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Summer 2003, Vol. 2, No. 2

Editor’s Memo   PAH-Associated Congenital Heart Disease: Finding Common Ground With Primary Pulmonary Hypertension As we planned this issue on pulmonary arterial hypertension (PAH) in congenital heart disease and in children, we sensed the long shadows of physicians who have helped establish the parame-ters of its diagnosis. There were giants like Viktor Eisenmenger, who described the clinical features of a patient with PAH more than 100 years ago, and Paul Wood, who subsequently used the term “Eisenmenger syndrome” to describe PAH with reversal of a systemic to pulmonary shunt. As the nomenclature has evolved so has our understanding of the conditions seen in association with pulmonary hypertension. A reclassification by the World Health Organization in 1998 brought us further along, as it emphasized similarities between primary pulmonary hypertension and PAH of certain known etiologies. An improved understanding of the mechanisms underlying the vas-cular changes in PAH has been an important factor in the emergence of new therapies. Notably, some medical therapies for the treatment of primary pulmonary hypertension may benefit patients with PAH asso-ciated with congenital heart disease. It is intriguing to find the diversi-ty in the etiology of PAH and yet some uniformity in treatment approaches. Our Roundtable Discussion so capably managed by Robyn Barst, MD, explored all of these issues. We have all recognized how difficult it may be to differentiate whether a patient with a congenital heart defect has Eisenmenger syndrome as opposed to primary pul-monary hypertension. The experts in pediatric cardiology we brought together for the Roundtable, as well as the authors of our articles in this issue, addressed this and many other difficult questions and pres-ent a comprehensive discussion of vital topics essential to our under-standing of PAH in congenital heart disease. Hopefully, the insights presented will give us a clearer perspective on how to classify and treat patients who have these diseases. Vic Tapson, MD Editor-in-Chief   Take it With You   Download this issue in PDF   Issue Sponsor Ads   Actelion(PDF) Accredo Therapeutics(PDF)	Articles   Advances in Pulmonary Hypertension, Summer 2003, Vol. 2, No. 2 cover close-up Click titles to read stories... Profiles in Pulmonary Hypertension: Remembering Paul Wood: Relentlessly Driven, Dazzling Diagnostician Forty years after he died at the tragically early age of 54, the reputation of Dr Paul Wood as a showman and con-summate physician, so dazzling in his ability to diagnose that he is recognized as one of the greatest diagnosticians ever, still serves as a reminder of what he means to contemporary cardiology... Pulmonary Hypertension in Children: New Insights Offer Opportunity to Reverse the Disease Process Severe, sustained pulmonary hypertension is potentially fatal. It is, however, time to adopt a more positive and aggressive approach to the management of pulmonary hypertension in children. Recent advances in genetics and cell biology provide insights into the pathogenesis of this disease. New therapies offer an improved quality of life and increased survival... Eisenmenger Syndrome in Adults: Strategies to Correct Congenital Defects Before Fixed Vascular Disease Develops Congenital heart defects (CHDs) associated with large systemic-to- pulmonary shunts, (eg, atrial septal defect, ventricular sep-tal defect, patent ductus arteriosus) can lead to pulmonary vas-cular disease, which is characterized by bidirectional shunting or reversal of the shunt. This phenomenon, which is associated with progressive cyanosis, polycythemia, and ultimately, func-tional limitation, was first described in 1897 by Viktor Eisenmenger and later termed the Eisenmenger syndrome (ES)... Pulmonary Hypertension Roundtable Pulmonary Arterial Hypertension in Congenital Heart Disease: Controversies and Consensus Featuring: Robyn Barst, MD David Wessel, MD Nancy Bridges, MD Dunbar Ivy, MD Clinical Algorithm Evaluation of Pulmonary Hypertension in Children (PDF)     PHA Announcements   PHA's 2004 Sixth International Pulmonary Hypertension Conference
