title: Sildenafil for long-term treatment of nonoperable chronic thromboembolic pulmonary hypertension.
abstract: Only a small percentage of patients with chronic thromboembolic pulmonary hypertension are eligible for pulmonary thrombendarterectomy. We investigated the effects of oral sildenafil on hemodynamics and exercise capacity in 12 nonoperable chronic thromboembolic pulmonary hypertension patients. All patients were in disease progression despite sufficient long-term anticoagulation and the best supportive care and suffered from severe pulmonary hypertension (pulmonary vascular resistance index 1,935 +/- 228 dyn. s. cm-5. m2, cardiac index 2.0 l. min-1. m-2, 6-minute walking distance 312 +/- 30 m). After approximately 6 months of sildenafil treatment, pulmonary hemodynamics and exercise capacity improved significantly (pulmonary vascular resistance index 1,361 +/- 177 L. min-1. m2, p = 0.004, cardiac index 2.4 +/- 0.2 L. min-1. m-2, p = 0.009, 6-minute walking distance 366 +/- 28 m, p = 0.02). Therefore, oral sildenafil may offer a new option for medical treatment of this devastating disease.
authors.affiliation: Department of Internal Medicine, University Hospital, Justus-Liebig-University Giessen, Germany.
authors.forename: Hossein A
authors.initials: HA
authors.lastname: Ghofrani
authors.forename: Ralph T
authors.initials: RT
authors.lastname: Schermuly
authors.forename: Frank
authors.initials: F
authors.lastname: Rose
authors.forename: Ralph
authors.initials: R
authors.lastname: Wiedemann
authors.forename: Markus G
authors.initials: MG
authors.lastname: Kohstall
authors.forename: André
authors.initials: A
authors.lastname: Kreckel
authors.forename: Horst
authors.initials: H
authors.lastname: Olschewski
authors.forename: Norbert
authors.initials: N
authors.lastname: Weissmann
authors.forename: Beate
authors.initials: B
authors.lastname: Enke
authors.forename: Stefanie
authors.initials: S
authors.lastname: Ghofrani
authors.forename: Werner
authors.initials: W
authors.lastname: Seeger
authors.forename: Friedrich
authors.initials: F
authors.lastname: Grimminger
mesh_terms: D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications / Q000188:drug therapy*; D008297:Male; D010726:Phosphodiesterase Inhibitors / Q000627:therapeutic use*; D010879:Piperazines / Q000627:therapeutic use*; D011655:Pulmonary Embolism / Q000150:complications / Q000188:drug therapy*; D011687:Purines; D000068677:Sildenafil Citrate; D013450:Sulfones; D013997:Time Factors
chemical_list: D010726:Phosphodiesterase Inhibitors; D010879:Piperazines; D011687:Purines; D013450:Sulfones; D000068677:Sildenafil Citrate