In 2000, The International Nomenclature Committee for Pediatric and Congenital Heart Disease was established. This committee eventually evolved into the International Society for Nomenclature of Paediatric and Congenital Heart Disease. The working component of this international nomenclature society has been The International Working Group for Mapping and Coding of Nomenclatures for Paediatric and Congenital Heart Disease, also known as the Nomenclature Working Group. The Nomenclature Working Group created the International Paediatric and Congenital Cardiac Code, which is available for free download from the internet at [http://www.IPCCC.NET].

In previous publications from the Nomenclature Working Group, unity has been produced by cross-mapping separate systems for coding, as for example in the treatment of the functionally univentricular heart, hypoplastic left heart syndrome, or congenitally corrected transposition. In this manuscript, we review the nomenclature, definition, and classification of heterotaxy, also known as the heterotaxy syndrome, placing special emphasis on the philosophical approach taken by both the Bostonian school of segmental notation developed from the teachings of Van Praagh, and the European school of sequential segmental analysis. The Nomenclature Working Group offers the following definition for the term “heterotaxy”: “Heterotaxy is synonymous with ‘visceral heterotaxy’ and ‘heterotaxy syndrome’. Heterotaxy is defined as an abnormality where the internal thoraco-abdominal organs demonstrate abnormal arrangement across the left-right axis of the body. By convention, heterotaxy does not include patients with either the expected usual or normal arrangement of the internal organs along the left-right axis, also known as ‘situs solitus’, nor patients with complete mirror-imaged arrangement of the internal organs along the left-right axis also known as ‘situs inversus’.” “Situs ambiguus is defined as an abnormality in which there are components of situs solitus and situs inversus in the same person. Situs ambiguus, therefore, can be considered to be present when the thoracic and abdominal organs are positioned in such a way with respect to each other as to be not clearly lateralised and thus have neither the usual, or normal, nor the mirror-imaged arrangements.”

The heterotaxy syndrome as thus defined is typically associated with complex cardiovascular malformations. Proper description of the heart in patients with this syndrome requires complete description of both the cardiac relations and the junctional connections of the cardiac segments, with documentation of the arrangement of the atrial appendages, the ventricular topology, the nature of the unions of the segments across the atrioventricular and the ventriculoarterial junctions, the infundibular morphologies, and the relationships of the arterial trunks in space. The position of the heart in the chest, and the orientation of the cardiac apex, must also be described separately. Particular attention is required for the venoatrial connections, since these are so often abnormal. The malformations within the heart are then analysed and described separately as for any patient with suspected congenital cardiac disease. The relationship and arrangement of the remaining thoraco-abdominal organs, including the spleen, the lungs, and the intestines, also must be described separately, because, although common patterns of association have been identified, there are frequent exceptions to these common patterns. One of the clinically important implications of heterotaxy syndrome is that splenic abnormalities are common. Investigation of any patient with the cardiac findings associated with heterotaxy, therefore, should include analysis of splenic morphology. The less than perfect association between the state of the spleen and the form of heart disease implies that splenic morphology should be investigated in all forms of heterotaxy, regardless of the type of cardiac disease. The splenic morphology should not be used to stratify the form of disease within the heart, and the form of cardiac disease should not be used to stratify the state of the spleen. Intestinal malrotation is another frequently associated lesion that must be considered. Some advocate that all patients with heterotaxy, especially those with isomerism of the right atrial appendages or asplenia syndrome, should have a barium study to evaluate for intestinal malrotation, given the associated potential morbidity. The cardiac anatomy and associated cardiac malformations, as well as the relationship and arrangement of the remaining thoraco-abdominal organs, must be described separately. It is only by utilizing this stepwise and logical progression of analysis that it becomes possible to describe correctly, and to classify properly, patients with heterotaxy.

How best to analyse and describe the features of the situation commonly known as “visceral heterotaxy” remains controversial. Much of the disagreement devolves on how to deal with the concept of isomerism. In the opinion of some, the concept of bilateral right-sidedness and bilateral left-sidedness, while useful in helping to remember which abnormalities are likely to occur in asplenia or polysplenia, should not be granted the status of a specific “situs”, since there are numerous examples of exceptions to these patterns. On the other hand, those who favour the concept of isomerism point out that, when describing only the heart, and taking the structure of the atrial appendages as the starting point for analysis, basing this on the extent of the pectinate muscles relative to the atrioventricular junctions, then the only possible arrangements for the appendages are the usual one, its mirror-image, and the two situations in which appendages of comparable morphology are found on both sides of the heart, these being the arrangements of right or left isomerism. It is certainly the case that the arrangement of the organs is not always in harmony with the arrangement of the atrial appendages, but those circumstances, in which there is disharmony, can readily be described by paying specific attention to each series of organs. On this basis, in this review, we describe the approach to heterotaxy, and isomerism of the atrial appendages, in terms of the genetic background, the diagnosis, and outcomes after cardiac surgery. Attention is given to the various diagnostic modalities, including fetal and postnatal echocardiography, recent tomographic and magnetic resonance imaging techniques, and the time-honoured approach using angiography.

