Henning Gall1, Janine F Felix2, Franziska K Schneck3, Katrin Milger4, Natascha Sommer3, Robert Voswinckel3, Oscar H Franco2, Albert Hofman5, Ralph T Schermuly3, Norbert Weissmann3, Friedrich Grimminger3, Werner Seeger3, Hossein A Ghofrani6. 1. 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. 2. Department of Epidemiology, Erasmus MC, University Medical Center Rotterdam, Rotterdam, The Netherlands. 3. 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. 4. 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. 5. 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. 6. 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.
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.
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.
Authors: Lauren Rose; Kurt W Prins; Stephen L Archer; Marc Pritzker; E Kenneth Weir; Jeffrey R Misialek; Thenappan Thenappan Journal: J Heart Lung Transplant Date: 2018-09-14 Impact factor: 10.247