Risk stratification and treatment goals in pulmonary arterial hypertension.

Risk stratification has gained an increasing role in predicting outcomes and guiding the treatment of patients with pulmonary arterial hypertension (PAH). The most predictive prognostic factors are three noninvasive parameters (World Health Organization functional class, 6-min walk distance and natriuretic peptides) that are included in all currently validated risk stratification tools. However, suffering from limitations mainly related to reduced specificity of PAH severity, these variables may not always be adequate in isolation for guiding individualised treatment decisions. Moreover, with effective combination treatment regimens and emerging PAH therapies, markers associated with pulmonary vascular remodelling are expected to become of increasing relevance in guiding the treatment of patients with PAH. While reaching a low mortality risk, assessed with a validated risk tool, remains an important treatment goal, preliminary data suggest that invasive haemodynamics and cardiac imaging may add incremental value in guiding treatment decisions.
Competing Interests: Conflict of interest: F. Dardi reports consultancy fees from Janssen and Chiesi Farmaceutici, and payment or honoraria for lectures, presentations, manuscript writing or educational events from Janssen. A. Boucly reports grants from Acceleron, Janssen and MSD, payment or honoraria for lectures, presentations, manuscript writing or educational events from Janssen, Merck, AOP Orphan, Ferrer and AstraZeneca, and support for attending meetings from Janssen, MSD, Ferrer and AOP Orphan. R. Benza reports consultancy fees from Cereno, Gossamer and United Therapeutics, and participation on a data safety monitoring board or advisory board with Altavant. R. Frantz reports grants from NHLBI and Gossamer Bio, royalties or licences from UpToDate, consultancy fees from Gossamer Bio, Insmed, Merck and Liquidia, participation on a data safety monitoring board or advisory board with Aerovate Pharmaceuticals, leadership role with Pulmonary Hypertension Association Scientific Leadership Council, and stock (or stock options) with Merck. V. Mercurio reports consultancy fees from MSD, payment or honoraria for lectures, presentations, manuscript writing or educational events from Janssen, and support for attending meetings from Janssen and MSD. H. Olschewski reports consultancy fees from Actelion, AstraZeneca, Bayer, Boehringer, Janssen, MSD, Chiesi, GSK, Inventiva, Ferrer, Menarini and Sanofi, payment or honoraria for lectures, presentations, manuscript writing or educational events from Springer, Medupdate and Mondial, support for attending meetings from Boehringer, Menarini and MSD, participation on a data safety monitoring board or advisory board with Aerovate, Bayer, Pfizer and IQVIA, receipt of equipment, materials, drugs, medical writing, gifts or other services from Boehringer, and the following financial (or non-financial) interests: Deputy Director, Ludwig Boltzmann Institute for Lung Vascular Research, Graz. G. Rådegran reports grants from Nordic Infucare, payment or honoraria for lectures, presentations, manuscript writing or educational events from Janssen, MSD, Nodic Infucare and Orpha Care/AOP Health, and participation on a data safety monitoring board or advisory board with Janssen, MSD and Orpha Care/AOP Health. L.J. Rubin reports consultancy fees from Gossamer and SoniVie, payment for expert testimony from Sandoz, and is a member of the Organizing and Founders Committees, WSPH. M.M. Hoeper reports consultancy fees from Acceleron, Actelion, AOP Health, Bayer, Ferrer, Gossamer Bio, Janssen, Keros and MSD, and payment or honoraria for lectures, presentations, manuscript writing or educational events from Actelion, AOP Health, Bayer, Ferrer, Janssen and MSD.
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