The coronary arteries, the vessels through which both substrate and oxygen are provided to the cardiac muscle, normally arise from paired stems, right and left, each arising from a separate and distinct sinus of the aortic valve. The right coronary artery runs through the right atrioventricular groove, terminating in the majority of instances in the inferior interventricular groove. The main stem of the left coronary artery bifurcates into the anterior descending, or interventricular, and the circumflex branches. Origin of the anterior descending and circumflex arteries from separate orifices from the left sinus of Valsalva occurs in about 1% of the population, while it is also frequent to find the infundibular artery arising as a separate branch from the right sinus of Valsalva.

Anomalies of the coronary arteries can result from rudimentary persistence of an embryologic coronary arterial structure, failure of normal development or normal atrophy as part of development, or misplacement of connection of a an otherwise normal coronary artery. Anomalies, therefore, can be summarized in terms of abnormal origin or course, abnormal number of coronary arteries, lack of patency of the orifice of coronary artery, or abnormal connections of the arteries.

Anomalous origin of the left coronary artery from the pulmonary trunk occurs with an incidence of approximately 1 in 300,000 children. The degree of left ventricular dysfunction produced likely relates to the development of collateral vessels that arise from the right coronary artery, and provide flow into the left system. Anomalous origin of either the right or the left coronary artery from the opposite sinus of Valsalva can be relatively innocuous, but if the anomalous artery takes an interarterial course between the pulmonary trunk and the aorta, this can underlie sudden death, almost invariably during or immediately following strenuous exercise or competitive sporting events. Distal anomalies of the coronary arteries most commonly involve abnormal connections, or fistulas, between the right or left coronary arterial systems and a chamber or vessel.

We discuss the current techniques available for imaging these various lesions, along with their functional assessment, concluding with a summary of current strategies for management.

Persistent patency of the arterial duct represents one of the most common lesions in the field of congenital cardiac disease. The strategies for management continue to evolve. In this review, we focus on management beyond the neonatal period. We review the temporal evolution of strategies for management, illustrate the currently available the techniques for permanent closure of the patent arterial duct, review the expected outcomes after closure, discuss the current controversy over the appropriate treatment of the so-called “silent” duct, and provide recommendations for the current state of management of patients with persistent patency of the arterial duct outside of the neonatal period.

At the Congenital Heart Institute of Florida, we now recommend closure of all patent arterial ducts, regardless of their size. Before selecting and performing the type of procedure, we explain the natural history of the persistently patent arterial duct to the parents or legal guardian of the child. Particular emphasis is placed on the risks of endocarditis, including the recognition that many cases of endocarditis may not be preventable.

The devastating effects of endocarditis, coupled with the perception of more anecdotal reports of endocarditis with the silent duct, as well as the low risk of interventions, has led us to recommend closure of the patent arterial duct in these situations. We now recommend intervention, after informed consent, for all patients with a patent arterial duct regardless of size, including those in which the patent duct is “silent”. We recognize, however, that this remains a controversial topic, especially given the new recommendations for endocarditis prophylaxis from American Heart Association.

Since 2003, our strategy for closure of the patent arterial duct has changed subsequent to the availability of the Amplatzer occluder. This new device has allowed significantly larger patent arterial ducts to be closed with interventional catheterization procedures that in the past would have been closed at surgery. During the interval between 2002 and 2006 inclusive, the overall surgical volume at our Institute has been stable. Over this period, the number of patients undergoing surgical ligation of the patent arterial duct has decreased, with this decline in volume most notable for the subgroup of patients weighing more than five kilograms. This decrease has been especially notable in thoracoscopic procedures and is attributable to the increased ability to close larger ducts using the Amplatzer occluder.

For infants with symptomatic pulmonary overcirculation weighing less than 5 kilograms, our preference is for the surgical approach. For patients who have ductal calcification, significant pleural scarring, or “window-like” arterial ducts, video-assisted ligation is not an option and open surgical techniques are used. When video-assisted ligation is possible, the approach is based on family and surgeon preference. When open thoracotomy is selected, we usually use a muscle-sparing left posterolateral thoracotomy.

