RT Journal Article
SR Electronic
T1 Long-term treatment with sildenafil in chronic thromboembolic pulmonary hypertension
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP 922
OP 927
DO 10.1183/09031936.00039007
VO 30
IS 5
A1 F. Reichenberger
A1 R. Voswinckel
A1 B. Enke
A1 M. Rutsch
A1 E. El Fechtali
A1 T. Schmehl
A1 H. Olschewski
A1 R. Schermuly
A1 N. Weissmann
A1 H. A. Ghofrani
A1 F. Grimminger
A1 E. Mayer
A1 W. Seeger
YR 2007
UL http://erj.ersjournals.com/content/30/5/922.abstract
AB 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.