PMID- 28302503 OWN - NLM STAT- MEDLINE DCOM- 20180611 LR - 20180611 IS - 1557-3117 (Electronic) IS - 1053-2498 (Linking) VI - 36 IP - 9 DP - 2017 Sep TI - The Giessen Pulmonary Hypertension Registry: Survival in pulmonary hypertension subgroups. PG - 957-967 LID - S1053-2498(17)31625-X [pii] LID - 10.1016/j.healun.2017.02.016 [doi] AB - 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. CI - Copyright (c) 2017 The Authors. Published by Elsevier Inc. All rights reserved. FAU - Gall, Henning AU - Gall H AD - Pulmonary Hypertension Division, Medical Clinic II/V, University of Giessen, Marburg Lung Center, German Center for Lung Research, Giessen, Germany, and Department of Medicine, Imperial College London, UK; Department of Epidemiology, Erasmus MC, University Medical Center Rotterdam, Rotterdam, The Netherlands. FAU - Felix, Janine F AU - Felix JF AD - Department of Epidemiology, Erasmus MC, University Medical Center Rotterdam, Rotterdam, The Netherlands. FAU - Schneck, Franziska K AU - Schneck FK AD - Pulmonary Hypertension Division, Medical Clinic II/V, University of Giessen, Marburg Lung Center, German Center for Lung Research, Giessen, Germany, and Department of Medicine, Imperial College London, UK. FAU - Milger, Katrin AU - Milger K AD - Pulmonary Hypertension Division, Medical Clinic II/V, University of Giessen, Marburg Lung Center, German Center for Lung Research, Giessen, Germany, and Department of Medicine, Imperial College London, UK; Comprehensive Pneumology Center, Ludwig-Maximilians University, Asklepios Clinic Gauting and Helmholtz Center Munich, Munich, Germany. FAU - Sommer, Natascha AU - Sommer N AD - Pulmonary Hypertension Division, Medical Clinic II/V, University of Giessen, Marburg Lung Center, German Center for Lung Research, Giessen, Germany, and Department of Medicine, Imperial College London, UK. FAU - Voswinckel, Robert AU - Voswinckel R AD - Pulmonary Hypertension Division, Medical Clinic II/V, University of Giessen, Marburg Lung Center, German Center for Lung Research, Giessen, Germany, and Department of Medicine, Imperial College London, UK. FAU - Franco, Oscar H AU - Franco OH AD - Department of Epidemiology, Erasmus MC, University Medical Center Rotterdam, Rotterdam, The Netherlands. FAU - Hofman, Albert AU - Hofman A AD - Department of Epidemiology, Erasmus MC, University Medical Center Rotterdam, Rotterdam, The Netherlands; Department of Epidemiology, T.H. Chan School of Public Health, Harvard University, Boston, Massachusetts, USA. FAU - Schermuly, Ralph T AU - Schermuly RT AD - Pulmonary Hypertension Division, Medical Clinic II/V, University of Giessen, Marburg Lung Center, German Center for Lung Research, Giessen, Germany, and Department of Medicine, Imperial College London, UK. FAU - Weissmann, Norbert AU - Weissmann N AD - Pulmonary Hypertension Division, Medical Clinic II/V, University of Giessen, Marburg Lung Center, German Center for Lung Research, Giessen, Germany, and Department of Medicine, Imperial College London, UK. FAU - Grimminger, Friedrich AU - Grimminger F AD - Pulmonary Hypertension Division, Medical Clinic II/V, University of Giessen, Marburg Lung Center, German Center for Lung Research, Giessen, Germany, and Department of Medicine, Imperial College London, UK. FAU - Seeger, Werner AU - Seeger W AD - Pulmonary Hypertension Division, Medical Clinic II/V, University of Giessen, Marburg Lung Center, German Center for Lung Research, Giessen, Germany, and Department of Medicine, Imperial College London, UK. FAU - Ghofrani, Hossein A AU - Ghofrani HA AD - Pulmonary Hypertension Division, Medical Clinic II/V, University of Giessen, Marburg Lung Center, German Center for Lung Research, Giessen, Germany, and Department of Medicine, Imperial College London, UK. Electronic address: ardeschir.ghofrani@innere.med.uni-giessen.de. LA - eng PT - Comparative Study PT - Journal Article DEP - 20170217 PL - United States TA - J Heart Lung Transplant JT - The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation JID - 9102703 SB - IM CIN - J Heart Lung Transplant. 2017 Sep;36(9):938-939. PMID: 28551352 MH - Adult MH - Age Factors MH - Aged MH - *Cause of Death MH - Cohort Studies MH - Female MH - Germany MH - Hospital Mortality/*trends MH - Hospitals, University MH - Humans MH - Hypertension, Pulmonary/*diagnosis/*mortality/therapy MH - Male MH - Middle Aged MH - Multivariate Analysis MH - Proportional Hazards Models MH - *Registries MH - Risk Assessment MH - Severity of Illness Index MH - Sex Factors MH - Survival Analysis OTO - NOTNLM OT - biobank OT - biomarker OT - etiology subtypes OT - hypertension OT - pulmonary OT - registries OT - survival EDAT- 2017/03/18 06:00 MHDA- 2018/06/12 06:00 CRDT- 2017/03/18 06:00 PHST- 2016/08/28 00:00 [received] PHST- 2017/02/09 00:00 [revised] PHST- 2017/02/15 00:00 [accepted] PHST- 2017/03/18 06:00 [pubmed] PHST- 2018/06/12 06:00 [medline] PHST- 2017/03/18 06:00 [entrez] AID - S1053-2498(17)31625-X [pii] AID - 10.1016/j.healun.2017.02.016 [doi] PST - ppublish SO - J Heart Lung Transplant. 2017 Sep;36(9):957-967. doi: 10.1016/j.healun.2017.02.016. Epub 2017 Feb 17.