For patients weighing more than 5 kilograms, we currently recommend percutaneous closure for all patent arterial ducts as the first intervention, reserving surgical treatment for those cases that are not amenable to the percutaneous approach. For symptomatic infants weighing greater than 5 kilogram with large ducts, we prefer to use the Amplatzer occluder. In rare instances, the size of the required ductal occluder is so large that either encroachment into the aorta or pulmonary arteries is noted, and the device is removed. The child is then referred for surgical closure. We can now often predict via echocardiography that a duct is too large for transcatheter closure, even with the Amplatzer occluder, and refer these patients directly to surgery.

For patients with an asymptomatic patent arterial duct, we prefer to wait until the weight is from 10 to 12 kilograms, or they are closer to 2 years of age. If the patent arterial duct is greater than 2.0 to 2.5 millimetres in diameter, our preference is to use the Amplatzer occluder. For smaller ducts, we typically use stainless steel coils. Using this strategy, we close all patent arterial ducts, regardless of their size.

Variability in practice can be considered to foster clinical innovation, and allow for individualized therapeutic plans and independence of practitioners. The Institute of Medicine, however, has issued a report suggesting that variability in patterns of practice are “illogical”, and should be avoided whenever possible. Perhaps nowhere in the field of congenital cardiac disease is variability in practice more apparent than in the management of hypoplastic left heart syndrome. This review assesses the variability in practice at a large number of centres that manage neonates with hypoplastic left heart syndrome, with an emphasis on practice before, during, and after the first stage of the Norwood sequence of operations. We also suggest changes in future strategies for research.

In March, 2007, colleagues were contacted to respond to an internet-based survey using commercially available software (www.surveymonkey.com) to collect responses about the management practices for neonates with “straight-forward” hypoplastic left heart syndrome. No attempt was made to correlate management practices with any measures of outcome, as neither the practices themselves, nor the outcomes of interest, could be externally validated. Data is reported from 52 centers thought to manage over 1000 neonates with hypoplastic left heart syndrome on an annual basis. The first stage of the Norwood sequence was “recommended” to families by approximately five-sixths (86.5%) of the centres. No centre recommended primary cardiac transplantation, a “hybrid” approach, or non-intervention. In 7 centres (14.5%), it was reported that there was discussion of some or all of the above options, but ultimately the families decided upon the appropriate strategy.

Most centres preferentially used antegrade cerebral perfusion (54%) in contrast to deep hypothermia with circulatory arrest (24%), albeit that 11% of centres used a combination of these techniques and in 9% the support strategy was based on surgeon preference. The source of flow of blood for the lungs following the first stage of reconstruction was also highly variable. Of the 51 centres that responded to the question, 13 (25.5%) were participating in a multi-centric randomized clinical trial comparing the modified Blalock-Taussig shunt to the conduit placed from the right ventricle to the pulmonary arteries, the so-called “Sano” modification. Of the remaining 38 centres, 18 “usually” placed a conduit from the right ventricle to the pulmonary artery, 14 “usually” placed a modified Blalock-Taussig shunt, and at six centres, the decision was made “based upon the preference of the surgeon and/or the cardiologist”. Similarly, significant variability in practice was evident in preoperative management, other surgical strategies, postoperative medical support, monitoring and discharge planning. Other than the randomized clinical trial of shunt type, no other medical or surgical management strategy was currently under investigation in a multi-centric or randomized trial in the centres who responded to the survey.

The survey emphasises the extreme variability in our current practices for treatment of children with hypoplastic left heart syndrome. While there are some areas for which there is consensus in management, the majority of our practices are variable between and within centres. These results emphasize that large multicentric trials and registries are necessary to improve care, and to answer important clinical questions, emphasizing the need to shift from analysis of experiences of single centres to multi-centric and multi-disciplinary collaboration.

Patients with congenital cardiac disease require lifelong medical care. Current challenges that face practitioners who care for adults with congenital heart disease include identifying the best location for procedures, which could be a children’s hospital, an adult hospital, or a tertiary care facility; providing appropriate antenatal management of pregnant women with congenitally malformed hearts, and continuing this care in the peripartum period; and securing the infrastructure and expertise of the non-cardiac subspecialties, such as nephrology, hepatology, pulmonary medicine, and haematology. The objectives of this review are to outline the common problems that confront this population of patients and the medical community, to identify challenges encountered in establishing a programme for care of adults with congenitally malformed hearts, and to review the spectrum of disease and operations that have been identified in a high volume tertiary care centre for adult patients with congenital cardiac disease. Three chosen examples of the fundamental problems facing the practitioner and patient in the United States of America in 2007 are the neglected patient with congenital cardiac disease, weak infrastructure for adults with congenital cardiac disease, and family planning and management of pregnancy for patients with congenital cardiac disease.

