Otto N. Krogmann

The nomenclature, definition and classification of hypoplastic left heart syndrome

The hypoplastic left heart syndrome encompasses a spectrum of cardiac malformations that are characterized by significant underdevelopment of the components of the left heart and the aorta, including the left ventricular cavity and mass. At the severe end of the spectrum is found the combination of aortic and mitral atresia, when the left ventricle can be close to non-existent. At the mild end are the patients with hypoplasia of the aortic and mitral valves, but without intrinsic valvar stenosis or atresia, and milder degrees of left ventricular hypoplasia. Although the majority of the patients are suitable only for functionally univentricular repair, a small minority may be candidates for biventricular repair. The nature of the syndrome was a topic for discussion at the second meeting of the International Working Group for Mapping and Coding of Nomenclatures for Paediatric and Congenital Heart Disease, the Nomenclature Working Group, held in Montreal, Canada, over the period January 17 through 19, 2003. Subsequent to these discussions, the Nomenclature Working Group was able to create a bidirectional crossmap between the nomenclature initially produced jointly on behalf of the European Association for Cardio-Thoracic Surgery and the Society of Thoracic Surgeons, and the alternative nomenclature developed on behalf of the Association for European Paediatric Cardiology. This process is a part of the overall efforts of the Nomenclature Working Group to create a comprehensive and all-inclusive international system of nomenclature for paediatric and congenital cardiac disease, the International Paediatric and Congenital Cardiac Code. In this review, we discuss the evolution of nomenclature and surgical treatment for the spectrum of lesions making up the hypoplastic left heart syndrome and its related malformations. We also present the crossmap of the associated terms for diagnoses and procedures, as recently completed by the Nomenclature Working Group.

Nomenclature and databases for the surgical treatment of congenital cardiac disease - an updated primer and an analysis of opportunities for improvement

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 finalizing 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 current status and future directions of efforts to create a global database for the outcomes of therapy for congenital heart disease

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:

Report from The International Society for Nomenclature of Paediatric and Congenital Heart Disease: cardiovascular catheterisation for congenital and paediatric cardiac disease (Part 2 – Nomenclature of complications associated with interventional cardiology)

Interventional cardiology for paediatric and congenital cardiac disease is a relatively young and rapidly evolving field. As the profession begins to establish multi-institutional databases, a universal system of nomenclature is necessary for the field of interventional cardiology for paediatric and congenital cardiac disease. The purpose of this paper is to present the results of the efforts of The International Society for Nomenclature of Paediatric and Congenital Heart Disease to establish a system of nomenclature for cardiovascular catheterisation for congenital and paediatric cardiac disease, focusing both on procedural nomenclature and the nomenclature of complications associated with interventional cardiology. This system of nomenclature for cardiovascular catheterisation for congenital and paediatric cardiac disease is a component of The International Paediatric and Congenital Cardiac Code. This manuscript is the second part of the two-part series. Part 1 covered the procedural nomenclature associated with interventional cardiology as treatment for paediatric and congenital cardiac disease. Part 2 will cover the nomenclature of complications associated with interventional cardiology as treatment for paediatric and congenital cardiac disease.

Classification of the functionally univentricular heart: unity from mapped codes.

The nomenclature and classification of patients with a functionally univentricular heart has been debated for decades. We review here the approach taken for dealing with this group of patients by the International Working Group for Mapping and Coding of Nomenclatures for Paediatric and Congenital Cardiac Disease. We discuss the approach of this Nomenclature Working Group in the context of other historical and contemporary ideas about this topic.

The nomenclature, definition and classification of discordant atrioventricular connections

During the process of creation of a bidirectional crossmap between the system emerging, on the one hand, from the initiative sponsored by the Congenital Heart Committees of the European Association for Cardio-Thoracic Surgery and the Society of Thoracic Surgeons, and on the other hand, from that formulated by the Coding Committee of the European Association for Pediatric Cardiology, the Nomenclature Working Group has successfully created the International Paediatric and Congenital Cardiac Code. As would be expected, during the process of crossmapping it became clear that, for most lesions, the European Pediatric Cardiac Code was more complete in its description of the diagnoses, while the International Congenital Heart Surgery Nomenclature and Database Project was more complete in its description of the procedures. This process of crossmapping exemplifies the efforts of the Nomenclature Working Group to create a comprehensive and all-inclusive international system for the naming of paediatric and congenital cardiac disease, the International Pediatric and Congenital Cardiac Code. Although names and classification for paediatric and congenital cardiac disease will continue to evolve over time, we are now closer than ever to reaching uniform international agreement and standardization. The International Paediatric and Congenital Cardiac Code can be downloaded from the Internet, free of charge, at www.ipccc. net.

Report from The International Society for Nomenclature of Paediatric and Congenital Heart Disease: cardiovascular catheterisation for congenital and paediatric cardiac disease (Part 1 - Procedural nomenclature)

Interventional cardiology for paediatric and congenital cardiac disease is a relatively young and rapidly evolving field. As the profession begins to establish multi-institutional databases, a universal system of nomenclature is necessary for the field of interventional cardiology for paediatric and congenital cardiac disease. The purpose of this paper is to present the results of the efforts of The International Society for Nomenclature of Paediatric and Congenital Heart Disease to establish a system of nomenclature for cardiovascular catheterisation for congenital and paediatric cardiac disease, focusing both on procedural nomenclature and on the nomenclature of complications associated with interventional cardiology. This system of nomenclature for cardiovascular catheterisation for congenital and paediatric cardiac disease is a component of The International Paediatric and Congenital Cardiac Code. This manuscript is the first part of a two-part series. Part 1 will cover the procedural nomenclature associated with interventional cardiology as treatment for paediatric and congenital cardiac disease. This procedural nomenclature of The International Paediatric and Congenital Cardiac Code will be used in the IMPACT Registry™ (IMproving Pediatric and Adult Congenital Treatment) of the National Cardiovascular Data Registry® of The American College of Cardiology. Part 2 will cover the nomenclature of complications associated with interventional cardiology as treatment for paediatric and congenital cardiac disease.

Report From The International Society for Nomenclature of Paediatric and Congenital Heart Disease: Creation of a Visual Encyclopedia Illustrating the Terms and Definitions of the International Pediatric and Congenital Cardiac Code

Tremendous progress has been made in the field of pediatric heart disease over the past 30 years. Although survival after heart surgery in children has improved dramatically, complications still occur, and optimization of outcomes for all patients remains a challenge. To improve outcomes, collaborative efforts are required and ultimately depend on the possibility of using a common language when discussing pediatric and congenital heart disease. Such a universal language has been developed and named the International Pediatric and Congenital Cardiac Code (IPCCC). To make the IPCCC more universally understood, efforts are under way to link the IPCCC to pictures and videos. The Archiving Working Group is an organization composed of leaders within the international pediatric cardiac medical community and part of the International Society for Nomenclature of Paediatric and Congenital Heart Disease (www.ipccc.net). Its purpose is to illustrate, with representative images of all types and formats, the pertinent aspects of cardiac diseases that affect neonates, infants, children, and adults with congenital heart disease, using the codes and definitions associated with the IPCCC as the organizational backbone. The Archiving Working Group certifies and links images and videos to the appropriate term and definition in the IPCCC. These images and videos are then displayed in an electronic format on the Internet. The purpose of this publication is to report the recent progress made by the Archiving Working Group in establishing an Internet-based, image encyclopedia that is based on the standards of the IPCCC.

Complex atrial arrhythmias as first manifestation of catecholaminergic polymorphic ventricular tachycardia: an unusual course in a patient with a new mutation in ryanodine receptor type 2 gene.

Catecholaminergic polymorphic ventricular tachycardia is a rare life-threatening arrhythmogenic disorder. An association with paroxysmal atrial fibrillation and other atrial arrhythmias has been described, but in all published cases the initial manifestation of the disease was ventricular arrhythmia. This is the first report about a patient who presented with complex atrial tachycardia and sinus node dysfunction about 1 year before the typical ventricular arrhythmias were observed, leading to the diagnosis of catecholaminergic polymorphic ventricular tachycardia. In this girl, a mutation of the ryanodine receptor type 2 gene, which has not been described so far, was discovered.

Mapping and coding of nomenclatures for paediatric and congenital heart disease.

Vera D. Aiello Sao Paulo, Brazil Marie J. Béland* Montreal, Canada (Secretary-Treasurer) Steven D. Colan Boston, USA Rodney C.G. Franklin* London, England J. William Gaynor Philadelphia, USA Jeffrey P. Jacobs* St. Petersburg, USA Otto N. Krogmann Duisburg, Germany Hiromi Kurosawa Tokyo, Japan Bohdan Maruszewski Warsaw, Poland Giovanni Stellin Padova, Italy Christo I. Tchervenkov* Montreal, Canada (Chairman) Paul Weinberg Philadelphia, USA * Executive Committee Members