Details

Autor(en) / Beteiligte

• Gall, Henning, MD PhD
• Felix, Janine F., PhD
• Schneck, Franziska K., MD
• Milger, Katrin, MD
• Sommer, Natascha, PhD
• Voswinckel, Robert, MD
• Franco, Oscar H., PhD
• Hofman, Albert, PhD
• Schermuly, Ralph T., PhD
• Weissmann, Norbert, PhD
• Grimminger, Friedrich, PhD
• Seeger, Werner, MD
• Ghofrani, Hossein A., MD

Titel

The Giessen Pulmonary Hypertension Registry: Survival in Pulmonary Hypertension Subgroups

Ist Teil von

• The Journal of heart and lung transplantation, 2017-09, Vol.36 (9), p.957-967

Ort / Verlag

United States: Elsevier Inc

Erscheinungsjahr

2017

Quelle

MEDLINE

Beschreibungen/Notizen

• Background Pulmonary hypertension (PH) is a severe progressive disease. Though five subgroups are recognised, reports on survival focus 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, 2067 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 etiological 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, sex, 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 reported single-center PH cohort. Some parameters used in clinical practice do not independently predict survival. Age, sex, and 6MWD outperformed NYHA functional class in predicting survival across all etiologic groups.