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