Advances in Pulmonary Hypertension - PHA Online University

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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 Advisory Board Editor-in-Chief Victor F. Tapson, MD Associate Professor of Medicine Division of Pulmonary and Critical Care Medicine Duke University Medical Center Durham, North Carolina Sean Gaine, MD, PhD Director, Pulmonary Hypertension Unit Mater Misericordiae Hospital University College Dublin, Ireland Vallerie McLaughlin, MD Assistant Professor of Medicine Associate Director Center for Pulmonary Heart Disease Coronary Heart Disease Detection and Treatment Center Rush Heart Institute Rush Presbyterian-St. Luke’s Medical Center Chicago, Illinois Ivan M. Robbins, MD Director, Pulmonary Hypertension Center Allergy/Pulmonary and Critical Care Medicine Vanderbilt University Nashville, Tennessee Publisher Pulmonary Hypertension Association Bruce Brundage, MD, President Rino Aldrighetti, Executive 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 850 Sligo Avenue, Suite 800 Silver Spring, MD 20910 301-565-3004, 301-565-3994 (fax) www.phassociation.org Provided through an unrestricted educational grant from Actelion Pharmaceuticals, U.S., Inc. and Accredo Therapeutics. © 2002 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 the executive editor. Editorial Offices Advances in Pulmonary Hypertension, DataMedica, 424 Dune Road, Westhampton Beach, NY 11978 Tel (631) 288-4702 Advances in Pulmonary Hypertension is circulated to cardiologists, pulmonologists, rheumatologists and other selected physicians by the Pulmonary Hypertension Association. The contents are independently determined by the Editor and the Editorial Advisory Board. Cover Photo: Chest x-ray depicts pulmonary hypertension and a Hickman catheter. In lower left, computed tomography shows interstitial lung disease and the dilated esophagus from scleroderma. (Courtesy of Victor F. Tapson, MD) Printed on recycled paper. The Scientific Leadership Council of the Pulmonary Hypertension Association The scientific program of the Pulmonary Hypertension Association is guided by the association’s Scientific Leadership Council. The Council includes the following health care professionals: Michael McGoon, MD SLC Chair Pulmonary Hypertension Clinic/Mayo Clinic Rochester, Minnesota Robyn Barst, MD SLC Vice-Chair Columbia Presbyterian Medical Center Babies Hospital New York, New York David B. Badesch, MD Chair, SLC Nominations Committee University of Colorado Health Sciences Center Denver, Colorado Joy Beckmann, RN, MSN Pulmonary Hypertension Program Harbor-UCLA Medical Center Torrance, California C. Gregory Elliott, MD Chair, SLC Publications Committee LDS Hospital University of Utah School of Medicine Salt Lake City, Utah Adaani Frost, MD Baylor College of Medicine Houston, Texas Sean Gaine, MD, PhD Mater Misericordiae Hospital Dublin, Ireland Nazzareno Galiè, MD Istituto di Malattie dell’ Apparato Cardiovascolare Universita di Bologna Bologna, Italy Abby Krichman, RRT Division of Pulmonary and Critical Care Medicine Duke University Medical Center Durham, North Carolina David Langleben, MD Jewish General Hospital Montreal, Quebec, Canada James E. Loyd, MD Pulmonary and Critical Care Medicine Vanderbilt University Medical Center Nashville, Tennessee Vallerie McLaughlin, MD Rush Presbyterian-St. Lukes Medical Center Chicago, Illinois John H. Newman, MD Nashville VA Hospital Nashville, Tennessee Horst Olschewski, MD Justus-Liebig Universitat Gessen, Germany Harold I. Palevsky, MD University of Pennsylvania School of Medicine Presbyterian Medical Center Philadelphia, Pennsylvania Stuart Rich, MD Vice-Chair, SLC Research Committee Rush Heart Institute- Center for Pulmonary Heart Disease Chicago, Illinois Ivan M. Robbins, MD Chair, SLC Consensus Committee Vanderbilt University Nashville, Tennessee Lewis J. Rubin, MD Chair, SLC Research Committee University of California at San Diego San Diego, California Cathy J. Severson, RN, BSN Pulmonary Hypertension Clinic/Mayo Clinic Rochester, Minnesota Victor F. Tapson, MD Division of Pulmonary and Critical Care Medicine Duke University Medical Center Durham, North Carolina Allison Widlitz, PA Pediatric Cardiology New York Presbyterian Hospital New York, New York Carol E. Vreim, PhD NHLBI Liaison Deputy Director Division of Lung Diseases, NHBLI Bethesda, Maryland Emeritus Bruce H. Brundage, MD Heart Institute of the Cascades Bend, Oregon Alfred P. Fishman, MD University of Pennsylvania Health System Philadelphia, Pennsylvania
