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Junior Faculty Clinical Case Reports: Pulmonary Hypertension (Banda Sea 1)
(PH)   Poster Numbers: 1333-1336

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Primary Audience: PH
Secondary Audience: CTEPH

Session Summary: Exercise testing plays a key role in the management of PAH and CTEPH, diseases with exercise-induced symptoms. In contrast with 6MWD, CPET provides unique insights in the pathophysiology of PAH and CTEPH. In addition, the role of key variables derived from CPET on outcome may be useful in assessing treatment efficacy in clinical practice and maybe clinical trials. This symposium will review the various aspects of this technique, from pathophysiology to outcome prediction.

Chairs: Marco Guazzi, MD, PhD and William Auger, MD
 

7:00 AM	Bicycle Race: Basic Principles of CPET Lisa Mielniczuk, MD, University of Ottawa Heart Institute, Ottawa, ON, Canada
7:15 AM	Under Pressure: CPET in PAH and CTEPH Marco Guazzi, MD, PhD, IRCCS Pol San Donato, Milan, Italy
7:30 AM	Don’t Stop Me Now: CPET to Predict Outcome in PAH - Time for a Change! Jean-Luc Vachiery, MD, Erasme University Hospital, Brussels, Belgium
7:45 AM	15-min Panel Discussion

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Primary Audience: PH
Secondary Audiences: CTEPH, PATH

Session Summary: This symposium, in collaboration with the Pulmonary Vascular Research Institute (PVRI), will focus on controversies and new proposed developments under review since the recent World Symposium on Pulmonary Hypertension, exploring the concept of changing the definition to mean pulmonary artery pressure of 20mmHg and the implications for future management of patients, risk stratification of patients with PAH and best approach, and which best clinical trial endpoint should be adopted.

Chairs: Colin Church, MD, PhD and Marc A. Simon, MD
 

1:45 PM	Feeling the Pressure: New Hemodynamic Classification of Pulmonary Vascular Disease Robert P. Frantz, MD, Mayo Clinic, Rochester, MN, USA
2:00 PM	Q&A
2:05 PM	The Drugs Don’t (or might not) Work Anymore! Implications for Treatments in PAH with the New Hemodynamic Definition Mardi Gomberg-Maitland, MD, Inova Fairfax Hospital, Falls Church, VA, USA
2:20 PM	Q&A
2:25 PM	Too Risky for PAH? Patient Stratification and Implications for Treatment Paul Hassoun, MD, Johns Hopkins University, Baltimore, MD USA
2:40 PM	Q&A
2:45 PM	DEBATE: Quality Over Quantity? Future Trial Primary Endpoints Should Be QOL Focused (PRO) Ioana R. Preston, MD, Tufts Medical Center, Boston, MA, USA
3:00 PM	DEBATE: Quality over Quantity? Future Trial Primary Endpoints Should be QOL Focused (CON) Ardeschir Ghofrani, MD, University of Giessen, Giessen, Germany
3:15 PM	Debate Rebuttal (PRO) - Ioana R. Preston, MD, Tufts Medical Center, Boston, MA, USA
3:20 PM	Debate Rebuttal (CON) - Ardeschir Ghofrani, MD, University of Giessen, Giessen, Germany

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Primary Audience: PH
Secondary Audience: PHARM

Chairs: C. Dario Vizza, MD and Amresh Raina, MD
 

4:00 PM	(210) Treatment with Oral Treprostinil Delays Time to Clinical Worsening in Patients with Pulmonary Arterial Hypertension - Results from FREEDOM-EV V. F. Tapson1, C. J. Sanchez Diaz2, G. M. Bohns Meyer3, T. Pulido4, P. Sepulveda5, K. Y. Wang6, C. Q. Deng7, R. Grover7, D. Solum7, A. Ousmanou7, R. J. White8. 1Cedars Sinai, Los Angeles, CA, 2Unidad De Inv Clinica En Medicina, Monterrey, Mexico, 3Complexo Hospitalar Santa Casa de Porto Alegre, Porto Alegre, Brazil, 4Instituto Nacional de Cardiologia Ignacio Chavez, Mexico City, Mexico, 5Hospital San Juan De Dios, University of Chile, Santiago, Chile, 6Taichung Veterans General Hospital, Taichung, Taiwan, 7United Therapeutics, Research Triangle Park, NC, 8University of Rochester Medical Center, Rochester, NY
4:15 PM	(211) Risk Assessment in Functional Class (FC) II Pulmonary Arterial Hypertension (PAH) Patients: Comparison of Physician Gestalt with ESC/ERS-Guidelines and REVEAL 2.0 S. Sahay1, A. Tonelli2, T. Kung3, Z. Watson4, R. L. Benza5. 1Houston Methodist Hospital, Houston, TX, 2Cleveland Clinic, Cleveland, OH, 3Actelion Pharmaceuticals US, Inc., South San Francisco, CA, 4Putnam Associates, Boston, MA, 5Allegheny General Hospital, Pittsburgh, PA
4:30 PM	(212) Clinical Surrogates of Right Ventricular-Pulmonary Arterial Uncoupling C. G. Ireland1, R. L. Damico1, T. M. Kolb1, S. Mathai1, S. Zimmerman1, A. A. Shah1, M. Mukherjee1, F. Wigley1, P. M. Hassoun1, D. A. Kass1, R. J. Tedford2, S. Hsu1. 1The Johns Hopkins Hospital, Baltimore, MD, 2Medical University of South Carolina, Charleston, SC
4:45 PM	(213) Relationship between Race/Ethnicity and Survival in Patients with Pulmonary Arterial Hypertension (PAH) S. Medrek1, S. Sahay2, T. Kung3, C. Zhao3, A. E. Frost2. 1Division of Pulmonary and Critical Care Medicine, University of New Mexico, Albuquerque, NM, 2Houston Methodist Hospital, Houston, TX, 3Actelion, South San Francisco, CA
5:00 PM	(214) Suppressor of Tumorigenicity 2 as a Biomarker in Pulmonary Arterial Hypertension and its Association with REVEAL Risk Score in Riociguat-Treated Patients in the RESPITE Study R. L. Benza1, J. R. Klinger2, H. A. Ghofrani3, P. Jansa4, E. Grünig5, D. Vizza6, M. M. Hoeper7. 1The Cardiovascular Institute, Allegheny General Hospital, Pittsburgh, PA, 2Division of Pulmonary, Sleep, and Critical Care Medicine, Rhode Island Hospital, Alpert Medical School of Brown University, Providence, RI, 3University of Giessen and Marburg Lung Center (UGMLC), Member of the German Center for Lung Research (DZL), Giessen, Germany, 4Clinicial Department of Cardiology and Angiology, 1st Faculty of Medicine, Charles University, Prague, Czech Republic, 5Centre for Pulmonary Hypertension, Thorax Clinic at the University Hospital, Heidelberg, Germany, 6Pulmonary Hypertension Unit, Department of Cardiovascular and Respiratory Disease, La Sapienza University of Rome, Rome, Italy, 7Clinic for Respiratory Medicine, Hannover Medical School, Member of the German Center for Lung Research (DZL), Hannover, Germany
5:15 PM	(215) Long-Term Outcomes in Pulmonary Arterial Hypertension by Functional Class: A Meta-Analysis of Randomized Controlled Trials and Observational Registries N. H. Kim1, M. Fisher2, D. S. Poch1, C. Zhao3, M. Shah3, S. Bartolome4. 1University of California, San Diego, La Jolla, CA, 2Emory School of Medicine, Atlanta, GA, 3Actelion, South San Francisco, CA, 4University of Texas, Southwestern Medical Center, Dallas, TX
5:30 PM	(216) Beta Receptor Blockade Fails to Recover Ventricular Function Despite Increased Pre-Synaptic Sympathetic Nervous System Function in Experimental Pulmonary Arterial Hypertension J. Zelt, A. Ahmadi, J. Renaud, R. deKemp, R. Beanlands, L. Mielniczuk. University of Ottawa Heart Institue, Ottawa, ON, Canada

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Primary Audience: PH
Secondary Audience: HTX

Chairs: John Granton, MD and Joanna Pepke-Zaba, MD
 

6:00 PM	(489) Demographic, Hemodynamic, and HRQL Differences between Methamphetamine-Associated and Idiopathic PAH: The Pulmonary Hypertension Association Registry N. A. Kolaitis1, R. T. Zamanian2, V. A. de Jesus Perez2, D. B. Badesch3, R. L. Benza4, C. D. Burger5, M. M. Chakinala6, J. Feldman7, M. R. Lammi8, S. C. Mathai9, K. W. Presberg10, J. C. Robinson11, J. S. Sager12, O. A. Shlobin13, M. A. Simon14, S. M. Kawut15, J. P. Singer1, T. De Marco1. 1University of California, San Francisco, San Francisco, CA, 2Stanford University, Palo Alto, CA, 3University of Colorado Denver, Aurora, CO, 4Allegheny General Hospital, Pittsburgh, PA, 5Mayo Clinic Florida, Jacksonville, FL, 6Washington University in St. Louis, St. Louis, MO, 7Arizona Pulmonary Specialists, Phoenix, AZ, 8Louisiana State University, New Orleans, LA, 9Johns Hopkins University, Baltimore, MD, 10Medical College of Wisconsin, Milwaukee, WI, 11The Oregon Clinic, Portland, OR, 12Cottage Pulmonary Hypertension Center, Santa Barbara, CA, 13Inova Fairfax Medical Campus, Falls Church, VA, 14University of Pittsburgh, Pittsburgh, PA, 15University of Pennsylvania, Philadelphia, PA
6:05 PM	(490) Comparison of Outcomes of Living-Donor Lobar and Cadaveric Lung Transplantation in Patients with Pulmonary Arterial Hypertension S. Ueda, T. Chen-Yoshikawa, Y. Yokoyama, H. Oda, M. Ikeda, J. Tokuno, H. Kayawake, H. Yamagishi, F. Gochi, R. Okabe, D. Nakajima, A. Ohsumi, H. Date. Thoracic Surgery, Kyoto University Hospital, Kyoto, Japan
6:10 PM	(491) Do Pulmonary Hypertensive Patients Have Worse Post Transplant Survival Than Non-Pulmonary Hypertensive Patients? An ISHLT Registry Analysis J. Lasky1, K. Tsui2, C. Alex2, M. Dia2, E. Jweied2, P. Pappas2, C. Wigfield2. 1Rosalind Franklin University of Medicine and Science, Chicago, IL, 2Lung Transplant, Advocate Christ Medical Center, Chicago, IL
6:15 PM	(492) Exercise Pulmonary Hypertension and Progression to Resting Pulmonary Hypertension - Is TPR the Best Predictor? K. Kearney, N. Bart, V. Shah, P. Jain, E. Kotlyar, A. Keogh. St Vincents Hospital, Darlinghurst, Australia
6:20 PM	(1228) Severe Pulmonary Arterial Hypertension Treated with ABI-009, nab-Sirolimus, an mTOR Inhibitor M. Simon1, M. Gomberg-Maitland2, R. J. Oudiz3, R. Machado4, F. Rischard5, J. M. Elinoff6, B. Grigorian7, A. N. Schmid7, S. Hou7, N. Desai7, M. Gladwin1. 1Pittsburgh Heart, Lung, Blood and Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, 2MSc. Inova Heart and Vascular Institute, VCU Medicine, Falls Church, VA, 3LA Biomedical Research Institute, Harbor-UCLA Medical Center, Los Angeles, CA, 4Indiana University, Indianapolis, IN, 5Department of Medicine, University of Arizona, Tucson, AZ, 6The NIH Clinical Center, Bethesda, MD, 7Aadi Bioscience, Pacific Palisades, CA
6:25 PM	(495) Two Polymorphic Gene Loci Associated with Treprostinil Dose in Pulmonary Arterial Hypertension M. A. Psotka1, V. Thomeas-McEwing2, E. R. Gamazon3, T. Hirota4, P. N. Friedman5, A. Konkashbaev3, M. Kubo4, Y. Nakamura6, M. J. Ratain7, R. L. Benza8, N. J. Cox3, M. I. Gomberg-Maitland1, M. L. Maitland2. 1Inova Heart and Vascular Institute, Falls Church, VA, 2Center for Personalized Health, Inova Schar Cancer Institute, Falls Church, VA, 3Division of Genetic Medicine, Vanderbilt University School of Medicine, Nashville, TN, 4Center for Integrative Medical Sciences, RIKEN, Tokyo, Japan, 5Feinberg School of Medicine, Northwestern Univeristy, Chicago, IL, 6Cancer Precision Medicine Research Center, Japanese Foundation for Cancer Research, Tokyo, Japan, 7Department of Medicine, University of Chicago, Chicago, IL, 8Cardiovascular Institute, Allegheny General Hospital, Pittsburgh, PA
6:30 PM	(496) Ralinepag Plasma Levels Correlate with Improvements in Functional and Hemodynamic Parameters in Patients with Pulmonary Arterial Hypertension H. W. Farber1, N. Sood2, I. R. Preston1, J. Adams3, J. Grundy3, C. King3, P. Klassen3, V. F. Tapson4, V. McLaughlin5, R. J. Oudiz6. 1Professor of Medicine, Pulmonary, Critical Care and Sleep Division, Tufts Medical Center, Boston, MA, 2University of Texas Health Science Center of Houston, Houston, TX, 3Arena Pharmaceuticals, Inc, San Diego, CA, 4Cedars-Sinai Medical Center, Los Angeles, CA, 5Kim A. Eagle, MD, Endowed Professor of Cardiovascular Medicine, Director of Pulmonary Hypertension Program, University of Michigan, Ann Arbor, MI, 6Department of Medicine, Division of Cardiology, Los Angeles Biomedical Research Institute at Harbor-UCLA Medical Center, Torrence, CA
6:35 PM	(497) The Effects of Inhaled Sodium Nitrite on Pulmonary Vascular Impedance in Patients with Pulmonary Hypertension Associated with HFpEF M. J. Bashline1, T. N. Bachman2, N. L. Helbling3, M. T. Gladwin4, M. A. Simon3. 1Department of Internal Medicine, University of Pittsburgh Medical Center, Pittsburgh, PA, 2Department of Bioengineering, Uinversity of Pittsburgh, Pittsburgh, PA, 3Vascular Medicine Institute, UPMC Heart and Vascular Institute, Pittsburgh, PA, 4Division of Pulmonary, Allergy, and Critical Care Medicine, University of Pittsburgh Medical Center, Pittsburgh, PA

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Primary Audience: PH
Secondary Audience: CTEPH, LF/LTX

Session Summary: This symposium will explore the complex decision making in the care of patients with end stage pulmonary hypertension awaiting lung transplantation. Should patients with pulmonary hypertension ever be offered combined heart and lung transplantation? Should patients with Group 3 pulmonary hypertension be offered vasodilators as they become sicker before transplantation? Can urgency-based allocation systems be fair to patients with pulmonary hypertension?

Chairs: Paul A. Corris, MB, FRCP and Oksana A. Shlobin, MD
 

10:30 AM	CASE PRESENTATION: PAH Patient with Severe RV Dysfunction Prior to Transplant Nicholas Kolaitis, MD, UCSF Medical Center, San Francisco, CA, USA
10:35 AM	DEBATE: ‘All You Need is Lungs’: Lung Transplantation Alone is the Only Type of Organ Transplantation that Should Be Offered in Patients with End Stage PAH Jerome Le Pavec, Sr, MD. Hôpital Marie Lannelongue, Le Plessis Robinson, France
10:50 AM	DEBATE: ‘All You Need is Lungs’: Combined Heart/Lung Transplant Should Be Offered in Patients with End Stage PAH Fernando Torres, MD, UT Southwestern Medical Center, Dallas, TX, USA
11:05 AM	CASE PRESENTATION: Patient with Group 3 Disease and RV Dysfunction Awaiting Transplant Caroline M. Patterson, MD, Papworth Hospital, Cambridge, UK
11:10 AM	DEBATE: Severe PH in Advanced Lung Disease (Group 3) Should Be Treated with Vasodilators (PRO) Steven D. Nathan, MD, Inova Fairfax Hospital, Falls Church, VA, USA
11:25 AM	DEBATE: Severe PH in Advanced Lung Disease (Group 3) Should Be Treated with Vasodilators (CON) Adaani Frost, MD, Baylor College of Medicine, Houston, TX, USA
11:40 AM	CASE PRESENTATION: Patient with PAH Listed for Lung Transplant Who is Getting Sicker Errol L. Bush, MD, Johns Hopkins University, Baltimore, MD, USA
11:45 AM	DEBATE: Let It Be, Let It Be, Speaking of Urgency Allocation, Let It Be: Urgency-Based Allocation Can Be Fair for Patients with PAH (PRO) Thomas M. Egan, MD, MSC. University of North Carolina School of Medicine, Chapel Hill, NC, USA
12:00 PM	DEBATE: Let It Be, Let It Be, Speaking of Urgency Allocation, Let It Be: Urgency-Based Allocation Can Be Fair for Patients with PAH (CON) Helen M. Whitford, MBBS, FRACP, Alfred Hospital, Melbourne, Australia

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Primary Audience: PH
Secondary Audience: CTEPH, MCS

Session Summary: In collaboration with the Pulmonary Vascular Research Institute (PVRI), this symposium will provide an overview into the mechanisms, clinical diagnosis and management of chronic right heart failure in left heart disease, scleroderma, and pulmonary hypertension. There will be a focus on the identification of maladaptive remodeling with discussion of the mechanisms contributing to maladaptive remodeling in right heart failure. We will explore the relationship of right ventricular function to functional capacity and highlight a debate on exercise challenge during right heart catheterization.

Chairs: Arun Jose, MD and Marc Simon, MD
 

4:15 PM	Back to the Future 1: Mechanisms of Chronic RV Failure Ryan J. Tedford, MD, Medical University of South Carolina, Charleston, NC, USA
4:30 PM	Back to the Future 2: Metabolic Changes in the Failing Right Heart Evangelos Michelakis, MD, University of Alberta, Alberta, AB, Canada
4:45 PM	Don’t Go Breaking My Heart: Scleroderma Related - a Specific Entity Steven Hsu, MD, Johns Hopkins University, Baltimore, MD, USA
5:00 PM	When the Going Gets Tough, the Tough Get Going: Management of Acute RV Failure John Granton, MD, University of Toronto, Toronto, ON, Canada
5:15 PM	DEBATE: We Should Exercise Our PH Patients During Right Heart Catheterization (PRO) Susanna Mak, MD, PhD, Mount Sinai Hospital, Toronto, ON, Canada
5:30 PM	DEBATE: We Should Exercise Our PH Patients During Right Heart Catheterization (CON) Ivan Robbins, MD, Vanderbilt University, Nashville, TN, USA
5:45 PM	15-min Panel Discussion

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Pulmonary Hypertension and CTEPH (Gardenia 2)
(PH, CTEPH)   Poster Numbers: 1218-1244