Expert Consensus

Strongly agree 5

Strongly disagree -5

Neither agree nor disagree 0

Consensus is reached and recommendation is provided when the mean score for each statement is > 2.5 (recommended) or < -2.5 (not recommended)

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1

In PAH the regular clinical assessment must be integrated with the evaluation of right ventricular structure and function, that largely determine PAH patients prognosis.

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2

The echocardiographic assessment should not be downsized to measurements of right atrial surface, pericardial effusion and TAPSE/PAPs but should combine other parameters reflecting RV function, strongly associated with transplant-free survival and able to improve the risk stratification of PAH patients (e.g. TAPSE and degree of tricuspid regurgitation).

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3

A close follow-up is pivotal to early identify PAH patients needing treatment escalation to a third add-on therapy.

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4

The switch from ERA or PDE5-i to another oral drug of the same class is not recommended due to lack of specific head-to-head comparisons of safety.

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5

The switch from PDE5-i to riociguat should be considered as it resulted to improve hemodynamic variables, cardiac biomarkers and clinical endpoints.

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6

The REVEAL risk score, NT-proBNP and GDF-15 levels could be useful to predict response to the switch from PDE5-i to riociguat.

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7

The addition of inhaled prostacyclin analogs (iloprost and treprostinil) may be considered for PAH patients requiring therapies targeting the prostacyclin metabolic pathways although with some concerns, since studies evaluating their efficacy in patients treated with oral combination therapy are limited.

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8

Inhaled treprostinil at doses > 9 breaths per treatment session quarter in die should be considered thus in clinical practice they resulted associated to higher rate of survival and a longer time to transition to parenteral therapy compared to the lower doses tested in RCTs.

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9

The addition of oral treprostinil, preferably adopting a three times daily dosing with a 6 month target of 6 mg TID, is recommended in order to reduce the risk of clinical worsening and to improve exercise capacity and hemodynamics.

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10

The addition of selexipag may be considered for its benefit effects on morbidity/mortality event rate.

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11

Given the lack of head-to-head comparison among oral therapies targeting the prostacyclin pathway, no definitive recommendation can be provided on the best solution between selexipag and oral treprostinil.

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12

The addition of parenteral prostanoids is recommended for patients remaining at intermediate- or high- risk status despite escalation to triple oral combination therapy.

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