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United States Pulmonary Hypertension Scientific Registry: Baseline Characteristics.

Publication ,  Journal Article
Badlam, JB; Badesch, DB; Austin, ED; Benza, RL; Chung, WK; Farber, HW; Feldkircher, K; Frost, AE; Poms, AD; Lutz, KA; Pauciulo, MW; Yu, C ...
Published in: Chest
January 2021

BACKGROUND: The treatment, genotyping, and phenotyping of patients with World Health Organization Group 1 pulmonary arterial hypertension (PAH) have evolved dramatically in the last decade. RESEARCH QUESTION: The United States Pulmonary Hypertension Scientific Registry was established as the first US PAH patient registry to investigate genetic information, reproductive histories, and environmental exposure data in a contemporary patient population. STUDY DESIGN AND METHODS: Investigators at 15 US centers enrolled consecutively screened adults diagnosed with Group 1 PAH who had enrolled in the National Biological Sample and Data Repository for PAH (PAH Biobank) within 5 years of a cardiac catheterization demonstrating qualifying hemodynamic criteria. Exposure and reproductive histories were collected by using a structured interview and questionnaire. The biobank provided genetic data. RESULTS: Between 2015 and 2018, a total of 499 of 979 eligible patients with clinical diagnoses of idiopathic PAH (IPAH) or familial PAH (n = 240 [48%]), associated PAH (APAH; n = 256 [51%]), or pulmonary venoocclusive disease/pulmonary capillary hemangiomatosis (n = 3 [1%]) enrolled. The mean age was 55.8 years, average BMI was 29.2 kg/m2, and 79% were women. Mean duration between symptom onset and diagnostic catheterization was 1.9 years. Sixty-six percent of patients were treated with more than one PAH medication at enrollment. Past use of prescription weight loss drugs (16%), recreational drugs (27%), and oral contraceptive pills (77%) was common. Women often reported miscarriage (37%), although PAH was rarely diagnosed within 6 months of pregnancy (1.9%). Results of genetic testing identified pathogenic or suspected pathogenic variants in 13% of patients, reclassifying 18% of IPAH patients and 5% of APAH patients to heritable PAH. INTERPRETATION: Patients with Group 1 PAH remain predominately middle-aged women diagnosed with IPAH or APAH. Delays in diagnosis of PAH persist. Treatment with combinations of PAH-targeted medications is more common than in the past. Women often report pregnancy complications, as well as exposure to anorexigens, oral contraceptives, and/or recreational drugs. Results of genetic tests frequently identify unsuspected heritable PAH.

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Published In

Chest

DOI

EISSN

1931-3543

Publication Date

January 2021

Volume

159

Issue

1

Start / End Page

311 / 327

Location

United States

Related Subject Headings

  • Young Adult
  • United States
  • Symptom Assessment
  • Respiratory System
  • Reproductive History
  • Registries
  • Mutation
  • Middle Aged
  • Male
  • Hypertension, Pulmonary
 

Citation

APA
Chicago
ICMJE
MLA
NLM
Badlam, J. B., Badesch, D. B., Austin, E. D., Benza, R. L., Chung, W. K., Farber, H. W., … USPHSR Investigators, . (2021). United States Pulmonary Hypertension Scientific Registry: Baseline Characteristics. Chest, 159(1), 311–327. https://doi.org/10.1016/j.chest.2020.07.088
Badlam, Jessica B., David B. Badesch, Eric D. Austin, Raymond L. Benza, Wendy K. Chung, Harrison W. Farber, Kathy Feldkircher, et al. “United States Pulmonary Hypertension Scientific Registry: Baseline Characteristics.Chest 159, no. 1 (January 2021): 311–27. https://doi.org/10.1016/j.chest.2020.07.088.
Badlam JB, Badesch DB, Austin ED, Benza RL, Chung WK, Farber HW, et al. United States Pulmonary Hypertension Scientific Registry: Baseline Characteristics. Chest. 2021 Jan;159(1):311–27.
Badlam, Jessica B., et al. “United States Pulmonary Hypertension Scientific Registry: Baseline Characteristics.Chest, vol. 159, no. 1, Jan. 2021, pp. 311–27. Pubmed, doi:10.1016/j.chest.2020.07.088.
Badlam JB, Badesch DB, Austin ED, Benza RL, Chung WK, Farber HW, Feldkircher K, Frost AE, Poms AD, Lutz KA, Pauciulo MW, Yu C, Nichols WC, Elliott CG, USPHSR Investigators. United States Pulmonary Hypertension Scientific Registry: Baseline Characteristics. Chest. 2021 Jan;159(1):311–327.

Published In

Chest

DOI

EISSN

1931-3543

Publication Date

January 2021

Volume

159

Issue

1

Start / End Page

311 / 327

Location

United States

Related Subject Headings

  • Young Adult
  • United States
  • Symptom Assessment
  • Respiratory System
  • Reproductive History
  • Registries
  • Mutation
  • Middle Aged
  • Male
  • Hypertension, Pulmonary