TY - JOUR T1 - Severe pulmonary hypertension associated with lung disease is characterised by a loss of small pulmonary vessels on quantitative CT JF - ERJ Open Research JO - erjor DO - 10.1183/23120541.00503-2021 SP - 00503-2021 AU - Dheyaa Alkhanfar AU - Yousef Shahin AU - Faisal Alandejani AU - Krit Dwivedi AU - Samer Alabed AU - Chris Johns AU - Allan Lawrie AU - AA Roger Thompson AU - Alexander MK Rothman AU - Juerg Tschirren AU - Johanna M Uthoff AU - Eric Hoffman AU - Robin Condliffe AU - Jim M Wild AU - David G Kiely AU - Andrew J Swift Y1 - 2022/01/01 UR - http://openres.ersjournals.com/content/early/2022/02/18/23120541.00503-2021.abstract N2 - Background Pulmonary hypertension (PH) in patients with chronic lung disease (CLD) predicts reduced functional status, clinical worsening and increased mortality, with patients with severe PH-CLD (≥35 mmHg) having a significantly worse prognosis than mild to moderate PH-CLD (21–34 mmHg). The aim of this cross-sectional study was to assess the association between computed tomography (CT) derived quantitative pulmonary vessel volume, PH severity and disease aetiology in CLD.Methods Treatment naïve patients with CLD who underwent CT pulmonary angiography, lung function testing and right heart catheterisation were identified from the ASPIRE Registry between October 2012 and July 2018. Quantitative assessments of total pulmonary vessel and small pulmonary vessel volume were performed.Results Ninety patients had PH-CLD including 44 associated with COPD/emphysema and 46 with interstitial lung disease. Patients with severe PH-CLD (n=40) had lower small pulmonary vessel volume compared to patients with mild to moderate PH-CLD (n=50). Patients with PH-ILD had significantly reduced small pulmonary blood vessel volume, compared to PH-COPD/emphysema. Higher mortality was identified in patients with lower small pulmonary vessel volume.Conclusion Patients with severe PH-CLD, regardless of aetiology, have lower small pulmonary vessel volume compared to patients with mild-moderate PH-CLD and this is associated with a higher mortality. Whether pulmonary vessel changes quantified by CT are a marker of remodelling of the distal pulmonary vasculature requires further study.FootnotesThis manuscript has recently been accepted for publication in the ERJ Open Research. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJOR online. Please open or download the PDF to view this article.Conflict of interest: Dr. Alkhanfar has nothing to disclose.Conflict of interest: Dr. Shahin has nothing to disclose.Conflict of interest: Dr. Alandejani has nothing to disclose.Conflict of interest: Dr. Dwivedi has nothing to disclose.Conflict of interest: Dr. Alabed has nothing to disclose.Conflict of interest: Dr. Johns has nothing to disclose.Conflict of interest: Dr. Lawrie has nothing to disclose.Conflict of interest: Dr. Thompson has nothing to disclose.Conflict of interest: Dr. Rothman has nothing to disclose.Conflict of interest: Dr. Tschirren has nothing to disclose.Conflict of interest: Dr. Uthoff has nothing to disclose.Conflict of interest: Dr. Hoffman has nothing to disclose.Conflict of interest: Dr. Condliffe has nothing to disclose.Conflict of interest: Dr. Wild has nothing to disclose.Conflict of interest: Dr. Kiely has nothing to disclose.Conflict of interest: Dr. Swift has nothing to disclose. ER -