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ABSTRACT: Background
PVDOMICS (Pulmonary Vascular Disease Phenomics) is a precision medicine initiative to characterize pulmonary vascular disease (PVD) using deep phenotyping. PVDOMICS tests the hypothesis that integration of clinical metrics with omic measures will enhance understanding of PVD and facilitate an updated PVD classification.Objectives
The purpose of this study was to describe clinical characteristics and transplant-free survival in the PVDOMICS cohort.Methods
Subjects with World Symposium Pulmonary Hypertension (WSPH) group 1-5 PH, disease comparators with similar underlying diseases and mild or no PH and healthy control subjects enrolled in a cross-sectional study. PH groups, comparators were compared using standard statistical tests including log-rank tests for comparing time to transplant or death.Results
A total of 1,193 subjects were included. Multiple WSPH groups were identified in 38.9% of PH subjects. Nocturnal desaturation was more frequently observed in groups 1, 3, and 4 PH vs comparators. A total of 50.2% of group 1 PH subjects had ground glass opacities on chest computed tomography. Diffusing capacity for carbon monoxide was significantly lower in groups 1-3 PH than their respective comparators. Right atrial volume index was higher in WSPH groups 1-4 than comparators. A total of 110 participants had a mean pulmonary artery pressure of 21-24 mm Hg. Transplant-free survival was poorest in group 3 PH.Conclusions
PVDOMICS enrolled subjects across the spectrum of PVD, including mild and mixed etiology PH. Novel findings include low diffusing capacity for carbon monoxide and enlarged right atrial volume index as shared features of groups 1-3 and 1-4 PH, respectively; unexpected, frequent presence of ground glass opacities on computed tomography; and sleep alterations in group 1 PH, and poorest survival in group 3 PH. PVDOMICS will facilitate a new understanding of PVD and refine the current PVD classification. (Pulmonary Vascular Disease Phenomics Program PVDOMICS [PVDOMICS]; NCT02980887).
SUBMITTER: Hemnes AR
PROVIDER: S-EPMC9897285 | biostudies-literature | 2022 Aug
REPOSITORIES: biostudies-literature
Hemnes Anna R AR Leopold Jane A JA Radeva Milena K MK Beck Gerald J GJ Abidov Aiden A Aldred Micheala A MA Barnard John J Rosenzweig Erika B EB Borlaug Barry A BA Chung Wendy K WK Comhair Suzy A A SAA Desai Ankit A AA Dubrock Hilary M HM Erzurum Serpil C SC Finet J Emanuel JE Frantz Robert P RP Garcia Joe G N JGN Geraci Mark W MW Gray Michael P MP Grunig Gabriele G Hassoun Paul M PM Highland Kristin B KB Hill Nicholas S NS Hu Bo B Kwon Deborah H DH Jacob Miriam S MS Jellis Christine L CL Larive A Brett AB Lempel Jason K JK Maron Bradley A BA Mathai Stephen C SC McCarthy Kevin K Mehra Reena R Nawabit Rawan R Newman John H JH Olman Mitchell A MA Park Margaret M MM Ramos Jose A JA Renapurkar Rahul D RD Rischard Franz P FP Sherer Susan G SG Tang W H Wilson WHW Thomas James D JD Vanderpool Rebecca R RR Waxman Aaron B AB Wilcox Jennifer D JD Yuan Jason X-J JX Horn Evelyn M EM
Journal of the American College of Cardiology 20220801 7
<h4>Background</h4>PVDOMICS (Pulmonary Vascular Disease Phenomics) is a precision medicine initiative to characterize pulmonary vascular disease (PVD) using deep phenotyping. PVDOMICS tests the hypothesis that integration of clinical metrics with omic measures will enhance understanding of PVD and facilitate an updated PVD classification.<h4>Objectives</h4>The purpose of this study was to describe clinical characteristics and transplant-free survival in the PVDOMICS cohort.<h4>Methods</h4>Subjec ...[more]