, Volume 99, Issue 9, pp 553-556
Date: 25 Apr 2010

Standard PAH therapy improves long term survival in CTEPH patients

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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.

Patients and 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.


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 ≤ 0.0002).


Our data may generate the hypothesis that specific vasodilative treatment improves outcome in patients with inoperable CTEPH.