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Journal of the American College of Cardiology Journal of the American College of Cardiology Copyright © 2013 American College of Cardiology. All rights reserved Volume 62, Issue 25, Supplement, Pages A1-A8, D1-D128 (24 December 2013) Updates in Pulmonary Hypertension Edited by Gerald Simonneau and Nazzareno Galiè INTRODUCTION 1 The Fifth World Symposium on Pulmonary Hypertension Review Article Pages D1-D3 Nazzareno Galiè, Gerald Simonneau Show preview | PDF (98 K) | Recommended articles | Related reference work articles STATE-OF-THE-ART PAPERS 2 Relevant Issues in the Pathology and Pathobiology of Pulmonary Hypertension Review Article Pages D4-D12 Rubin M. Tuder, Stephen L. Archer, Peter Dorfmüller, Serpil C. Erzurum, Christophe Guignabert, Evangelos Michelakis, Marlene Rabinovitch, Ralph Schermuly, Kurt R. Stenmark, Nicholas W. Morrell Show preview | PDF (601 K) | Recommended articles | Related reference work articles 3 Genetics and Genomics of Pulmonary Arterial Hypertension Review Article Pages D13-D21 Florent Soubrier, Wendy K. Chung, Rajiv Machado, Ekkehard Grünig, Micheala Aldred, Mark Geraci, James E. Loyd, C. Gregory Elliott, Richard C. Trembath, John H. Newman, Marc Humbert Show preview | PDF (267 K) | Recommended articles | Related reference work articles 4 Right Heart Adaptation to Pulmonary Arterial Hypertension: Physiology and Pathobiology Review Article Pages D22-D33 Anton Vonk-Noordegraaf, François Haddad, Kelly M. Chin, Paul R. Forfia, Steven M. Kawut, Joost Lumens, Robert Naeije, John Newman, Ronald J. Oudiz, Steve Provencher, Adam Torbicki, Norbert F. Voelkel, Paul M. Hassoun Show preview | PDF (953 K) | Recommended articles | Related reference work articles 5 Updated Clinical Classification of Pulmonary Hypertension Review Article Pages D34-D41 Gerald Simonneau, Michael A. Gatzoulis, Ian Adatia, David Celermajer, Chris Denton, Ardeschir Ghofrani, Miguel Angel Gomez Sanchez, R. Krishna Kumar, Michael Landzberg, Roberto F. Machado, Horst Olschewski, Ivan M. Robbins, Rogiero Souza Show preview | PDF (221 K) | Recommended articles | Related reference work articles 6 Definitions and Diagnosis of Pulmonary Hypertension Review Article Pages D42-D50 Marius M. Hoeper, Harm Jan Bogaard, Robin Condliffe, Robert Frantz, Dinesh Khanna, Marcin Kurzyna, David Langleben, Alessandra Manes, Toru Satoh, Fernando Torres, Martin R. Wilkins, David B. Badesch Show preview | PDF (688 K) | Recommended articles | Related reference work articles 7 Pulmonary Arterial Hypertension: Epidemiology and Registries Review Article Pages D51-D59 Michael D. McGoon, Raymond L. Benza, Pilar Escribano-Subias, Xin Jiang, Dave P. Miller, Andrew J. Peacock, Joanna Pepke-Zaba, Tomas Pulido, Stuart Rich, Stephan Rosenkranz, Samy Suissa, Marc Humbert Show preview | PDF (228 K) | Recommended articles | Related reference work articles 8 Updated Treatment Algorithm of Pulmonary Arterial Hypertension Review Article Pages D60-D72 Nazzareno Galiè, Paul A. Corris, Adaani Frost, Reda E. Girgis, John Granton, Zhi Cheng Jing, Walter Klepetko, Michael D. McGoon, Vallerie V. McLaughlin, Ioana R. Preston, Lewis J. Rubin, Julio Sandoval, Werner Seeger, Anne Keogh Show preview | PDF (1226 K) | Recommended articles | Related reference work articles 9 Treatment Goals of Pulmonary Hypertension Review Article Pages D73-D81 Vallerie V. McLaughlin, Sean Patrick Gaine, Luke S. Howard, Hanno H. Leuchte, Michael A. Mathier, Sanjay Mehta, Massimillano Palazzini, Myung H. Park, Victor F. Tapson, Olivier Sitbon Show preview | PDF (228 K) | Recommended articles | Related reference work articles 10 New Trial Designs and Potential Therapies for Pulmonary Artery Hypertension Review Article Pages D82-D91 Mardi Gomberg-Maitland, Todd M. Bull, Rajeev Saggar, Robyn J. Barst, Amany Elgazayerly, Thomas R. Fleming, Friedrich Grimminger, Maurizio Rainisio, Duncan J. Stewart, Norman Stockbridge, Carlo Ventura, Ardeschir H. Ghofrani, Lewis J. Rubin Show preview | PDF (280 K) | Recommended articles | Related reference work articles 11 Chronic Thromboembolic Pulmonary Hypertension Review Article Pages D92-D99 Nick H. Kim, Marion Delcroix, David P. Jenkins, Richard Channick, Philippe Dartevelle, Pavel Jansa, Irene Lang, Michael M. Madani, Hitoshi Ogino, Vittorio Pengo, Eckhard Mayer Show preview | PDF (485 K) | Recommended articles | Related reference work articles 12 Pulmonary Hypertension Due to Left Heart Diseases Review Article Pages D100-D108 Jean-Luc Vachiéry, Yochai Adir, Joan Albert Barberà, Hunter Champion, John Gerard Coghlan, Vincent Cottin, Teresa De Marco, Nazzareno Galiè, Stefano Ghio, J. Simon R. Gibbs, Fernando Martinez, Marc Semigran, Gerald Simonneau, Athol Wells, Werner Seeger Show preview | PDF (455 K) | Recommended articles | Related reference work articles 13 Pulmonary Hypertension in Chronic