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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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Open Access Green möglich sobald Postprint bei der ZB eingereicht worden ist.
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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Publikationstyp Artikel: Journalartikel
Dokumenttyp Wissenschaftlicher Artikel
Schlagwörter 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
ISSN (print) / ISBN 1053-2498
e-ISSN 1557-3117
Zeitschrift Journal of Heart and Lung Transplantation, The
Quellenangaben Band: 36, Heft: 9, Seiten: 957-967 Artikelnummer: , Supplement: ,
Verlag Elsevier
Verlagsort New York
Begutachtungsstatus