title: Intravenous epoprostenol in inoperable chronic thromboembolic pulmonary hypertension.
abstract: BACKGROUND
  Pulmonary thromboendarterectomy may be a cure for patients with chronic thromboembolic pulmonary hypertension (CTEPH) with proximal obstruction. Conversely, short-term prognosis is poor for inoperable CTEPH patients with distal obstruction. Vascular remodeling lesions can be found in lungs of patients with CTEPH. Little is known about epoprostenol therapy in inoperable distal CTEPH patients.
  
  
  METHODS
  We conducted a retrospective analysis of 27 consecutive patients with inoperable distal CTEPH, who were in New York Heart Association Functional Class (NYHA FC) III (n = 20) or IV (n = 7) and treated with long-term intravenous epoprostenol.
  
  
  RESULTS
  Before epoprostenol initiation (baseline), 6-minute walk distance (6MWD) was 265 +/- 117 m, right atrial pressure 13 +/- 5 mm Hg, mean pulmonary artery pressure (mPAP) 56 +/- 9 mm Hg, cardiac index (CI) 1.9 +/- 0.3 liters/min/m2, total pulmonary resistance (TPR) 29.3 +/- 7.0 U/m2 and mixed venous oxygen saturation 52 +/- 8%. After 3 months of epoprostenol (n = 23; mean epoprostenol dose 16 +/- 2 ng/kg/min), NYHA FC improved by one class in 11 of 23 patients, 6MWD increased by 66 m (p < 0.0001), and hemodynamics also improved (mPAP: 51 +/- 8 mm Hg, p = 0.001; CI: 2.3 +/- 0.4 liters/min/m2, p = 0.0003; TPR: 23.0 +/- 5.0 U/m2, p < 0.0001). At last evaluation (n = 18; 20 +/- 8 months), mean dose of epoprostenol was 30 +/- 8 ng/kg/min, NYHA FC was improved in 9 of 18 patients and there was sustained improvement in 6MWD (+46 m, p = 0.03) and hemodynamic parameters. Survival at 1, 2 and 3 years was 73%, 59% and 41%, respectively.
  
  
  CONCLUSIONS
  Long-term treatment with intravenous epoprostenol improves exercise capacity and hemodynamic parameters in patients with inoperable distal CTEPH. Epoprostenol may be used as bridge therapy in CTEPH patients awaiting lung transplantation.
authors.affiliation: Centre des Maladies Vasculaires Pulmonaires (UPRES EA 2705), Service de Pneumologie et Réanimation, AP-HP, Université Paris-Sud, Hôpital Antoine Béclère, Clamart, France.
authors.forename: Ségolène
authors.initials: S
authors.lastname: Cabrol
authors.forename: Rogerio
authors.initials: R
authors.lastname: Souza
authors.forename: Xavier
authors.initials: X
authors.lastname: Jais
authors.forename: Elie
authors.initials: E
authors.lastname: Fadel
authors.forename: Ratiba Haddad Si
authors.initials: RH
authors.lastname: Ali
authors.forename: Marc
authors.initials: M
authors.lastname: Humbert
authors.forename: Philippe
authors.initials: P
authors.lastname: Dartevelle
authors.forename: Gérald
authors.initials: G
authors.lastname: Simonneau
authors.forename: Olivier
authors.initials: O
authors.lastname: Sitbon
mesh_terms: D000328:Adult; D000959:Antihypertensive Agents / Q000008:administration & dosage* / Q000009:adverse effects / Q000627:therapeutic use; D002302:Cardiac Output; D002908:Chronic Disease; D004334:Drug Administration Schedule; D011464:Epoprostenol / Q000008:administration & dosage* / Q000009:adverse effects / Q000627:therapeutic use; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology* / Q000503:physiopathology / Q000601:surgery; D007275:Injections, Intravenous; D053208:Kaplan-Meier Estimate; D016040:Lung Transplantation; D008297:Male; D008875:Middle Aged; D010807:Physical Endurance; D010975:Platelet Aggregation Inhibitors / Q000008:administration & dosage* / Q000009:adverse effects / Q000627:therapeutic use; D012189:Retrospective Studies; D013923:Thromboembolism / Q000150:complications* / Q000188:drug therapy*; D014655:Vascular Resistance
chemical_list: D000959:Antihypertensive Agents; D010975:Platelet Aggregation Inhibitors; D011464:Epoprostenol