The Giessen Pulmonary Hypertension Registry: Survival in pulmonary hypertension subgroups

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). Long-term transplant-free survival and its determinants were investigated in patients with PH (diagnosed by right he...

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Published in:The Journal of heart and lung transplantation Vol. 36; no. 9; p. 957
Main Authors: Gall, Henning, Felix, Janine F, Schneck, Franziska K, Milger, Katrin, Sommer, Natascha, Voswinckel, Robert, Franco, Oscar H, Hofman, Albert, Schermuly, Ralph T, Weissmann, Norbert, Grimminger, Friedrich, Seeger, Werner, Ghofrani, Hossein A
Format: Journal Article
Language:English
Published: United States 01.09.2017
Subjects:
Adult
Age Factors
Aged
Cause of Death
Cohort Studies
Female
Germany
Hospital Mortality - trends
Hospitals, University
Humans
Hypertension, Pulmonary - diagnosis
Hypertension, Pulmonary - mortality
Hypertension, Pulmonary - therapy
Male
Middle Aged
Multivariate Analysis
Proportional Hazards Models
Registries
Risk Assessment
Severity of Illness Index
Sex Factors
Survival Analysis
survival
pulmonary
registries
biomarker
biobank
etiology subtypes
hypertension
ISSN:1557-3117, 1557-3117
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Summary: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). 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. 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. 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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ISSN:1557-3117
1557-3117
DOI:10.1016/j.healun.2017.02.016