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9.2.3 Recommendations on the use of drugs

approved for PAH in PH associated with lung disease The recommendations on the use of drugs approved for PAH in patients with PH associated with COPD and ILD have been established based on key narrative questions 6 and 7 (Supplementary Data, Sections 9.1 and 9.2, respectively). The recommendations on the use of PDE5is in patients with severe PH associated with ILD are based on PICO question III (Supplementary Data, Section 9.3). There are no direct data from RCTs on the safety, tolerability, and efficacy of PDE5is in patients with PH associated with ILD. The indirect data included in the guidelines do not enable firm conclusions to be drawn. Given the lack of robust evidence, the Task Force members felt unable to provide a recommendation for or against the use of PDE5is in patients with ILD and severe PH, and recommend that these patients are referred to a PH centre for individualized decision-making.

10 Chronic thrombo-embolic

pulmonary hypertension (group 4)

All patients whose symptoms can be attributed to post-thrombo-embolic fibrotic obstructions within the PA are

Recommendation Table 23 — Recommendations for pulmonary hypertension associated with lung disease and/or hypoxia Recommendation Table 23A

Recommendations Classa Levelb If PH is suspected in patients with lung disease, it is recommended that echocardiographyc be performed and the results interpreted in conjunction with ABG, PFTs including DLCO, and CT imaging I C In patients with lung disease and suspected PH, it is recommended to optimize treatment of the underlying lung disease and, where indicated, hypoxaemia, sleep-disordered breathing, and/or alveolar hypoventilation I C In patients with lung disease and suspected severe PH, or where there is uncertainty regarding the treatment of PH, referral to a PH centre is recommendedd I C In patients with lung disease and severe PH, an individualized approach to treatment is recommended I C

It is recommended to refer eligible patients with lung disease and PH for LTx evaluation I C

In patients with lung disease and suspected PH, RHC is recommended if the results are expected to aid management decisions I C

Inhaled treprostinil may be considered in patients with PH associated with ILD734 IIb B

The use of ambrisentan is not recommended in patients with PH associated with IPF740 III B

The use of riociguat is not recommended in patients with PH associated with IIP181 III B

The use of PAH medication is not recommended in patients with lung disease and non-severe PHe III C © ESC/ERS 2022

Recommendation Table 23B

GRADE

Recommendations Quality of

evidence Strength of

recommendation Classa Levelb

PDE5is may be considered in patients with severe PH associated with ILD (individual decision-making in PH centres) Very low Conditional IIb C The use of PDE5is in patients with ILD and non-severe PH is not recommended Very low Conditional III C © ESC/ERS 2022 ABG, arterial blood gas analysis; CT, computed tomography; DLCO, Lung diffusion capacity for carbon monoxide; IIP, idiopathic interstitial pneumonia; IPF, idiopathic pulmonary fibrosis; ILD, interstitial lung disease; LTx, lung transplantation; PAH, pulmonary arterial hypertension; PDE5i, phosphodiesterase 5 inhibitor; PFT, pulmonary function test; PH, pulmonary hypertension; RHC, right heart catheterization. aClass of recommendation. bLevel of evidence. cAssessments should ideally be made when the patient is clinically stable, as exacerbations can significantly raise pulmonary artery pressure. dThis recommendation does not apply to patients with end-stage lung disease who are not considered candidates for LTx. eThis does not include inhaled treprostinil, which may be considered in patients with PH associated with ILD, irrespective of PH severity. Downloaded from academic.oup.com/eurheartj/article/43/38/3618/6673929 by guest on 07 October 2026