Notes   Editorial Advisory Board Editor-in-Chief Ronald J. Oudiz, MD Associate Professor of Medicine UCLA School of Medicine Director, Liu Center for Pulmonary Hypertension Division of Cardiology Los Angeles Biomedical Research Institute at Harbor-UCLA Medical Center Torrance, California Immediate Past Editor Vallerie V. McLaughlin, MD Associate Professor of Medicine Director, Pulmonary Hypertension Program University of Michigan Health System Ann Arbor, Michigan Editor-in-Chief Elect Richard Channick, MD Professor of Clinical Medicine Pulmonary and Critical Care Division University of California, San Diego Medical Center San Diego, California Associate Editors Erika Berman Rosenzweig, MD Assistant Professor of Pediatrics Department of Pediatrics Columbia College of Physicians and Surgeons New York, New York Robert Frantz, MD Consultant in Cardiovascular Diseases and Internal Medicine Assistant Professor of Medicine Mayo Clinic College of Medicine Rochester, Minnesota Srinivas Murali, MD, FACC Professor of Medicine Drexel University College of Medicine Director, Division of Cardiovascular Medicine Medical Director, Gerald McGinnis Cardiovascular Institute Allegheny General Hospital Pittsburgh, Pennsylvania Publisher Pulmonary Hypertension Association Jack Stibbs, Board Chair Rino Aldrighetti, President Rachel Pokorney, Medical Services Associate Director Publishing Staff Stu Chapman, Executive Editor Susan Chapman, Managing Editor Heidi Green, Associate Editor Gloria Catalano, Production Director Michael McClain, Design Director PHA Office Pulmonary Hypertension Association 801 Roeder Road., St.e 400 Silver Spring, MD 20910 301-565-3004, 301-565-3994 (fax) www.PHAssociation.org Provided with an unrestricted educational grant from Actelion Pharmaceuticals, U.S., Inc. and Accredo Therapeuticss. © 2006 by Pulmonary Hypertension Association and DataMedica. All rights reserved. None of the contents may be reproduced in any form whatsoever without the written permission of PHA. Editorial Offices Advances in Pulmonary Hypertension DataMedica 424 Dune Road Westhampton Beach, NY 11978 Tel: 631-288-7733 Fax: 631- 288-7744 Advances in Pulmonary Hypertension is circulated to cardiologists, pulmonologists, rheumatologists and other selected physicians by the Pulmonary Hypertension Association . The contents of the articles are independently determined ly the Editor and the Editorial Advisory Board. PHA's Scientific Leadership Council Editorial Mission Advances in Pulmonary Hypertension is committed to help physicians in their clinical decision making by informing them of important trends affecting their practice. Analyzing the impact of new findings and covering current information in the peer-reviewed literature, Advances in Pulmonary Hypertension is published four times a year. Advances in Pulmonary Hypertension is the official journal of the Pulmonary Hypertension Association. Each article in this journal has been reviewed and approved by members of the Editorial Advisory Board.   Editorial Board Todd Bull, MD Division of Pulmonary Sciences and Critical Care Medicine University of Colorado Health Sciences Center Denver, Colorado Murali Chakinala, MD Director, Pulmonary Hypertension Clinic Washington University School of Medicine St. Louis, Missouri Kristin Highland, MD Assistant Professor Division of Pulmonary and Critical Care Director, Pulmonary Hypertension Clinic Medical University of South Carolina Charleston, South Carolina Jim Maloney, MD Associate Professor of Pulmonary and Critical Care Medicine University of Colorado Health Sciences Center Denver, Colorado Ioana Preston, MD Assistant Professor of Medicine Tufts - New England Medical Center Boston, Massachusetts Zeenat Safdar, MD Assistant Professor of Medicine Department of Medicine, Pulmonary & Critical Care Section Pulmonary Hypertension Center Baylor College of Medicine Houston, Texas Rajan Saggar, MD Assistant Professor Division of Pulmonary and Critical Care Medicine and Hospitalists David Geffen School of Medicine UCLA Los Angeles, California Robert Schilz, DO, PhD Medical Director of Lung Transplantation and Pulmonary Vascular Disease University Hospital of Cleveland Case Western Reserve University Cleveland, Ohio Francisco Soto, MD, MS Assistant Professor Director, Pulmonary Hypertension Program Medical College of Wisconsin Milwaukee, Wisconsin Roxana Sulica, MD Assistant Professor of Medicine Albert Einstein College of Medicine Director, Beth Israel Pulmonary Hypertension Program Beth Israel Medical Center New York, New York

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