Patients with adult congenital cardiac disease often do not receive appropriate surveillance. Three fundamental reasons for this problem are, first, that most adults with congenitally malformed hearts have been lost to follow-up by specialists, and are either receiving community care or no care at all. Second, patients and their families have not been educated about their malformed hearts, what to expect, and how to protect their interests most effectively. Third, adult physicians have not been educated about the complexity of the adult with a congenitally malformed heart. This combination can be fatal for adults with complications related to their congenitally malformed heart, or its prior treatment. Two solutions would improve surveillance and care for the next generation of patients coming out of the care of paediatric cardiologists. The first would be to educate patients and their families during childhood and adolescence. They would learn the names of the diagnoses and treatments, the problems they need to anticipate and avoid, the importance of expert surveillance, career and family planning information, and appropriate self-management. The second solution would be to encourage an orderly transfer of patients from paediatric to adult practice, usually at about 18 years of age, and at the time of graduation from high school.

Clinics for adults with congenital cardiac disease depend upon multidisciplinary collaboration with specialties in areas such as congenital cardiac imaging, diagnostic and interventional catheterization, congenital cardiac surgery and anaesthesia, heart failure, transplantation, electrophysiology, reproductive and high risk pregnancy services, genetics, pulmonary hypertension, hepatology, nephrology, haematology, and others. None of these services are easily available “off the rack”, although with time, experience, and determination, these services can develop very well. Facilities with experienced personnel to provide competent care for adults with congenital cardiac disease are becoming increasingly available. Parents and patients should learn that these facilities exist, and be directed to one by their paediatric caregivers when the time comes for transition to adult care.

With the steady increase in the number of adults with congenital heart disease, an ever increasing number of women with such disease are becoming pregnant. Services are not widely available to assess competently and plan a pregnancy for those with more complex disease. It is essential to have a close interplay between the obstetrician, the adult congenital cardiologist, the fetal medicine perinatologist, and neonatologist.

In both a community based programme and a tertiary care centre, the nuances and complexities of congenital cardiac anatomy, coupled with the high probability of previous operation during childhood, makes the trained congenital cardiothoracic surgeon best suited to deal with the surgical needs of this growing population. It is clear that the majority of adults with congenital heart disease are not “cured”, but require lifelong comprehensive care from specialists who have expertise in this complex arena. There is a growing cadre of healthcare professionals dedicated to improving the care of these patients. More information has become available about their care, and will be improved upon in the next decade. With the support of the general paediatric and paediatric cardiologic communities, and of the Adult Congenital Heart Association, and with the persistence of the providers of care for adults with congenital cardiac disease currently staffing clinics, the care of these patients should become more secure in the next decade as we mature our capabilities.

Concerns have emerged in recent years with regard to the role to be played by the pathologist in reaching a final diagnosis. When considering the field of the congenitally malformed heart, it is true that the richness of detail now provided by imaging techniques is truly amazing. Alongside these developments, there has also been a significant decline in the number of autopsies performed in tertiary medial centres around the World. In this review, we consider some of the factors that have contributed to this decrease in autopsies, and review the reasons why strong steps should be taken to reverse this trend.

When considering the reasons for the decline in autopsies, there can be little doubt that the scandal devolving on inappropriate retention of organs, which came to light in the United Kingdom, but which had reverberations throughout the World, contributed in no small way to the reticence of families to grant appropriate permission to conduct a post-mortem examination. It is sincerely hoped that the changes in practise that followed these revelations will stop, and indeed reverse, this unfortunate decline. The inappropriate retention of organs came into the public domain in an attempt to emphasise the value of the autopsy in clinical practise, research, and education. All of these good reasons for performing the autopsy remain.

From the stance of education, we emphasise the importance of retaining existing archives, which have long since proved their value. From the stance of improving clinical practise, we reiterate that the attitude of the morphologist, working side-by-side with the clinician or surgeon, has always been fundamental in expanding this aspect of knowledge. We recognise, nonetheless, that performance of the autopsy still carries financial considerations. In this respect, when considering the congenitally malformed heart, we stress the option of having the pathologist working in harness with an experienced cardiac morphologist, or alternatively with a properly trained pathologist’s assistant. In terms of training, we show how, with the advantage of a few simple rules, it becomes an easy matter to describe and analyse the congenitally malformed heart. Thereafter, having reviewed means of increasing the number of autopsies, and discussing new techniques, we complete our review with a detailed account of the fetal, perinatal, and paediatric autopsy in the patients with a congenitally malformed heart, taking particular account of the role to be played by the properly trained pathologist’s assistant.