Lung Diseases Review Article Pages D109-D116 Werner Seeger, Yochai Adir, Joan Albert Barberà, Hunter Champion, John Gerard Coghlan, Vincent Cottin, Teresa De Marco, Nazzareno Galiè, Stefano Ghio, Simon Gibbs, Fernando J. Martinez, Marc J. Semigran, Gerald Simonneau, Athol U. Wells, Jean-Luc Vachiéry Show preview | PDF (242 K) | Recommended articles | Related reference work articles 14 Pediatric Pulmonary Hypertension Review Article Pages D117-D126 D. Dunbar Ivy, Steven H. Abman, Robyn J. Barst, Rolf M.F. Berger, Damien Bonnet, Thomas R. Fleming, Sheila G. Haworth, J. Usha Raj, Erika B. Rosenzweig, Ingram Schulze Neick, Robin H. Steinhorn, Maurice Beghetti Show preview | PDF (952 K) | Recommended articles | Related reference work articles Journal of the American College of Cardiology Vol. 62, No. 25, Suppl D, 2013 Ó 2013 by the American College of Cardiology Foundation ISSN 0735-1097/$36.00 Published by Elsevier Inc. http://dx.doi.org/10.1016/j.jacc.2013.10.030 INTRODUCTION The Fifth World Symposium on Pulmonary Hypertension Nazzareno Galiè, MD,* Gerald Simonneau, MDy Bologna, Italy; and Paris, France Sometimes pivotal events can be identified in the history of The well-known National Institutes of Health Registry on a disease that mark the borders of different eras in the primary pulmonary hypertension was launched after this first diagnosis or treatment. One of these pivotal events in the symposium (4). history of pulmonary arterial hypertension (PAH) was the The second WSPH was held 25 years later in 1998 in publication in 1996 of the paper of Barst et al. (1) on Evian, France. There were multiple reasons for starting the continuous intravenous epoprostenol therapy for primary modern series of WSPH, including the availability of 2 very pulmonary hypertension. For the first time, a medical therapy effective treatments such as epoprostenol (1) and high doses was shown to reduce substantially the mortality of a disease of calcium channel blockers in patients responding to acute defined as the “kingdom of the near dead” (2). In her out- vasoreactivity tests (5). A more comprehensive clinical standing career, Dr. Barst made considerable contributions to classification, including 5 groups, was proposed for the first the additional pivotal events in the PAH history, which can time at the symposium, and this facilitated both clinical be identified in the World Symposia on Pulmonary Hyper- practice and the clinical research. tension (WSPH) that has been held since 1973. The influ- In fact, in the third WSPH, which was held in Venice, ence of Dr. Barst spanned every aspect of the disease from Italy, in 2003, there were already 3 classes of drugs effective pediatrics to adulthood and from genetics to randomized, in the treatment of PAH (prostanoids, endothelin receptor controlled trials (RCTs). Her interventions in the discussions antagonists, and phosphodiesterase type 5 inhibitors), and were unmistakable and full of energy and passion, and this a specific treatment algorithm was proposed (6). The clinical passion also characterized her last days when the energy was classification was refined, including the familial form char- fading. We miss her, and we would like to remember her with acterized by the BMPR2 gene mutations (7). the last gift she has left us: the example of a physician and The fourth WSPH was held in Dana Point, California, in a scientist fully engaged in her activity until the end of her life. 2008. New management strategies were introduced such as Even if she was not able to personally attend the meeting, we the treatment of mildly symptomatic patients and the dedicate the 5th WSPH to her memory. combination and goal-oriented treatment strategies (8). The The achievements of the different WSPH have marked the RCT design was also discussed, including the need to use progress made on this condition. The first WSPH was held in time to clinical worsening as primary endpoint in phase III Geneva, Switzerland, in 1973 and was organized by the registration studies (9). World Health Organization because of an epidemic of PAH The fifth WSPH was held in Nice, France, from February 27 cases due to the use of aminorex, an anorexigen drug (3).A to March 1, 2013; the findings and recommendations of 129 simple clinical classification was proposed at this meeting worldwide experts divided into 12 task forces operating for (primary, secondary, and associated pulmonary hypertension 12 months were discussed in front of an audience of w1,000 [PH]) together with the hemodynamic definition of the physicians and representatives of the industry, patient asso- disease as a mean pulmonary arterial pressure 25 mm Hg. ciations, and regulatory agencies. The main conclusions of the task forces and the meeting discussions are reported in the 13 articles published
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