Pulmonary Hypertension CE/CME ACCREDITED Watch Time: 33 mins

touchEXPERT OPINIONS Improving outcomes in pulmonary arterial hypertension: A multidisciplinary approach

Watch three experts share their perspectives and best-practice guidance on how to optimize the diagnosis and management of pulmonary arterial hypertension (PAH).

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Prof. Harrison Farber
Tufts Medical Center, Boston, MA, USA
Best practice for early recognition and prompt diagnosis of PAH
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In this interview Prof. Farber answers the following questions:

  • What are the causes and clinical characteristics of PAH?
  • What are the most important tools for diagnosing PAH?
  • In your opinion, what are the challenges associated with making a prompt diagnosis of PAH?
  • Who should be screened for PAH?

Prof. Harrison Farber is a professor of medicine and director of the Pulmonary Embolism Emergency Response Team (PERT) and the Pulmonary Hypertension Fellowship at Tufts Medical Center, Tufts Medical Center, Boston, USA. read more

Prof. Farber’s research focuses on the response of the pulmonary vasculature to injury and endothelial cell biology. His laboratory investigates the response of the pulmonary vasculature in different aetiologies of pulmonary hypertension (PH), in particular, scleroderma, using genomic and proteomic approaches to identify unique molecules as potential therapeutic targets for PH and associated diseases. Prof. Farber serves on the steering committees for several registries for PH and pulmonary arterial hypertension (PAH) and is very active in developing risk assessment tools for both conditions.

Prof. Harrison Farber discloses: Advisory board or panel fees from Acceleron Pharma, Actelion, Aerami Therapeutics, Aerovate Therapeutics, Altavant Sciences, and United Therapeutics Corporation. Speaker’s bureau fees from Bayer.

 
Prof. Marion Delcroix
University Hospitals Leuven, Belgium
Practical management with pharmacotherapy in PAH: A patient-centric approach
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In this interview Prof. Delcroix answers the following questions:

  • What is the best approach for risk stratification in PAH?
  • How should we set treatment goals for patients with PAH?
  • What treatments are approved for PAH and which pathological pathways do they target?
  • What are the latest guideline-recommended treatment options for PAH?

Prof. Marion Delcroix is a professor of medicine and respiratory physiology at the Universities of Leuven and Kortrijk, Belgium. She is head of the pulmonary hypertension (PH) programme, in charge of the respiratory high care unit, and vice chair of the Council for Rare Diseases at the University Hospitals Leuven. read more

Prof. Delcroix’s research interests focus on pulmonary circulation and gas exchange, cardiac imaging, and the role of inflammation in the pathogenesis of PAH and chronic thromboembolic pulmonary hypertension (CTEPH). Prof. Delcroix is a founding member and chair of the International CTEPH Association. She was a core PH member of the European Reference Network-LUNG, former assembly head of the European Respiratory Society (ERS), Pulmonary Vascular Diseases Assembly, and co-chair of the severe Pulmonary Hypertension mAnagement acROSs Europe (PHAROS) ERS Clinical Research Collaboration. Prof. Delcroix was also a scientific board member of the World Symposia on Pulmonary Hypertension Association. She is a fellow of the European Society of Cardiology (ESC) and the ERS, and co-chair of the ESC/ERS 2022 PH guidelines. Prof. Delcroix was a former associate editor of the European Respiratory Journal and is currently a deputy editor of the Journal of Heart and Lung Transplantation.

Prof. Marion Delcroix discloses: Speaker or consultancy fees from Acceleron Pharma, Altavant Sciences, AOP Health, Bayer, Ferrer, Gossamer Bio, Inari Medical, Janssen, MSD and United Therapeutics. Grants/research support from Janssen.

 
Prof. Stephan Rosenkranz
University of Cologne, Germany
Optimizing pharmacotherapy in PAH
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In this interview Prof. Rosenkranz answers the following questions:

  • What novel pharmacotherapies are in development for PAH?
  • What data support the potential future use of these novel agents for PAH?
  • What is the psychosocial impact of PAH and how can patients best be supported?

Prof. Stephan Rosenkranz is an interventional cardiologist and head of the Pulmonary Hypertension Center at the University of Cologne, Germany, and also leads the Cologne Cardiovascular Research Center (CCRC). read more

Prof. Rosenkranz is a past chair of the European Society of Cardiology (ESC) Working Group on Pulmonary Circulation and Right Ventricular Function, and served as co-chair of the 2022 ESC/European Respiratory Society guidelines for pulmonary hypertension (PH). His research interests include cardiopulmonary interaction and right ventricular function in left-sided heart disease, as well as signal transduction and the biological importance of receptor tyrosine kinases and stress signalling in cardiovascular disease. He has been involved in many clinical trials in the fields of atherosclerosis, heart failure and PH, and has received numerous scientific awards.

