Respiratory Trials·org

Topic

Pulmonary hypertension

Vasodilator therapy, combination strategies, and activin signalling.

Start two drugs, not one. Initial combination ambrisentan and tadalafil halved clinical failure compared with either as monotherapy in treatment-naive pulmonary arterial hypertension (AMBITION), ending sequential add-on therapy as the default.

Sotatercept works on remodelling rather than tone, and in high-risk patients already on maximum tolerated background therapy it reduced death, transplantation or hospitalisation by 76% — an effect size rare in this field, and large enough that the trial was stopped early (ZENITH).

Group 3 disease finally has a positive trial. Systemic vasodilators had repeatedly failed or harmed patients with pulmonary hypertension due to lung disease by worsening ventilation- perfusion matching. Inhaled treprostinil, delivered to ventilated lung, improved six-minute walk distance by 31 m (INCREASE) — and prompted the trial of the same drug in IPF without requiring pulmonary hypertension at all (TETON-2).

Activin signalling inhibition

Trial Year Therapy Effect Finding
ZENITH 2025 Sotatercept Sotatercept cut death, transplant or hospitalisation by three quarters in high-risk PAH

Initial combination therapy

Trial Year Therapy Effect Finding
AMBITION 2015 Ambrisentan plus tadalafil Starting two vasodilators at once halved clinical failure compared with either alone

Pulmonary hypertension in lung disease

Trial Year Therapy Effect Finding
INCREASE 2021 Inhaled treprostinil Inhaled treprostinil improved walk distance in PH due to interstitial lung disease

Key references

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