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Pulmonary arterial hypertension (PAH) diagnosis and management have evolved over the years. Despite advances in management that resulted in an overall improvement of disease outcomes, there remain challenges to be addressed, including more accurate disease phenotyping, earlier diagnosis with better screening, and novel therapeutic approaches. In this issue of the Journal of Investigative Medicine, Deshwal1 review the changing landscape of PAH with a special emphasis on clinical risk assessment as a tool to guide treatment options.
Further disease phenotyping is needed beyond the current Sixth World Symposium on Pulmonary Hypertension subgroups to better understand the disease and response to therapies.2 The Redefining Pulmonary Hypertension through Pulmonary Vascular Disease Phenomics program aims to compare ‘omics’ data to better phenotype patients with pulmonary vascular disease to advance a precision medicine approach.3 4 The lack of consistent response to multiple PAH-specific therapies across the PAH spectrum may be …
Contributors KE-K wrote the editorial. JDD edited and revised the editorial.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests KE-K served on advisory boards for United Therapeutics and Actelion and as consultant for Acceleron Pharma, and received institutional funded research from United Therapeutics and Actelion.
Provenance and peer review Commissioned; externally peer reviewed.
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