Pre-Capillary, Combined, and Post-Capillary Pulmonary Hypertension: A Pathophysiological Continuum
File(s)
Published version
Author(s)
Type
Journal Article
Abstract
BACKGROUND: Pulmonary hypertension (PH) is hemodynamically classified as pre-capillary (as seen in idiopathic pulmonary arterial hypertension [IPAH]) or post-capillary (as seen in heart failure with preserved ejection fraction [HFpEF]). Overlaps between these conditions exist. Some patients present with risk factors for left heart disease but pre-capillary PH, whereas patients with HFpEF may have combined pre- and post-capillary PH. OBJECTIVES: This study sought to further characterize similarities and differences among patient populations with either PH-HFpEF or IPAH. METHODS: We used registry data to analyze clinical characteristics, hemodynamics, and treatment responses in patients with typical IPAH (<3 risk factors for left heart disease; n = 421), atypical IPAH (≥3 risk factors for left heart disease; n = 139), and PH-HFpEF (n = 226) receiving PH-targeted therapy. RESULTS: Compared with typical IPAH, patients with atypical IPAH and PH-HFpEF were older, had a higher body mass index, had more comorbidities, and had a lower 6-min walking distance, whereas mean pulmonary artery pressure (46.9 ± 13.3 mm Hg vs. 43.9 ± 10.7 mm Hg vs. 45.7 ± 9.4 mm Hg, respectively) and cardiac index (2.3 ± 0.8 l/min/m(2) vs. 2.2 ± 0.8 l/min/m(2) vs. 2.2 ± 0.7 l/min/m(2), respectively) were comparable among groups. After initiation of targeted PH therapies, all groups showed improvement in exercise capacity, functional class, and natriuretic peptides from baseline to 12 months, but treatment effects were less pronounced in patients with PH-HFpEF than typical IPAH; with atypical IPAH in between. Survival rates at 1, 3, and 5 years were almost identical for the 3 groups. CONCLUSIONS: Patients with atypical IPAH share features of both typical IPAH and PH-HFpEF, suggesting that there may be a continuum between these conditions.
Date Issued
2016-07-26
Date Acceptance
2016-05-03
Citation
Journal of the American College of Cardiology, 2016, 68 (4), pp.368-378
ISSN
1558-3597
Publisher
Elsevier
Start Page
368
End Page
378
Journal / Book Title
Journal of the American College of Cardiology
Volume
68
Issue
4
Copyright Statement
© 2016 BY THE AMERICAN COLLEGE OF CARD IOLOGY FOUNDATION. PUBLISHED BY
ELSEVIER. THIS IS AN OPEN ACCESS ARTI CLE UNDER THE CC BY-NC-ND LICENSE
ELSEVIER. THIS IS AN OPEN ACCESS ARTI CLE UNDER THE CC BY-NC-ND LICENSE
Subjects
heart failure with preserved ejection fraction
idiopathic pulmonary arterial hypertension
Cardiovascular System & Hematology
1102 Cardiovascular Medicine And Haematology
1117 Public Health And Health Services
Publication Status
Published