Key result
New USPHSR registry establishes deep clinical and genomic phenotyping in 499 PAH patients.
Why the study?
Past registries provided important insights into PAH, PVOD, and PCH, but did not include hormonal exposures or genomic data.
Observational (n=499)
Yes
The USPHSR is a contemporary multicenter registry designed to integrate demographic, physiologic, genomic, and hormonal exposure data to improve risk prediction and understand disease mechanisms in pulmonary arterial hypertension.
USPHSR may refine PAH risk models via genomic and hormonal data; extends prior registries but leaves open clinical utility.
Diagnostic World Health Organization (WHO) Group 1 pulmonary arterial hypertension (PAH) and Diagnostic Group 1' pulmonary veno-occlusive disease (PVOD) and/or pulmonary capillary hemangiomatosis (PCH) are progressive and fatal disorders. Past registries provided important insights into these disorders, but did not include hormonal exposures or genomic data. The United States Pulmonary Hypertension Scientific Registry (USPHSR) will provide demographic, physiologic, anorexigen and hormone exposure, genomic, and survival data in the current therapeutic era for 499 patients diagnosed with PAH, PVOD, or PCH. The USPHSR also will explore the relationship between pharmacologic, non-pharmacologic, and dietary hormonal exposures and the increased risk for women to develop idiopathic or heritable PAH.
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Elliott et al. (2019) conducted an observational in Pulmonary arterial hypertension (PAH), PVOD, or PCH (n=499). Hormonal and environmental exposures, genomic characteristics vs. Matched controls (for planned case-control analyses) was evaluated on Demographics, clinical course, and survival (study design only, no results reported). The United States Pulmonary Hypertension Scientific Registry (USPHSR) is a multicenter observational study designed to collect demographic, physiologic, genomic, and hormonal exposure data on 499 patients with PAH.
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