Clinical Characteristics and Transplant-Free Survival Across the Spectrum of Pulmonary Vascular Disease

Anna R Hemnes; Jane A Leopold; Milena K Radeva; Gerald J Beck; Aiden Abidov; Micheala A Aldred; John Barnard; Erika B Rosenzweig; Barry A Borlaug; Wendy K Chung; Suzy A A Comhair; Ankit A Desai; Hilary M Dubrock; Serpil C Erzurum; J Emanuel Finet; Robert P Frantz; Joe G N Garcia; Mark W Geraci; Michael P Gray; Gabriele Grunig; Paul M Hassoun; Kristin B Highland; Nicholas S Hill; Bo Hu; Deborah H Kwon; Miriam S Jacob; Christine L Jellis; A Brett Larive; Jason K Lempel; Bradley A Maron; Stephen C Mathai; Kevin McCarthy; Reena Mehra; Rawan Nawabit; John H Newman; Mitchell A Olman; Margaret M Park; Jose A Ramos; Rahul D Renapurkar; Franz P Rischard; Susan G Sherer; W H Wilson Tang; James D Thomas; Rebecca R Vanderpool; Aaron B Waxman; Jennifer D Wilcox; Jason X-J Yuan; Evelyn M Horn; PVDOMICS Study Group

doi:10.1016/j.jacc.2022.05.038

Clinical Characteristics and Transplant-Free Survival Across the Spectrum of Pulmonary Vascular Disease

J Am Coll Cardiol. 2022 Aug 16;80(7):697-718. doi: 10.1016/j.jacc.2022.05.038.

Authors

Anna R Hemnes¹, Jane A Leopold², Milena K Radeva³, Gerald J Beck³, Aiden Abidov⁴, Micheala A Aldred⁵, John Barnard³, Erika B Rosenzweig⁶, Barry A Borlaug⁷, Wendy K Chung⁸, Suzy A A Comhair⁹, Ankit A Desai⁵, Hilary M Dubrock¹⁰, Serpil C Erzurum¹¹, J Emanuel Finet¹², Robert P Frantz⁷, Joe G N Garcia¹³, Mark W Geraci¹⁴, Michael P Gray¹⁵, Gabriele Grunig¹⁶, Paul M Hassoun¹⁷, Kristin B Highland¹⁸, Nicholas S Hill¹⁹, Bo Hu³, Deborah H Kwon¹², Miriam S Jacob¹², Christine L Jellis¹², A Brett Larive³, Jason K Lempel²⁰, Bradley A Maron², Stephen C Mathai¹⁷, Kevin McCarthy²¹, Reena Mehra²², Rawan Nawabit²³, John H Newman²⁴, Mitchell A Olman⁹, Margaret M Park¹², Jose A Ramos¹⁸, Rahul D Renapurkar²⁰, Franz P Rischard²⁵, Susan G Sherer³, W H Wilson Tang¹², James D Thomas²⁶, Rebecca R Vanderpool²⁷, Aaron B Waxman²⁸, Jennifer D Wilcox²⁹, Jason X-J Yuan³⁰, Evelyn M Horn³¹; PVDOMICS Study Group

Affiliations

¹ Division of Allergy, Pulmonary and Critical Care Medicine, Vanderbilt University Medical Center, Nashville, Tennessee, USA. Electronic address: [email protected].
² Division of Cardiovascular Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
³ Department of Quantitative Health Sciences, Cleveland Clinic, Cleveland, Ohio, USA.
⁴ Division of Cardiology, Wayne State University, Detroit, Michigan, USA.
⁵ Department of Medicine, Indiana University School of Medicine, Indianapolis, Indiana, USA.
⁶ Department of Pediatrics and Medicine, Columbia University, New York, New York, USA.
⁷ Department of Cardiovascular Medicine, Mayo Clinic, Rochester, Minnesota, USA.
⁸ Department of Pediatrics, Columbia University, New York, New York, USA.
⁹ Department of Inflammation and Immunity, Cleveland Clinic, Cleveland, Ohio, USA.
¹⁰ Department of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, Minnesota, USA.
¹¹ Lerner Research Institute, Cleveland Clinic, Cleveland, Ohio, USA.
¹² Department of Cardiovascular Medicine, Cleveland Clinic, Cleveland, Ohio, USA.
¹³ Department of Medicine, University of Arizona, Tucson, Arizona, USA.
¹⁴ Department of Medicine, University of Pittsburgh, Pittsburgh, Pennsylvania, USA.
¹⁵ Department of Cardiology, The University of Sydney, Sydney, New South Wales, Australia.
¹⁶ Department of Environmental Medicine, New York University Grossman School of Medicine, New York, New York, USA.
¹⁷ Division of Pulmonary and Critical Care Medicine, Johns Hopkins University, Baltimore, Maryland, USA.
¹⁸ Respiratory Institute, Cleveland Clinic, Cleveland, Ohio, USA.
¹⁹ Division of Pulmonary, Critical Care, and Sleep Medicine, Tufts Medical Center, Boston, Massachusetts, USA.
²⁰ Imaging Institute, Cleveland Clinic, Cleveland, Ohio, USA.
²¹ Respiratory Solutions, ERT, Inc, Cleveland, Ohio, USA.
²² Neurologic and Respiratory Institutes, Cleveland Clinic, Cleveland, Ohio, USA.
²³ Pediatrics Institute, Cleveland Clinic, Cleveland, Ohio, USA.
²⁴ Division of Allergy, Pulmonary and Critical Care Medicine, Vanderbilt University Medical Center, Nashville, Tennessee, USA.
²⁵ Division of Pulmonary, Allergy, Critical Care and Sleep Medicine, University of Arizona, Tucson, Arizona, USA.
²⁶ Bluhm Cardiovascular Institute, Northwestern Medicine, Chicago, Illinois, USA.
²⁷ Division of Cardiovascular Medicine, The Ohio State University, Columbus, Ohio, USA.
²⁸ Division of Pulmonary and Critical Care Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
²⁹ Department of Cardiovascular and Metabolic Sciences, Cleveland Clinic Cleveland, Ohio, USA.
³⁰ Department of Medicine, University of California, San Diego, California, USA.
³¹ Perkin Heart Failure Center, Division of Cardiology, Weill Cornell Medicine, New York, New York, USA.

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).

Keywords: phenotyping; pulmonary hypertension.

Publication types

Clinical Study
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't

MeSH terms

Carbon Monoxide
Cross-Sectional Studies
Humans
Hypertension, Pulmonary* / etiology
Pulmonary Circulation
Vascular Diseases* / complications
Vascular Diseases* / diagnosis
Vascular Diseases* / surgery

Clinical Characteristics and Transplant-Free Survival Across the Spectrum of Pulmonary Vascular Disease

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