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Tutorial

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Poll

How confident are you in treating patients with PAH?

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Not confident
   
A little confident
   
Moderately confident
   
Very confident
   

Tutorial

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Poll

Which risk stratification method do you use most often for your patients with PAH?

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2022 ESC/ERS risk score
   
COMPERA
   
REVEAL
   
Other
   

Tutorial

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Poll

Which symptom is typically noticed first by your patients with suspected PAH?

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Dizziness
   
Fatigue
   
Shortness of breath
   
Other
   
 
Clinical diagnosis of PAH: Signs and steps to diagnosis
Risk stratification of PAH: Working towards individualized treatment
Treatment algorithms for PAH: An evolving landscape
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Pulmonary Hypertension, Rare Diseases CE/CME accredited

touchIN CONVERSATION
A relaxed discussion between two faculty focussed on real world clinical issues. Useful tips below will show how to navigate the activity. Join the conversation. Close

Pulmonary arterial hypertension: A practical roadmap from diagnosis to treatment

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  • A practice aid is available for this activity in the Toolkit
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Learning Objectives

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

  • Outline the clinical features of PAH and the steps to diagnosis
  • Recognize the importance of risk stratification of patients with PAH to optimize treatment management
  • Apply current guideline-recommended treatment algorithms and emerging therapeutic agents for PAH
Overview

In this activity, two experts share their insights on the diagnosis of PAH, patient risk stratification as part of treatment decision-making and considerations for optimal patient treatment and management in an evolving treatment landscape.

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

Target Audience

Pulmonologists, cardiologists, and the multidisciplinary team involved in the management of suspected PAH.

USF Accreditation

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. Jean-Luc Vachiéry Discloses: Advisory Board or Panel fees from Acceleron (relationship terminated), Aerovate, Bayer HealthCare (relationship terminated), Boehringer Ingelheim, Enzyvant (now Sumitomo Dainippon Pharma; relationship terminated), Gossamer Bio, Insmed, Janssen, Liquidia and Merck/MSD. Consultancy fees from Acceleron (relationship terminated), Bayer HealthCare (relationship terminated), Gossamer Bio, Insmed, Janssen, Keros, Liquidia and Merck/MSD.

Prof. Manreet Kanwar Discloses: Advisory Board or Panel fees from Abbott and Abiomed. Consultancy fees from Bivacor. Speaker’s Bureau fees from Abiomed.

Content reviewer

Danielle Walker, DNP, APRN, AGNP-C has no financial interests/relationships or affiliations in relation to this activity.

Touch Medical Contributors

Johanna Barry has no financial interests/relationships or affiliations in relation to this activity.

Adriano Boasso has 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.

Nurses

USF Health is accredited as a provider of nursing continuing professional development by the American Nurses Credentialing Center’s Commission on Accreditation.

A maximum of 0.75 contact hour may be earned by learners who successfully complete this continuing nursing education activity. USF Health, the accredited provider, acknowledges touchIME as the joint provider in the planning and execution of this CNE activity.

This activity is awarded 0.75 ANCC pharmacotherapeutic contact hour.

Date of original release: 24 October 2024. Date credits expire: 24 October 2025.

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

EBAC® Accreditation

touchIME is an EBAC® accredited provider since 2023.

This programme is accredited by the European Board for Accreditation of Continuing Education for Health Professionals (EBAC®) for 0.75 hour of effective education time.

The Accreditation Council for Continuing Medical Education (ACCME®), and the Royal College of Physicians and Surgeons of Canada hold an agreement on mutual recognition on substantive equivalency of accreditation systems with EBAC®.

Through an agreement between the European Board for Accreditation of Continuing Education for Health Professionals and the American Medical Association (AMA), physicians may convert EBAC® CE credits to AMA PRA Category 1 CreditTM. Information on the process to convert EBAC® credit to AMA credit can be found on the AMA website. Other health care professionals may obtain from the AMA a certificate of having participated in an activity eligible for conversion of credit to AMA PRA Category 1 CreditTM.

Faculty Disclosure Statement/Conflict of Interest Policy

In compliance with EBAC® guidelines, all speakers/chairpersons participating in this programme have disclosed or indicated potential conflicts of interest which might cause a bias in the presentations. The Organizing Committee/Course Director is responsible for ensuring that all potential conflicts of interest relevant to the event have been mitigated and declared to the audience prior to the CME activities.

Requirements for Successful Completion

Certificates of Completion may be awarded upon successful completion of the post-test and evaluation form. If you have completed one hour or more of effective education through EBAC® accredited CE activities, please contact us at accreditation@touchime.org to receive your EBAC® CE credit certificate. EBAC® grants 1 CE credit for every hour of education completed.

Date of original release: 24 October 2024. Date credits expire: 24 October 2025.

Time to Complete: 45 minutes

If you have any questions regarding the EBAC® credits, please contact accreditation@touchime.org 

This activity is CE/CME accredited

To obtain the CE/CME credit(s) from this activity, please complete this post-activity test.

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  • Select in the video player controls bar to choose subtitle language. Subtitles available in English, French, German, Italian, Portuguese, Spanish.
  • A practice aid is available for this activity in the Toolkit
  • Downloads including slides are available for this activity in the Toolkit

Topics covered in this activity

Pulmonary Hypertension / Rare Diseases
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touchIN CONVERSATION
Pulmonary arterial hypertension: A practical roadmap from diagnosis to treatment
0.75 CE/CME credit

Question 1/5
What three symptoms are most commonly noticed first by patients with PAH?

