Conference Guide for the Pulmonary Hypertension Association

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Lung Transplantation: From Listing to Management

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.

Saturday, June 30 2 - 3 p.m. Speakers: Deborah J. Levine, M.D. University Health System in San Antonio San Antonio

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

Dana McGlothlin, M.D. Kaiser Permanente - California San Francisco

Moderators:

Sangeeta M. Bhorade, M.D. Northwestern University Chicago

Zeenat Safdar, M.D., M.S., FCCP Houston Methodist Hospital-Weill Cornell College Houston

Oksana A. Shlobin, M.D. Inova Fairfax Hospital Falls Church, Va.

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.

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.

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

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 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

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 Speaker: Namita Sood, M.D., FCCP • Increase awareness of patient assistance programs University of Texas Health Science Center at Houston • Review new therapies in the pipeline Houston Sunday, July 1 9:30 - 10:30 a.m.

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All participants seeking continuing education credit must record their attendance and complete an activity evaluation at http://pha.cds.pesgce.com. Please complete this process and print your CE certificate no later than August 15, 2018.

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 1004 1005

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

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

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 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

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

2018 PHA SCIENTIFIC SESSIONS AND EDUCATION FOR MEDICAL PROFESSIONALS

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