Open AccessUnited States Pulmonary Hypertension Scientific Registry (USPHSR): rationale, design, and clinical implications
Author(s) -
Elliott C. Gregory,
Austin Eric D.,
Badesch David,
Badlam Jessica,
Benza Raymond L.,
Chung Wendy K.,
Farber Harrison W.,
Feldkircher Kathy,
Frost Adaani E.,
Poms Abby D.,
Lutz Katie A.,
Pauciulo Michael W.,
Yu Chang,
Nichols William C.
Publication year - 2019
Publication title -
pulmonary circulation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.791
H-Index - 40
ISSN - 2045-8940
DOI - 10.1177/2045894019851696
Subject(s) - medicine , pulmonary hypertension , pulmonary veno occlusive disease , disease , hormone , intensive care medicine , pediatrics
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.