Mechanical circulatory support is an invaluable tool in the care of children with severe refractory cardiac and or pulmonary failure. Two forms of mechanical circulatory support are currently available to neonates, infants, and smaller children, namely extracorporeal membrane oxygenation and use of a ventricular assist device, with each technique having unique advantages and disadvantages. The intra-aortic balloon pump is a third form of mechanical support that has been successfully used in larger children, adolescents, and adults, but has limited applicability in smaller children. In this review, we discuss the current experiences with extracorporeal membrane oxygenation and ventricular assist devices in children with cardiac disease.

A variety of forms of mechanical circulatory support are available for children with cardiopulmonary dysfunction refractory to conventional management. These devices require extensive resources, both human and economic. Extracorporeal membrane oxygenation can be effectively used in a variety of settings to provide support to critically-ill patients with cardiac disease. Careful selection of patients and timing of intervention remains challenging. Special consideration should be given to children with cardiac disease with regard to anatomy, physiology, cannulation, and circuit management. Even though exciting progress is being made in the development of ventricular assist devices for long-term mechanical support in children, extracorporeal membrane oxygenation remains the mainstay of mechanical circulatory support in children with complex anatomy, particularly those needing rapid resuscitation and those with a functionally univentricular circulation.

As the familiarity and experience with extracorporeal membrane oxygenation has grown, new indications have evolved, including emergent resuscitation. This utilization has been termed extracorporeal cardiopulmonary resuscitation. The literature supporting emergent cardiopulmonary support is mounting. Reasonable survival rates have been achieved after initiation of support during active compressions of the chest following in-hospital cardiac arrest. Due to the limitations of conventional circuits for extracorporeal membrane oxygenation, some centres have developed novel systems for rapid cardiopulmonary support.

Many centres previously considered a functionally univentricular circulation to be a contraindication to extracorporeal membrane oxygenation, but improved results have been achieved recently with this complex subset of patients. The registry of the Extracorporeal Life Support Organization recently reported the outcome of extracorporeal life support used in neonates for cardiac indications from 1996 to 2000. Of the 740 neonates who were placed on extracorporeal life support for cardiac indications, 118 had hypoplastic left heart syndrome. There was no significant difference in survival between these patients and those with other defects. It is now common to use extracorporeal membrane oxygenation to support patients with a functionally univentricular circulation, and reasonable survival rates are to be expected.

Although extracorporeal membrane oxygenation has become a standard of care for many paediatric centres, its use is limited to those patients who require only short-term cardiopulmonary support. Mechanical ventricular assist devices have become standard therapy for adults with cardiac failure refractory to maximal medical management. Several devices are readily available in the United States of America for adults, but there are fewer options available to children. Over the last few years, substantial progress has been made in paediatric mechanical support. Ventricular assist devices are being used with increasing frequency in children with cardiac failure refractory to medical therapy for primary treatment as a long-term bridge to recovery or transplantation. The paracorporeal, pneumatic, pulsatile “Berlin Heart” ventricular assist device is being used with increasing frequency in Europe and North America to provide univentricular and biventricular support. With this device, a patient can be maintained on mechanical circulatory support while extubated, being mobilized, and feeding by mouth.

Mechanical circulatory support should be anticipated, and every attempt must be made to initiate support “urgently” rather than “emergently”, before the presence of dysfunction of end organs or circulatory collapse. In an emergency, these patients can be resuscitated with extracorporeal membrane oxygenation and subsequently transitioned to a long-term ventricular assist device after a period of stability.

