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.
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
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.
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
The
information provided on the PHA website is provided for general information
only. It is not intended as legal, medical or other professional
advice, and should not be relied upon as a substitute for consultations
with qualified professionals who are familiar with your individual
needs.
PAH-Associated
Congenital Heart Disease: Finding Common Ground With
Primary Pulmonary Hypertension
