About This Activity

Activity Description and 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 CARDIO. Oakstone Publishing is accredited by the ACCME to provide continuing medical education to physicians.

After watching this activity, participants should be better able to:

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. A score of 70% or higher is needed to obtain CME credit. 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.

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CME Post-test

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Q1. Which of the following general criteria would you require a patient with CTEPH to meet before assessing them as operable?

  1. A. Patient age under 60 years old
  2. B. WHO functional class I disease
  3. C. Pulmonary vascular resistance >3 WU
  4. D. Surgical accessibility of thrombi in the main, lobar or segmental pulmonary arteries

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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.

Q2. 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?

  1. A. An increase in the 6-minute walk distance for the riociguat arm
  2. B. No change in pulmonary vascular resistance in the riociguat arm
  3. C. An increase in the 6-minute walk distance for the placebo arm
  4. D. No change in WHO functional class for the placebo arm

Please try again

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.

Q3. If your patient is assessed as inoperable, which of the following would be your choice of disease management?

  1. A. Life-long anticoagulation alone
  2. B. Riociguat and consideration of balloon pulmonary angioplasty
  3. C. Macitentan
  4. D. Diuretics and oxygen alone

Please try again

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.

Q4. How would you initiate treatment for your patient with CTEPH and who is eligible to receive riociguat?

  1. A. In patients with low blood pressure, riociguat should be administered at a fixed-dose throughout the course of the treatment
  2. B. Individualized dose-adjustment scheme at treatment initiation
  3. C. Treatment should be initiated at the maximum dose of riociguat and then adjusted down following adverse events

Please try again

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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Medical therapy for CTEPH:
what is the standard of care in 2020?