Pulseless cardiac arrest, defined as the cessation of cardiac mechanical activity, determined by unresponsiveness, apneoa, and the absence of a palpable central pulse, accounts for around one-twentieth of admissions to paediatric intensive care units, be they medical or exclusively cardiac. Such cardiac arrest is higher in children admitted to a cardiac as opposed to a paediatric intensive care unit, but the outcome of these patients is better, with just over two-fifths surviving when treated in the cardiac intensive care unit, versus between one-sixth and one-quarter of those admitted to paediatric intensive care units. Children who receive chest compressions for bradycardia with pulses have a significantly higher rate of survival to discharge, at 60%, than do those presenting with pulseless cardiac arrest, with only 27% surviving to discharge. This suggests that early resuscitation before the patient becomes pulseless, along with early recognition and intervention, are likely to improve outcomes. Recently published reports of in-hospital cardiac arrests in children can be derived from the multi-centric National Registry of Cardiopulmonary Resuscitation provided by the American Heart Association. The population is heterogeneous, but most arrests occurred in children with progressive respiratory insufficiency, and/or progressive circulatory shock. During the past 4 years at the Children’s Hospital of Philadelphia, 3.1% of the average 1000 annual admissions to the cardiac intensive care unit have received cardiopulmonary resuscitation. Overall survival of those receiving cardiopulmonary resuscitation was 46%. Survival was better for those receiving cardiopulmonary resuscitation after cardiac surgery, at 53%, compared with survival of 33% for pre-operative or non-surgical patients undergoing resuscitation. Clearly there is room for improvement in outcomes from cardiac resuscitation in children with cardiac disease. In this review, therefore, we summarize the newest developments in paediatric resuscitation, with an expanded focus upon the unique challenges and importance of anticipatory care in infants and children with cardiac disease.

The complexity of the modern systems providing health care presents a unique challenge in delivering care of the required quality in a safe environment. Issues of safety have been thrust into the limelight because of adverse events highly publicized in the general media.

In the United States of America, improving the safety and quality in health care has been set forth as a priority for improvements in the 21st century in the report from the Institute of Medicine. Many measures have now been initiated for improving the safety of patients at hospital, regional, and national level, and through initiatives sponsored by governments and private organizations. In this review, we summarize known concepts and current issues on the safety of patients, and their applicability to children with congenital cardiac disease. Prior to examining the issues of medical error and safety, it is important to define the terminology.

An error is defined as the failure of a planned action to be completed as intended, also known as an execution error, or the use of a wrong plan to achieve an aim, this representing a planning error. An active error is an error that occurs at the level of the frontline operator, and the effects of which are felt immediately. A latent error is an error in the design, organization, training and maintenance, that leads to operator errors, and the effects of which are typically dormant in the system for lengthy periods of time. Latent errors may cause harm given the right circumstances and environment.

An adverse event is defined as an injury resulting from medical intervention. A preventable adverse event is an adverse event that occurs due to medical error. Negligent adverse events are a subset of preventable adverse events where the care provided did not meet the standard of care expected of that practitioner.

The study of improving the delivery of safe care for our patients is a rapidly growing field. Important components for development of programmes to improve the safety of patients include the leadership for the programme, the implementation of process design based on human limitations, the promotion of teamwork and function, the anticipation of unexpected events, and the creation of a learning environment.

Much is yet to be learned about the risk and incidence of adverse events during hospitalization of children with congenital cardiac disease. Errors due to human factors, such as poor communication, poor coordination, and suboptimal team work, have shown to be important causes of adverse outcomes in children undergoing cardiac surgery, and should be a focus for improvement. Future research on evaluating causes and prevention of medical errors and adverse events in this population at high risk, and consuming high resources, is essential.

Issues of inadequate safeguards for patients have been prominent in the media, and have been highlighted in reports from the Institute of Medicine. Our review discusses research on the causes of medical error, and proposes concepts to design successful programmes to improve safety for the patients on a local level.

Our review summarizes the thoughts we shared in presenting the 8th annual Daicoff lecture. It is fitting, therefore, to begin with a few comments about George Daicoff. One of us (RU) first met George at a meeting, which might have been the Southern Thoracic Surgical Association. He was very kind and gracious, and made me feel welcome. We would like for each of you now reading this review to think, for a moment, of when you have had an experience like that from someone you didn’t know well, and how it made you feel comfortable, and that maybe you “fit in.” George, we thank you for that memory. Our field needs more graciousness. As all of us function in our everyday world, we should remember that we never know when our acts of graciousness one to another will be remembered and acknowledged. Our review summarises five concepts that we have found helpful in our work with similar groups of busy professionals.

