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Published online before print August 9, 2007
Eur Respir J 2007, doi:10.1183/09031936.00039007
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ORIGINAL ARTICLE

Long-term treatment with sildenafil in chronic thromboembolic pulmonary hypertension

F. Reichenberger 1*, R. Voswinckel 1, B. Enke 1, M. Rutsch 1, E.E. Fechtali 1, T. Schmehl 1, H. Olschewski 2, R. Schermuly 1, N. Weissmann 1, H.A. Ghofrani 1, F. Grimminger 1, E. Mayer 3, W. Seeger 1

1 University of Giessen Lung Centre, Dept of Internal Medicine, University Hospital Giessen, Klinikstrasse 36, 35392 Giessen, Germany
2 Division of Pulmonology, University Clinic for Internal Medicine, Medical University Graz, Austria
3 Dept of Cardio-Thoracic Surgery, University Hospital Mainz, Germany

* To whom correspondence should be addressed. E-mail: Frank.Reichenberger{at}uglc.de.


   Abstract

For chronic thromboembolic pulmonary hypertension (CTEPH) not amenable to pulmonary endarterectomy, effective medical therapy is desired.

In an open label, uncontrolled clinical trial 104 patients (mean age 62 [SD 11] years) with non-operable CTEPH were treated with 50 mg sildenafil TID. At baseline, patients had severe pulmonary hypertension (pulmonary vascular resistance (PVR) mean 863 [38] dynes * s * cm-5), a six minute walking distance (6MWD) of 310 [11] meters. Eight patients were in WHO functional class II, 76 patients in class III, and 20 patients in class IV.

After 3 months treatment, there was a significant haemodynamic improvement with reduction of PVR to 759 [62] dynes*s*cm-5 (p<0,001). The 6MWD significantly increased to 361 [15] meters after 3 months, and to 366 [18] meters after 12 months treatment (p=0.001 compared to baseline).

A subset of 67 patients received a single dose of 50mg sildenafil during initial right heart catheter. The acute haemodynamic effect was not predictive for long-term outcome.

In this large series of patients with non-operable CTEPH, open label treatment with sildenafil led to significant long-term functional improvement. The acute effect to sildenafil may not predict the long-term outcome of therapy.

Keywords:  Chronic thromboembolism, pulmonary hypertension, sildenafil




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