatomic residuals include supravalvar pulmonary stenosis, neoaortic valve insufficiency, and coronary ostial stenosis. Reinterventions and rare, but challenging surgical reoperations address these residual findings with satisfactory outcomes. Quality of life into young adulthood is satisfactory, but functional problems include reduced exercise capacity, diffuse coronary insufficiency, and neurodevelopmental shortcomings, of which the true incidence and potential clinical implications are still unknown. The arterial switch is a spectacular anatomic correction for a once lethal condition and currently the best surgical solution for patients with discordant ventriculoarterial connections. It is, however, far from a true cure; closer and ongoing follow-up for future care will continue to be required.

The arterial switch operation has become a safe operation in many centres. The complexity of the procedure has evolved over the last two decades. Several anatomical features can hardly be considered complex today, namely, normal coronary anatomy, circumflex coming off the right coronary artery, eccentric ostium, and early take-off of an infundibular artery. In addition, as peri-operative mortality becomes very low, the outcomes need to be evaluated on the peri-operative morbidity, late reoperations, and late deaths. The arterial switch operation remains complex in around 20% of the cases, where one or several complexity factors are associated. The complexity of the coronary arteries is a major factor. According to a classification essentially based on the course of the coronary arteries, complex coronaries include: double-looping coronaries, anterior-looping coronaries, intramural coronaries, and single coronary ostium. The most challenging coronary pattern remains the association of a single ostium with intramural course. Other features are equally complex: severe malalignment of the commissures, aortic arch obstruction, multiple ventricular septal defect, Taussig–Bing with subaortic obstruction, double-outlet right ventricle non-committed ventricular septal defect, transposition of the great arteries-intact ventricular septum >3 weeks, transposition of the great arteries-ventricular septal defect with high lung resistances and weight <2.5 kg. Owing to the fact that the risks of arterial switch operation vary according to the experience of the centres, we defined the arterial switch operation complexity based on a subjective approach as proposed by the Aristotle comprehensive score. The recent introduction of a morbidity score will allow to stratify more accurately the outcomes when the peri-operative mortality is very low or nil. The complexity of the coronary patterns tends to be well controlled today. It remains that rare coronary failures and aortic root dilation will occur in the long term, requiring a close follow-up of the most complex patients. Successfully achieving complex arterial switch operation implies a second learning curve.

Arrhythmias in patients with congenital heart disease present a challenge to the care of these patients and can result in significant morbidity and mortality. Transposition of the great arteries and common arterial trunk are no exceptions. It is important to identify risk factors for arrhythmia development in the peri-operative period. The peri-operative arrhythmia burden may relate to the underlying congenital heart disease, haemodynamic perturbations, operative events, and potential residual lesions. In addition, these patients are at risk for developing arrhythmias later in life, and non-invasive and potentially invasive arrhythmia surveillance should be a routine part of the care of these patients. This article highlights important strategies to manage arrhythmia development and prevention in this patient population.