Prof. Stephan Rosenkranz discloses: Speaker or consultancy fees from Abbott, Acceleron Pharma, Actelion, Aerovate Therapeutics, Altavant Sciences, AOP Health, AstraZeneca, Bayer, Boehringer Ingelheim, Edwards Lifesciences, Ferrer, Gossamer Bio, Janssen, MSD, United Therapeutics and Vifor Pharma. Grants/research support from AstraZeneca, Bayer and Janssen.

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Overview & Learning Objectives
Overview

Expert pulmonologists and a cardiologist discuss the importance of early diagnosis for patients with PAH, review guideline-recommended therapeutic strategies and consider strategies to improve patient outcomes.

This activity is jointly provided by USF Health and touchIME. read more

Target Audience

This activity has been designed to meet the educational needs of cardiologists, cardiology nurses, hypertension specialists, internal medicine specialists, pharmacists, pulmonologists and radiologists involved in the management of PAH.

Disclosures

USF Health adheres to the Standards for Integrity and Independence in Accredited Continuing Education. All individuals in a position to influence content have disclosed to USF Health any financial relationship with an ineligible organization. USF Health has reviewed and mitigated all relevant financial relationships related to the content of the activity.  The relevant relationships are listed below. All individuals not listed have no relevant financial relationships.

Faculty

Prof. Harrison Farber discloses: Advisory board or panel fees from Acceleron Pharma, Actelion, Aerami Therapeutics, Aerovate Therapeutics, Altavant Sciences and United Therapeutics. Speaker’s bureau fees from Bayer.

Prof. Marion Delcroix discloses: Speaker or consultancy fees from Acceleron Pharma, Altavant Sciences, AOP Health, Bayer, Ferrer, Gossamer Bio, Inari Medical, Janssen, MSD and United Therapeutics. Grants/research support from Janssen.

Prof. Stephan Rosenkranz discloses: Speaker or consultancy fees from Abbott, Acceleron Pharma, Actelion, Aerovate Therapeutics, Altavant Sciences, AOP Health, AstraZeneca, Bayer, Boehringer Ingelheim, Edwards Lifesciences, Ferrer, Gossamer Bio, Janssen, MSD, United Therapeutics and Vifor Pharma. Grants/research support from AstraZeneca, Bayer and Janssen.

Content reviewer

Christy Thai, PharmD, BCPS has no financial interests/relationships or affiliations in relation to this activity.

Touch Medical Director

Katrina Lester and Anne Nunn have no financial interests/relationships or affiliations in relation to this activity.

USF Health Office of Continuing Professional Development and touchIME staff have no financial interests/relationships or affiliations in relation to this activity.

Requirements for Successful Completion

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

If you have questions regarding credit please contact cpdsupport@usf.edu.

Accreditations

Physicians

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 a joint providership of USF Health and touchIME. USF Health is accredited by the ACCME to provide continuing medical education for physicians.

USF Health designates this enduring material for a maximum of 0.75 AMA PRA Category 1 CreditTM.  Physicians should claim only the credit commensurate with the extent of their participation in the activity.

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 (AMA). European physicians interested in converting AMA PRA Category 1 CreditTM into European CME credit (ECMEC) should contact the UEMS (www.uems.eu).

Advanced Practice Providers

Physician Assistants may claim a maximum of 0.75 Category 1 credits for completing this activity. NCCPA accepts AMA PRA Category 1 CreditTM from organizations accredited by ACCME or a recognized state medical society.

The AANPCP accepts certificates of participation for educational activities approved for AMA PRA Category 1 CreditTM by ACCME-accredited providers. APRNs who participate will receive a certificate of completion commensurate with the extent of their participation.

Date of original release: 24 April 2023. Date credits expire: 24 April 2024.

If you have any questions regarding credit please contact cpdsupport@usf.edu.

