Medical
Professionals - Articles and Presentations
The purpose of this section of the PHA website is to provide
information about the activities of PH Centers and progress
on the study of this disease. Here's a list of what's included and a link down to the material:
PHRN Dinner
Research Opportunities in Pulmonary Hypertension for Nurses and Allied Health Professionals (Diane L. Carroll, PhD, ACNS-BC, FAAN, FAHA and Yvonne L. Munn, Nurse Researcher)
Scientific Sessions
Scientific Sessions Introduction Animal Models of Human Severe PAH (Ivan F. McMurtry, PhD)Bosentan improves hemodynamics and delays time to clinical worsening in patients with mildly symptomatic pulmonary arterial hypertension: Results of the EARLY study (Galie N, Benza R, Rubin LJ, Hoeper MM, Jansa P, Kusic-Pajic A, Simonneau G)Long-term Ambrisentan Therapy Provides Sustained Benefit in Patients with Pulmonary Arterial Hypertension (RJ Oudiz)Extramural Research
Pulmonary Arterial Hypertension (James P. Kiley, M.S., Ph.D.)Pharmacogenomics (Richard Weinshilboum, M.D.)Potential Role for the Bone Morphogenetic Antagonist Gremlin in Pulmonary Hypertension (Dr. Sean Gaine)Molecular and Hemodynamic Determinants of Right Ventricular Response to Pulmonary Hypertension (Hunter C. Champion, MD, PhD, FAHA) Mechanisms for Cardiac Dysfunction in the
Metabolic Syndrome (E. Dale Abel MD, Ph.D.)
Idiopathic Pulmonary Hypertension is Caused by a Fetal Phenotype of Arterial Diameter Network Organization Remodeling Under the Principle of Minimum Work (Allen R.P., Milstein J.M., Moon-Grady A.J., Raff G.W., Black S.M., Fineman J.R., Bennett S.H., Eldridge M.W.) Functional Genetic Variations in BMPR2 and Other Signaling Pathways Modify Clinical Expression of Familial Pulmonary Arterial Hypertension (Austin E.D., Phillips J.A. III, Cogan J.D., Stanton K.S., Phillips C.A., Yu C., Wheeler L.A., Newman J.H., Loyd J.E.) HHV-8 Infection of Pulmonary Microvascular Endothelial Cells – A Potential Mechanism for the Development of Pulmonary Arterial Hypertension (Bull T.M., Meadows C.A., Campbell T.B., Geraci M.A.) Induced Metabolic Acidosis is Protective in a Rat Model of Hypoxic Pulmonary Hypertension: Direct Effect on Vascular Smooth Muscle Cell (VSMC) Proliferation and Migration (Christou H., Arons E., Mitsialis S.A.) Potential Role for the Bone Morphogenetic Antagonist Gremlin in Pulmonary Hypertension (Costello C.M., Howell K., Cahill E., McBryan J., Koenigshoff M., Eickelberg O., Gaine S., Martin F., McLoughlin P.) Protease Activated Receptor Ca2+-Linked Mitochondrial Reactive Oxygen Species are Essential for Endothelial/Leukocyte Adherence (Hawkins B.J., Solt L.A., Chowdhury I., Kazi A.S., Abid M.R., Aird W.C., May M.J.,
Foskett J.K., Madesh M.)PY-STAT3: an Upstream Regulator in Pulmonary Artery Hypertension (Mathew R., Huang J., Olson S., Gewitz M.)Progressive Dysfunction of Nitric Oxide Synthase in a Lamb Model of Chronically Increased Pulmonary Blood Flow: A role for oxidant stress (Oishi P.E., Wiseman D.A., Sharma S., Azakie A., Black S.M., Fineman J.R.)Ixolaris, a Tissue Factor Inhibitor, Reduces Vascular Pruning and Delays Death in Rats with Established Pulmonary Arterial Hypertension (Swarthout, R.F., Meoli, D.F., Taubman, M.B., Francischetti, I., Awad, H., White, R.J.) Enhancement of TRPC6-mediated Ca2+ Entry in Pulmonary Artery Smooth Muscle Cells of Idiopathic Pulmonary Arterial Hypertension Patients (Yu Y., Safrina O., Rubin L.J., Cahalan M.D., Yuan, J.X.J.) Gene Variants in Caveolin-1 and Caveolin-2 in Patients with Idiopathic Pulmonary Arterial Hypertension (Zellner C., Liao W., Pullinger C.R., Chan S., Poon A., Kwok P., Malloy M.J., Kane J.P., McGlothlin D., Chatterjee K., DeMarco T.D.) Uric Acid Decreases NO Availability and Increases Arginase Activity in Cultured Pulmonary Artery Endothelial Cells (Zharikov S., Krotova K., Hu H., Patel J.M., Baylis C., Johnson R.J., Block E.R.) Hemolysis-Induced Pulmonary Hypertension and Treatment with Infused or Inhaled Nitrite, or Inhaled NO in the Newborn Lamb (Blood A.B., Schroeder H., Terry M.H., Merrill-Henry J., Power G.G.) A Long-acting Prostacyclin Agonist with Thromboxane Inhibitory Activity for Pulmonary Hypertension (Kataoka M., Satoh T., Nagaya N., Ogawa S.) Cell Therapy Confers Protection from Immunodeficiency-Associated Pulmonary Hypertension and Prevents Activation of Right Ventricular Fetal Gene Program (Nicolls M.R., Long C., Amber K., Tamosiuniene R.) Pharmacogenomics in PAH: Roles of Downstream Polymorphisms of the ET-1 Pathway (Olave N.C., Benza R.L., Wolkowicz P.E., Grenett H.E.)Vascular Pharmacogenomics in a Surgical Shunt Model of Pulmonary Arterial Hypertension (PAH): Analysis by Endoarterial Biopsy (Rothman A., Mann D., Davidson S., Wiencek R., Restrepo H., Sarukhanov V., Evans W.E., Williams R., Rouslahti E.) Serotonin 2B Receptor Antagonist PRX-08066 Inhibits Monocrotaline-Induced Pulmonary Hypertension in Rats (Warburton R.R., Gannon K.S., Guevara O., Liu T., Preston I.R., Fanburg B.L.,
Hill N.S.) High-Altitude Pulmonary Edema Susceptibility in Relation to Epigenetic Patterns of Pulmonary Distensibility and Arterial Fractal Network Design (Allen R.P., Milstein J.M., Moon-Grady A.J., Raff G.W., Black S.M., Fineman J.R., Bennett S.H., Eldridge M.W.)Post-Splenectomy Pulmonary Hypertension: Characteristics of 49 Patients (Golbin J.M., McGoon M.D., Krowka M.J.) Pulmonary Hypertension is Common in Patients with Valvular Heart Disease and Persists Following Valve Surgery (Hurewitz A.N., Bartlett M., Schubach S.) Effect of Hemodynamics and Functional Capacity on Survival in Patients with Sickle Cell Disease and Pulmonary Hypertension (Machado R.F., Cuttica M., Hunter L., Coles W., Gladwin M.T.) Macrophage Migration Inhibitory Factor (Mif) as a Biomarker in the Assessment of Pulmonary Arterial Hypertension and Interstitial Lung Disease: a Preliminary Study (Omonuwa K., Talwar A., Hu M., Lin X., Berkoski P., Dedopoulos S., Miller E.J.) Degree of Reduction in Functional Pulmonary Microvascular Surface Area in Pulmonary Arterial Hypertension Related to CTD is Proportional for Two Pulmonary Metabolic Activities (Orfanos S.E., Langleben D., Dupuis J., Hirsch A. M., Baron M., Senecal J.L., Giovinazzo M., Catravas J.D.) Exercise Echocardiograms (EE) in Scleroderma Patients with Different Auto-antibodies (Steen, V.) REVEAL Registry: Baseline Characteristics of the First 1226 Enrolled Patients (Badesch D.B., Barst R.J., Benza R.L., Elliott C.G., Farber H., Frost A.E., Krichman A., Liou T.G., Raskob G.E., Giles S., Feldkircher K., Miller D.P., McGoon,M.D.) REVEAL Registry: Baseline Characteristics of Patients with Childhood-Onset PAH (Barst R.J., Ivy D., Badesch D.B., Benza R.L., Elliott C.G., Farber H., Frost A.E., Krichman A., Liou T.G., Raskob G.E., Giles S., Feldkircher K., Miller D.P., McGoon M.D.) TOPP: The First Multinational Registry in Pediatric Pulmonary Hypertension (Barst R.J., Beghetti M., Berger R.M.F., Humpl T., Raskob G., Schulze-Neick I.) REVEAL: A Registry to Evaluate PAH Disease Management (McGoon M.D., Badesch D.B., Barst R.J., Benza R.L., Elliott C.G., Farber H., Frost A.E., Krichman A., Liou T.G., Raskob G.E., Giles S., Feldkircher K., Miller D.P.) 2007 Review of Utah Pulmonary Hypertension Genetics Project: Updated Revisions and Findings (Schmidt J.W., Glissmeyer E.W., Adams D., Wojciechowski B.L., Elliott C.G., Hegewald, M.J., Carlquist J., Mead M.) Survival of Patients with Pulmonary Arterial Hypertension (PAH) Associated with Anorexigens vs. Survival Predicted From NIH Registry on Primary (Schmidt J.W., Glissmeyer E.W., Wojciechowski B.L., Adams D., Elliott C.G., Jensen R.L. Hegewald M.J., Carlquist J., Scholand M.B., Liou T., Mead M.) Thyroperoxidase Antibody is Not a Useful Screening Test for Thyroid Disease in Patients with Pulmonary Hypertension (Burger C.D.) Endothelin-1 and BNP Plasma Levels Predict Survival in Patients with Pulmonary Arterial Hypertension (Frantz R.P., Robbins I.M., Durst L.A., Grill D.E., McGoon M.D., Burnett J.C.) Misclassification of Pulmonary Arterial Hypertension Due to Use of Pulmonary Capillary Wedge Pressure (PCWP) Rather Than Left-Ventricular End-Diastolic Pressure (LVEDP) (Halpern S.D., Taichman D.B.) What Patients and their Relatives Think About Testing for BMPR2 (Jones D.L., Sandberg J.C., Rosenthal M.J., Saunders R.C., Hannig V.L., Clayton E.W.) Intra- and Inter-grader Agreement for World Health Organization Functional Class Assignment Based on a Novel Questionnaire in Patients with Pulmonary Arterial Hypertension (Murugappan M., Taichman D., Williamson T., Sundy R., Byers B., Duncan M., Zheng J., Chakinala M.) Role of BNP in Identifying Elevated Pulmonary Wedge Pressure in Patients with Pulmonary Hypertension (Navas V., Rahaghi N., Ramirez J., Smolley L., Oliveira E., Areces M., Scridon C.,
Fromkin K., Novaro G. and Rahaghi F.F.) Elevated Indices of Collagen Synthesis in Pulmonary Arterial Hypertension (Zeenat S., Frost. A.E.) In Patients with Pulmonary Hypertension, Right Heart Failure is Diagnosed by Precise Estimation of Right Atrial Pressure with Inspection of the Internal Jugular Vein and Additional Use of the Hepatojugular Reflux Technique (Satoh T., Niino K., Kawakami T., Karaoke M.., Yoshikawa T., Ogawa S.) Evaluating the Right Ventricle: Volume, Function, and Shape (Sheehan, F.H.) External Doppler is an Accurate and Reproducible Method of Measuring Cardiac Output in Patients with Pulmonary Hypertension (Sundy R., Billadello J., Zhen J., Ahrens T., Chakinala M.) Wide Variation in Clinicians’ Assessments of NYHA/WHO Functional Class (Taichman D.B., McGoon M.D., Harhay M.O., Sager J., Gallop R., Palevsky H.I.) Baseline Hemodynamic Measurement as a Predictor for Heart-Lung Transplantation versus Lung Transplantation in Pulmonary Arterial Hypertension (Ishizawar D., Zhang J., Simon M.A., McCurry K.R., Mathier M.A.) Idiopathic Pulmonary Arterial Hypertension (IPAH) Recurring Within One Year After Bilateral Lung Transplantation (LT) (Snow J.L., Palevsky H.I., Archer-Chicko C., Carney K.C., Sager J.S.) Prognostic Factors Associated with Survival in 860 PAH Patients Treated With Subcutaneous Treprostinil Over a 3 Year Period (Arneson C.) Methods of Increasing Comprehensive and Efficient Patient Care: The Enhancement of a Pediatric Pulmonary Hypertension Program (Bagby M., Giver J., Hirsch R.) Maintenance of Intravenous Prostacyclin Infusions during Magnetic Resonance Imaging Scan (Bair N., Banjac S., Minai O., Dweik R.) TRIUMPH I: Efficacy and Safety of Inhaled Treprostinil Sodium in Patients with Pulmonary Arterial Hypertension (PAH) (Benza R., Rubin L., McLaughlin V., Channick R., Vosswinkel R., Tapson V., Robbins I., Olschewski H., Seeger W.) Comparable Efficacy of Bosentan in Eisenmenger’s Syndrome Patients with Atrial Septal Defects and Ventricular Septal Defects (Berger R.M.F., Beghetti M., Galiè N., Gatzoulis M.A., Granton J., Lauer A., Chiossi E., Landzberg M.) Comprehensive Pulmonary Rehabilitation Improves Exercise Capacity and Quality of Life in Patients with Pulmonary Hypertension (Connors G.L., Hess S., Shumway J., Lamberti J.P.) Clinicopathologic Correlation of a Calcium Channel Responsive Patient with Idiopathic Pulmonary Arterial Hypertension (IPAH) (Cooke R.K., Duarte A.G., Eltorky M.A., Boroumand N., Cowan D.F.) Multi-center Experience with the Transition to Treprostinil from Inhaled Iloprost in Pulmonary Arterial Hypertension (Frantz R., McGoon M., Waxman A., Kim N., Torres F., Rollins K., Strootman D., Nathan S.) Bosentan Improves Hemodynamics and Delays Time to Clinical Worsening in Patients with Mildly Symptomatic Pulmonary Arterial Hypertension: Results of the EARLY Study (Galie N., Rubin L.J., Hoeper M.M., Jansa P., Kusic-Pajic A., Simonneau G.) Inhaled Nitric Oxide in the Assessment of Pulmonary Hypertension (Hunt J.M., Barker S.A., Messenger J.C., Groves B.M., Lowes B.D., Carol J.D., Gruber M.P., Bull T.M.) Prevention of Catheter Related Bloodstream Infections with a Closed Hub System and Connection Protection in Patients Receiving Prostanoid Therapy for Pulmonary Arterial Hypertension (Ivy D., Doran A., Calderbank M., Dolan S., Nyquist A.) Bosentan for Inoperable Chronic Thromboembolic Pulmonary Hypertension: A Randomized, Placebo-Controlled Trial (BENEFiT) (Jaïs X., Ghofrani A., Hoeper M.M., Lang I., Mayer E., Pepke-Zaba J., Rubin L.J., Simonneau G.) Clinical and Hemodynamic Factors Associated with Prognosis in Pulmonary Arterial Hypertension (PAH) (Kane G.C., Slusser J.P., Scott C.G., Maradit-Kremers H., McGoon M.D.) Treatment Patterns and Predictors of Drug Therapy in Pulmonary Arterial Hypertension (PAH) Between 1995 and 2005 (Maradit-Kremers H., Golbin J.M., Slusser J.P., Scott C.G., Kane G.C., McGoon M.D.) Long-term Ambrisentan Therapy Provides Sustained Benefit in Patients with Pulmonary Arterial Hypertension (Oudiz R.J., on behalf of the ARIES Study Group) Goal-Directed Combination Therapy in Pulmonary Arterial Hypertension (PAH): Study Design of COMPASS-3 (Park M., Torres F., Gupta H., Soto F., Lubeck C., Benza R.L., Murali S.) Change in Diastolic Pulmonary Arterial Pressure (dPAP) Predicts Changes in Cardiac Index (CI) in Pulmonary Arterial Hypertension (PAH): Hemodynamic Data Analysis of the Initial Randomized Placebo-Controlled Bosentan Study (Soto F.J., Girotra S.) A 36-Month Survival Analysis of Patients Beginning Oral PAH Monotherapy: An Indication for Escalation of Therapy? (Tankersley M.A., D’Albini L.D., Ozanich A.N., Whitman A.J.) Management of Pulmonary Arterial Hypertension During Pregnancy (Thomas S., Duarte A.G., Chin K., Torres F., Wen T., Vadhera R.B.) Oral Treprostinil Diethanolamine Provides Sustained Therapeutic Plasma Concentrations Over a Wide Range of Doses in Patients with Pulmonary Arterial Hypertension (White R.J., Allen R., Yehle D., Laliberte K., Mottola D., Galie N., Simmoneau G., Tapson V.F.) What is the Most Effective Way to Market a New Pulmonary Hypertension Center? (Zeiger T.K., Safford R.E., Burger C.D.)
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General Sessions
How Did We Get Here? History and Classification of PAH (PDF) Associate Professor of Medicine, Director, Pulmonary Hypertension Program, University of Michigan Health System
The Hemodynamics of PAH: Interpreting the Numbers (PDF) Associate Professor of Medicine, Medical Director, ICU Anshutz Inpatient Pavilion
ACCP Updated Guidelines for PAH Treatment (PDF) Professor of Medicine, Clinical Director, Pulmonary Hypertension Center, University of Colorado Health Sciences Center REVEAL Registry: What Can It Reveal? (PDF) Professor of Medicine, Boston University School of Medicine; Director, Pulmonary Hypertension Center, Boston Medical Center Professor of Pediatrics, Columbia University College of Physicians & Surgeons; Research Associate, Manager, Pulmonary Vascular Disease Center, Duke University
Combination Therapy: Is the Sum Better Than the Parts? (PDF) Associate Professor of Medicine, David Geffen School of Medicine at UCLA; Director, Liu Center for Pulmonary Hypertension, Division of Cardiology, Los Angeles Biomedical Research Institute at Harbor-UCLA Medical Center
Medicare Part D (PDF) Managing Partner, Argenta Reimbursement Advisors, L.L.C. and Argenta T.E.C. Advisors, L.L.C. Reimbursement Manager, Accredo Health Group Government Accounts Manager, Gilead Sciences, Inc.
Quality Care to the End of Life (PDF) PH Nurse Clinician, Park Nicollet Heart & Vascular Center Director, HealthPartners Simulation Center for Patient Safety, Metropolitan State University Introduction to PH Resource Network Committees (PDF) PH Resource Network Chair
Breakout Sessions
Liver Disease (PDF) Assistant Professor of Pulmonary and Critical Care Medicine, Mayo Clinic College of Medicine Nurse Practitioner, Virginia Commonwealth University Health System
Lung Transplant: The Only Definitive Therapy (PDF) Senior Cardiothoracic Transplant Coordinator/PH Coordinator, Childrens Hospital Los Angeles Associate Professor of Medicine, Pulmonary Critical Care Division, University of Washington Medical Center Director, Pulmonary Hypertension Program; Childrens Hospital Los Angeles; Keck School of Medicine at the University of Southern California Cardiothoracic Surgeon, University of Minnesota
Scleroderma: Common Association, Uncommon Implications (PDF)Nurse Practitioner, University of Texas, Southwestern Medical Center at Dallas Assistant Professor of Medicine, Division of Pulmonary and Critical Care, Oregon Health and Science University
Methamphetamine & PH: An Emerging Population (PDF) Jane Janusz, R.N., B.S.N.Clinical Nurse Coordinator, Medical College of Wisconsin Assistant Professor of Medicine, University of Texas Southwestern Pulmonary Hypertension Program
Free Your Fears of Flolan (Epoprostenol) (PDF) Pulmonary Vascular Nurse Practitioner, Barnes-Jewish Hospital Nurse Coordinator, Pulmonary Hypertension Center, University of Colorado Hospital
IV & SQ Remodulin (Treprostinil): Challenges and Solutions (PDF) Nurse Coordinator, Pulmonary Vascular Disease, University of Washington Medical Center Nurse Practitioner, Vera M. Wall Center for Pulmonary Vascular Disease Pulmonary Hypertension Clinical Nurse Coordinator, LDS Hospital/Pulmonary Division, Intermountain Healthcare
Inhaled Therapies: The Noninvasive Option (PDF) Abby Krichman, R.R.T.Research Associate, Manager, Pulmonary Vascular Disease Center, Duke University Johanna van Dijk, RN Baylor University Medical Center
Managing Multiple Oral Therapies (PDF) Nurse Practitioner, PENN Presbyterian Medical Center Nurse Practitioner, Massachusetts General Hospital, Pulmonary and Critical Care Coordinator, Winthrop Pulmonary Hypertension Center
Managing Other Forms of PH: Do Therapies Help these Patients? (PDF)Research Nurse Coordinator, Rhode Island Hospital Assistant Professor; Director, Pulmonary Hypertension Center Washington University Coordinator, Winthrop Pulmonary Hypertension Center
Chronic Thromboembolic Disease: When Do We Operate? (PDF) Pulmonary Hypertension Clinical Nurse Coordinator, LDS Hospital/Pulmonary Division, Intermountain Healthcare University of California, San Diego Professor of Clinical Medicine, University of California, San Diego Medical Center
Changing Tracks: Transitioning From One Therapy to Another (PDF) PAH Clinical Nurse Specialist, Greater Los Angeles Veterans Affairs/ University of California, Los Angeles Director, Pulmonary Hypertension Program, Baylor College of Medicine
PHA’s Public Policy Agenda and the Importance of Grassroots Advocacy (PDF)President, Health and Medicine Council of Washington
Conducting Clinical Research (PDF) Research Nurse, Massachusetts General Hospital Research Nurse Coordinator, Harbor University of California, Los Angeles Medical Center Director, Pulmonary Vascular Diseases Program, University of Maryland, School of Medicine
Getting Started: Strategies for Establishing or Expanding a PH Program (PDF)PH Nurse Clinician Specialist, University of Iowa Clinical Nurse Coordinator, Bryan LGH Medical Center Medical Director, Pulmonary Hypertension Program, Florida Mayo Clinic
Nurses Exerting an Influence: Certification and Beyond (PDF) Cardiothoracic Clinical Nurse Specialist, University of Minnesota Medical Center, Fairview
cosponsored by PHA
Audio & Video Podcasts
Day 1
Day 2
New PAH Scientific Updates are being added monthly.
Each update lists the most recently published articles
on pulmonary arterial hypertension, the journal it
was published in, and when possible, the article abstract.
These summaries are provided in Rich Text Format -
to follow links provided, hold down the CTRL button
while clicking.
PowerPoint Presentations
Scientific Sessions Opening , C. Gregory ElliotCharacteristics of Unstable and Stable Plaques , Paul M Ridker, MDInflammation in PH , Norbert F. Voelkel, MDGenetics of PAH , John H. Newman, MDClinical Information Needed for Approval of PAH Drugs , Salma Lemtouni, MD, MPHStress Echo in PAH, Ekkehard Gruenig, MD part A | part B
Magnetic Resonance Imaging in Pulmonary Arterial Hypertension , Valentin Fuster, MD, PhD Abstract Presentation 1049: Relationship of BMRP2 Mutations and Vasoreactivity in PAH , Eric Glissmeyer
Abstract Presentation 1050: Comparison of Sitaxentan and Bosentan in PAH Associated with CTD , Reda E. Girgis, MD
Abstract Presentation 1051: Sildenafil Improves Exercise Ability and Hemodynamics in Patients with PAH Associated with CTD , Nicholas Hill, MD
Abstract Presentation 1052: Diagnostic Criteria for IPAH do not Reflect Patients Treated for IPAH at a Referral PH Clinic , Michael McGoon, MD
Clinical Science Delivery of Intravenous Treprostinil at Low Infusion Rates Using a Miniaturized Infusion Pump in Patients with Pulmonary Arterial Hypertension (Victor Tapson, M.D., Vallerie McLaughlin, M.D., Mardi Gomberg-Maitland, M.D. M.Sc., Allison Widlitz, P.A.-C, Abby Krichman, RRT, Kevin Laliberte, Pharm.D., David Zaccardelli, Pharm.D., Robyn J. Barst, M.D.) "Unexplained” Pulmonary Hypertension and Normal Ejection Fraction in Elderly Patients: Idiopathic Pulmonary Arterial Hypertension or Diastolic Heart Failure? (Shapiro BP, Redfield MM) Calcium Channel Blockers Impair Right Atrial Contractility and Cardiac Output in Non-Responders with Chronic Pulmonary Hypertension (Zierer A., Gaynor S.L., Maniar H.S., Steendijk P., Patterson G.A., Moon M.R.) Short-Term Mortality in Patients After Single or Bilateral Lung Transplantation for Idiopathic Pulmonary Arterial Hypertension: Analysis of the UNOS Database (S.A. Daud, MD, R.R. Hachem, MD, R.D. Yusen, MD and M.M. Chakinala, MD) Diagnostic Criteria for
Clinical Science Idiopathic Pulmonary Arterial Hypertension (IPAH) Do Not Reflect Patients Treated for IPAH at a Referral Pulmonary Hypertension (PH) Clinic (McGoon M, Frantz R, Tointon S, Severson, C, Durst L, Murphy J, Krowka M, Kushwaha S, Swanson K, McCully R, Edwards B) Basic Science A Single-Nucleotide Polymorphism (-254 C to G) in the TRPC6 Gene is Associated with Idiopathic Pulmonary Arterial Hypertension (Ying Yu, Nivruthi Vangala, Judd W. Landsberg, Ann Nicholson, Lewis J. Rubin, and Jason X.-J. Yuan) Correction of rheological properties of blood in severe chronic obstructive pulmonary disease patients with pulmonary hypertension (Gavrisyuk V.K., Gumenyuk N.I., Yachnik A.I., Dzyublik Ya.A.) Rapid switch from Intravenous Epoprostenol to Intravenous Treprostinil in patients with pulmonary arterial hypertension (Sitbon O., Manes A., Jais X., Pallazini M., Humbert M., Presotto L., de Paillette L., Zaccardelli D., Davis G., Jeffs, R., Simonneau G., Galie N.) Long-term follow-up and survival of BMPR2 mutation positive vs. negative patients (J.W. Schmidt, E.W. Glissmeyer, B.S., M. Kim, BS, B.L. Wojciechowski, C.G.Elliott, MD R. Trembath, MD, J. Carlquist, PhD, M. Scholand, MD, Leppert, PhD, M. Mead, K. Ward, MD) BMPR2 Mutations Are Not Found In Portopulmonary Hypertension Patients (M. Kim, C.G. Elliott, J. Carlquist, J. Schmidt, E. Glissmeyer) Preparatory clinical study on treatment of pulmonary arterial hypertension associated with connective tissue diseases with sildenafil: experience from a Chinese academic hospital (Li Mengtao, Zeng Xiaofeng, Wang Qian, Tian Zhuang, Zhao Yan and Zhang Fengchun) Dramatic Pulmonary Vascular Changes in a Liver Transplant Candidate (Jason Golbin, D.O., M.S. and Michael J. Krowka, M.D.) Long-Term Benefits of Sildenafil Treatment on Health-Related Quality of Life in Patients With Pulmonary Arterial Hypertension (Doughty N., Collings L., Gilbert C., Pepke-Zaba J.) Hemodynamic Comparisons Between Transthoracic Doppler Echocardiography and Right Heart Catheterization in Patients with Pulmonary Hypertension and Idiopathic Pulmonary Fibrosis (Swanson KL, Utz JP, Krowka MJ) BREATHE-5: Bosentan improves hemodynamics and exercise capacity in the first randomized placebo-controlled trial in Eisenmenger physiology (Michael Landzberg, Maurice Beghetti, Michael Gatzoulis, John Granton, Rolf Berger, Andrea Lauer, Eleonora Chiossi, Nazzareno Galiè) Combination of Bosentan with Prostanoids or Sildenafil in Pulmonary Arterial Hypertension was Well Tolerated: Results from the TRAX Database (J. Carlsen, MD, D. G. Kiely, MD, M. M. Hoeper, MD, E. Segal, MD and M. Humbert, MD) Long-Term Safety Profile of Bosentan in Patients With Systemic Sclerosis and Pulmonary Arterial Hypertension: Results From the TRAX Database (C. P. Denton, J. Coghlan, C. M. Black, M. M. Hoeper, D. G. Kiely, J. Carlsen, E. Segal, M. Humbert) Ambrisentan Improves Exercise Capacity and Time to Clinical Worsening in Patients with Pulmonary Arterial Hypertension: Results of the ARIES-2 Study (Oudiz RJ, Olschewski H, Galiè N, Frost AE, Badesch DB, McGoon MD, McLaughlin VV, Rubin LJ and the ARIES Study Group) Do Liver Function Abnormalities With Bosentan Recur With Sitaxsentan? (T.C. Coyne, MD, K.L. Alford, RN, BS, N.S. Henry, RN, BSN, Richard Dixon, PhD) No Clinical Interaction between Sitaxsentan and Sildenafi l
(T.C. Coyne, MD and W. Kramer, PhD)Sildenafil Improves Exercise Ability and Hemodynamics in Patients With Pulmonary Arterial Hypertension Associated With Connective Tissue Disease
(Hill N., Burgess G., Badesch D., on behalf of the SUPER Study Group)Severe Chronic Pulmonary Hypertention Sheep Model by Continuous Intravenous Air Infusion Induced Pulmonary Air Embolism (Zhou X, Wang D, Castro C, Hawkins H, JB Zwischenberger)Brain Natriuretic Peptide Levels in Patients With PAH Attending the 2004 Miami PHA Meeting: Relationship to Diuretic Requirement and WHO Class (Frantz RP, Anderson Severson, CJ, Durst LA, Tointon SK, McGoon MD)Relation Between Pulmonary Arterial Pressure Response and Exercise Tolerance in Pulmonary Arterial Hypertension Patients Treated with Bosentan or Treprostinil
(Frantz R, McGoon M, Severson C, Benza R, Bourge R, Barst R, Kjellstrom B, Roettger A, Bennett T.)Hemodynamic response during six-minute walk tests and during daily living in patients with pulmonary arterial hypertension
(Kjellstrom B, Frantz R, McGoon M, Severson C, Benza R, Bourge R, Barst R, Roettger A, Bennett T.)Comparison of Sitaxentan and Bosentan in Pulmonary Arterial Hypertension Associated with Connective Tissue Diseases (K. B. Highland, C. Strange, R. Girgis, C. Black)Ambulatory right ventricular and pulmonary artery pressure response to treatment with bosentan and treprostinil in patients with pulmonary arterial hypertension
(McGoon M, Frantz R, Severson C, Benza R, Bourge R, Barst R, Kjellstrom B, Roettger A, Bennett T.)Relationship of BMRP2 Mutations and Vasoreactivity in Pulmonary Arterial Hypertension (Eric W. Glissmeyer, BS, Gregory T. Havlena, BS, John Carlquist, PhD, Jason T. McKinney, MS, Stuart Rich, MD, Michael D. McGoon, MD, Mary Beth Scholand, MD, Miryoung Kim, BS, Robert L. Jensen, PhD, Jon W. Schmidt, Kenneth Ward, MD, C. Gregory Elliott, MD)Single Center Experience with Rapid Dose Escalation of Sub-Cutaneous (SQ) Treprostinil for the Treatment of Pulmonary Arterial Hypertension (Soto F.J., Derksen T., Janusz J., Presberg K.)Changes in V Wave During a Nitric Oxide Vasodilator Trial for Pulmonary Hypertension: Unmasking Left Heart Disease (Soto F.J., Zouras W., Siegel R., Cinquegrani M., Marks D., Kleczka J., Presberg K., Woods T.)Estimation of Cardiac Index in Patients with Pulmonary Hypertension: Thermal Dilution vs. Fick's Method (Soto F.J., Kleczka J., Siegel R., Presberg K., Marks D.)PRX-08066: A Potent 5-HT2B Receptor Antagonist with a dual disease modifying/vasodilating mechanism for the Treatment of Pulmonary Hypertension (Sharon Shacham, Pini Orbach, Yael Marantz, Sekar Reddy, Joseph Rutkowski, Oren Becker, Luc Maroteaux, Michael Ward, Hwee Teoh, Jean-Marie Launay, Nicolas Delacotte, Georges-Alexendre Guérin and Silvia Noiman)Right Index of Myocardial Performance (RIMP) as a Predictor of Death or Lung/Heart Transplantation in Patients with Pulmonary Hypertension (PH) (Abhayaratna WP, Tsang TS, Abhayaratna K, Ness S-A, Wiste JA, Barnes ME,
Murphy JG.)Left Atrial Volume as an Indicator of Left Heart Disease in Patients with Pulmonary Hypertension (Abhayaratna WP, Tsang TS, Abhayaratna K, Ness S-A, Wiste JA, Barnes ME,
Murphy JG.)Efficacy of Sildenafil Treatment and Improved Survival After 1 Year in Patients With Pulmonary Arterial Hypertension (PAH) (McLaughlin, V., Chong, C., Collings, L. Rubin, L.J., on behalf of the SUPER Study Group)R egistry to EV E A L REVEAL Registry™) (McGoon M., Barst R., Badesch, D., Benza, R, Doyle, R., Frost, A., Elliot G., Farber, H., Krichman, A, Pennington, J., Feldkircher, K.)Etiology specific reductions in pulmonary endothelial angiotensin converting enzyme activity in patients with PAH and Chronic Thromboembolic PH (Langleben D., Orfanos S., Giovinazzo M., Hirsch A., Catravas J.)One Year Experience with Intravenous Treprostinil in Pulmonary Arterial Hypertension (PAH) Patients (VV McLaughlin, RJ Barst, M Gomberg-Maitland, VF Tapson, A Krichman, AC Widlitz, RL Benza)Quercetin Represses NADPH oxidase subunit p22phox Gene Expression: A Molecular Mechanism to Control ROS-Linked Functions and Dysfunction (Grenett H.E., Olave N.C., Pasten M.C., Benza R.L., Wolkowicz P.E.)Acute Vasoreactivity and Genetic Polymorphisms in IPAH/FPAH Children and Adults (Rosenzweig EB, Morse J, Khan M, Diamond B, Knowles J, Barst RJ)Pulmonary Hypertension in Children with Sickle Cell Disease (Muhammad A Khan, MD, Erika Berman Rosenzweig, MD, Robyn J Barst, MD, Margaret T Lee, MD, Tania Small, Mitchell S Cairo, MD and Sujit S Sheth, MD)Combination Therapy with Sildenafil and Bosentan and Epoprostenol Weaning in Patients with Pulmonary Arterial Hypertension (Robert P. Frantz, MD, Thomas A. DeZiel RN, Luanne J. Koenig RN, Michael D. McGoon MD)The effects of eNOS deficiency on pulmonary vascular remodeling in pulmonary hypertension (Vanderpool R, Tuchscherer H, Kersten E, Kobs R, Chesler N.)Effect of Prostacyclins on Thrombocytopenia in Portopulmonary Hypertension (Jason M. Golbin, D.O. and Michael J. Krowka, M.D.)Right Heart Catheterization is Necessary to Confirm and Further Characterize Portopulmonary Hypertension
(Krowka MJ, Swanson KL, Frantz RP, McGoon MD)The Plasma Proteome in Pediatric Idiopathic Pulmonary Arterial Hypertension: A Compelling Case for Angiogenesis in Patients who Respond to Vasodilator Therapy (Davie NJ, Colvin KL, Duncan MW, Vaughn J, Fox S, Clausen L, Edwards M, Ivy DD)Endothelin-1 Up-regulates Plasminogen Activators and Plasminogen Activator Inhibitor- type1 Gene Expression in Cultured Human Pulmonary Endothelial Cells (Tabengwa E., Barchue, J, Benza, R., Pan, W.)Human Analogue BNP in the Management of Right Heart Failure due to Secondary Pulmonary Hypertension (Mohammad N Jameel, MD and Monica M Colvin-Adams, MD)Ultrafiltration: A Safe and Effective Treatment for Right Ventricular Failure due to Pulmonary Hypertension (Monica M. Colvin-Adams, Mohammed N. Jameel, Andrew J. Boyle, Emil Missov, Anne Taylor, Leslie W. Miller)Successful Liver Transplantation Following Medical Management of Portopulmonary Hypertension; a Single Center Series (Norman L. Sussman, MD, Vaidehi Kaza, MD, Neal R. Barshes, MD, Rise J. Stribling, MD, John A. Goss, MD, Christine A. O’Mahony, MD, Q. Ed Zhang, MD, PhD, John M. Vierling, MD, and Adaani E. Frost, MD)B-type natriuretic peptide (BNP) response to six-minute walk test (6MWT) in Pulmonary Arterial Hypertension (PAH) (Safdar Z., Frost A E.)Pulmonary vascular adaptations in swine with secondary pulmonary hypertension after myocardial infarction- role of endothelin (B. Houweling, D. Merkus, F. Boomsma, D.J. Duncker)
rhVEGF Treatment Preserves Pulmonary Vascular Reactivity and Structure in an Experimental Model of Pulmonary Hypertension in Fetal Sheep (Theresa R. Grover, Thomas A. Parker, Neil E. Markham, Steven H. Abman)
Cognitive Impairment is Common in Patients with Pulmonary Arterial Hypertension (Darren Taichman, Rose Biester, Gail Mancini, Michael Harhay, John Hansen-Flaschen, Harold Palevsky, Ramona Hopkins, and Jason Christie)Simvastatin Potently Suppresses Rho-Kinase in Chronic Hypoxic Pulmonary Hypertension (RE Girgis, S Mozammel, HC Champion, X Peng, L Shimoda, RM Tuder, RA Johns, PM Hassoun)Elevated plasma endothelin-1 (ET1) levels are associated with death in newborns with congenital diaphragmatic hernia (CDH) (Keller RL, Tacy TA, Xu J, Hendricks-Munoz K, Hawgood S, Fineman JR)A Novel Gas Exchange Assist Device in Juvenile Porcine Acute Lung Injury (G T El-Ferzli, M.D., N Ambalavanan, M.D, A Bulger, B.S. and J B Philips III, M.D.)Role of integrins in hypoxic pulmonary hypertension: expression and mobilization of intracellular Ca2+ in rat pulmonary arterial smooth muscle cells (PASMCs) (Anita Umesh, Michael A. Thompson, Kay-Pong Yip, Eduardo N. Chini, and James S.K. Sham)Thrombospondin-1 in Hypoxic Pulmonary Vascular Remodeling (Ochoa C.D; Hasak S.L; Al-Ansari E.; Yu L; Hales C.A. and Quinn D.A.)The bone morphogenetic protein type II receptor regulates bone morphogenetic protein signaling and function in pulmonary vascular smooth muscle cells (Yu P.B., Deng D.Y., Beppu H.B., Kawai N.K., Ichinose F., Bloch K.D.)
PAH:
Diagnosis and Treatment (including combination)
(Michael McGoon, MD) Right
Heart Catheterization and Pulmonary Hemodynamics
(Hunter Champion, MD)
Congenital
Heart Disease (Robyn J. Barst, MD; Allison
Widlitz, MS, PA-C)
Genomics of PAH (Greg Elliott ,
MD, MACP; Lisa Wheeler ,
MT)
Scleroderma (Debbie McCloskey ,
RN; James R. Seibold ,
MD)
Chronic Thromboembolic PH (Maureen Cavanagh ,
RN; Kim Kerr ,
MD, FCCP)
“Pulmonary
Hypertension: An Emerging Picture” (Janet
Croft, PhD) Psychosocial Issues of Chronically Ill Patients,
End of Life Issues, and Planning (Paul Fairman ,
MD; Natalie Kitterman ,
RN, BSN)
Pediatric PH (Aimee Doran, RN, MS, CPNP; Jeffrey
A. Feinstein ,
MD, MPH; Monica Horn ,
RN, CCRN, CCTC)
Liver
Disease (Harold Palevsky, MD; Karen Swanson,
DO) IV Flolan (Chris Archer-Chicko ,
MSN, CRNP; Paul Fairman ,
MD; Rhonda Groebner ,
RN, MSN, ANP; Janet Pinson ,
NP)
IV
Remodulin (James A. Lee, Jr., RN, BSN; Vallerie
V. McLaughlin, MD, FACC, FAHA, FCCP; Ginger Ward,
RN; Allison Widlitz, MS, PA-C) SQ Remodulin (Wendy Hill, NP; Martha Kingman ,
FNP-C; Michael McGoon ,
MD)
Inhaled
Iloprost (Carrie Melegari, RN, BSN; Harold
Palevsky, MD; Helena Purl, RN, BSN, CCRC) Managing
Multiple Oral Therapies (Joy Beckmann, RN,
MSN; Greg Elliott, MD, MACP; Stephanie Harris, RN,
BSN; Lian Latham, BA, RN) Managing
other forms of PH (PVOD, COPD, ILD, Sleep Apnea)
(Paul Fairman, MD; Harold Palevsky, MD) Establishing
and Expanding a PH Program (Ann Gihl, RN,
BSN; Abby Krichman, RRT) Anesthetic
Concerns (Greg Elliott, MD, MACP; Michael
McGoon, MD; Vallerie V. McLaughlin, MD, FACC, FAHA,
FCCP) New
Areas of Research in PAH (Vallerie V. McLaughlin,
MD, FACC, FAHA, FCCP) The
Anatomy of Medical Mistakes: Keeping PH Patient
Safe(r) (Bruce Gordon, Pharm D) Insurance
Issues and How to Access Patient Assistance Programs
(Penni Potter, JD) Managing Unique Patient Situations (Joy Beckmann ,
RN, MSN; Raquel Clayton, RN, BSN; Susie McDevitt ,
RN, MSN, ACNP)
Line
Care (Nadine Nakazawa-Carpol, RN, BS, OCN) Good Clinical Practices in Clinical Trials (Greg
Elliott ,
MD, MACP; Deborah McCollister ,
RN, BSN)
Assessing
Quality of Life in PAH (Natalie Doughty,
RN) “Caring
for the Caregiver” (James Avery, MD)
Presentation Summaries:
Abstracts Chosen for Oral
Presentation:
Abstracts:
Comparing the prevalence of
acute vasoresponsiveness using three different
definitions in patients with pulmonary arterial
hypertension. Long-Term Efficacy of Continuous Subcutaneous
Infusion of Treprostinil in Pulmonary Arterial
Hypertension
Heart Lung Transplantation for Primary
Pulmonary Hypertension. Epidemiological Features,
Clinical
Monitoring Pulmonary Hemodynamic Responses
to Epoprostenol Infusions with an Implantable
Hemodynamic, Echocardiographic,
and Genetic Profile of PAH Related to
Scleroderma
Successful Weaning of Epoprostenol
in Severe Pulmonary Arterial Hypertension?
Neurohumoral Profiles in Pulmonary
Arterial Hypertension: Clinical,
Hemodynamic
and Echocardiographic Correlations
Monitoring Pulmonary Artery
and Right Ventricular Hemodynamic
Responses to Six-minute
Walk Tests
with an Implantable Monitoring
Device in PAH Patients
Endogenous Nitric Oxide
in Primary Pulmonary Hypertension:
Response
to
Bosentan Therapy
Chronic thromboembolic
pulmonary disease. An infrequent
cause of
pulmonary hypertension.
Pulmonary Thromboendarterectomy
as an Alternative to Heart-Lung
or Lung
Transplantation.
A single center experience.
Pediatric Lung Transplant:
Do Primary Pulmonary Hypertension
Patients
Have Worse
Outcome?
Sustained Clinical,
Functional and Hemodynamic
Benefit in Patients
with Pulmonary
Arterial
Hypertension
after One Year of Therapy
with the Selective Orally-Active
Endothelin -A Receptor
Antagonist, Sitaxsentan
Continuous Monitoring
of Pulmonary Hemodynamics
with an
Implantable
Device: Initial Experience
Range of Pulmonary
Artery-Right Heart
Pressures in Ambulatory
Patients with Pulmonanry
Arterial Intra-Aortic
Balloon Pump (IABP)
Counterpulsation
Might
Help to Bridge
Emergency Status
Weaning of
Continuously Infused
Prostacyclin
(Flolan) Following
Addition of Oral
Bosentan (Tracleer)
in Patients With
Pulmonary Arterial
Hypertension
Impact of
Pulmonary Hypertension
on the
Short-Term Morbidity
and
Mortality of
Noncardiac Surgery
Effects
of Long-term
Bosentan in
Children with Pulmonary
Arterial
Hypertension Crosstalk
between the
Bone Morphogenetic
Protein Receptor
II (BMPR-II)
and Mts1,
a novel gene
in
Pulmonary
Hypertension
Thrombin-antithrombin
(TAT) complexes
in pulmonary
hypertension
Safety
and Efficacy
of Chronic
Intravenous
Treprostinil
in Pulmonary
Arterial
Hypertension
An
Analysis
of
Employment Status,
Social
Activity,
and
Adverse Drug
Effects
after
Epoprostenol
Initiation:
Results from
the
LIFE
(Life
in
the
Flolan
Era)
Survey
Update of
the
Familial
Primary
Pulmonary
Hypertension
Registry:
New
Mutations,
Modifier
Genes,
Outcomes
in Children
with Idiopathic
Pulmonary Arterial
Hypertension
Transitioning
From Intravenous
Epoprostenol to
Subcutaneous Treprostinil
in Stable
Pulmonary Arterial
Hypertension Outpatients
Enhanced
TRPC Expression
in Pulmonary
Artery Smooth
Muscle Cells
of Patients
with
Primary Pulmonary
Hypertension
Long-Term
Treatment of
Pulmonary Arterial
Hypertension With
Treprostinil: Echocardiographic
Evaluation
of
Efficacy
Functional
analysis of
disease causing
mutations in
the bone
morphogenetic protein
type II
receptor
Successful
Management of
Pregnancy in
Six Patients
With Pulmonary
Arterial Hypertension
(PAH) Comprehensive
Mutation Scanning
in the
BMPR2 Gene
using High
Resolution Melting
Curve Analysis
Pulmonary
Endothelin-1
Clearance
in Human
Pulmonary
Arterial
Hypertension
Susceptibility
to Pulmonary
Hypertension
in
BMPR2+/-
Mice
Circulating
Mediators
in
Portopulmonary
Hypertension
Mechanisms
of Decreased
NO
in
Lungs
of
Individuals
with
Pulmonary
Arterial
Hypertension
Efficacy
of
Epoprostenol
in
Patients
with
PAH
Associated
with
Interstitial
Lung
Disease
Protocol
for
Reduction
of
Site
Pain
in
the
Administration
of
Remodulin
Brain
Natriuretic
Peptide
Levels
in
Pulmonary
Arterial
Hypertension:
An
Evolving
Role?
Transition
from
Epoprostenol
and
Treprostinil
to
the
Oral
Endothelin
Receptor
Antagonist
Bosentan
Safety
of
Bosentan
in
Pulmonary
Arterial
Hypertension
Related
to
Eisenmenger
Physiology
6MW
as
an
Efficacy
Endpoint
in
PAH
Clinical
Trials:
Demonstration
of
a
ceiling
effect Chronic
Sitaxsentan
Therapy
in
PAH
L-arginine
Plasma
Levels
correlate
with
Severity
of
Primary
Pulmonary
Hypertension
Power Point Presentations:
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