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