Pulmonary Hypertension CME ACCREDITED Watch Time: 30 mins

touchPANEL DISCUSSION Medical therapy for CTEPH: What is the standard of care in 2020?

Watch a panel of world-leading experts discuss the multidisciplinary approach to the diagnosis and treatment of CTEPH, including emerging targeted medical therapies for technically inoperable patients.

Prof. Irene Lang

University of Vienna, Vienna, Austria


Prof. Nick Kim, Prof. Hiromi Matsubara

Prof. Irene Lang chairs a discussion with Prof. Nick Kim and Prof. Hiromi Matsubara exploring medical therapy approaches for CTEPH, and considers the current standard of care using case-based studies to provide practical guidance on the optimal clinical management of patients with CTEPH.

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1/4 Next Chapter
Multidisciplinary assessment of CTEPH

How are multidisciplinary approaches to CTEPH assessment and treatment decisions best employed, particularly when presented with technically inoperable cases?

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2/4 Next Chapter
Targeted medical therapy in technically inoperable patients

When should medical therapy be considered as a treatment option in patients with CTEPH?

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3/4 Next Chapter
Persistent/recurrent symptomatic disease following PEA

What strategies can be employed to clinically manage patients with persistent or recurrent CTEPH following pulmonary endarterectomy?

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Overview & Learning Objectives

In this activity, world-leading experts in CTEPH discuss medical therapy for CTEPH, and consider the current standard of care using case-based studies to provide practical guidance on the optimal clinical management of patients with CTEPH.

This activity has been jointly provided by Oakstone and touchIME RESPIRATORY. Oakstone Publishing is accredited by the ACCME to provide continuing medical education to physicians.

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Target Audience

This activity has been designed to meet the educational needs of pulmonologists, cardiologists, cardiopulmonary surgeons, rheumatologists and other healthcare professionals involved in the multidisciplinary management of patients with CTEPH, globally.


Oakstone Publishing has assessed conflict of interest with its faculty, authors, editors, and any individuals who were in a position to control the content of this CME activity. Any identified relevant conflicts of interest were resolved for fair balance and scientific objectivity of studies utilized in this activity. Oakstone Publishing’s planners, content reviewers, and editorial staff disclose no relevant commercial interests.


Prof. Lang disclosures
Academic funding and consultancy or speaker fees from Actelion, AOP Orphan Pharmaceuticals, AstraZeneca, Medtronic, Ferrer and United Therapeutics.

Prof. Kim disclosures
Research grants from Gossamer Bio, SoniVie and United Therapeutics. Speaker fees from Actelion and Bayer. Consulting services for Actelion, Bayer and Merck.

Prof. Matsubara disclosures
Speaker fees from Actelion, Bayer Yakuhin and Nippon Shinyaku.

Content Reviewer

Walter Murray Yarbrough, MD, has no financial interests/relationships or affiliations in relation to this activity.

Touch Medical Director

Paul Taylor, PhD, has no financial interests/relationships or affiliations in relation to this activity.

Requirements for Successful Completion

Oakstone Publishing designates this enduring material for a maximum of 0.5 AMA PRA Category 1 Credit™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

In order to receive credit for this activity, participants must review and complete the post-test and evaluation form. Statements of credit are awarded upon successful completion of the post-test and evaluation form.

This activity has been planned and implemented in accordance with the accreditation requirements and policies of the Accreditation Council for Continuing Medical Education (ACCME) through the joint providership of Oakstone Publishing and touchIME. Oakstone Publishing is accredited by the ACCME to provide continuing medical education for physicians.

The European Union of Medical Specialists (UEMS) – European Accreditation Council for Continuing Medical Education (EACCME) has an agreement of mutual recognition of continuing medical education (CME) credit with the American Medical Association (AME). European physicians interested in converting AMA PRA Category 1 Credit™ into European CME credit (ECMEC) should contact the UEMS (www.uems.eu).

Date of original release: 12 August 2020. Date credits expire: 12 August 2021.

Learning Objectives

After watching this touchPANEL DISCUSSION, you should be able to:

  • Discuss best practice for the multidisciplinary assessment of CTEPH
  • Apply medical therapy for inoperable patients with CTEPH
  • Perform medical therapy for patients with post-operative persistent/recurrent symptomatic disease
Faculty & Disclosures
Prof. Irene Lang

Medical University of Vienna, Vienna, Austria

Irene M. Lang is a clinical cardiologist and Professor of Vascular Biology at the Medical University of Vienna, Austria. She has been nominated as one of the World Medical Association’s Caring Physicians of the World in 2005. Prof. Lang directs an out-patient clinic for pulmonary vascular diseases and is an active interventional and structural cardiologist. She also leads basic research on the biology of vascular occlusions, with a special focus on CTEPH.

Prof. Lang is a Member of the Austrian and European societies of cardiology, member of senate of the Medical University of Vienna and Editor for Pulmonary Circulation, the European Heart Journal and Atherosclerosis.

Academic funding and consultancy or speaker fees from Actelion, AOP Orphan Pharmaceuticals, Astra-Zeneca, Medtronic, Ferrer and United Therapeutics.

Prof. Nick Kim

UC San Diego Health, CA, USA

Nick Kim, Professor of Medicine and Section Chief of Pulmonary Vascular Medicine at UC San Diego Health, USA, is a pulmonary and critical care specialist. Prof. Kim is an internationally known expert in the diagnosis and treatment of pulmonary hypertension including chronic thromboembolic pulmonary hypertension (CTEPH).
Prof. Kim’s investigative interests include clinical trials in pulmonary hypertension, pre- operative evaluation and treatment outcomes in CTEPH, and balloon pulmonary angioplasty. Prof. Kim has published extensively in peer-reviewed medical journals and has lectured worldwide on pulmonary hypertension..

Research grants from Bellerophon, Eiger, Gossamer Bio, Lung Biotechnology, and SoniVie. Speaker fees from Actelion and Bayer. Consulting services for Actelion, Bayer, Merck and United Therapeutics.

Prof. Hiromi Matsubara

National Hospital Organization Okayama Medical Center, Okayama, Japan

Hiromi Matsubara is Clinical Professor of Cardiovascular Medicine and Director of Cardiology and Clinical Science at the National Hospital Organization Okayama Medical Center, Okayama, Japan, where he practices as an interventional cardiologist. He has extensive experience in the clinical management of CTEPH and is an expert in balloon pulmonary angioplasty.

Active in clinical and translational research to improve outcomes for patients with pulmonary hypertension, Prof. Matsubara has published extensively on the clinical and physiological aspects of pulmonary hypertension, including CTEPH. He sits on the Executive Board of the International CTEPH Association and the Editorial Board for Pulmonary Circulation. Prof. Matsubara is currently Director of the Japanese Pulmonary Circulation and Pulmonary Hypertension Society.

Speaker fees from Actelion, Bayer Yakuhin and Nippon Shinyaku.


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Question 1/4
Which of the following general criteria would you require a patient with CTEPH to meet before assessing them as operable?

The 2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension state that general criteria for the patient operability include preoperative WHO functional class II–IV and surgical accessibility of thrombi in the main, lobar or segmental pulmonary arteries. There is no pulmonary vascular resistance threshold or specific age of patient that can be considered to preclude surgery per se.

CTEPH, chronic thromboembolic pulmonary hypertension; ERS, European Respiratory Society; ESC, European Society of Cardiology; WHO, World Health Organization; WU, Wood units.

Galiè N, et al. Eur Respir J. 2015;46:903–75.

Question 2/4
Which of the following was a reported outcome of the phase III CHEST-1 placebo-controlled study of riociguat in patients with inoperable CTEPH or persistent or recurrent pulmonary hypertension after pulmonary endarterectomy?

The CHEST-1 trial reported 6-minute walk distance had a mean increase in the riociguat arm compared with a mean decrease in the placebo arm (least-squares mean difference, 46 m; 95% confidence interval, 25 to 67; P<0.001). Pulmonary vascular resistance decreased in the riociguat arm and increased in the placebo arm (least-squares mean difference, -246 dyn/s/cm-5; 95% confidence interval, -303 to -190; P<0.001). Riociguat was also associated with significant improvements in WHO functional class (P=0.003), whereas in the placebo arm 15% of patients moved to a lower functional class, 78% stayed in the same functional class and 7% moved to a higher functional class.

CTEPH, chronic thromboembolic pulmonary hypertension; WHO, World Health Organization.

Ghofrani HG, et al. N Engl J Med. 2013;369:319–29.

Question 3/4
If your patient is assessed as inoperable, which of the following would be your choice of disease management?

Riociguat is the currently approved medical therapy in many countries for inoperable CTEPH and its use should be considered in combination with balloon pulmonary angioplasty.1 Macitentan is being considered for potential CTEPH registration, but is currently not licensed.1 Life-long anticoagulation is recommended in all patients with CTEPH even after pulmonary endarterectomy.2 Diuretics and oxygen are recommended in cases of heart failure or hypoxemia in combination with other therapy.2

CTEPH, chronic thromboembolic pulmonary hypertension.


  1. 1 Kim NH, et al. Eur Respir J. 2019;53:1801915.
  2. 2Galiè N et al. Eur Respir J. 2015;46:903–75.
Question 4/4
How would you initiate treatment for your patient with CTEPH and who is eligible to receive riociguat?

Riociguat exposure shows pronounced interindividual variability in patients with PAH and it is, therefore, administered using an individualized dose-adjustment scheme at treatment initiation. The recommended starting dose for riociguat is 1 mg tid for 2 weeks, with tablets being taken approximately 6–8 h apart. For patients with low blood pressure, physicians may consider starting riociguat at 0.5 mg tid. The dose should then be adjusted up to a maximum of 2.5 mg tid in 0.5 mg increments, according to signs and symptoms of hypotension.

CTEPH, chronic thromboembolic pulmonary hypertension; PAH, pulmonary arterial hypertension; tid, three times daily.


  1. Bayer AG. Adempas (riociguat tablets): EU summary of product characteristics, www.ema.europa.eu/docs/en_GB/document_library/EPAR_-_Product_Information/human/002737/WC500165034.pdf (accessed July 2020).
  2. Bayer AG. Adempas (riociguat tablets), US prescribing information 2018, http://labeling.bayerhealthcare.com/html/products/pi/Adempas_PI.pdf (accessed July 2020).
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