The first is mindfulness, sometimes referred to as being conscious of the present moment. It is an irony of the training of health care professionals that we are constantly being directed towards a future focus. We readily don the blinders of a professional life that keeps us focusing on what lies ahead. Although some element of this is essential for professional success, we run the risk of missing out on the richness of our everyday experiences. The second is intentionality. In our work with busy professionals, we have found that so many have drifted into the automaticity of patterned responses. This gets us in so much trouble because we forget that we always have choice. The third is mindsight. This is about empathy, and the ability to connect to the experiences of others. Mindsight is about connecting to our differences. The fourth is forgiveness and shared meanings. It is important to practice forgiveness, and to create shared meanings in relationships. These processes allow us to reconnect to people who have hurt, disappointed, or angered us. When we don’t forgive, we create toxic relationships, both with ourselves and with others. It is the heaviness of resentment that prevents us from being at ease. The final concept concerns management of stress. We should learn to recognize when we are stressed. We cannot manage what we don’t know. It is our belief that attention to these features will help you better manage the numerous demands of your life.

This review discusses the historical aspects, current state of the art, and potential future advances in the areas of nomenclature and databases for the analysis of outcomes of treatments for patients with congenitally malformed hearts. We will consider the current state of analysis of outcomes, lay out some principles which might make it possible to achieve life-long monitoring and follow-up using our databases, and describe the next steps those involved in the care of these patients need to take in order to achieve these objectives. In order to perform meaningful multi-institutional analyses, we suggest that any database must incorporate the following six essential elements: use of a common language and nomenclature, use of an established uniform core dataset for collection of information, incorporation of a mechanism of evaluating case complexity, availability of a mechanism to assure and verify the completeness and accuracy of the data collected, collaboration between medical and surgical subspecialties, and standardised protocols for life-long follow-up. During the 1990s, both The European Association for Cardio-Thoracic Surgery and The Society of Thoracic Surgeons created databases to assess the outcomes of congenital cardiac surgery. Beginning in 1998, these two organizations collaborated to create the International Congenital Heart Surgery Nomenclature and Database Project. By 2000, a common nomenclature, along with a common core minimal dataset, were adopted by The European Association for Cardio-Thoracic Surgery and The Society of Thoracic Surgeons, and published in the Annals of Thoracic Surgery. In 2000, The International Nomenclature Committee for Pediatric and Congenital Heart Disease was established. This committee eventually evolved into the International Society for Nomenclature of Paediatric and Congenital Heart Disease. The working component of this international nomenclature society has been The International Working Group for Mapping and Coding of Nomenclatures for Paediatric and Congenital Heart Disease, also known as the Nomenclature Working Group. By 2005, the Nomenclature Working Group crossmapped the nomenclature of the International Congenital Heart Surgery Nomenclature and Database Project of The European Association for Cardio-Thoracic Surgery and The Society of Thoracic Surgeons with the European Paediatric Cardiac Code of the Association for European Paediatric Cardiology, and therefore created the International Paediatric and Congenital Cardiac Code, which is available for free download from the internet at [http://www.IPCCC.NET].

This common nomenclature, the International Paediatric and Congenital Cardiac Code, and the common minimum database data set created by the International Congenital Heart Surgery Nomenclature and Database Project, are now utilized by both The European Association for Cardio-Thoracic Surgery and The Society of Thoracic Surgeons. Between 1998 and 2007 inclusive, this nomenclature and database was used by both these two organizations to analyze outcomes of over 100,000 patients undergoing surgical treatment for congenital cardiac disease. Two major multi-institutional efforts that have attempted to measure the complexity of congenital heart surgery are the Risk Adjustment in Congenital Heart Surgery-1 system, and the Aristotle Complexity Score. Current efforts to unify the Risk Adjustment in Congenital Heart Surgery-1 system and the Aristotle Complexity Score are in their early stages, but encouraging. Collaborative efforts involving The European Association for Cardio-Thoracic Surgery and The Society of Thoracic Surgeons are under way to develop mechanisms to verify the completeness and accuracy of the data in the databases. Under the leadership of The MultiSocietal Database Committee for Pediatric and Congenital Heart Disease, further collaborative efforts are ongoing between paediatric and congenital cardiac surgeons and other subspecialties, including paediatric cardiac anaesthesiologists, via The Congenital Cardiac Anesthesia Society, paediatric cardiac intensivists, via The Pediatric Cardiac Intensive Care Society, and paediatric cardiologists, via the Joint Council on Congenital Heart Disease and The Association for European Paediatric Cardiology.

In finalising our review, we emphasise that analysis of outcomes must move beyond mortality, and encompass longer term follow-up, including cardiac and non cardiac morbidities, and importantly, those morbidities impacting health related quality of life. Methodologies must be implemented in these databases to allow uniform, protocol driven, and meaningful, long term follow-up.

