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Gall, H.* ; Felix, J.F.* ; Schneck, F.K.* ; Milger, K. ; Sommer, N.* ; Voswinckel, R.* ; Franco, O.H.* ; Hofman, A.* ; Schermuly, R.T.* ; Weissmann, N.* ; Grimminger, F.* ; Seeger, W.* ; Ghofrani, H.A.*

The Giessen Pulmonary Hypertension Registry: Survival in pulmonary hypertension subgroups.

J. Heart Lung Transpl. 36, 957-967 (2017)
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BACKGROUND: Pulmonary hypertension (PH) is a severe, progressive disease. Although 5 PH subgroups are recognized, reports on survival have focused mainly on pulmonary arterial hypertension (PAH). METHODS: Long-term transplant-free survival and its determinants were investigated in patients with PH (diagnosed by right heart catheterization) within a prospective registry at a single referral center in Giessen, Germany. RESULTS: In total, 2,067 patients were enrolled (PAH, 685 patients [33.1%]; pulmonary venous hypertension, 307 patients [14.9%]; PH due to lung diseases (LD-PH), 546 patients [26.4%; mainly interstitial lung disease and chronic obstructive pulmonary disease]; chronic thromboembolic PH, 459 patients [22.2%]; PH owing to miscellaneous/unknown causes, 70 patients [3.4%]). Median follow-up was 37 months. Differences in transplant-free survival between etiologic groups were highly significant (p < 0.001), with 1-, 3- and 5-year survival rates of 88.2%, 72.2% and 59.4%, respectively, for those with PAH compared with 79.5%, 52.7% and 38.1%, respectively, for patients with LD-PH. Patients' age, gender and 6-minute walk distance (6MWD), but not New York Heart Association (NYHA) functional class, associated significantly with survival across all PH subtypes in multivariate Cox regression analyses. CONCLUSIONS: This is the largest single-center PH cohort described so far. Some parameters used in clinical practice do not independently predict survival. Age, gender and 6MWD outperformed NYHA functional class in predicting survival across all etiologic groups.
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Publication type Article: Journal article
Document type Scientific Article
Keywords Biobank ; Biomarker ; Etiology Subtypes ; Hypertension ; Pulmonary ; Registries ; Survival; Disease Updated Recommendations; Right-ventricular Function; Consensus Conference 2011; 6-minute Walk Test; Left-heart Disease; Arterial-hypertension; Risk Stratification; National Registry; Pressure-gradient; Prevalent Cohorts
Language english
Publication Year 2017
HGF-reported in Year 2017
ISSN (print) / ISBN 1053-2498
e-ISSN 1557-3117
Journal Journal of Heart and Lung Transplantation, The
Quellenangaben Volume: 36, Issue: 9, Pages: 957-967 Article Number: , Supplement: ,
Publisher Elsevier
Publishing Place New York
Reviewing status Peer reviewed
Institute(s) Institute of Lung Health and Immunity (LHI)
POF-Topic(s) 30202 - Environmental Health
Research field(s) Lung Research
PSP Element(s) G-501600-001
DOI 10.1016/j.healun.2017.02.016
WOS ID WOS:000409395200007
Scopus ID 85019698880
PubMed ID 28302503
Erfassungsdatum 2017-06-13
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