Not all patients with pulmonary arterial hypertension respond to vasodilator testing — and those who do not, particularly when presenting at high risk, require a distinct and more aggressive therapeutic strategy from the outset.
This protocol applies to patients with idiopathic, heritable, or drug-associated PAH, or PAH associated with connective tissue disease, who have a negative acute pulmonary vasoreactivity test result and no cardiopulmonary comorbidities, and who are stratified at high risk of death at presentation.
In this high-risk, non-vasoreactive population, the recommended strategy involves starting with a combination of agents from multiple drug classes simultaneously — including a component that requires intravenous or subcutaneous delivery. The complete regimen, including agent selection and full clinical detail, is available in the structured protocol.
Treatment goals: Achieving and maintaining a low-risk profile on optimised medical therapy, assessed using WHO functional class, six-minute walk distance, and BNP/NT-proBNP.
DOI: 10.1093/eurheartj/ehac237
In patients with IPAH/HPAH/DPAH who present at high risk of death, initial combination therapy with a PDE5i, an ERA, and i.v./s.c. prostacyclin analogues should be considered.
In patients presenting at high risk, initial triple-combination therapy including an i.v./s.c. prostacyclin analogue should be considered.
Intravenous epoprostenol or i.v./s.c. treprostinil.
Achieving and maintaining a low-risk profile on optimized medical therapy is recommended as a treatment goal in patients with PAH.
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