Expert Consensus Opinion

Creating global unity on the early use of oral prostacyclin pathway agents (PPA) in a broad range of patients with PAH

Consensus Opinion Results

Your Next Patient With PAH And WHO FC II Or FC III Symptoms On Double Combination Therapy Could Benefit From Early Initiation Of Triple Combination Therapy[1][2][3]

A panel of 19 international PAH experts clarified and combined guidelines from around the globe on the initiation of oral PPAs in the treatment of patients with PAH and WHO FC II or FC III symptoms.[3]

The panel combined the best available scientific evidence with their expertise to identify and form a consensus on 13 patient profiles in which UPTRAVI® (selexipag) has shown to benefit treatment outcomes.[3]

In the McLaughlin et al. Consensus Opinion,[3] IPAH+ was defined as patients with idiopathic, heritable, repaired congenital heart defect, or drug- or toxin-induced PAH. The use of UPTRAVI® is not recommended for patients with WHO FC I or FC IV symptoms.[3]


Adapted from McLaughlin VV et al. 20203

*Panelists were asked questions sequentially about the appropriateness of adding an oral PPA in patients in each clinical scenario regarding clinical factors in the following order: (1) haemodynamic risk assessed with ESC/ERS thresholds, (2) hospitalisation within the past 6 months, yes or no, (3) RV function within previous month, normal, moderate/severe based on echocardiogram or MRI, (4) BNP or NT-proBNP within previous month; normal, abnormal based on local standards, (5) 6MWD within previous month ≤440 m or >440 m.[3]


Prof. McLaughlin and Prof. Gaine, two of the experts on the panel, were interviewed by the European Medical Journal. They highlighted the importance of the Consensus in making real-world treatment decisions and identifying patients that could benefit from early UPTRAVI® treatment.[4]

“This paper helps exemplify which patients are appropriate for oral PPA based on risk assessment, and I think it goes into some granular detail of even patients at lower risk that might be appropriate candidates for oral PPA.”

Expert Consensus Opinion’s author Prof. McLaughlin[4]

International experts: Bringing clarity to PAH treatment for you and your patients










V. McLaughlin

S. Gaine

N. Galiè

R. Souza

J. Coghlan

I. Lang

D. Langleben

M. Li

O. Sitbon

R. Channick

T. De Marco

H. Farber

R. Krasuski

I. Preston

R. Frantz

A. Hemnes

N. Kim

V. Tapson

A. Frost

Clinical Factors Taken Into Consideration During The Development Of The Consensus Statements

In order to develop the Consensus statements, the panel identified and agreed on 5 clinical factors commonly used to make the treatment decision on initiating an oral PPA.[3] They ranked them in order of clinical importance:

Haemodynamic Risk

Assessed with ESC/ERS thresholds

PAH-Related Hospitalisation

Within the past 6 months; yes or no

Right Ventricular (RV) Function

Within the previous month; normal, mild dysfunction or moderate/severe dysfunction based on echocardiogram or MRI

NT-proBNP/BNP Levels

Within previous month; normal, abnormal based on local standards

6-Minute Walk Distance (6MWD)

Within previous month; ≤440 m or

>440 m

Beyond Tomorrow

Why Continuous Risk Assessment Is Critical

The goal of PAH treatment is to increase the quality of life and decrease the risk profile for patients in the long term.[2] In order to achieve this, Prof. McLaughlin and Prof. Gaine stress the importance of continuous multiparameter risk assessments and taking into consideration the longer-term implications of treatment when evaluating patients with PAH.[4]

This not only allows the optimisation of care but also increases the number of patients that could be brought into the low-risk state, the recommended state according to the ESC/ERS 2015 Guidelines.[2][3][5]

“I also think at this point we’re thinking not just about short-term outcomes such as improving symptoms, we’re thinking about long-term outcomes in improving morbidity and mortality in these patients.”

Expert Consensus Opinion’s author Prof. McLaughlin[4]

You May Also Be Interested In


Details of the GRIPHON study, where the safety and efficacy of UPTRAVI® were investigated in a Phase 3 study to assess the long-term benefits for pulmonary arterial hypertension (PAH) patients.

Patient Management

Advice on how to manage your UPTRAVI® patients, including details on dosage and side effects.


Details from the 2022 ESC/ERS guidelines to help you achieve and/or maintain a low-risk status for your patients with PAH.


A list of useful links to management guidelines, scientific and patient associations, the full Summary of Product Characteristics for UPTRAVI® and the patient information leaflet.

BNP, B-type natriuretic peptide; CTD, connective tissue disease; ERS, European Respiratory Society; ESC, European Society of Cardiology; FC, functional class; IPAH, idiopathic pulmonary arterial hypertension; MRI, magnetic resonance imaging; NT-proBNP, N-terminal pro-B-type natriuretic peptide; PAH, pulmonary arterial hypertension; PPA, prostacyclin pathway agent; RV, right ventricle; 6MWD, 6-minute walk distance; WHO, World Health Organization

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