Treatment of Pulmonary Hypertension with Interstitial Lung Disease After Inhaled Treprostinil
Clinical scenario
This protocol covers pulmonary hypertension (PH) secondary to lung disease specifically in patients with interstitial lung disease (ILD) — a sub-population in which management requires specialist oversight and individualised evaluation at a dedicated PH centre.
Previous treatment did not meet targets
Where inhaled treprostinil has been used as initial therapy in PH associated with ILD, response is evaluated at week 16. If the target goals of improving 6-minute walk distance and reducing NT-proBNP are not achieved, escalation to the next line of management is warranted.
Next-step approach
For patients with severe PH associated with ILD who have not achieved adequate response, a specific class of targeted oral agents may be considered as the next step — though only with case-by-case assessment at a PH centre.
The complete evidence-based regimen is available via the structured protocol below.
References
DOI: 10.1093/eurheartj/ehac237
- Inhaled treprostinil may be considered in patients with PH associated with ILD.
- PDE5is may be considered in patients with severe PH associated with ILD (individual decision-making in PH centres).
- 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.
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