Reaching and keeping low-risk status is now the main treatment goal in pulmonary arterial hypertension, but the evidence on which treatments help patients get there is spread across many studies using different risk tools. A systematic review was conducted by researchers in the UK following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) 2020 statement. PubMed, Scopus, Dimensions, and the Cochrane Central Register of Controlled Trials (CENTRAL) were searched from inception to August 27, 2026, for studies of adults with Group 1 pulmonary arterial hypertension that evaluated a treatment strategy and reported a validated risk-stratification outcome. Results were synthesised narratively under the Synthesis Without Meta-analysis (SWiM) guideline, with certainty graded using Grading of Recommendations Assessment, Development and Evaluation (GRADE).
Forty-eight reports were included: 10 randomised or prospective interventional studies, 12 secondary analyses of trial data, and 26 observational cohorts.
Key messages:
- Low risk is the right goal, but hard to reach. Most patients don’t get there, even with combination therapy from the start, and those who do often don’t stay there.
- Some treatments help more. The strongest evidence for improving risk status is for stepping up prostacyclin treatments and, for patients not yet at goal, adding sotatercept.
- The first year matters most. Risk at the first annual review is the best predictor of how the disease will progress, so key treatment decisions should be made early and risk should be assessed with a validated tool at every visit.
- Low risk is not the whole story. It doesn’t yet fully reflect long-term outcomes, and it’s still unclear whether patients already at low risk benefit from more treatment.
- The tools have limits. For older patients, those with other illnesses and those with certain forms of the disease, standard risk scores may not fully capture how they are really doing.
Citation
Chaoddhary S, Yatiraju S, Chellamani A, et al. (October 18, 2026) Treatment Strategies for Achieving and Maintaining Low-Risk Status in Pulmonary Arterial Hypertension: A Systematic Review. Cureus 18(10): e118129. doi:10.7759/cureus.118129