The problems that exist in maintaining a partnership in paediatric cardiac surgery are considerable. They relate to fairness in allocation of time for leisure, the apportioning of cases between the partners, internal competition between them, financial considerations, and promotion of the ego. In this review, we discuss our own experiences in maintaining a partnership over a period of more than 18 years, relating such a “virtuous partnership” to the writings of Plato and Aristotle, and setting it against the tenets of the ancient Greek polis.

The polis, or city state, came to prominence in ancient Greece during the golden age of Pericles, this period seeing the initial evolution of Western philosophy, as well as numerous other scientific, artistic and architectural advances. The concept of the polis was to create a natural association with its citizens that nurtured all that is best in people, at the same time defining their character. In this respect, according to Plato, the person and the polis are mirror images. Aristotle then expanded this notion to incorporate the various forms of friendship, which he pointed out last only as long as the interrelated pleasure survives.

Using these principles as the point of departure, we argue that cardiac surgeons should respect moral virtue in each other. Extending this process means that we should also respect, and celebrate, our relationships with affiliated physicians, nurses, perfusionists, administrators, and all concerned in the care of children with congenitally malformed hearts. In this way, we create a virtuous partnership for congenital cardiac surgery that promotes all that was good, as engendered in the ancient Greek polis.

As we extend these observations to the modern world, we discuss some of the features that have permitted us to work so well together. One of the most important is a summoning and unwritten tenet that greets us as we enter the operating room, namely “check your ego at the door”. The operative choice should always be dictated by discussion, citations of literature, considered opinions, and relevance to the particular patient. Continuity of care should lead inexorably to the paediatric intensive care unit, where collegial relationships should be maintained with all those working therein. We need to recognize that there are various “captains of the ship”, who must work in harmony so as to bring the best possible care to our patients. We always endeavour to empower others to act on our behalf, based on their experience and training.

Whether we have achieved our desired moral excellence, and produced the completed or perfected friendship, is for others to judge. From our stance, we believe we have created a favourable environment by hard work, unselfish attitudes, and celebration of our mutual accomplishments. We were not the first to forge this kind of professional association, and happily we will not be the last.

Professionals working in the arena of health care face a variety of challenges as their careers evolve and develop. In this review, we analyze the role of mentorship, learning curves, and balance in overcoming challenges that all such professionals are likely to encounter. These challenges can exist both in professional and personal life.

As any professional involved in health care matures, complex professional skills must be mastered, and new professional skills must be acquired. These skills are both technical and judgmental. In most circumstances, these skills must be learned. In 2007, despite the continued need for obtaining new knowledge and learning new skills, the professional and public tolerance for a “learning curve” is much less than in previous decades. Mentorship is the key to success in these endeavours. The success of mentorship is two-sided, with responsibilities for both the mentor and the mentee. The benefits of this relationship must be bidirectional. It is the responsibility of both the student and the mentor to assure this bidirectional exchange of benefit. This relationship requires time, patience, dedication, and to some degree selflessness. This mentorship will ultimately be the best tool for mastering complex professional skills and maturing through various learning curves. Professional mentorship also requires that mentors identify and explicitly teach their mentees the relational skills and abilities inherent in learning the management of the triad of self, relationships with others, and professional responsibilities.

Up to two decades ago, a learning curve was tolerated, and even expected, while professionals involved in healthcare developed the techniques that allowed for the treatment of previously untreatable diseases. Outcomes have now improved to the point that this type of learning curve is no longer acceptable to the public. Still, professionals must learn to perform and develop independence and confidence. The responsibility to meet this challenge without a painful learning curve belongs to both the younger professionals, who must progress through the learning curve, and the more mature professionals who must create an appropriate environment for learning.

In addition to mentorship, the detailed tracking of outcomes is an essential tool for mastering any learning curve. It is crucial to utilize a detailed database to track outcomes, to learn, and to protect both yourself and your patients. It is our professional responsibility to engage in self-evaluation, in part employing voluntary sharing of data. For cardiac surgical subspecialties, the databases now existing for The European Association for CardioThoracic Surgery and The Society of Thoracic Surgeons represent the ideal tool for monitoring outcomes. Evolving initiatives in the fields of paediatric cardiology, paediatric critical care, and paediatric cardiac anaesthesia will play similar roles.

A variety of professional and personal challenges must be met by all those working in health care. The acquisition of learned skills, and the use of special tools, will facilitate the process of conquering these challenges. Choosing appropriate role models and mentors can help progression through any learning curve in a controlled and protected fashion. Professional and personal satisfaction are both necessities. Finding the satisfactory balance between work and home life is difficult, but possible with the right tools, organization skills, and support system at work and at home. The concepts of mentorship, learning curves and balance cannot be underappreciated.