Journal
JOURNAL OF HEART AND LUNG TRANSPLANTATION
Volume 36, Issue 9, Pages 957-967Publisher
ELSEVIER SCIENCE INC
DOI: 10.1016/j.healun.2017.02.016
Keywords
biomarker; biobank; etiology subtypes; hypertension; pulmonary; registries; survival
Funding
- Actelion
- AstraZeneca
- Bayer
- GlaxoSmithKline
- Janssen Cilag
- Lilly
- Pfizer
- United Therapeutics/OMT
- Bayer Healthcare
- Novartis
- Noxxon
- Merck
- Universities of Giessen
- Marburg Lung Center within Landes-Offensive zur Entwicklung Wissenschaftlichokonomischer Exzellenz (LOEWE) program of the state of Hesse
- Deutsche Forschungsgemeinschaft (DFG
- German Research Foundation) Excellence Cluster Cardio Pulmonary System (ECCPS)
- Bundesministerium fur Bildung and Forschung (Federal Ministry of Education and Research)
- German Lung Center
- University of Giessen
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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. (C) 2017 The Authors. Published by Elsevier Inc.
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