Advances in Pulmonary Hypertension
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 in the last 10 years the discovery of new medications have positively influenced the prognosis and survival of patients with PAH.
This self-study activity is based on 4 articles that review the management of patients with congenital heart disease and pulmonary hypertension.
This activity is jointly sponsored by Washington University School of Medicine and the Pulmonary Hypertension Association.
Target Audience: This self-study activity is appropriate for cardiologists, pulmonologists, rheumatologists, and other physicians who treat patients with PH.
Learning Objectives: Upon completion of this activity, participants will be able to:
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Describe the anatomy of the most common congenital heart defects (and their repairs) that are associated with the development of PAH.
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Demonstrate the importance of simple and advanced imaging technique in adults with congenital heart disease and PH as diagnostic and risk stratifying tools.
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Understand the complex interplay between pulmonary blood flow and pulmonary vascular resistance and the techniques (medicinal, catheter-based, and surgical) that are used to modify it.
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Discuss the clinical studies that have established the basis for pharmacology therapy, and explore the new therapeutic frontiers in patients with congenital heart disease and PH.
Self-Assessment Examination: See pages 196 and 197 for self-assessment questions, answer key, and evaluation form.
Faculty
Chair
Richard A. Krasuski, MD, FACC, FAHA, Director of Adult CongenitalHeart Disease Services, Cardiovascular Medicine, Cleveland Clinic Foundation, Cleveland, Ohio
Contributing Authors
Jamil A. Aboulhosn, MD, FACC, FSCAI, Director, Ahmanson/UCLA Adult Congenital Heart Disease Center, Los Angeles, California
Sonya V. Babu-Narayan, MRCP, PhD, Royal Brompton and Harefield NHSFoundation Trust, National Heart and Lung Institute, Imperial College London, NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital and Imperial College London, London, United Kingdom
Thomas Bashore, MD, Professor of Medicine, Senior Vice Chief, Division of Cardiology, Duke University Medical Center, Durham, North Carolina
Michael A. Gatzoulis, MD, PhD, FESC, FACC, Royal Brompton and Harefield NHS Foundation Trust, National Heart and Lung Institute, Imperial College London, NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital and Imperial College London, London, United Kingdom
Todd L. Kiefer, MD, Assistant Professor, Division of Cardiology, Duke University Medical Center, Durham, North Carolina
Wei Li, MD, PhD, FESC, FACC, Royal Brompton and Harefield NHS Foundation Trust, London, United Kingdom
Erika B. Rosenzweig, MD, Columbia University College of Physicians and Surgeons, Pulmonary Hypertension Center, New York, New York
Michael B. Rubens, FRCR, Royal Brompton and Harefield NHS Foundation Trust, London, United Kingdom
Giancarlo Scognamiglio, MD, PhD, Royal Brompton and Harefield NHS Foundation Trust, London, United Kingdom
Warren A. Zuckerman, MD, Columbia University College of Physicians and Surgeons, Pulmonary Hypertension Center, New York, New York
Accreditation Statement: This activity has been planned and implemented in accordance with the Essential Areas and Policies of the Accreditation Council for Continuing Medical Education (ACCME) through the joint sponsorship of Washington University School of Medicine and the Pulmonary Hypertension Association. Washington University School of Medicine is accredited by the ACCME to provide continuing medical education to physicians.
Credit Designation: Washington University School of Medicine designates this enduring material for a maximum of 2.0 AMA PRA Category 1 Credits.™ Physicians should claim only the credit commensurate with the extent of their participation in the activity.
Instructions for Earning Credit: This activity is a self-study program; a self-assessment examination is included on page 196 to help physicians review important points. A form is also included on page 197 for physicians to evaluate the CME activity. Completion of this activity involves reading the journal and completing the self-assessment examination and evaluation form with a passing grade of 70% or higher, which may take up to 2 hours. Credits for this self-study program are available from May 31, 2013 through April 30, 2014. There is no fee for this program. Please note that this self-study program may also be viewed online at https://cme-online.wustl.edu/pha.
Accreditation Statement: Department of Continuing Medical Education, Washington University School of Medicine, Campus Box 8063, 660 South Euclid Ave., St. Louis, MO 63110
Disclosures: The Accreditation Council for Continuing Medical Education and the Association of American Colleges have standards and guidelines to ensure that individuals participating in CME activities are aware of relationships between authors and commercial companies that could potentially affect the information presented. To be disclosed to participants are all personal financial relationships with a commercial interest whose products are relevant to the content of this CME activity. It is the policy of Washington University School of Medicine, Continuing Medical Education, to ensure balance, independence, objectivity, and scientific rigor in all its educational activities. All faculty participating in this activity are expected to disclose to the audience any financial interest or other potential conflict. Each author was asked to complete a disclosure information form for this activity. Disclosures are reported below:
Dr Aboulhosn has no financial relationships to disclose. Dr Babu-Narayan has no financial relationships to disclose. Dr Bashore has no financial relationships to disclose. Dr Gatzoulis has no financial relationships to disclose. Dr Kiefer has no financial relationships to disclose. Dr Li has no financial relationships to disclose. Dr Rubens has no financial relationships to disclose. Dr Scognamiglio has no financial relationships to disclose. Dr Zuckerman has no financial relationships to disclose.
Dr Rosenzweig has served as a consultant/advisory board/steering committee member for United Therapeutics, Actelion, and Gilead; and received institutional grant/research support from Actelion, Gilead, United Therapeutics, GSK, Bayer, and Novartis.
Dr Dodson has no financial relationship to disclose. Dr Chakinala receives research support or serves as a consultant to Actelion, Gilead, United Therapeutics, Lung LLC, Novartis, and Ikaria.
CME Reviewers
W. Edwin Dodson, MD, Associate Vice Chancellor and Associate Dean for Admissions and Continuing Medical Education, Professor of Pediatrics and Neurology, Washington University School of Medicine.
Murali Chakinala, MD, Associate Professor, Division of Pulmonary and Critical Care Medicine, Washington University School of Medicine.