United States Pulmonary Hypertension Scientific Registry (USPHSR): rationale, design, and clinical implications

C Gregory Elliott; Eric D Austin; David Badesch; Jessica Badlam; Raymond L Benza; Wendy K Chung; Harrison W Farber; Kathy Feldkircher; Adaani E Frost; Abby D Poms; Katie A Lutz; Michael W Pauciulo; Chang Yu; William C Nichols

doi:10.1177/2045894019851696

United States Pulmonary Hypertension Scientific Registry (USPHSR): rationale, design, and clinical implications

Pulm Circ. 2019 Apr-Jun;9(2):2045894019851696. doi: 10.1177/2045894019851696.

Authors

Affiliations

¹ 1 Intermountain Medical Center Department of Medicine and the University of Utah, Pulmonary Division, Salt Lake City, UT, USA.
² 2 Vanderbilt University Medical Center Department of Pediatrics, Nashville, TN, USA.
³ 3 University of Colorado, Denver, CO, USA.
⁴ 4 University of Vermont, Burlington, VT, USA.
⁵ 5 Allegheny General Hospital, Pittsburgh, PA, USA.
⁶ 6 Columbia University Medical Center, New York, NY, USA.
⁷ 7 Tufts University School of Medicine, Boston, MA, USA.
⁸ 8 E Squared Trials and Registries, Inc., Half Moon Bay, CA, USA.
⁹ 9 Houston Methodist Hospital Lung Center, Houston, TX, USA.
¹⁰ 10 Division of Human Genetics, Cincinnati Children's Medical Center and Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA.
¹¹ 11 Department of Biostatistics, Vanderbilt University, Nashville, TN, USA.

Abstract

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.

Keywords: Phenotype; genotype; hormones; pulmonary hypertension; toxins.

Grants and funding

R24 HL105333/HL/NHLBI NIH HHS/United States