Vol 7, No 3 - PHA Online University

Advances in Pulmonary Hypertension CME Section
Program Overview
Pulmonary arterial hypertension (PAH), an incurable
disease, is characterized by medial hypertrophy, intimal
fibrosis, and in situ thrombi in small muscular pulmonary
arteries. PAH was considered a rapidly fatal illness with
a median survival of 2.8 years in the 1980s when no
evidence-based therapies were available. Since then
the treatment of this disease has made tremendous
advances, and the last 10 years have seen the discovery
of new medications that have positively influenced the
prognosis and survival of patients with PAH.
This self-study activity is based on 5 articles that review
the latest information on new treatments, combinations
of therapies, and data from phase 1 and 2 clinical trials.
This activity is jointly sponsored by the University of
Michigan Medical School and the Pulmonary Hypertension
Association and supported by an unrestricted
education grant from Actelion Pharmaceuticals US, Inc,
Encysive Pharmaceuticals, Inc, Gilead Sciences, Inc,
Pfizer, Inc, and United Therapeutics Corporation.
Target Audience
This self-study activity is appropriate for cardiologists,
pulmonologists, rheumatologists, and other physicians
who treat patients with pulmonary hypertension.
Learning Objectives
Upon completion of this activity participants will be
able to:
1. Review the various animal models used in PH
research to date
2. Compare and contrast the different pathological
findings in animal models of PH
3. Define the metabolic syndrome
4. Define “diabetic cardiomyopathy”
5. Review the effects of metabolic syndrome on
energy production in cardiac myocytes
6. Identify novel hemodynamic measurements that
can be made during right heart catheterization
Self-Assessment Examination
See pages 351 and 352 for self-assessment questions,
answer key, and evaluation form.
Faculty
Karen Fagan, MD
Chief, Division of Pulmonary and
Critical Care Medicine
University of South Alabama
Mobile, Alabama
Contributing Authors
Heiko Bugger, MD, PhD
Division of Endocrinology, Metabolism and
Diabetes
Program in Human Molecular Biology and
Genetics
University of Utah School of Medicine
E. Dale Abel, MD, PhD
Division of Endocrinology, Metabolism and
Diabetes
Program in Human Molecular Biology and
Genetics
University of Utah School of Medicine
Paul M. Hassoun, MD
Professor of Medicine and Director of the
Pulmonary Hypertension Program
Division of Pulmonary and Critical Care Medicine
Johns Hopkins University, School of Medicine
Kurt Stenmark, MD
Department of Pediatrics
Developmental Lung Biology Laboratory
University of Colorado at Denver and
Health Sciences Center
Hunter C. Champion, MD, PhD
Pulmonary Hypertension Program and
Division of Cardiology
Department of Medicine
Johns Hopkins Medical Institutions
Ivan F. McMurtry, PhD
Departments of Pharmacology and
Medicine and Center for Lung Biology
University of South Alabama
Agenda
The Metabolic Syndrome and Cardiac Function
Heiko Bugger, MD, PhD and
E. Dale Abel, MD, PhD
National Heart Lung and Blood Institute Hopkins
Specialized Center in Clinical Oriented Research
(SCCOR): Molecular Determinants of Pulmonary
Arterial Hypertension
Paul M. Hassoun, MD
Specialized Center in Clinical Oriented Research
(SCCOR) Update: Mechanisms and Treatment of
Long Vascular Disease in Infants and Children
Kurt Stenmark, MD
Getting More From Right Heart Catheterization:
A Focus on the Right Ventricle
Hunter C. Champion, MD, PhD
Animal Models of Human Severe PAH
Ivan F. McMurtry, PhD
330 Advances in Pulmonary Hypertension