Opitz, Christian F., Hoeper, Marius M., Gibbs, J. Simon R., Kaemmerer, Harald, Pepke-Zaba, Joanna, Coghlan, J. Gerry, Scelsi, Laura ORCID: 0000-0001-9409-691X, D'Alto, Michele ORCID: 0000-0001-5729-1038, Olsson, Karen M., Ulrich, Silvia ORCID: 0000-0002-5250-5022, Scholtz, Werner, Schulz, Uwe, Gruenig, Ekkehard, Vizza, Carmine D., Staehler, Gerd, Bruch, Leonhard, Huscher, Doerte, Pittrow, David and Rosenkranz, Stephan (2016). Pre-Capillary, Combined, and Post-Capillary Pulmonary Hypertension A Pathophysiological Continuum. J. Am. Coll. Cardiol., 68 (4). S. 368 - 379. NEW YORK: ELSEVIER SCIENCE INC. ISSN 1558-3597

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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 precapillary 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. (C) 2016 by the American College of Cardiology Foundation. Published by Elsevier.

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Opitz, Christian F.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hoeper, Marius M.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gibbs, J. Simon R.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kaemmerer, HaraldUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Pepke-Zaba, JoannaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Coghlan, J. GerryUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Scelsi, LauraUNSPECIFIEDorcid.org/0000-0001-9409-691XUNSPECIFIED
D'Alto, MicheleUNSPECIFIEDorcid.org/0000-0001-5729-1038UNSPECIFIED
Olsson, Karen M.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Ulrich, SilviaUNSPECIFIEDorcid.org/0000-0002-5250-5022UNSPECIFIED
Scholtz, WernerUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Schulz, UweUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gruenig, EkkehardUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Vizza, Carmine D.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Staehler, GerdUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Bruch, LeonhardUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Huscher, DoerteUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Pittrow, DavidUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Rosenkranz, StephanUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
URN: urn:nbn:de:hbz:38-269597
DOI: 10.1016/j.jacc.2016.05.047
Journal or Publication Title: J. Am. Coll. Cardiol.
Volume: 68
Number: 4
Page Range: S. 368 - 379
Date: 2016
Publisher: ELSEVIER SCIENCE INC
Place of Publication: NEW YORK
ISSN: 1558-3597
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
PRESERVED EJECTION FRACTION; SYSTOLIC HEART-FAILURE; ARTERIAL-HYPERTENSION; EXERCISE CAPACITY; PHOSPHODIESTERASE-5 INHIBITION; CLINICAL STATUS; SILDENAFIL; DIAGNOSIS; REGISTRY; HEMODYNAMICSMultiple languages
Cardiac & Cardiovascular SystemsMultiple languages
Refereed: Yes
URI: http://kups.ub.uni-koeln.de/id/eprint/26959

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