PAH, pulmonary arterial hypertension.

In a recent study in five European countries, which included information from 1,152 physicians and 572 patients, the three symptoms of PAH that were most commonly noticed first by patients were shortness of breath (91.1%), fatigue (62.8%) and general weakness (32.2%).

Abbreviation

PAH, pulmonary arterial hypertension.

Reference

Small M, et al.Ther Adv Respir Dis. 2024;18:17534666231218886.

Question 2/5
You have a patient with unexplained dyspnoea and you suspect PAH and left heart failure. According to the 2022 ESC/ERS guidelines for the diagnosis and treatment of PH what should your next steps be to confirm the diagnosis?

CPET, cardiopulmonary exercise testing; CT, computed tomography; ERS, European Respiratory Society; ESC, European Society of Cardiology; PAH, pulmonary arterial hypertension; PFT, pulmonary function test; PH, pulmonary hypertension.

According to the diagnostic algorithm in the 2022 ESC/ERS guidelines for the diagnosis and treatment of PH, patients with unexplained dyspnoea and/or suspected PH should undergo echocardiogram followed by CPET when heart disease is suspected.

Abbreviations

CPET, cardiopulmonary exercise testing; ERS, European Respiratory Society; ESC, European Society of Cardiology; PH, pulmonary hypertension.

Reference

Humbert M, et al. Eur Heart J.2022;43:3618–731.

Question 3/5
Which of the following variables have a high predictive value when carrying out risk stratification in patients with PAH?

6MWD, 6-minute walking distance; BNP, brain natriuretic peptide; CPET, cardiopulmonary exercise testing; NT-proBNP, N-terminal pro-BNP; PAH, pulmonary arterial hypertension; PFT, pulmonary function test.

Several modified versions of the 2015 ESC/ERS risk-stratification tool have been developed, including the Swedish Pulmonary Arterial Hypertension Registry (SPAHR), the Comparative, Prospective Registry of Newly Initiated Therapies for PH (COMPERA), and the French PH Registry (FPHR). Other risk-stratification tools have been developed from the US REVEAL, including the REVEAL 2.0 risk score calculator, and an abridged version (REVEAL Lite 2). In all these studies, WHO-FC, 6MWD, and BNP/NT-proBNP emerged as the variables with the highest predictive value.

Abbreviations

6MWD, 6-minute walking distance; BNP, brain natriuretic peptide; CPET, cardiopulmonary exercise testing; ERS, European Respiratory Society; ESC, European Society of Cardiology; NT-proBNP, N-terminal pro-brain natriuretic peptide; PAH, pulmonary arterial hypertension; PFT, pulmonary function test; PH, pulmonary hypertension; WHO-FC, World Health Organization functional class.

Reference

Humbert M, et al. Eur Heart J. 2022;43:3618–731.

Question 4/5
Your 40 year old female patient is presenting with shortness of breath, fatigue, general weakness and dyspnoea on exertion. Upon further investigation, she also has signs of right HF, syncope, a 6MWD of 150 m and BNP of 810 ng/L. How do you interpret these observations?

6MWD, 6-minute walking distance; BNP, brain natriuretic peptide; HF, heart failure; PAH, pulmonary arterial hypertension.

The 2022 ESC/ERS guidelines for the diagnosis and treatment of PH classify a patient with PAH at high risk (defined as a 1-year mortality risk >20%) if they have clinical observations that include signs of right HF, repeated syncope, a 6MWD <165 m and BNP >800 ng/L or NT-proBNP >1,100 ng/L.

Abbreviations

6MWD, 6-minute walking distance; BNP, brain natriuretic peptide; ERS, European Respiratory Society; ESC, European Society of Cardiology; HF, heart failure; NT-proBNP, N-terminal pro-BNP; PAH, pulmonary arterial hypertension.

Reference

Humbert M, et al. Eur Heart J. 2022;43:3618–731.

Question 5/5
A patient who was recently diagnosed with PAH has WHO-FC III symptoms and evidence of rapid disease progression, and is considered to be in the high-risk strata. In addition to initial combination therapy with ambrisentan and tadalafil, assuming the patient can tolerate them, which of the following should you consider as an additional therapy option?

CCB, calcium channel blocker; PAH, pulmonary arterial hypertension; sGC, soluble guanylate cyclase; WHO-FC, World Health Organization functional class.

According to the 2019 CHEST guidelines for therapy for PAH in adults, initial treatment with a parenteral prostanoid is advised for patients with PAH who have WHO-FC III symptoms and evidence of rapid disease progression or other markers of poor clinical prognosis.1 In the 2022 ESC/ERS guidelines for the diagnosis and treatment of PH, it is recommended that patients with PAH that are in the high-risk strata have initial therapy consisting of an ERA + PDE5i and IV/SC PCA.2

Abbreviations

CHEST, American College of Chest Physicians; ERA, endothelin receptor antagonist; ERS, European Respiratory Society; ESC, European Society of Cardiology; IV, intravenous; PAH, pulmonary arterial hypertension; PCA, prostacyclin analogue; PDE5i, phosphodiesterase-5 inhibitor; PH, pulmonary hypertension; SC, subcutaneous; WHO-FC, World Health Organization functional class.

References

  1. Klinger JR, et al. Chest. 2019;155:565–86.
  2. Humbert M, et al. Eur Heart J. 2022;43:3618–731.
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