Ground Glass Opacities in Pulmonary Arterial Hypertension-Results from the PVDOMICS Study.

RATIONALE AND OBJECTIVES

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. We sought to characterise the clinical features and outcomes of Group 1 PH patients with and without GGOs in the PVDOMICS study, a prospective multicentre cohort study aimed at deep phenotyping PH.

STUDY DESIGN AND METHODS

Incident and prevalent PH patients were enrolled across 7 US centres. We included Group 1 PH patients and excluded those with parenchymal lung disease or without chest imaging, resulting in a cohort of 242 patients.

RESULTS

GGOs were common among Group 1 PH patients (43% prevalence), associated with female sex, younger age, prostanoid use, longer disease duration and were more common among patients with familial PAH (FPAH) 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 [HR 2.49 (95% CI 1.43-4.32, p = 0.001)] and had independent prognostic value for predicting transplant-free survival after adjusting for European Society of Cardiology (ESC)/European Respiratory Society (ERS) risk stratification (HR 2.19, 95% CI 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.

CLINICAL TRIAL REGISTRATION

Brief Title: Pulmonary Vascular Disease Phenomics Program (PVDOMICS) Official Title: Redefining Pulmonary Hypertension Through Pulmonary Vascular Disease Phenomics (PVDOMICS) ID: NCT02980887. https://clinicaltrials.gov/study/NCT02980887?term=NCT02980887&rank=1.