The diagnosis and treatment for paediatric and congenital cardiac disease has undergone remarkable progress over the last 60 years. Unfortunately, this progress has been largely limited to the developed world. Yet every year approximately 90% of the more than 1,000,000 children who are born with congenital cardiac disease across the world receive either suboptimal care or are totally denied care.While in the developed world the focus has changed from an effort to decrease post-operative mortality to now improving quality of life and decreasing morbidity, which is the focus of this Supplement, the rest of the world still needs to develop basic access to congenital cardiac care. The World Society for Pediatric and Congenital Heart Surgery [http://www.wspchs.org/] was established in 2006. The Vision of the World Society is that every child born anywhere in the world with a congenital heart defect should have access to appropriate medical and surgical care. The Mission of the World Society is to promote the highest quality comprehensive care to all patients with pediatric and/or congenital heart disease, from the fetus to the adult, regardless of the patient's economic means, with emphasis on excellence in education, research and community service.We present in this article an overview of the epidemiology of congenital cardiac disease, the current and future challenges to improve care in the developed and developing world, the impact of the globalization of cardiac surgery, and the role that the World Society should play. The World Society for Pediatric and Congenital Heart Surgery is in a unique position to influence and truly improve the global care of children and adults with congenital cardiac disease throughout the world [http://www.wspchs.org/].

Double-outlet right atrium (DORA) is characterized by simultaneous right atrial emptying into both ventricles. Ventriculoatrial septal malalignment is the cardinal morphological feature. Three cases are presented to depict two major types of DORA-DORA with a malaligned atrial septum and DORA with a malaligned ventricular septum. We describe two subtypes of each form of DORA: DORA with a malaligned atrial septum presents with either a common atrioventricular (AV) junction (guarded by a common AV valve) or with a single AV junction (due to the absence of the left AV junction). DORA with a malaligned ventricular septum may be associated with a right ventricle (RV) that is adequate for biventricular repair or a severely hypoplastic RV not compatible with biventricular repair. DORA with a malaligned ventricular septum is closely related to typical straddling of the tricuspid valve. Peculiarly, DORA with a malaligned ventricular septum presents three AV valves at the AV junction and is associated with an abnormal disposition of the AV conduction axis. Clear understanding of the morphology of these lesions is important in preventing a surgical misadventure at the crux of the heart.

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In patients with transposition of the great arteries with intact ventricular septum (TGA-IVS), late presentation for surgical treatment is not uncommon. Earlier experience suggested an excess mortality for the primary arterial switch operation (ASO) when performed after the third week of life in such patients. The true age limit for the primary ASO, however, remains speculative. A best evidence topic was written to determine the safety of extending the age limit of the primary ASO for TGA-IVS beyond three weeks of age. Eight of 74 relevant papers constituted the best evidence to address the issue. Sufficient level II evidence was found to suggest that in experienced institutions in the current era, the ASO for TGA-IVS may be performed primarily in infants three to eight weeks of age with comparable early outcomes to younger patients. Kang et al. investigated 275 neonates (younger than 21 days) and 105 infants (age range 21-185 days) undergoing the primary ASO for TGA-IVS. They found no significant difference in terms of in-hospital mortality (5.5% vs. 3.8%) or need for mechanical left ventricular (LV) support (3.6% vs. 5.7%) between the younger and older groups, respectively. Of the younger group, 3.8% developed postoperative LV failure leading to death or mechanical LV support. All nine patients older than two months (age range 61-185 days) survived to discharge although two (aged 69 and 86 days) required mechanical LV support for postoperative LV failure. The postoperative course of the late ASO group, however, was significantly prolonged as reflected in the duration of postoperative ventilation (4.9 vs. 7.1 days, P=0.012) and length of postoperative stay (12.5 vs. 18.9 days, P=0.001). In the report of Sarris et al., 52 patients with TGA-IVS who were older than four weeks of age (36 were older than eight weeks) underwent a primary ASO with early mortality comparable with younger patients (2% vs. 3%, respectively). For infants between the ages of two and six months, the strength of the evidence favoring this management approach is limited by the small sample size of reported series and the increased requirement for mechanical circulatory support for postoperative LV failure.

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