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Long-term treatment with sildenafil in chronic thromboembolic pulmonary hypertension

¹ University of Giessen Lung Centre, Dept of Internal Medicine, Giessen University Hospital, Giessen, ³ Dept of Cardiothoracic Surgery, Mainz University Hospital, Mainz, Germany. ² Division of Pulmonology, University Clinic for Internal Medicine, Graz Medical University, Graz, Austria.

CORRESPONDENCE: F. Reichenberger, University of Giessen Lung Centre, University Hospital Giessen, Klinikstrasse 36, 35392 Giessen, Germany. Fax: 49 6419942599. E-mail: Frank.Reichenberger{at}innere.med.uni-giessen.de

Keywords: Chronic thromboembolism, pulmonary hypertension, sildenafil

Received: April 2, 2007
Accepted July 25, 2007

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

In an open-label uncontrolled clinical trial, 104 patients (mean±SEM age 62±11 yrs) with inoperable chronic thromboembolic pulmonary hypertension were treated with 50 mg sildenafil t.i.d. At baseline, patients had severe pulmonary hypertension (pulmonary vascular resistance 863±38 dyn·s·cm^–5) and a 6-min walking distance of 310±11 m. Eight patients were in World Health Organization functional class II, 76 in class III and 20 in class IV.

After 3 months’ treatment, there was significant haemodynamic improvement, with reduction of pulmonary vascular resistance to 759±62 dyn·s·cm^–5. The 6-min walking distance increased significantly to 361±15 m after 3 months’ treatment, and to 366±18 m after 12 months’ treatment. A subset of 67 patients received a single dose of 50 mg sildenafil during initial right heart catheterisation. The acute haemodynamic effect of this was not predictive of long-term outcome.

In this large series of patients with inoperable chronic thromboembolic pulmonary hypertension, open-label treatment with sildenafil led to significant long-term functional improvement. The acute effect of sildenafil may not predict the long-term outcome of therapy.

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