The number of children now surviving surgical interventions because of congenital cardiac disease continues to increase. Indeed, it is estimated that, by the end of 2005, there will be more adults than children with congenital cardiac malformations. The complex challenges facing this growing population are only now becoming apparent, including the continued hazard for mortality, the risk of reoperation, and newly-emerging late sequels of surgery. In particular, children with severe lesions requiring intervention in the neonatal period are surviving at levels thought to be highly improbable no more than two decades ago. Heralded by the availability of prostaglandin E1 in the late 1970s, improvements in technology, such as echocardiography, and color Doppler in particular, intensive care and intraoperative support, have recently allowed surgeons to reconstruct the hearts of many children with otherwise lethal defects. Much of the published literature has focused on the cardiac sequels in this population. Increasing attention has recently been given, nonetheless, to the neurodevelopmental outcomes in this population of patients.

As the overall mortality declines following repair of complex congenital cardiac malformations, attention has focused on reducing the lasting morbidity of these interventions, particularly the observed neurodevelopmental deficiencies. Both cardiopulmonary bypass and deep hypothermic circulatory arrest produce transient alterations in cerebral hemodynamics and metabolism. In studies performed in animals, deep hypothermic circulatory arrest, as compared to cardiopulmonary bypass alone, has been shown to produce excess injury to, and death of, neuronal and glial cells.1 In neonates, deep hypothermic circulatory arrest of greater duration than one hour is a risk factor for early post-operative seizures, and for subsequent neurodevelopmental deficits.2 The Boston Circulatory Arrest Study suggests that, at follow-up of eight years, infants subjected to greater than 41 minutes of deep hypothermic circulatory arrest had excess deficits in full-scale, verbal and performance intelligence quotient, the Mayo apraxia test, and grooved pegboard testing.3 The independent adverse effects of deep hypothermic circulatory arrest have encouraged clinicians to develop the alternative technique of intermittent global perfusion, or continuous regional perfusion at low flow perfusion, in an attempt to reduce the degree of injury to the central nervous system.4–7

The evolution of cardiac surgery has led to increasing emphasis on complete repair of congenital heart defects early in life, nowadays increasingly performed in neonates or small infants. Good results have been achieved because of innovative techniques permitting reconstruction of normal anatomy, and restoration of normal physiology, before either the heart or the patient undergo deleterious adaptation to the congenitally abnormal physiology. Despite the ability surgically to correct complex defects in such small patients, limitations in outcome are sometimes encountered related to the systems necessary for repair. In particular, exposure to cardiopulmonary bypass may present the greatest challenge for these tiny patients.

Survivors of repairs of complex congenital cardiac malformations in infancy have an increased risk of permanent abnormalities in motor, cognitive, expressive, and behavioral functioning. These functional deficits are expressions of complex interactions of environment, including prolonged hospitalization and conditioned child–parental behaviours, alterations of social environment, the effects of physical limitations, biological influences including genetic determinants, prenatal injury, and acquired reversible and irreversible neuronal injury.1,2 The magnitude of the problem is large, with incidence dependent upon the measures used for assessment. Overt postoperative neurologic signs have been recorded in up to one-tenth of postoperative infants and children, with double that rate found in those with abnormalities of the aortic arch.3 A decreased potential for development, based upon parent-sibling models, has been estimated to occur in one-third of survivors.4,5 Evidence of injury is provided by magnetic resonance imaging in up to one-third of patients preoperatively, and between half and nine-tenths postoperatively, although most of these early postoperative changes will disappear.5 Although recent changes in perioperative management are likely to reduce such neurologic injury, their significance remains high.

Due to many innovative techniques and technologies, the overall rate of survival for infants undergoing surgical repair of congenital cardiac malformations is excellent. Despite these excellent outcomes, neonates with hypoplasia of the left heart represent a very unique population, and pose difficult challenges for the entire team involved in care. Despite some centers reporting outstanding outcomes, overall rate of survival rate is reported at only 53 percent,1 and survivors often show neurodevelopmental deficits. Many attempts have been made to identify predictors of neurologic outcomes, with heavy concentration around the pre- and peri-operative periods. Little attention has been paid, however, to assessing how attempts at balancing the systemic and pulmonary circulation affect neurodevelopmental outcomes. In the past, we have reported excellent rates of survival with use of routine postoperative circulatory support to augment cardiac output.2,3 This strategy, nonetheless, is still widely debated. It seems plausible that, by augmenting cardiac output in the immediate postoperative period, cerebral perfusion and oxygenation can be optimized, thereby improving neurologic outcomes. In this review, we describe our experience to date in assessing neurological outcomes, and discuss whether outcomes are associated with cerebral saturation and extraction of oxygen during the period of postoperative circulatory assist.

