The Multi-Societal Database Committee for Pediatric and Congenital Heart Disease was established in 2005 with the goal of providing the infrastructure, spanning geographical and subspecialty boundaries, for collaboration between health care professionals interested in the analysis of outcomes of treatments provided to patients with congenital cardiac disease, with the ultimate aim of improvement in the quality of care provided to these patients. The purpose of these collaborative efforts is to promote the highest quality comprehensive cardiac care to all patients with congenital heart disease, from the fetus to the adult, regardless of the patient’s economic means, with an emphasis on excellence in teaching, research and community service. This manuscript provides the Introduction to the 2008 Supplement to Cardiology in the Young titled: “Databases and The Assessment of Complications associated with the Treatment of Patients with Congenital Cardiac Disease”. This Supplement was prepared by The Multi-Societal Database Committee for Pediatric and Congenital Heart Disease.

The Multi-Societal Database Committee for Pediatric and Congenital Heart Disease offers the following definition of the term “Complication”: “A complication is an event or occurrence that is associated with a disease or a healthcare intervention, is a departure from the desired course of events, and may cause, or be associated with, suboptimal outcome. A complication does not necessarily represent a breech in the standard of care that constitutes medical negligence or medical malpractice. An operative or procedural complication is any complication, regardless of cause, occurring (1) within 30 days after surgery or intervention in or out of the hospital, or (2) after 30 days during the same hospitalization subsequent to the operation or intervention. Operative and procedural complications include both intraoperative/intraprocedural complications and postoperative/postprocedural complications in this time interval.”

The Multi-Societal Database Committee for Pediatric and Congenital Heart Disease offers the following definition of the term “Adverse Event”: “An adverse event is a complication that is associated with a healthcare intervention and is associated with suboptimal outcome. Adverse events represent a subset of complications. Not all medical errors result in an adverse event; the administration of an incorrect dose of a medication is a medical error, but it does not always result in an adverse event. Similarly, not all adverse events are the result of medical error. A child may develop pneumonia after an atrial septal defect repair despite intra- and peri-operative management that is free of error. Complications of the underlying disease state, which are not related to a medical intervention, are not adverse events. For example, a patient who presents for medical care with metastatic lung cancer has already developed a complication (Metastatic spread) of the primary lung cancer without any healthcare intervention. Furthermore, complications not associated with suboptimal outcome or harm are not adverse events and are known as no harm events. The patient who receives an incorrect dose of a medication without harm has experienced a no harm event, but not an adverse event.”

Based on the above definitions, it is apparent that The Multi-Societal Database Committee for Pediatric and Congenital Heart Disease has taken an inclusive approach to defining the universe of complications. Complications may or may not be associated with healthcare intervention and may or may not be associated with suboptimal outcome. Meanwhile, adverse events must be associated with healthcare intervention and must be associated with suboptimal outcome.

The persistence of so-called spongy myocardium was first reported in humans in the 1960s. Subsequently, a few reports described the condition, utilizing inconsistent facts and terminology. In 1990 the first report appeared using the term “noncompaction”, and detailed its clinical implications. Following this report, more descriptions of patients with noncompaction entered the literature. Prior to the availability of high resolution cross-sectional echocardiography, most cases of noncompacted myocardium escaped detection, as few patients underwent angiography. In this essay, I seek to adjudicate and clarify several confusions and controversies in the current literature including the acquired nature of hypertrabeculation, the fate of the so-called sinusoids, the relationship to coronary arteries, and noncompaction as another congenital malformation. The embryonic timing of cessation and reinitiation of compaction is conjectured as an explanation of the varied configurations of noncompaction as seen as a clinical entity. The clinical outcomes of decreased contractility, arrhythmia, and thromboembolism, have been stressed in the current literature, but more study is needed of the gray area between the normal variant and minimal noncompaction. A plea is made for the standardization of methods and terminology.

In this review, I summarize observations suggesting that social cognition deficits are an important element of the neurodevelopmental morbidities of children with congenital cardiac malformations. Referred to as “theory of mind” deficits, they pertain to the ability to “read” other people, that is, to infer their internal states and thus to interpret their actions appropriately. I also hypothesize that children with such congenital cardiac malformations have difficulty identifying and describing their own internal states, which is referred to as alexithymia. These hypotheses are based on data collected as part of a prospective study of the neurologic outcomes of children with concordant atrioventricular and discordant ventriculo-arterial connections, or transposition. On a variety of tasks that assess pragmatic language and discourse skills, such as elicited personal narratives, free play with a parent, and written narratives, the children consistently performed below age-expected levels and in ways that suggested the presence of “theory of mind” deficits. Their narratives lacked coherence, failed to include critical information required by a listener, and made infrequent reference to internal states. In ongoing evaluations of the children as adolescents, we are testing these hypotheses more systematically by administering several instruments specifically developed to assess “theory of mind” and alexithymia.

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 of these two organizations to analyze outcomes of over 150,000 operations involving 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 congenital and paediatric 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.

A large number of patients in developing countries require surgical correction of congenitally malformed hearts. Unfortunately, only a limited number of centres offer these patients surgery at an affordable cost. In this review, we discuss the problems in managing these patients, with an emphasis on reduction of costs, so that the maximum number of patients can benefit. It is apparent that containing costs requires a multipronged approach, which begins with timely referral, and continues with early surgical correction and adequate postoperative care carried out in a scientific manner. Indigenization, innovation, training of manpower, and building a team, are essential to cut the costs, and to improve the quality of care.

The literature has scant documentation of the relationship between the important founders of paediatric cardiology, Maude Abbott and Helen Taussig. Recently discovered entries in the diaries kept by Maude Abbott provide evidence for a close connection between them. Further evidence suggests that their association was complex, and influenced by outside factors, such as their difference in age and era-related notoriety. In this review, I attempt to define more clearly the relationship between these two early icons of paediatric cardiology.

When there is obstruction of the left ventricular outflow tract, there are several surgical approaches to the repair of discordant ventriculo-arterial connections in the setting of concordant atrioventricular connections. Choosing the optimal technique demands not only knowledge of the different surgical procedures, but also the understanding of the particular anatomic features present in a specific patient. These requisites are then essential to plan the operation, to foresee some difficult situations, and to avoid post-operative complications. In this review, we assess all these surgical and anatomic aspects, focussing on their relative importance in clinical assessment.

Pulmonary hypertension associated with congenital systemic-to-pulmonary shunts has been classified, in the Evian-Venice classification, as Pulmonary Arterial Hypertension, which includes a heterogeneous group of conditions. Emerging options for treatment of patients with pulmonary arterial hypertension are related to those with the idiopathic form of the disease, but may also improve quality of life and survival in patients with pulmonary arterial hypertension associated with congenital cardiac disease. Despite the evident similarities in pulmonary vascular disease, it is important also to recognise the differences between patients in whom pulmonary arterial hypertension is the consequence of systemic-to-pulmonary shunts as opposed to those with other conditions. Patients with pulmonary hypertension associated with congenital cardiac disease themselves constitute a heterogeneous population, in which generalisation may be hazardous. Specific considerations need to be given to the type of cardiac diagnosis, the prognosis and evolution of pulmonary vascular disease, and the circulatory physiology before embarking on new strategies for medical treatment in the individual patient. In this review, we highlight the features that require specific attention in these patients. In addition, we discuss briefly the data currently available on the effectiveness of the new anti-proliferative drugs in patients with the Eisenmenger syndrome.