Learning Objectives

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

  • Recognize the importance of early diagnosis and rapid classification of PAH
  • Discuss the setting of effective treatment goals and implementing appropriate therapeutic strategies
  • Evaluate the data for emerging therapies and strategies for improving quality of life for patients with PAH
Faculty & Disclosures
Prof. Harrison Farber

Tufts Medical Center, Boston, MA, USA

Prof. Harrison Farber is a professor of medicine and director of the Pulmonary Embolism Emergency Response Team (PERT) and the Pulmonary Hypertension Fellowship at Tufts Medical Center, Boston, USA. read more

Prof. Farber’s research focuses on the response of the pulmonary vasculature to injury and endothelial cell biology. His laboratory investigates the response of the pulmonary vasculature in different aetiologies of pulmonary hypertension (PH), in particular, scleroderma, using genomic and proteomic approaches to identify unique molecules as potential therapeutic targets for PH and associated diseases. Prof. Farber serves on the steering committees for several registries for PH and PAH and is very active in developing risk assessment tools for both conditions.

Prof. Harrison Farber discloses

Advisory board or panel fees from Acceleron Pharma, Actelion, Aerami Therapeutics, Aerovate Therapeutics, Altavant Sciences and United Therapeutics. Speaker’s bureau fees from Bayer.

Prof. Marion Delcroix

University Hospitals Leuven, Belgium

Prof. Marion Delcroix is a professor of medicine and respiratory physiology at the Universities of Leuven and Kortrijk, Belgium. She is head of the pulmonary hypertension (PH) programme, in charge of the respiratory high care unit, and vice chair of the Council for Rare Diseases at the University Hospitals Leuven. read more

Prof. Delcroix’s research interests focus on pulmonary circulation and gas exchange, cardiac imaging, and the role of inflammation in the pathogenesis of PAH and chronic thromboembolic pulmonary hypertension (CTEPH). Prof. Delcroix is a founding member and chair of the International CTEPH Association. She was a core PH member of the European Reference Network-LUNG, former assembly head of the European Respiratory Society (ERS), Pulmonary Vascular Diseases Assembly, and co-chair of the severe Pulmonary Hypertension mAnagement acROSs Europe (PHAROS) ERS Clinical Research Collaboration. Prof. Delcroix was also a scientific board member of the World Symposia on Pulmonary Hypertension Association. She is a fellow of the European Society of Cardiology (ESC) and the ERS, and co-chair of the ESC/ERS 2022 PH guidelines. Prof. Delcroix was a former associate editor of the European Respiratory Journal and is currently a deputy editor of The Journal of Heart and Lung Transplantation.

Prof. Marion Delcroix discloses: Speaker or consultancy fees from Acceleron Pharma, Altavant Sciences, AOP Health, Bayer, Ferrer, Gossamer Bio, Inari Medical, Janssen, MSD and United Therapeutics. Grants/research support from Janssen.

Prof. Stephan Rosenkranz

University of Cologne, Germany

Prof. Stephan Rosenkranz is an interventional cardiologist and head of the Pulmonary Hypertension Center at the University of Cologne, Germany, and also leads the Cologne Cardiovascular Research Center (CCRC). read more

Prof. Rosenkranz is a past chair of the European Society of Cardiology (ESC) Working Group on Pulmonary Circulation and Right Ventricular Function, and served as co-chair of the 2022 ESC/European Respiratory Society guidelines for pulmonary hypertension (PH). His research interests include cardiopulmonary interaction and right ventricular function in left-sided heart disease, as well as signal transduction and the biological importance of receptor tyrosine kinases and stress signalling in cardiovascular disease. He has been involved in many clinical trials in the fields of atherosclerosis, heart failure and PH, and has received numerous scientific awards.

Prof. Stephan Rosenkranz discloses: Speaker or consultancy fees from Abbott, Acceleron Pharma, Actelion, Aerovate Therapeutics, Altavant Sciences, AOP Health, AstraZeneca, Bayer, Boehringer Ingelheim, Edwards Lifesciences, Ferrer, Gossamer Bio, Janssen, MSD, United Therapeutics and Vifor Pharma. Grants/research support from AstraZeneca, Bayer and Janssen.

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Question 1/5
Which of the following symptoms would indicate urgent referral for evaluation for PAH in patients experiencing dyspnoea and exercise-limiting fatigue?

PAH, pulmonary arterial hypertension.
Correct

Syncope after exercise is usually indicative of an underlying cardiac or cardiopulmonary condition, such as aortic stenosis, hypertrophic obstructive cardiomyopathy, ventricular tachycardia or PAH.1 A patient presenting with severe exercise limitation (WHO FC IV), presyncopal symptoms, syncope, or lower extremity oedema suggests advanced disease that requires urgent evaluation for PAH.2

Abbreviations

PAH, pulmonary arterial hypertension; WHO FC, World Health Organization functional class.