The sixth, and final, section of this Supplement is titled “Caring for Patients with Congenital Cardiac Disease”. In it, we have included reviews that address a variety of issues related to caring for patients with congenital cardiac disease, including analysis of data, training, organization of the cardiac intensive care unit, quality of life, intraoperative imaging, and databases relating to congenital cardiac malformations. The purpose of this section of the Supplement is to take time to ponder the future of our professions, and what will be required to improve this future. My own belief is that, to quote the American football coach Jon Gruden, “I think the future's so bright here I've got to wear shades (sunglasses)”.1 Still, we all must work, ideally together, to ensure this bright future. This final section of the Supplement reviews several ideas and concepts which are representative of areas that our professional collaboration and work will lead to a better future for both patients with congenital cardiac disease, and the professionals caring for these often challenging patients. Indeed, part of being a Professional is self-regulating the profession, and taking measures to improve the state of the art in this profession, and in the process, “raising the bar”.

For many years, surgeons have been able to implement new techniques and treatments with minimal oversight. Although regulatory control over application of new strategies for treatment is far different today, surgeons have enjoyed a greater degree of freedom than other specialists. The laboratory of the surgeon, and particularly the congenital cardiac surgeon, is often the operating room, since appropriate animal models are not available. Thus, the congenital cardiac surgeon has often been stimulated to modify existing techniques, or create new surgical approaches, to improve the perceived limitations of previously known surgical strategies.1 The introduction of a new surgical technique has often taken the strong ego and persistence of a surgical innovator, and has subjected the surgeon to criticism from medical colleagues. Although innovation is a proud heritage of the development of congenital cardiac, it now seems appropriate, as we are more cognizant of the past accomplishments in the development of repairs for congenital cardiac malformations, to evaluate how surgical techniques become widely adopted, often with only minimal data to suggest improvement over previously accepted procedures.

As once observed by Tom Spray, “When they invite you to give the history talk, you're finished!” Those words flashed through my mind when I was invited to deliver the version of the lecture celebrating George Daicoff, and forced me to think carefully about what I do, and what I believe about doing it. I have been doing cardiac surgery for 25 years, and paediatric cardiac surgery for 20 of those. Looking back, it would be tempting to think that I am here to follow in the illustrious footsteps of Kirklin, Debakey, Lillehei, Cooley, Ross, Yacoub or Daicoff. But I have to admit that would not be the truth. I was more influenced by Dr Kildare and M.A.S.H., driven by the drama of their lives and the beauty of their nurses. I have not been disappointed.

There are many daunting challenges that need to be met by any seeking to achieve leadership in the area of cardiac intensive care. Paediatric cardiac intensive care has now emerged as a specialized clinical area, focusing on the unique needs of critically-ill neonates, children, and adults with congenital and acquired heart disease.1 Although an increasing number of centres dealing with paediatric cardiology have either started, or are considering starting, a dedicated cardiac intensive care unit and programme, there are no published primers regarding the logistical aspects of such a challenging endeavour. While there may be an ongoing debate about the preferred strategy in the clinical ownership of the patients treated in intensive care, most authorities agree that it is beneficial to have a dedicated team devoted to the needs of critically-ill children and adults with congenital and acquired cardiac disease.

The past two decades have witnessed a major shift towards repair of most congenital cardiac malformations during the neonatal or infant periods of life.1 Early anatomic correction or palliation, dramatic improvements in survival, and reduced morbidity due to improvements in perioperative and long-term medical management, have resulted in new populations of children that have reaped the benefits of the best care currently available for treatment of congenital cardiac disease. The impact of the congenital cardiac malformations, however, extends far beyond the walls of the hospital or clinic where we diagnose, treat, and follow our patients. The breakthrough of achieving predictable results with repair or palliation of most lesions during the neonatal and infant periods mandates us to look beyond survival, and to examine the lives our patients lead when they are outside of our care. Our purpose in this review is to discuss the measures of psychosocial outcome that are appropriate for exploration in those neonates and infants who survive cardiac surgery, to explore what is known about the psychosocial outcomes and quality of life for these patients, and what needs exist for future research.

The detail of cardiac morphology, with its subtle complexity and variation, is difficult to convey to colleagues and families, and even more time-consuming to learn. The terminology is occasionally unresolved, visual representations challenging, and there is often a mismatch between the presentation of the defect by the morphologist, the echocardiographic impression gained by the cardiologist, and the view obtained by the surgeon in the operating room. It can be said, therefore, that size, complexity and three-dimensional configuration, the three most important concepts to grasp when describing the congenitally malformed heart, are seldom touched upon with traditional illustrations or texts.

The abilities for both computer technology, and intra-operative video-imaging, are evolving rapidly. The merger of these two sciences can be very beneficial, both to congenital cardiac surgeons in general, and in facilitating the creation of a cardioscopic database in particular.

There are many reasons for seeking to create a global database with which to record the outcomes of therapy for congenital heart disease. Such a database can function as a tool to support a variety of purposes: