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Nomenclature for Pediatric and Congenital Cardiac Care: Unification of Clinical and Administrative Nomenclature – The 2021 International Paediatric and Congenital Cardiac Code (IPCCC) and the Eleventh Revision of the International Classification of Diseases (ICD-11)
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- Jeffrey P. Jacobs, Rodney C. G. Franklin, Marie J. Béland, Diane E. Spicer, Steven D. Colan, Henry L. Walters III, Frédérique Bailliard, Lucile Houyel, James D. St. Louis, Leo Lopez, Vera D. Aiello, J. William Gaynor, Otto N. Krogmann, Hiromi Kurosawa, Bohdan J. Maruszewski, Giovanni Stellin, Paul Morris Weinberg, Marshall Lewis Jacobs, Jeffrey R. Boris, Meryl S. Cohen, Allen D. Everett, Jorge M. Giroud, Kristine J. Guleserian, Marina L. Hughes, Amy L. Juraszek, Stephen P. Seslar, Charles W. Shepard, Shubhika Srivastava, Andrew C. Cook, Adrian Crucean, Lazaro E. Hernandez, Rohit S. Loomba, Lindsay S. Rogers, Stephen P. Sanders, Jill J. Savla, Elif Seda Selamet Tierney, Justin T. Tretter, Lianyi Wang, Martin J. Elliott, Constantine Mavroudis, Christo I. Tchervenkov
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- Journal:
- Cardiology in the Young / Volume 31 / Issue 7 / July 2021
- Published online by Cambridge University Press:
- 29 July 2021, pp. 1057-1188
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Substantial progress has been made in the standardization of nomenclature for paediatric and congenital cardiac care. In 1936, Maude Abbott published her Atlas of Congenital Cardiac Disease, which was the first formal attempt to classify congenital heart disease. The International Paediatric and Congenital Cardiac Code (IPCCC) is now utilized worldwide and has most recently become the paediatric and congenital cardiac component of the Eleventh Revision of the International Classification of Diseases (ICD-11). The most recent publication of the IPCCC was in 2017. This manuscript provides an updated 2021 version of the IPCCC.
The International Society for Nomenclature of Paediatric and Congenital Heart Disease (ISNPCHD), in collaboration with the World Health Organization (WHO), developed the paediatric and congenital cardiac nomenclature that is now within the eleventh version of the International Classification of Diseases (ICD-11). This unification of IPCCC and ICD-11 is the IPCCC ICD-11 Nomenclature and is the first time that the clinical nomenclature for paediatric and congenital cardiac care and the administrative nomenclature for paediatric and congenital cardiac care are harmonized. The resultant congenital cardiac component of ICD-11 was increased from 29 congenital cardiac codes in ICD-9 and 73 congenital cardiac codes in ICD-10 to 318 codes submitted by ISNPCHD through 2018 for incorporation into ICD-11. After these 318 terms were incorporated into ICD-11 in 2018, the WHO ICD-11 team added an additional 49 terms, some of which are acceptable legacy terms from ICD-10, while others provide greater granularity than the ISNPCHD thought was originally acceptable. Thus, the total number of paediatric and congenital cardiac terms in ICD-11 is 367. In this manuscript, we describe and review the terminology, hierarchy, and definitions of the IPCCC ICD-11 Nomenclature. This article, therefore, presents a global system of nomenclature for paediatric and congenital cardiac care that unifies clinical and administrative nomenclature.
The members of ISNPCHD realize that the nomenclature published in this manuscript will continue to evolve. The version of the IPCCC that was published in 2017 has evolved and changed, and it is now replaced by this 2021 version. In the future, ISNPCHD will again publish updated versions of IPCCC, as IPCCC continues to evolve.
Assessing implementation fidelity in the First Episode Rapid Early Intervention for Eating Disorders service model
- Katie L. Richards, Michaela Flynn, Amelia Austin, Katie Lang, Karina L. Allen, Ranjeet Bassi, Gabrielle Brady, Amy Brown, Frances Connan, Mary Franklin-Smith, Danielle Glennon, Nina Grant, William Rhys Jones, Kuda Kali, Antonia Koskina, Kate Mahony, Victoria A. Mountford, Nicole Nunes, Monique Schelhase, Lucy Serpell, Ulrike Schmidt
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- BJPsych Open / Volume 7 / Issue 3 / May 2021
- Published online by Cambridge University Press:
- 07 May 2021, e98
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Background
The First Episode Rapid Early Intervention for Eating Disorders (FREED) service model is associated with significant reductions in wait times and improved clinical outcomes for emerging adults with recent-onset eating disorders. An understanding of how FREED is implemented is a necessary precondition to enable an attribution of these findings to key components of the model, namely the wait-time targets and care package.
AimsThis study evaluated fidelity to the FREED service model during the multicentre FREED-Up study.
MethodParticipants were 259 emerging adults (aged 16–25 years) with an eating disorder of <3 years duration, offered treatment through the FREED care pathway. Patient journey records documented patient care from screening to end of treatment. Adherence to wait-time targets (engagement call within 48 h, assessment within 2 weeks, treatment within 4 weeks) and care package, and differences in adherence across diagnosis and treatment group were examined.
ResultsThere were significant increases (16–40%) in adherence to the wait-time targets following the introduction of FREED, irrespective of diagnosis. Receiving FREED under optimal conditions also increased adherence to the targets. Care package use differed by component and diagnosis. The most used care package activities were psychoeducation and dietary change. Attention to transitions was less well used.
ConclusionsThis study provides an indication of adherence levels to key components of the FREED model. These adherence rates can tentatively be considered as clinically meaningful thresholds. Results highlight aspects of the model and its implementation that warrant future examination.
Nomenclature for congenital and paediatric cardiac disease: the International Paediatric and Congenital Cardiac Code (IPCCC) and the Eleventh Iteration of the International Classification of Diseases (ICD-11)*
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- Rodney C. G. Franklin, Marie J. Béland, Steven D. Colan, Henry L. Walters III, Vera D. Aiello, Robert H. Anderson, Frédérique Bailliard, Jeffrey R. Boris, Meryl S. Cohen, J. William Gaynor, Kristine J. Guleserian, Lucile Houyel, Marshall L. Jacobs, Amy L. Juraszek, Otto N. Krogmann, Hiromi Kurosawa, Leo Lopez, Bohdan J. Maruszewski, James D. St. Louis, Stephen P. Seslar, Shubhika Srivastava, Giovanni Stellin, Christo I. Tchervenkov, Paul M. Weinberg, Jeffrey P. Jacobs
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- Cardiology in the Young / Volume 27 / Issue 10 / December 2017
- Published online by Cambridge University Press:
- 29 December 2017, pp. 1872-1938
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An internationally approved and globally used classification scheme for the diagnosis of CHD has long been sought. The International Paediatric and Congenital Cardiac Code (IPCCC), which was produced and has been maintained by the International Society for Nomenclature of Paediatric and Congenital Heart Disease (the International Nomenclature Society), is used widely, but has spawned many “short list” versions that differ in content depending on the user. Thus, efforts to have a uniform identification of patients with CHD using a single up-to-date and coordinated nomenclature system continue to be thwarted, even if a common nomenclature has been used as a basis for composing various “short lists”. In an attempt to solve this problem, the International Nomenclature Society has linked its efforts with those of the World Health Organization to obtain a globally accepted nomenclature tree for CHD within the 11th iteration of the International Classification of Diseases (ICD-11). The International Nomenclature Society has submitted a hierarchical nomenclature tree for CHD to the World Health Organization that is expected to serve increasingly as the “short list” for all communities interested in coding for congenital cardiology. This article reviews the history of the International Classification of Diseases and of the IPCCC, and outlines the process used in developing the ICD-11 congenital cardiac disease diagnostic list and the definitions for each term on the list. An overview of the content of the congenital heart anomaly section of the Foundation Component of ICD-11, published herein in its entirety, is also included. Future plans for the International Nomenclature Society include linking again with the World Health Organization to tackle procedural nomenclature as it relates to cardiac malformations. By doing so, the Society will continue its role in standardising nomenclature for CHD across the globe, thereby promoting research and better outcomes for fetuses, children, and adults with congenital heart anomalies.
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- By Mohamed Aboulghar, Ahmed Abou-Setta, Mary E. Abusief, G. David Adamson, R. J. Aitken, Hesham Al-Inany, Baris Ata, Hamdy Azab, Adam Balen, David H. Barad, Pedro N. Barri, C. Blockeel, Giuseppe Botta, Mark Bowman, Chris Brewer, Dominique M. Butawan, Sandra A. Carson, Hai Ying Chen, Anne Clark, Buenaventura Coroleu, S. Das, C. Dechanet, H. Déchaud, Cora de Klerk, Sheryl de Lacey, S. Deutsch-Bringer, P. Devroey, Didier Dewailly, Hakan E. Duran, Walid El Sherbiny, Tarek El-Toukhy, Johannes L. H. Evers, Cynthia Farquhar, Rodney D. Franklin, Juan A. Garcia-Velasco, David K. Gardner, Norbert Gleicher, Gedis Grudzinskas, Roger Hart, B Hédon, Colin M. Howles, Jack Yu Jen Huang, N. P. Johnson, Hey-Joo Kang, Gab Kovacs, Ben Kroon, Anver Kuliev, William H. Kutteh, Nick Macklon, Ragaa Mansour, Lamiya Mohiyiddeen, Lisa J. Moran, David Mortimer, Sharon T. Mortimer, Luciano G. Nardo, Robert J. Norman, Willem Ombelet, Luk Rombauts, Zev Rosenwaks, Francisco J. Ruiz Flores, Anthony J. Rutherford, Gavin Sacks, Denny Sakkas, M. W. Seif, Ayse Seyhan, Caroline Smith, Kate Stern, Elizabeth A. Sullivan, Sesh Kamal Sunkara, Seang Lin Tan, Mohamed Taranissi, Kelton P. Tremellen, Wendy S. Vitek, V. Vloeberghs, Bradley J. Van Voorhis, S. F. van Voorst, Amr Wahba, Yueping A. Wang, Klaus E. Wiemer
- Edited by Gab Kovacs, Monash University, Victoria
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- How to Improve your ART Success Rates
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- 05 July 2011
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- 30 June 2011, pp viii-xii
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- By Rose Teteki Abbey, K. C. Abraham, David Tuesday Adamo, LeRoy H. Aden, Efrain Agosto, Victor Aguilan, Gillian T. W. Ahlgren, Charanjit Kaur AjitSingh, Dorothy B E A Akoto, Giuseppe Alberigo, Daniel E. Albrecht, Ruth Albrecht, Daniel O. Aleshire, Urs Altermatt, Anand Amaladass, Michael Amaladoss, James N. Amanze, Lesley G. Anderson, Thomas C. Anderson, Victor Anderson, Hope S. Antone, María Pilar Aquino, Paula Arai, Victorio Araya Guillén, S. Wesley Ariarajah, Ellen T. Armour, Brett Gregory Armstrong, Atsuhiro Asano, Naim Stifan Ateek, Mahmoud Ayoub, John Alembillah Azumah, Mercedes L. García Bachmann, Irena Backus, J. Wayne Baker, Mieke Bal, Lewis V. Baldwin, William Barbieri, António Barbosa da Silva, David Basinger, Bolaji Olukemi Bateye, Oswald Bayer, Daniel H. Bays, Rosalie Beck, Nancy Elizabeth Bedford, Guy-Thomas Bedouelle, Chorbishop Seely Beggiani, Wolfgang Behringer, Christopher M. Bellitto, Byard Bennett, Harold V. Bennett, Teresa Berger, Miguel A. Bernad, Henley Bernard, Alan E. Bernstein, Jon L. Berquist, Johannes Beutler, Ana María Bidegain, Matthew P. Binkewicz, Jennifer Bird, Joseph Blenkinsopp, Dmytro Bondarenko, Paulo Bonfatti, Riet en Pim Bons-Storm, Jessica A. Boon, Marcus J. Borg, Mark Bosco, Peter C. Bouteneff, François Bovon, William D. Bowman, Paul S. Boyer, David Brakke, Richard E. Brantley, Marcus Braybrooke, Ian Breward, Ênio José da Costa Brito, Jewel Spears Brooker, Johannes Brosseder, Nicholas Canfield Read Brown, Robert F. Brown, Pamela K. Brubaker, Walter Brueggemann, Bishop Colin O. Buchanan, Stanley M. Burgess, Amy Nelson Burnett, J. Patout Burns, David B. Burrell, David Buttrick, James P. Byrd, Lavinia Byrne, Gerado Caetano, Marcos Caldas, Alkiviadis Calivas, William J. Callahan, Salvatore Calomino, Euan K. Cameron, William S. Campbell, Marcelo Ayres Camurça, Daniel F. Caner, Paul E. Capetz, Carlos F. Cardoza-Orlandi, Patrick W. Carey, Barbara Carvill, Hal Cauthron, Subhadra Mitra Channa, Mark D. 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Hackett, Getatchew Haile, Douglas John Hall, Nicholas Hammond, Daphne Hampson, Jehu J. Hanciles, Barry Hankins, Jennifer Haraguchi, Stanley S. Harakas, Anthony John Harding, Conrad L. Harkins, J. William Harmless, Marjory Harper, Amir Harrak, Joel F. Harrington, Mark W. Harris, Susan Ashbrook Harvey, Van A. Harvey, R. Chris Hassel, Jione Havea, Daniel Hawk, Diana L. Hayes, Leslie Hayes, Priscilla Hayner, S. Mark Heim, Simo Heininen, Richard P. Heitzenrater, Eila Helander, David Hempton, Scott H. Hendrix, Jan-Olav Henriksen, Gina Hens-Piazza, Carter Heyward, Nicholas J. Higham, David Hilliard, Norman A. Hjelm, Peter C. Hodgson, Arthur Holder, M. Jan Holton, Dwight N. Hopkins, Ronnie Po-chia Hsia, Po-Ho Huang, James Hudnut-Beumler, Jennifer S. Hughes, Leonard M. Hummel, Mary E. Hunt, Laennec Hurbon, Mark Hutchinson, Susan E. Hylen, Mary Beth Ingham, H. Larry Ingle, Dale T. Irvin, Jon Isaak, Paul John Isaak, Ada María Isasi-Díaz, Hans Raun Iversen, Margaret C. 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Yee, Viktor Yelensky, Yeo Khiok-Khng, Gustav K. K. Yeung, Angela Yiu, Amos Yong, Yong Ting Jin, You Bin, Youhanna Nessim Youssef, Eliana Yunes, Robert Michael Zaller, Valarie H. Ziegler, Barbara Brown Zikmund, Joyce Ann Zimmerman, Aurora Zlotnik, Zhuo Xinping
- Edited by Daniel Patte, Vanderbilt University, Tennessee
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- The Cambridge Dictionary of Christianity
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- 05 August 2012
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- 20 September 2010, pp xi-xliv
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Nomenclature for congenital and paediatric cardiac disease: Historical perspectives and The International Pediatric and Congenital Cardiac Code
- Part of
- Rodney C.G. Franklin, Jeffrey Phillip Jacobs, Otto N. Krogmann, Marie J. Béland, Vera D. Aiello, Steven D. Colan, Martin J. Elliott, J. William Gaynor, Hiromi Kurosawa, Bohdan Maruszewski, Giovanni Stellin, Christo I. Tchervenkov, Henry L. Walters III, Paul Weinberg, Robert H. Anderson
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- Cardiology in the Young / Volume 18 / Issue S2 / December 2008
- Published online by Cambridge University Press:
- 01 December 2008, pp. 70-80
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Clinicians working in the field of congenital and paediatric cardiology have long felt the need for a common diagnostic and therapeutic nomenclature and coding system with which to classify patients of all ages with congenital and acquired cardiac disease. A cohesive and comprehensive system of nomenclature, suitable for setting a global standard for multicentric analysis of outcomes and stratification of risk, has only recently emerged, namely, The International Paediatric and Congenital Cardiac Code. This review, will give an historical perspective on the development of systems of nomenclature in general, and specifically with respect to the diagnosis and treatment of patients with paediatric and congenital cardiac disease. Finally, current and future efforts to merge such systems into the paperless environment of the electronic health or patient record on a global scale are briefly explored.
On October 6, 2000, The International Nomenclature Committee for Pediatric and Congenital Heart Disease was established. In January, 2005, the International Nomenclature Committee was constituted in Canada as The International Society for Nomenclature of Paediatric and Congenital Heart Disease. This International Society now has three working groups. The Nomenclature Working Group developed The International Paediatric and Congenital Cardiac Code and will continue to maintain, expand, update, and preserve this International Code. It will also provide ready access to the International Code for the global paediatric and congenital cardiology and cardiac surgery communities, related disciplines, the healthcare industry, and governmental agencies, both electronically and in published form. The Definitions Working Group will write definitions for the terms in the International Paediatric and Congenital Cardiac Code, building on the previously published definitions from the Nomenclature Working Group. The Archiving Working Group, also known as The Congenital Heart Archiving Research Team, will link images and videos to the International Paediatric and Congenital Cardiac Code. The images and videos will be acquired from cardiac morphologic specimens and imaging modalities such as echocardiography, angiography, computerized axial tomography and magnetic resonance imaging, as well as intraoperative images and videos.
Efforts are ongoing to expand the usage of The International Paediatric and Congenital Cardiac Code to other areas of global healthcare. Collaborative efforts are underway involving the leadership of The International Nomenclature Committee for Pediatric and Congenital Heart Disease and the representatives of the steering group responsible for the creation of the 11th revision of the International Classification of Diseases, administered by the World Health Organisation. Similar collaborative efforts are underway involving the leadership of The International Nomenclature Committee for Pediatric and Congenital Heart Disease and the International Health Terminology Standards Development Organisation, who are the owners of the Systematized Nomenclature of Medicine or “SNOMED”.
The International Paediatric and Congenital Cardiac Code was created by specialists in the field to name and classify paediatric and congenital cardiac disease and its treatment. It is a comprehensive code that can be freely downloaded from the internet (http://www.IPCCC.net) and is already in use worldwide, particularly for international comparisons of outcomes. The goal of this effort is to create strategies for stratification of risk and to improve healthcare for the individual patient. The collaboration with the World Heath Organization, the International Health Terminology Standards Development Organisation, and the healthcare industry, will lead to further enhancement of the International Code, and to its more universal use.
Nomenclature and databases for the surgical treatment of congenital cardiac disease – an updated primer and an analysis of opportunities for improvement
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- Jeffrey Phillip Jacobs, Marshall Lewis Jacobs, Constantine Mavroudis, Carl Lewis Backer, Francois G. Lacour-Gayet, Christo I. Tchervenkov, Rodney C. G. Franklin, Marie J. Béland, Kathy J. Jenkins, Hal Walters III, Emile A. Bacha, Bohdan Maruszewski, Hiromi Kurosawa, David Robinson Clarke, J. William Gaynor, Thomas L. Spray, Giovanni Stellin, Tjark Ebels, Otto N. Krogmann, Vera D. Aiello, Steven D. Colan, Paul Weinberg, Jorge M. Giroud, Allen Everett, Gil Wernovsky, Martin J. Elliott, Fred H. Edwards
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- Cardiology in the Young / Volume 18 / Issue S2 / December 2008
- Published online by Cambridge University Press:
- 01 December 2008, pp. 38-62
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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.
Databases for assessing the outcomes of the treatment of patients with congenital and paediatric cardiac disease – the perspective of cardiac surgery
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- Marshall Lewis Jacobs, Jeffrey Phillip Jacobs, Rodney C.G. Franklin, Constantine Mavroudis, Francois Lacour-Gayet, Christo I. Tchervenkov, Hal Walters III, Emile A. Bacha, David Robinson Clarke, J. William Gaynor, Thomas L. Spray, Giovanni Stellin, Tjark Ebels, Bohdan Maruszewski, Zdzislaw Tobota, Hiromi Kurosawa, Martin Elliott
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- Cardiology in the Young / Volume 18 / Issue S2 / December 2008
- Published online by Cambridge University Press:
- 01 December 2008, pp. 101-115
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This review includes a brief discussion, from the perspective of cardiac surgeons, of the rationale for creation and maintenance of multi-institutional databases of outcomes of congenital heart surgery, together with a history of the evolution of such databases, a description of the current state of the art, and a discussion of areas for improvement and future expansion of the concept. Five fundamental areas are reviewed: nomenclature, mechanism of data collection and storage, mechanisms for the evaluation and comparison of the complexity of operations and stratification of risk, mechanisms to ensure the completeness and accuracy of the data, and mechanisms for expansion of the current capabilities of databases to include comparison and sharing of data between medical subspecialties. This review briefly describes several European and North American initiatives related to databases for pediatric and congenital cardiac surgery the Congenital Database of The European Association for Cardio-Thoracic Surgery, the Congenital Database of The Society of Thoracic Surgeons, the Pediatric Cardiac Care Consortium, and the Central Cardiac Audit Database in the United Kingdom. Potential means of approaching the ultimate goal of acquisition of long-term follow-up data, and input of this data over the life of the patient, are also considered.
The nomenclature, definition and classification of cardiac structures in the setting of heterotaxy
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- Jeffrey P. Jacobs, Robert H. Anderson, Paul M. Weinberg, Henry L. Walters III, Christo I. Tchervenkov, Danny Del Duca, Rodney C. G. Franklin, Vera D. Aiello, Marie J. Béland, Steven D. Colan, J. William Gaynor, Otto N. Krogmann, Hiromi Kurosawa, Bohdan Maruszewski, Giovanni Stellin, Martin J. Elliott
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- Cardiology in the Young / Volume 17 / Issue S4 / September 2007
- Published online by Cambridge University Press:
- 26 November 2007, pp. 1-28
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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.
List of Contributors
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- By Ralph Adolphs, Bernard J. Baars, John A. Bargh, Jesse M. Bering, David F. Bjorklund, Joseph E. Bogen, Rebekah Bradley, Wallace Chafe, Michael C. Corballis, Diego Cosmelli, Jean-Marie Danion, Richard J. Davidson, Steven W. Day, Georges Dreyfus, John D. Dunne, Stan Franklin, Helena Hong Gao, Lisa Geraci, Deborah E. Hannula, J. Allan Hobson, Caroline Huron, John F. Kihlstrom, Asher Koriat, Uriah Kriegel, Jean-Philippe Lachaux, Charles D. Laughlin, Antoine Lutz, Drew McDermott, Katharine McGovern, Keith Oately, Suparna Rajaram, Henry L. Roediger III, Edmund T. Rolls, Daniel L. Schachter, William Seager, Daniel J. Simons, Scott D. Slotnick, Henry Stapp, Petra Stoerig, Ron Sun, Evan Thompson, C. Jason Throop, Rebecca Todd, Carlo Umiltà, David E. Warren, Joel Weinberger, Drew Westen, Dan Zahavi, Philip David Zelazo
- Edited by Philip David Zelazo, University of Toronto, Morris Moscovitch, University of Toronto, Evan Thompson, University of York
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- The Cambridge Handbook of Consciousness
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- 05 June 2012
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- 14 May 2007, pp -
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The nomenclature, definition and classification of discordant atrioventricular connections
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- Jeffrey P. Jacobs, Rodney C.G. Franklin, James L. Wilkinson, Andrew D. Cochrane, Tom R. Karl, Vera D. Aiello, Marie J. Béland, Steven D. Colan, Martin J. Elliott, J. William Gaynor, Otto N. Krogmann, Hiromi Kurosawa, Bohdan Maruszewski, Giovanni Stellin, Christo I. Tchervenkov, Paul M. Weinberg
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- Journal:
- Cardiology in the Young / Volume 16 / Issue S3 / September 2006
- Published online by Cambridge University Press:
- 13 October 2006, pp. 72-84
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Congenitally corrected transposition is a complex cardiac lesion that is often associated with ventricular septal defect, obstruction of the outflow tract of the morphologically left ventricle, and abnormalities of the morphologically tricuspid valve.1,2 Nomenclature for this lesion has been variable and confusing.1 In this review, we define, and hopefully clarify this terminology. The lesion is a combination of discordant union of the atrial chambers with the ventricles, and the ventricles with the arterial trunks.1,2 In rare circumstances, discordant atrioventricular connections can be associated with concordant ventriculo-arterial connections. This malformation has been called “isolated ventricular inversion”. The term is less than precise, and the descriptive approach using the phrase “discordant atrioventricular connections with concordant ventriculo-arterial connections” is preferred, as discussed below.
Classification of the functionally univentricular heart: unity from mapped codes
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- Jeffrey P. Jacobs, Rodney C. G. Franklin, Marshall L. Jacobs, Steven D. Colan, Christo I. Tchervenkov, Bohdan Maruszewski, J. William Gaynor, Thomas L. Spray, Giovanni Stellin, Vera D. Aiello, Marie J. Béland, Otto N. Krogmann, Hiromi Kurosawa, Paul M. Weinberg, Martin J. Elliott, Constantine Mavroudis, Robert H. Anderson
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- Journal:
- Cardiology in the Young / Volume 16 / Issue S1 / February 2006
- Published online by Cambridge University Press:
- 10 January 2006, pp. 9-21
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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 current status and future directions of efforts to create a global database for the outcomes of therapy for congenital heart disease
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- Jeffrey P. Jacobs, Bohdan Maruszewski, Christo I. Tchervenkov, Francois G. Lacour-Gayet, Marshall L. Jacobs, David R. Clarke, J. William Gaynor, Thomas L. Spray, Giovanni Stellin, Martin J. Elliott, Tjark Ebels, Rodney C. G. Franklin, Marie J. Béland, Hiromi Kurosawa, Vera D. Aiello, Steven D. Colan, Otto N. Krogmann, Paul Weinberg, Zdzislaw Tobota, Rachel S. Dokholyan, Eric D. Peterson, Constantine Mavroudis
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- Cardiology in the Young / Volume 15 / Issue S1 / February 2005
- Published online by Cambridge University Press:
- 08 September 2005, pp. 190-197
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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:
Predation of guanacos (Lama guanicoe) by southernmost mountain lions (Puma concolor) during a historically severe winter in Torres del Paine National Park, Chile
- Michael S. Bank, Ronald J. Sarno, Nichole K. Campbell, William L. Franklin
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- Journal:
- Journal of Zoology / Volume 258 / Issue 2 / October 2002
- Published online by Cambridge University Press:
- 30 September 2002, pp. 215-222
- Print publication:
- October 2002
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The effects of mountain lion Puma concolor predation on guanaco Lama guanicoe mortality was investigated during the historically severe winter of 1995 in Torres del Paine National Park, Chile. The 45 guanaco carcasses located represented 3% of the entire guanaco population of which 74% were mountain lion kills, 13% died from malnutrition, 2% died from fence entanglement and 11% died from unknown causes. Depleted bone marrow fat was observed in 20% of all carcasses. The number of guanaco deaths differed among sex and age classes, particularly in juveniles (< 1 year of age), which died more often than expected (P < 0.001). Fresh guanaco carcasses (n = 19) killed by mountain lions were located in tree and shrub habitats 79% of the time and were significantly greater than expected values based on the relative availability of these habitats (P < 0.001). Observations of the spatial distribution of mountain lion kills and guanaco mixed-sex groups were similar (P = 0.10), suggesting that mountain lions responded to winter migratory movements made by guanacos. Overall, adult guanacos experienced surprisingly lower levels of mortality (adult male = 1%, adult female = 2%) than expected, despite the severe winter conditions, while juveniles experienced higher levels of mortality (13%) than expected. In comparison to other years (1991–96), the severe winter of 1995 (i.e. this investigation) had the greatest total mortality for juveniles, although the proportion of deaths caused by mountain lion predation for juveniles was greatest during other winters.
Patterns of mtDNA and microsatellite variation in an island and mainland population of guanacos in southern Chile
- Ronald J. Sarno, William L. Franklin, Stephen J. O'Brien, Warren E. Johnson
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- Animal Conservation forum / Volume 4 / Issue 2 / May 2001
- Published online by Cambridge University Press:
- 18 May 2001, pp. 93-101
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- May 2001
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The archaeological record indicates that guanacos inhabited the Patagonia of Chile and Argentina about 13,600 years ago, but were unable to migrate further south owing to the presence of glacial and water barriers that covered much of southern South America including the island of Tierra del Fuego. As environmental and ecological conditions improved, guanacos, along with other large mammals including horses, colonized the area. As a result of continued world-wide glacial melting, ocean levels rose and Tierra del Fuego became isolated from the mainland approximately 8000 years ago. Although island populations generally exhibit lower levels of genetic variation than their counterpart mainland populations, it is difficult to predict how much less variation island populations will exhibit. An analysis of mitochondrial cytochrome b and ATPase-8 sequences and 15 nuclear microsatellite loci revealed that both populations retained appreciable genetic diversity. The island population, however, exhibited much less variation than the mainland population. Measures of genetic variation revealed modest, but significant genetic differentiation, consistent with separation of the two populations approximately 8000 years ago. The assessment of levels of genetic diversity and population differentiation among populations of the wild South American camelids is becoming increasingly important as interest mounts in their utilization as a renewable resource.
Guanacos in Peru
- William L. Franklin
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The guanaco, a wild South American camel, like the vicuña, is not on the IUCN endangered list, but numbers have decreased steadily, and it is on Peru's list of endangered species. The author, who describes a brief study of the largest population in Peru, suggests that protective measures are needed.
An analysis of the performance of an ultra-high-temperature milk sterilizing plant: IV. Comparison of experimental and calculated sporicidal effects for a strain of Bacillus stearothermophilus
- H. Burton, J. G. Franklin, D. J. Williams, Helen R. Chapman, A. Jean, W. Harrison, L. F. L. Clegg
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- Journal:
- Journal of Dairy Research / Volume 26 / Issue 3 / October 1959
- Published online by Cambridge University Press:
- 01 June 2009, pp. 221-226
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- October 1959
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This series of four papers has outlined a method for predicting the bactericidal efficiency of an ultra-high-temperature sterilizing plant. The method has been illustrated with reference to the performance of an A.P.V. 200 gal./hr. plate-type sterilizing plant, treating at different temperatures milk heavily inoculated with suspensions of B. subtilis and B. stearothermophilus spores.
The method involves the determination of the temperature and flow time distributions in the plant, and of the thermal death characteristics of the organism to be considered. The investigations of plant performance are considered in Part I. The determination of the sporicidal effect of the plant from this information is described in Part II. The effect is expressed in terms of the slope of the thermal death line for the organism considered, at the operation temperature of the plant. By expressing the result in this form it becomes general and applicable to any type of organism, at any operating temperature close to that for which the plant is designed.
The thermal death time of any organism at the operating temperature may be obtained by laboratory experiment, by extrapolation if necessary. In Parts III and IV, laboratory data for B. subtilis spores and B. Stearothermophilus spores are used to calculate the performance of the plant, and the calculated results are compared with the results of direct plant experiment. The agreement is satisfactory.
The interpretation of the results with spores of B. stearothermophilus is complicated by their very marked inhibition by u.h.t.-treated milk. Only about one spore per million germinates and grows in such milk. A false impression of the number of truly surviving organisms after the heat treatment may therefore be obtained. It is not known to what extent this effect occurs with spores of other strains of B. stearothermophilus or with other organisms.
It is not suggested that the theoretical method of estimation of performance will give reliable information on the spoilage level to be expected from a plant under practical dairy conditions. The uncertainties as to the number and types of incident organisms are too great. Comparisons can be made between different plants, however, by comparing the results of the theoretical analyses for the plants. No bacteriological data are then required. This may be a less difficult procedure for manufacturers than a direct bacteriological experiment using heavily inoculated milk. The analysis also enables the contribution to the overall lethal effect of the different part of a sterilizing plant to be assessed.
722. An analysis of the performance of an ultra-high-temperature milk sterilizing plant: III. Comparison of experimental and calculated sporicidal effects for a strain of Bacillus subtilis
- H. Burton, J. G. Franklin, D. J. Williams, Helen R. Chapman, A. Jean W. Harrison, L. F. L. Clegg
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- Journal of Dairy Research / Volume 25 / Issue 2 / June 1958
- Published online by Cambridge University Press:
- 01 June 2009, pp. 338-343
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- June 1958
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The previous papers of this series (1,2) have shown how the bactericidal efficiency of a flow-type heat exchanger may be predicted from physical measurements, and have given the necessary calculations for a small plate-type ultra-high-temperature sterilizing plant made by the A.P.V. Co. The purpose of this paper is to show the degree of agreement between practice and prediction from theory.