References

  1. Ziegelstein, RC. JAMA. 2004;292:1221–6.
  2. Edelman JD. Semin Cardiothorac Vasc Anesth. 2007;11:110–8.
Question 2/5
What factors would you take into consideration when setting treatment goals for patients with PAH?

PAH, pulmonary arterial hypertension.
Correct

The ESC/ERS guidelines recommend a risk-based, goal-oriented approach to treating patients with PAH. Individual factors such as age, sex, disease type, comorbidities and kidney function should be considered when setting goals of PAH therapy.1

 

Both the ESC/ERS and CHEST guidelines also recommend that patient values and preferences, and health-related quality of life assessments be considered.1,2

Abbreviations

CHEST, American College of Chest Physicians; ERS, European Respiratory Society; ESC, European Society of Cardiology; PAH, pulmonary arterial hypertension.

References

  1. Humbert M, et al. Eur Heart J. 2022;43:3618–731.
  2. Klinger JR, et al. Chest. 2019;155:565–86.
Question 3/5
Your 60-year-old male patient has recently been diagnosed with PAH. He has comorbid diabetes and hypertension, for which he is being treated with metformin and enalapril (an ACEi), respectively. At his initial PAH assessment, his symptoms placed him in WHO FC III. What treatment(s) would you prescribe to best manage PAH in this patient?

ACEi, angiotensin converting enzyme inhibitor; ERA, endothelin receptor antagonist; PAH, pulmonary arterial hypertension; PDE5i, phosphodiesterase 5 inhibitor; PRA, prostacyclin receptor agonist; sGC, soluble guanylate cyclase; WHO FC, World Health Organization functional class.
Correct

Patients with cardiopulmonary comorbidities tend not to respond as well to PAH medication compared with patients without comorbidities.1 The ESC/ERS guidelines suggest monotherapy with an ERA or PDE5i for treatment-naive patients with cardiopulmonary comorbidities, regardless of risk stratification.1 Registry data suggest most physicians use a PDE5i as a primary treatment for this patient group.1

The CHEST guidelines recommend monotherapy with an ERA, PDE5i or sGC stimulator for treatment-naive PAH patients with WHO FC III without progression, who are unable to tolerate combination therapy.2

Abbreviations

CHEST, American College of Chest Physicians; ERA, endothelin receptor antagonist; ERS, European Respiratory Society; ESC, European Society of Cardiology; PAH, pulmonary arterial hypertension; PDE5i, phosphodiesterase 5 inhibitor; sGC, soluble guanylate cyclase; WHO FC, World Health Organization functional class.

References

  1. Humbert M, et al. Eur Heart J. 2022;43:3618–73.
  2. Klinger JR, et al. Chest. 2019;155:565–86.
Question 4/5
In the phase III STELLAR trial, by what percentage did treatment with sotatercept reduce the risk of death or non-fatal clinical worsening events in patients with PAH vs placebo?

PAH, pulmonary arterial hypertension.
Correct

The phase III STELLAR trial (NCT04576988) investigated the efficacy and safety of subcutaneous sotatercept (n=163) vs placebo (n=160) in adult patients with symptomatic PAH (WHO FC II or III) on stable background therapy for PAH for ≥90 days. The risk of death or non-fatal clinical worsening events at 24 weeks was 84% lower with sotatercept vs placebo.

Abbreviations

PAH, pulmonary arterial hypertension; WHO FC, World Health Organization functional class.

Reference

Hoeper MM, et al. N Engl J Med. 2023:10.1056/NEJMoa2213558.

Question 5/5
When managing patients struggling with their PAH diagnosis, who report symptoms of depression or anxiety, what do you think you should offer them first?

PAH, pulmonary arterial hypertension.
Correct

There are a number of strategies to support patients with PAH, including use of empathic, hopeful communication and empowering patients through sharing awareness and knowledge of the disease.1

While nearly 40% of patients with PAH suffer a psychological disorder, not all will require psychopharmacological medication.2 Diagnostic screening tools should be employed to identify patients with PAH in need of referral for psychological assessment.1

Abbreviation

PAH, pulmonary arterial hypertension.

References

  1. Humbert M, et al. Eur Heart J. 2022;43:3618–73.
  2. Olsson KM, et al. Front Psychiatry. 2021;12:667602.
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