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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

 

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Articles
 
Advances in Pulmonary Hypertension, Summer 2003, Vol. 2, No. 2

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
Richard Channick, MD
Professor of Clinical Medicine
Pulmonary and Critical Care Division
University of California, San Diego Medical Center
San Diego, California

Immediate Past Editor
Ronald J. Oudiz, MD
Associate Professor of Medicine
The David Geffen School of Medicine at UCLA
Director, Liu Center for Pulmonary Hypertension
LA Biomedical Research Institute at Harbor-UCLA Medical Center

Editor-in-Chief Elect
Erika Berman Rosenzweig, MD
Columbia University,
College of Physicians and Surgeons
Morgan Stanley Children's Hospital of New York

Associate Editors

Kristin Highland, MD
Assistant Professor of Medicine
Division of Pulmonary, Critical Care, Allergy and Sleep Medicine
Medical University Of Carolina

Francisco Soto, MD, MS
Director, Pulmonary Hypertension Program
Pulmonary and Critical Care Medicine
Medical College of Wisconsin

Todd Bull, MD
Associate Professor of Medicine
Division of Pulmonary Sciences and Critical Care Medicine
University of Colorado Health Sciences Center

Robert Schilz, DO, PhD
Medical Director of Lung
Transplantation and Pulmonary Vascular Disease
University Hospital of Cleveland
Case Western Reserve University
Cleveland, Ohio


Publisher
Pulmonary Hypertension Association
Carl Hicks, Board Chair
Rino Aldrighetti, President
Donica Merhazion, Medical Services Associate Director

Publishing Staff
Deborah L. McBride
McBride Strategic Services
mcbridedeb@aol.com
P: 773-348-5455
C: 312-307-5455

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

© 2009 by Pulmonary Hypertension Association. All rights reserved. None of the contents may be reproduced in any form whatsoever without the written permission of PHA.

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

Charles Burger, MD
Medical Director, PH Clinic
Mayo Clinic
Jacksonville, FL

Karen Fagan, M.D.
Chief, Division of Pulmonary and Critical Care Medicine University of South Alabama

Eli Gabbay
Lung Transplant Unit
Royal Perth Hospital

Nick Kim, M.D.
Associate Clinical Professor of Medicine
University of California San Diego

Deborah Jo Levine, M.D.
University of Texas Health Science Center at San Antonio
San Antonio, TX

Omar Minai
Dept of Pulmonary, Allergy  and Critical Care Medicine

Myung Park
Associate Professor of Medicine
Division of Pulmonary Sciences and Critical Care Medicine
University of Colorado Health Sciences Center0

Fernando Torres, MD
Director Pulmonary Hypertension Clinic
UTSW Medical Center Dallas
University Hospital

Glenna Traiger, RN, MSN
Pulmonary & Critical Care
Pulmonary Hypertension CNS
University of California Los Angeles
Los Angeles, CA

R. James White, MD, PhD
Assistant Professor of Medicine, Pharmacology & Physiology
University of Rochester, Division of Pulmonary and CCM

Roham Zamanian, MD
Division of Pulmonary and Critical Care Medicine
Stanford University Medical Center

 

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