Although mortality rates for patients with d-transposition of the great arteries remain quite low, these patients have a unique circulation that requires careful management in the peri-operative period. Despite the improved mortality for patients with common arterial trunk, the course in the intensive care unit is remarkable for significant morbidity and utilisation of significant resources. Pre-operative patient management focuses on balancing competing circulations, pulmonary and systemic, which exist in parallel rather than in series, as in the normal circulation. Post-operative patient management in both lesions focuses on optimising systemic output, respiratory status, and mitigating the effects of cardiopulmonary bypass. In this article, we review pre- and post-operative intensive care management in neonates with d-transposition of the great arteries and common arterial trunk.

Primary care cardiology, also known as ambulatory or outpatient cardiology, for the longitudinal management of patients with common arterial trunk or with transposition of the great arteries is both poorly described and has limited evidence to justify its basis. This article discusses some of the various complications that these patients can develop, reviews the medical literature, and describes a framework for care of these complex patients from infancy to transition to adult congenital cardiac specialist care.

Improved survival in children with complex congenital cardiac disease, such as conotruncal abnormalities, has created a sub-population of children and young adults who need comprehensive multi-disciplinary long-term follow-up. Routine surveillance with comprehensive screening for structural heart disease, functional heart disease, thromboembolic disease, arrhythmias, and associated end-organ dysfunction is important. Future research will better define the care plans for routine surveillance in patients with conotruncal abnormalities.

Significant advances have been made in extracorporeal life support, which has resulted in the increased use of post-cardiotomy extracorporeal membrane oxygenation. Retrospective studies have contributed to the ongoing evolution of selection criteria for post-cardiotomy extracorporeal membrane oxygenation. Current indications include failure to wean from cardiopulmonary bypass, haemodynamic collapse, pulmonary hypertension, post-repair of hypoplastic left heart syndrome, or need for bridge to transplantation. Short- and mid-term results are improving. Ethical concerns still attend the process, however. Moral risks related to post-cardiotomy extracorporeal membrane oxygenation may be encountered before, during, and after the open heart procedure. At each stage of the decision-making process, moral risks are encountered by many factors that may result in decisions that may be contrary to the best interests of the patient, parents, or use of shared societal resources. These moral risks centre around the selection process, informed consent, decision making in the operating room, and post-operative maintenance of extracorporeal membrane oxygenation. Consideration of such risks is affected by questions of haemodynamic stability, haematologic compromise, neurologic status, and family concerns. We conclude that thorough understanding of the relevant scientific literature, heightened awareness of moral risks, and incorporation of ethical tenets in clinical deliberation will guide the clinician to do the right thing.

Significant challenges face developing countries as a result of the maldistribution of access to healthcare throughout the world, specifically access to paediatric cardiac care. Sustainable paediatric cardiac programmes must be established in developing countries to provide care to all children with congenital heart disease. Education and research are essential components to sustainable paediatric cardiac programmes in developing countries to define local problems and the incidence of disease, and to generate solutions thereto related. Research can contribute to developing local expertise, improving technology, providing opportunities for local talent, generating financial resources, enhancing the dignity of people, and the facilitating resolution of health problems throughout the world. Clinical trials conducted in developing countries should meet the same ethical standards as trials based in developed countries.

Although overall outcomes for children undergoing heart surgery have improved, there is a significant variation in outcomes across hospitals. This review discusses the variation in cost and outcomes across centres performing congenital heart surgery, potential underlying mechanisms, and efforts to reduce variation and improve outcome.

Computational fluid dynamics has been applied to the design, refinement, and assessment of surgical procedures and medical devices. This tool calculates flow patterns and pressure changes within a virtual model of the cardiovascular system. In the field of paediatric cardiac surgery, computational fluid dynamics is being used to elucidate the optimal approach to staged reconstruction of specific defects and study the haemodynamics of the resulting anatomical configurations after reconstructive or palliative surgery. In this paper, we review the techniques and principal findings of computational fluid dynamics studies as applied to a few representative forms of congenital heart disease.