title: Long-term treatment with sildenafil in chronic thromboembolic pulmonary hypertension.
abstract: 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.
authors: University of Giessen Lung Centre, University Hospital Giessen, Klinikstrasse 36, 35392, Giessen, Germany. Frank.Reichenberger@innere.med.uni-giessen.de
authors: F
authors: F
authors: Reichenberger
authors: R
authors: R
authors: Voswinckel
authors: B
authors: B
authors: Enke
authors: M
authors: M
authors: Rutsch
authors: E
authors: E
authors: El Fechtali
authors: T
authors: T
authors: Schmehl
authors: H
authors: H
authors: Olschewski
authors: R
authors: R
authors: Schermuly
authors: N
authors: N
authors: Weissmann
authors: H A
authors: HA
authors: Ghofrani
authors: F
authors: F
authors: Grimminger
authors: E
authors: E
authors: Mayer
authors: W
authors: W
authors: Seeger
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D006328:Cardiac Catheterization; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology; D008297:Male; D008875:Middle Aged; D010879:Piperazines / Q000627:therapeutic use*; D011687:Purines / Q000627:therapeutic use; D000068677:Sildenafil Citrate; D018709:Statistics, Nonparametric; D013450:Sulfones / Q000627:therapeutic use*; D013923:Thromboembolism / Q000150:complications / Q000188:drug therapy*; D016896:Treatment Outcome; D014655:Vascular Resistance / Q000187:drug effects; D014665:Vasodilator Agents / Q000627:therapeutic use*
chemical_list: D010879:Piperazines; D011687:Purines; D013450:Sulfones; D014665:Vasodilator Agents; D000068677:Sildenafil Citrate