Journal article

Publication year: 2017

Pages: 957-967

Journal: The Journal of Heart and Lung Transplantation

Volume number: 36

Issue number: 9

ISSN: 1053-2498

eISSN: 1557-3117

Open access status: Hybrid

DOI Link: https://doi.org/10.1016/j.healun.2017.02.016

Publisher: Elsevier

Abstract: 

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.

Harvard Citation style: Gall, H., Felix, J., Schneck, F., Milger, K., Sommer, N., Voswinckel, R., et al. (2017) The Giessen Pulmonary Hypertension Registry: Survival in pulmonary hypertension subgroups, The Journal of Heart and Lung Transplantation, 36(9), pp. 957-967. https://doi.org/10.1016/j.healun.2017.02.016

APA Citation style: Gall, H., Felix, J., Schneck, F., Milger, K., Sommer, N., Voswinckel, R., Franco, O., Hofman, A., Schermuly, R., Weissmann, N., Grimminger, F., Seeger, W., & Ghofrani, H. (2017). The Giessen Pulmonary Hypertension Registry: Survival in pulmonary hypertension subgroups. The Journal of Heart and Lung Transplantation. 36(9), 957-967. https://doi.org/10.1016/j.healun.2017.02.016