Standard PAH therapy improves long term survival in CTEPH patients.
BACKGROUND
Chronic thromboembolic pulmonary hypertension (CTEPH), subsequent to pulmonary embolism is a relatively frequent cause of pulmonary hypertension. Similar to patients with pulmonary arterial hypertension (PAH), CTEPH carries a poor prognosis. There is no hard evidence for any other therapy except pulmonary endarterectomy and none for those patients that are not eligible for this procedure.


METHODS
Fifty patients with confirmed, inoperable CTEPH receiving specific vasodilative therapy (prostanoids, endothelin receptor antagonists, PDE 5-inhibitors or combination) were included in this retrospective study (mean age 55 years, range 16-76 years; 36 female, 14 male). Kaplan-Meier plots of these patients were compared with Kaplan-Meier plots of two historical CTEPH patient groups without any specific vasodilative treatment by log rank tests.


RESULTS
CTEPH patients treated with specific vasodilative compounds as used for therapy of PAH were followed up for 52 +/- 30 months and had a significantly improved survival compared with patients treated without PAH type vasodilators (p < or = 0.0002).


CONCLUSIONS
Our data may generate the hypothesis that specific vasodilative treatment improves outcome in patients with inoperable CTEPH.
Seyfarth
Hans-Juergen
HJ
Department of Respiratory Medicine, University of Leipzig, Liebigstrasse 20, 04103, Leipzig, Germany. hans-juergen.seyfarth@uniklinik-leipzig.de
Halank
Michael
M
Wilkens
Heinrike
H
Schäfers
Hans-Joachim
HJ
Ewert
Ralf
R
Riedel
Martin
M
Schuster
Ernst
E
Pankau
Hans
H
Hammerschmidt
Stefan
S
Wirtz
Hubert
H
D000293:Adolescent; D000328:Adult; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology*; D008297:Male; D008875:Middle Aged; D011655:Pulmonary Embolism / Q000150:complications* / Q000401:mortality; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome; D014665:Vasodilator Agents / Q000627:therapeutic use*
D014665:Vasodilator Agents