Ground-Glass Opacities in Pulmonary Arterial Hypertension—Results From the PVDOMICS Study

Authors

• Divya Padmanabhan Menon, Mayo Clinic
• Robert P. Frantz, Mayo Clinic
• Benjamin R. Gochanour, Mayo Clinic
• Gerald J. Beck, Cleveland Clinic Foundation
• Erika S. Berman-Rosenzweig, New York Medical College
• Barry A. Borlaug, Mayo Clinic
• Serpil C. Erzurum, New York Medical College
• Samar Farha, New York Medical College
• J. Emanuel Finet, New York Medical College
• Gabriele Grunig, NYU Grossman School of Medicine
• Paul M. Hassoun, Johns Hopkins University
• Anna R. Hemnes, Vanderbilt University Medical Center
• Nicholas S. Hill, Tufts Medical Center
• Evelyn M. Horn, Weill Cornell Medicine
• Jason K. Lempel, Cleveland Clinic Foundation
• Jane A. Leopold, Harvard Medical School
• Stephen C. Mathai, Johns Hopkins University
• Rahul D. Renapurkar, Cleveland Clinic Foundation
• Franz P. Rischard, The University of Arizona
• Aaron B. Waxman, Harvard Medical School
• Hilary M. Dubrock, Mayo Clinic

DOI

10.1513/AnnalsATS.202503-333OC

Journal Title

Annals of the American Thoracic Society

First Page

1863

Last Page

1873

Document Type

Article

Publication Date

12-1-2025

Department

Pediatrics

Keywords

computed tomography, imaging, pulmonary arterial hypertension, right heart failure

Disciplines

Medicine and Health Sciences

Abstract

Rationale: Pulmonary hypertension (PH) is associated with significant morbidity and mortality. Ground-glass opacities (GGOs) are common in Group 1 PH, but their clinical significance is unclear. Objectives: We sought to characterize the clinical features and outcomes of patients with Group 1 PH with and without GGOs in the PVDOMICS study, a prospective multicenter cohort study aimed at deep phenotyping PH. Methods: Patients with incident and prevalent PH were enrolled across seven U.S. centers. We included patients with Group 1 PH and excluded those with parenchymal lung disease or without chest imaging, resulting in a cohort of 242 patients. Results: GGOs were common among patients with Group 1 PH (43% prevalence), associated with female sex, younger age, prostanoid use, and longer disease duration. GGOs were more common among patients with familial pulmonary arterial hypertension and pulmonary veno-occlusive disease. GGOs were associated with established markers of disease severity, including echocardiographic (right ventricular systolic pressure and tricuspid annular plane systolic excursion), biomarkers (N-terminal pro B-type natriuretic peptide), and worse hemodynamics (higher mean pulmonary artery pressure, pulmonary vascular resistance, and pulmonary artery wedge pressure). GGOs were associated with worse transplant-free survival (hazard ratio, 2.49; 95% confidence interval =1.43–4.32; P= 0.001) and had independent prognostic value for predicting transplant-free survival after adjusting for European Society of Cardiology and European Respiratory Society risk stratification (hazard ratio, 2.19; 95% confidence interval= 1.20–3.99; P=0.01). Conclusions: Overall, GGOs were associated with specific clinical characteristics and disease phenotypes, as well as worse hemodynamics, longer disease duration, prostanoid use, and worse survival. Future studies evaluating the pathophysiology and “omic” correlates of GGOs are warranted.

Recommended Citation

Menon, D., Frantz, R., Gochanour, B., Beck, G., Berman-Rosenzweig, E., Borlaug, B., Erzurum, S., Farha, S., Emanuel Finet, J., Grunig, G., Hassoun, P., Hemnes, A., Hill, N., Horn, E., Lempel, J., Leopold, J., Mathai, S., Renapurkar, R., Rischard, F., Waxman, A., & Dubrock, H. (2025). Ground-Glass Opacities in Pulmonary Arterial Hypertension—Results From the PVDOMICS Study. Annals of the American Thoracic Society, 22 (12), 1863-1873. https://doi.org/10.1513/AnnalsATS.202503-333OC