Editor’s Memo Bridging the Gap Between Specialists With Insights From an Expert Panel Often unsuspected and underappreciated, pulmonary arterial hypertension in some forms of scleroderma is much like primary pulmonary hypertension; the cause of the dyspnea and associated symptoms frequently eludes diagnosis until very late in the disease. Unfortunately, by that time, pulmonary hypertension may have reached an advanced stage less amenable to treatment. Although many patients with collagen vascular disease are considered at high risk for inflammatory pulmonary involvement, the disease process is often insidious and appropriate evaluation, including pulmonary function tests, echocardiography, and right-heart catheterization, may not be ordered when the benefit of treatment might be more substantial. One of the key issues concerns separating the potential causes of pulmonary hypertension that may occur in these patients and recognizing why they may require more aggressive follow-up to identify an evolving inflammatory component and potential progressive vasculopathy. Driven to Find Cure for PH, Brundage Leads New Quest for Breakthrough in Therapy Bruce Brundage, MD, has seen it all, from the first anecdotal cases when no treatment for pulmonary hypertension was available, to the landmark National Institutes of Health (NIH) registry, to the large scale clinical trials of today involving hundreds of patients. For anyone seeking to chart progress in the field, his career serves as a bridge, spanning milestones in the treatment of the disease. He has been associated with virtually every key development in the progress toward a cure, beginning in the 1970s when he served as the director of the cardiac catheterization laboratory at the University of California, San Francisco. It was during his study of those early cases, when he inserted catheters into the pulmonary artery to measure the effects of various vasodilating drugs, that he began focusing on pulmonary hypertension. During the last 25 For these reasons, the Pulmonary Hypertension Roundtable discussion in this issue will have broad appeal to our readership, which includes cardiologists, pulmonologists, and rheumatologists. Clearly, this is a disease that is likely to present to each of these groups, and by transcending the boundaries of these specialties, our discussion offers insights about its incidence, evolution, and appropriate evaluation. From the diverse backgrounds of our experts, you will find clinical pearls relevant to your practice regardless of your specialty. One of the goals of Advances in Pulmonary Hypertension is to bring you this kind of distilled knowledge, based on contributions from the Scientific Leadership Council of the Pulmonary Hypertension Association (PHA). These physicians are listed on page 2 and provide a tremendous breadth of experience gathered at leading institutions throughout the world. Their continued involvement with the journal gives us one of the clearest perspectives on the latest information in diagnosis and treatment. We appreciate the warm welcome Advances in Pulmonary Hypertension has received in the medical community and look forward to fulfilling the commitment PHA has made toward expanding your awareness of managing this disease. We also welcome your comments and suggestions regarding articles that appear in this issue by contacting PHA. Vic Tapson, MD Editor-in-Chief years, research has been his passion—leading him to serve on the steering committee of the national registry and to play a key role in the growth of the 5,000-member Pulmonary Hypertension Association (PHA), of which he is now president. “It was in the late 1970s that I discovered the NIH was starting its patient registry in pulmonary hypertension so I applied to have our center enrolled as part of the registry,” he said. “Soon afterward, I was invited to be on the steering committee for the patient registry at a time when we began collecting data in an organized manner.” Joining the faculty at the University of Illinois at Chicago, Dr Brundage teamed with Stuart Rich, MD, and Paul Levy, PhD, two leading investigators, as they explored the effects of high-dose calcium channel blockers in treating pulmonary hypertension. “This was the first breakthrough in the treatment because we found there was a percentage of patients who were helped.” In 1990 Dr Brundage was named chief of the Department of Cardiology at Harbor-UCLA Medical Center and became involved in the early studies of intravenous prostacyclin therapy. Enrolling 300 patients to receive what was a new infusion therapy at the time, he was a coauthor of a major paper published in the Journal of the American College of Cardiology demonstrating the longterm survival benefits of prostacyclin. At that point (continued on page 20) Advances in Pulmonary Hypertension 3
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Page 7 and 8: Table 2—Potential Therapeutic Opt
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Page 13 and 14: CO = oxygen consumption per minute
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