Abstract Winners and Presentations

Lung Transplantation: From Listing to Management

Saturday, June 30 2 - 3 p.m.

Speakers: Deborah J. Levine, M.D. University Health System in San Antonio San Antonio

Dana McGlothlin, M.D.

Kaiser Permanente - California San Francisco

Moderators:

Sangeeta M. Bhorade, M.D.

Northwestern University Chicago

Oksana A. Shlobin, M.D.

Inova Fairfax Hospital Falls Church, Va. This session will explore the entire spectrum of care for PAH patients who are candidates for lung transplantation, including indications for lung transplant, patient preparation and when to refer patients to an expert transplant center. Attendees will learn bridging strategies for PAH patients listed for transplantation, clinical management and recognition and treatment of progressive PAH and RV failure. At the conclusion of this session, participants will be able to: • Recognize when to refer patients with PAH for transplantation • Know the barriers and how to prepare patients for lung transplantation • Know how the LAS score affects PAH patients listed for transplantation • Recognize progressive PAH and RV failure and know how to treat it • Understand bridging strategies for PAH patients listed for transplantation, including the early use of ECMO

Zeenat Safdar, M.D., M.S., FCCP

Houston Methodist Hospital-Weill Cornell College Houston

Fernando Torres, M.D.

UT Southwestern Medical Center Dallas

Pediatric PH Patients: New Guidelines and Differences From Adult Care

Saturday, June 30 3:30 - 4:30 p.m.

Speakers: Erika S. Berman Rosenzweig, M.D. Columbia University Medical Center New York, N.Y.

Dunbar Ivy, M.D.

Children's Hospital Colorado Aurora, Colo. Pediatric pulmonary hypertension (PH) is organized into a clinical classification system similar to adult PH. However, important differences in developmental mechanisms and physiology, leading to unique clinical phenotypic presentations, complicate simply applying adult PH guidelines to the pediatric population. At the conclusion of this session, participants will be able to: • Provide a general overview of the most recent pediatric guidelines for diagnosis and treatment of PAH • Highlight updates in the recommendations for management of pediatric PAH • Provide a review of the similarities and differences of pediatric pulmonary hypertension care with the care of adults with PAH

Chronic Thromboembolic Pulmonary Hypertension: Cases, Controversies, Challenges and Conundrums

Sunday, July 1 8 - 9 a.m. Speakers: Josanna Rodriguez-Lopez, M.D. Massachusetts General Hospital Boston

Timothy L. Williamson, M.D.

Kansas University Hospital Kansas City, Kan. This session will be a case-based interactive session focusing on interesting and complex CTEPH cases. We will discuss the diagnostic work up, operability evaluation, options for inoperable CTEPH and some mimics of CTEPH.

At the conclusion of this session, participants will be able to: • Understand the diagnostic evaluation for a patient with suspected CTEPH • Review the different treatment options for CTEPH and inoperable CTEPH • Understand different imaging techniques used and how they can help to identify mimics of CTEPH

PAH Therapies: Insurance, Patient Assistance Programs and New Therapies on the Horizon

Sunday, July 1 9:30 - 10:30 a.m.

Speaker: Namita Sood, M.D., FCCP University of Texas Health Science Center at Houston Houston This session will very briefly review the current therapies available and the new therapies being developed. It will also discuss the insurance coverage process and the current patient assistance programs available. At the conclusion of this session, participants will be able to: •Understand the current therapies for PAH •Discuss cost and insurance challenges •Increase awareness of patient assistance programs •Review new therapies in the pipeline

BASIC SCIENCE ABSTRACTS

Associated Diseases and Conditions

1001 A Novel Paradigm and New Drug for the Management of Ductus-Dependent Congenital Heart Disease

Ye L, Li M, Ye X, Jiang C, Chen H, Zhang H, Liu J, Hong H, Yan X, Liu X

Databases and Registries

1002 Genetic Analysis of 2,592 WHO Group 1 PAH Patients Enrolled in the PAH Biobank

Lutz K, Walsworth A, Pauciulo M, Martin L, He H, Nichols W

Mechanistic Studies

1003 Bardoxolone Methyl Improves Molecular and Cellular Parameters Associated With Pulmonary Arterial Hypertension

Miller GA, Probst BL, Dulubova I, Trevino I, McCauley L, Ferguson DA, Wigley WC

1004 Endothelial and Smooth Muscle Cell Interaction Via FoxM1 Mediates Vascular Remodeling and Pulmonary Arterial Hypertension

Dai Z, Zhu MM, Peng Y, Jin H, Machireddy N, Qian Z, Zhang X, Zhao YY

1005 Gender Difference on the Cellular Level: Distinct Stress Responses in Male and Female Endothelial Cells Isolated From Mouse Lungs

Rafikova O, Zemskova M, Kurdyukov S, Rafikov R

1006 Heme-Induced Glycolytic Imbalance Contributes to Endothelial Cell Proliferation

Rafikov R, Srivastava A, Eccles C, Rafikova O

1007 Inflammatory Macrophage Expansion in Pulmonary Hypertension Depends Upon Mobilization of Blood-Borne Monocytes

Florentin J, Coppin E, Vasamsetti SB, Zhao J, Tai YY, Tang Y, Zhang Y, Watson A, Sembrat J, Rojas M, Vargas SO, Chan SY, Dutta P

1008 Insulin Receptor Substrate 2 is Decreased in Clinical Pulmonary Hypertension and its Anti-Inflammatory Role in the Pulmonary Vasculature Under Hypoxic Conditions

Yamaji-Kegan K, Johns RA

1009 Salutary Effects of Estrogen Receptor (ER)-α on the Pulmonary Vasculature in Experimental Pulmonary Hypertension (PH)

Frump AL, Yakubov B, Zeng L, Albrecht M, Essex A, Cook T, Fisher A, Martinez D, Phillips JL, Li R, Sun X, Chesler NC, Lahm T

1010 Stability of Treprostinil With Diluents and Medications

Doran A, Borg E, Rollins K, Phares K

1011 The Role of Perivascular Edema and Endothelial Barrier Function in Pulmonary Arterial Hypertension

Rafikov R, Srivastava A, Desai AA, Simon M, Rafikova O

Therapeutic Strategies

1012 The PPARγ Agonist Pioglitazone Reverses Pulmonary Arterial Hypertension and Prevents Right Heart Failure Through Fatty Acid Oxidation

Legchenko E, Chouvarine P, Borchert P, Fernandez-Gonzalez A, Snay E, Mägel L, Mitsialis SA, Meier M, Rog-Zielinska E, Kourembanas S, Jonigk D, Hansmann G

1013 Therapeutic MAb Targeting Endothelin Receptor A for Pulmonary Arterial Hypertension

Zhang C, Guo Y, Fan K, Jing S

CLINICAL SCIENCE ABSTRACTS

Associated Diseases and Conditions

1014 A Retrospective Comparison of Pulmonary Arterial Hypertension (PAH) Patients With Myositis-Related Autoantibodies to Patients With Idiopathic PAH and PAH Associated With Systemic Sclerosis

Si S, Weinmann S, Despotovic VN, McEvoy C, Chakinala MM

1015 Baseline Characteristics From a Pre-Specified Interim Analysis of a Phase IIb, Randomized, Double-Blind, Placebo-Controlled Trial of Sildenafil Added to Pirfenidone in Patients With Advanced Idiopathic Pulmonary Fibrosis and Risk of Pulmonary Hypertension

Behr J, Nathan SD, Harari S, Wuyts W, Stauffer JL, Kirchgaessler KU, Bengus M, Gilberg F, Wells AU

1016 Myositis-Related Autoantibodies and Pulmonary Arterial Hypertension (PAH): Another Sub-Type of PAH Associated With Autoimmune Conditions

Si S, Weinmann S, Despotovic VN, McEvoy C, Chakinala MM

1017 Risk of Pulmonary Hypertension in HIV Infected Patients - A Retrospective Analysis

Pyarali F, Iordanov R, Zablah G, Haque T, Parmar R, Boulanger C, Jayaweera D, Hurwitz B, Kolber M, De La Zerda D, Martinez C

Databases and Registries

1018 Advance Care Planning Completion in Pulmonary Hypertension Care Center

Rachu G, Whittenhall ME, Ventetuolo CE, Mullin CJ, Klinger JR, Allahua M, Ahearn M

1019 Baseline and Demographic Data on the First 250 Patients From SPHERE (Uptravi® [SelexiPag]: tHe usErs dRug rEgistry)

McLaughlin V, Kim NH, Chin K, Highland KB, Hemnes A, Chakinala MM, Keating M, Zhao C, Colvin J, Farber H

1020 Clinical Classification and Prevalence of Pulmonary Hypertension in Rural Minnesota

Mirfakhraie L, Dirks T, Ebnet S, Eckman P, Cabuay B, Garberich R, Reed S, Stokman P, Fenstad E

1021 Clinical, Hemodynamic, and Genetic Associations With Parenteral Prostacyclin Response in Pulmonary Arterial Hypertension

Halliday S, Faber-Eger E, Sheng Q, Xu M, Ye F, Pugh M, Robbins I, Assad T, Mosley J, West J, Brittain E, Hemnes A

1022 Electronic Medical Record (EMR) Tool for Hospitalized Patients to Facilitate Safe Management of Pulmonary Arterial Hypertension Patients on Prostacyclin Therapy

Driscoll T, Duncan M, Blakley A

1023 Health Care Costs and Resource Utilization Prior to Initiation of a Prostacyclin Receptor Agonist for Pulmonary Arterial Hypertension in a Real-World Database Representing a Large US Health Plan

Pruett J, Hull M, Elliott C, Tsang Y, Drake W

1024 Intersecting Identities and Patient Outcomes in Pulmonary Arterial Hypertension: Early Results in Derivation of a Model Using the Pulmonary Hypertension Association Registry (PHAR)

Grinnan D, Kang L, DeWilde C, Johnson D, Sager J, Badesch D, Bull T, Chakinala MM, DeMarco T, Feldman J, Fineman J, Ford J, Klinger J, McConnell J, Berman Rosenzweig E, Shlobin O, Zamanian R, Bartolome S, Elwing J, Franz R, and 9 more

1025 Medication Adherence and Risk of Hospitalization in Pulmonary Arterial Hypertension (PAH) Patients Treated With Endothelin Receptor Antagonists (ERAs) or Phosphodiesterase Type 5 Inhibitors (PDE5Is)

Cole MR, Hill JW, Lickert C, Wade RL, Drake W

1026 National Trends of Hospitalization Characteristics in Patients With Pulmonary Hypertension

Agarwal MA, Shah M, Patel B, Garg L, Khouzam RN

1027 Pulmonary Hypertension in Hereditary Hemorrhagic Telangiectasia

Harder E, Fares W

1028 Real-World Dosing and Titration of Selexipag in Patients With Pulmonary Arterial Hypertension in the SPHERE Registry (SelexiPag: tHe UsErs dRug rEgistry): Interim Analysis

Chakinala MM, Kim NH, Chin K, Farber H, Highland KB, Hemnes A, Carol Zhao C, Keating M, McLaughlin V

1029 Transitions From Inhaled, Intravenous, Subcutaneous, or Oral Prostacyclins to Selexipag: Interim Data From the SPHERE Registry (SelexiPag: tHe UsErs dRug rEgistry)

Hemnes A, Farber H, Kim NH, Chin K, Chakinala MM, Highland KB, Carol Zhao C, Keating M, McLaughlin V

1030 Treatment With Prostacyclins Reduces Hospital Readmissions Among Patients With Pulmonary Arterial Hypertension

Blanchette C, Noone J, Howden R, Classi P, Gordon K, Nelsen A

Diagnosis, Screening and Assessment

1031 Bone Morphogenetic Protein 9 is a Mechanistic Biomarker of Portopulmonary Hypertension

Nikolic I, Yung LM, Yang P, Malhotra R, Paskin-Flerlage SD, Dinter T, Bocobo GA, McNeil M, Faugno AJ, Lai CSC, Upton PD, Goumans MJ, Zamanian RT, Elliott G, Morrell NW, Chung RT, Channick RW, Roberts KE, Yu PB

1032 Changes of Plasma Angiotensin-(1-7) in Patients With Pulmonary Arterial Hypertension Due to Congenital Heart Disease Before and After Intervention Closure

Dai HL, Guang XF, Yin XL, Yang QF

1033 Digital Subtraction Pulmonary Angiography: An Old Technique With Novel Role in Children With Pulmonary Hypertension

Das BB, Mills J, Jadotte MM, Chan KC

1034 Early Pulmonary Vascular and Right Ventricular Dysfunction in Healthy Young Adults Born Prematurely

Goss KN, Beshish A, Macdonald J, Barton GP, Mulchrone AM, Chesler NC, Francois C, Wieben O, Eldridge MW

1035 Non-Invasive Ultrasonographic Cardiac Output Measurement in Pulmonary Arterial Hypertension

Matusov Y, Achamallah N, Sager JS

1036 Reliability of Systolic Pulmonary Artery Pressure by Doppler Echocardiography Compared to Right Heart Catheterization in Patients With Atrial Septal Defect: Analysis in a Large Patient Population

Dai HL, Guang XF, Yin XL, Yang QF

1037 Right Ventricular Transient Exertional Dilation (TED) in Pulmonary Hypertension – Is There Diagnostic Utility?

El-Yafawi R, Wirth J, Rancourt D, Hacobian M, Atherton D, Cohen M

1038 Risk Assessment and Prognosis Based on ESC/ERS Guidelines in Patients With Chronic Thromboembolic Pulmonary Hypertension Receiving Riociguat

Farber H, Humbert M, Hoeper MM, Busse D, Meier C, Ghofrani HA

1075 Risk Assessment and Prognosis Based on ESC/ERS Guidelines in Patients With Pulmonary Arterial Hypertension Receiving Riociguat

Humbert M, Farber H, Ghofrani HA, Busse D, Meier C, Hoeper MM