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.

An eight-month-old girl with hypoplastic left heart syndrome, who underwent a modified Norwood operation at the age of two days, and a Hemifontan operation at five months of age, had severely impaired ventricular function and new electrocardiographic changes. Coronary angiography demonstrated a small adherent thrombus in the non-coronary sinus of Valsalva of the native aortic root, which may be the cause of unexplained ventricular dysfunction.

Objective: To study the adaptive potential of the right ventricular myocardium after 30 days of mechanical-induced overload in rats from two different age groups. Materials and methods: We banded the pulmonary trunk, so as to increase the systolic work load of the right ventricle, in 19 adult Sprague-Dawley rats at the age of 10 weeks, and 16 weanlings when they were 3 weeks-old, using 10 adults and 10 weanlings as controls. We analysed the functional adaptation and structural changes of the right ventricular myocardium, blood vessels and interstitial tissue after 30 days of increased afterload. Results: The increased workload induced an increase of the right ventricular weight and free wall thickness in animals from both age groups when compared to controls. These changes were mostly related to cardiomyocytic hypertrophy, as confirmed by the expression of myocardial hypertrophic markers, without any apparent increase of their number, a “reactive” fibrosis especially evident in the adult rats, with p-value less than 0.0001, and a more extensive neocapillary network in the weanlings compared to the adults aubsequent to banding, the p-value being less than 0.0001. Conclusion: In response to right ventricular afterload, weanlings showed a higher adaptive capillary growth, which hampered the development of fibrosis as seen in the adult rats. Age seems to be a risk factor for adverse structural-functional changes of right ventricle subjected to increased workload.

We report a 2-month old female with a rare cardiac mass. She was evaluated echocardiographically after physical examination revealed a low grade cardiac murmur. The mass, located in right atrium, was shown to be the consequence of candida endocarditis by means of pathological study and blood culture.

We report on the management of Candida endocarditis in a 5-month old infant. The orifice of the tricuspid valve was totally obstructed, and the tension apparatus of the valve destroyed. Excision of the valve led to severe failure of the right heart. The combined use of anti-failure treatment, and reduction of right ventricular afterload with oxygen, nitric oxide and sildenafil, proved successful.

An intact atrial septum places infants with hypoplasia of the left heart into a group with an extremely high rate of mortality. We report a neonate, diagnosed antenatally, who was delivered by Caesarian section in the cardiac theatre, urgently placed onto cardiopulmonary bypass, and who then underwent an atrial septectomy and banding of both pulmonary arteries. After stablilisation, his arterial duct was stented, completing the first stage of hybrid palliation.

Noonan's syndrome is an autosomal dominant genetic disease, in which mutation of the PTPN11 gene is found in from one-third to half of all cases. Pulmonary valvar stenosis and myocardiopathy are frequently associated cardiac malformations, whereas aortic valvar stenosis is rarely described. We report, as far as we know, the first case of familial Noonan syndrome with severe aortic valvar stenosis, demonstrating mutation of the PTPN11 gene in the father of the patient.

We describe a child with tricuspid atresia associated with a porto-systemic shunt and complete absence of the portal vein, the latter known as the Abernethy malformation. As far as we are aware, this association has not previously been reported. We review the various cardiac defects that have been reported in children with the Abernethy malformation, emphasizing the interesting clinical presentations of this rare malformation of the portal venous system.

We report a patient in whom a cavopulmonary anastomosis had been constructed, along with repair of anomalous pulmonary venous drainage. Left-sided pulmonary venous obstruction led to redistribution of the flow to the right lung. The reversal of flow in the left pulmonary artery was accentuated by flow through collateral arteries feeding the left lung. Within 14 months, the collateral flow increased six-fold, resulting in a doubling of the cardiac output.

We report the first case of double-barrelled aorta associated with tetralogy of Fallot and pulmonary atresia, and we discuss the difficulties in the terminology of this fascinating anomaly.

Totally anomalous pulmonary venous return may present diagnostic difficulty to clinicians, as it often appears similar to severe neonatal pulmonary disease. We describe a neonate who presented with severely obstructed pulmonary venous return, but with a residual venous connection to the left atrium. The unusual anatomy confounded the diagnosis. To our knowledge, this particular permutation has not previously been described. Our experience indicates that echocardiographic interrogation should be repeated in cases where there is no response to conventional therapy, and all individual pulmonary veins should be observed carefully.

The role of familial disease in childhood dilated cardiomyopathy is unknown. A novel mutation in the cardiac Troponin C gene has been identified recently in a family with dilated cardiomyopathy. Here we present a subsequent case of dilated cardiomyopathy occurring in a child from the same family, and emphasise the implications for future screening and counselling.

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.

We report a 23-year-old patient undergoing functionally univentricular repair who developed cardiac failure due primarily to systemic ventricular dysfunction. The failing Fontan circulation was successfully re-established using intra-aortic balloon counterpulsation. Aortic counterpulsation facilitates recovery of systemic ventricular function, and appears to be a reasonable alternative in select instances of cardiac failure in patients with the Fontan circulation.

Primary cardiac tumours are rare in children. Of these, papillary fibroelastomas are unusual but benign, usually being found in adults. There are only sporadic cases reported in children. We diagnosed such a papillary fibroelastoma involving the tricuspid valve in an asymptomatic child during a routine cardiac investigation.

We describe a combination, unique as far as we are aware, of tetralogy of Fallot with so-called “absent” pulmonary valve and supracardiac totally anomalous pulmonary venous connection. The vertical vein was obstructed in a neonate born at term who presented with respiratory compromise.

Dilated cardiomyopathy as seen in children is clinically and genetically heterogeneous, with an increasing proportion of cases known to be caused by disorders of single genes. An autosomal recessive syndrome with a high incidence of dilated cardiomyopathy was recently described in the Canadian Dariusleut Hutterite population. It is caused by homozygous mutations in a novel gene, DNAJC19, presumed to play a role in importation of mitochondrial proteins. We discuss the cardiac features of this syndrome, and its relationship to cardiac mitochondrial function.

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.