title: Current epoprostenol use in patients with severe idiopathic, heritable or anorexigen-associated pulmonary arterial hypertension: data from the French pulmonary hypertension registry.
abstract: OBJECTIVE
  The current use of intravenous epoprostenol in patients with severe idiopathic, heritable or anorexigen-use associated pulmonary arterial hypertension (IHA-PAH) was investigated.
  
  
  METHODS
  This observational study evaluated newly diagnosed (≤1 year) patients with IHA-PAH, enrolled in the French pulmonary hypertension (PH) registry between 2006 and 2010 and treated with epoprostenol. Among 209 consecutive patients receiving epoprostenol for the treatment of severe PH, 78 had IHA-PAH, including 43 patients naïve of previous PAH-specific treatment.
  
  
  RESULTS
  After 4 months of epoprostenol therapy, improvement was observed for treatment naïve patients (n=43) and for patients who had received previous PAH-specific therapy (n=35): NYHA functional class improved in 79% and 44% of these patients, respectively, 6-minute walk distance increased by 146 (p<0.0001) and 41 m (p=0.03), cardiac index increased by 1.2 (p<0.0001) and 0.5 L·min(-1)·m(-2) (p=0.006), and pulmonary vascular resistance decreased by 700 (p<0.0001) and 299 dyn·s·cm(-5) (p=0.009). In the treatment-naïve patient group, upfront combination of epoprostenol and oral PAH therapy tended to be more beneficial compared with epoprostenol monotherapy and was associated with improvement in cardiac index (p=0.03). The observed 1- and 3-year survival estimates from epoprostenol initiation were 84% and 69%, respectively. The highest survival rates were observed for treatment-naïve patients receiving upfront combination of epoprostenol and oral PAH therapy (92% and 88% at 1 and 3 years, respectively).
  
  
  CONCLUSIONS
  First-line therapy with epoprostenol, especially when combined with oral PAH treatment, was associated with a substantial improvement in clinical and hemodynamic status and favorable survival estimates in patients with severe IHA-PAH.
authors: Hôpital Côte de Nacre, Centre de compétences Basse Normandie de l'Hypertension Pulmonaire, Université de Caen, Basse Normandie, Caen, France. Electronic address: bergot-e@chu-caen.fr.
authors: Emmanuel
authors: E
authors: Bergot
authors: University Paris-Sud, Le Kremlin-Bicêtre, France; APHP, Centre de Référence de l'Hypertension Pulmonaire Sévère, Le Kremlin-Bicêtre, France; Inserm U999, Le Plessis Robinson, France.
authors: Olivier
authors: O
authors: Sitbon
authors: Hôpital Louis Pradel, Centre de compétences Rhône-Alpes de l'hypertension Pulmonaire; Université Claude Bernard Lyon 1; Lyon, France.
authors: Vincent
authors: V
authors: Cottin
authors: Hôpital Larrey, Centre de compétences de l'Hypertension Pulmonaire, Toulouse, France.
authors: Grégoire
authors: G
authors: Prévot
authors: Hôpital Civil, Centre de compétences de l'Hypertension Pulmonaire, Strasbourg, France.
authors: Matthieu
authors: M
authors: Canuet
authors: Hôpital Arnaud de Villeneuve, Centre de compétences de l'Hypertension Pulmonaire, Montpellier, France.
authors: Arnaud
authors: A
authors: Bourdin
authors: Hôpital Cardiologique de Lille, Centre de compétences de l'Hypertension Pulmonaire, Lille, France.
authors: Pascal
authors: P
authors: de Groote
authors: APHP, Centre de Référence de l'Hypertension Pulmonaire Sévère, Le Kremlin-Bicêtre, France.
authors: Laurence
authors: L
authors: Rottat
authors: Actelion Pharmaceuticals France, Paris, France.
authors: Virginie
authors: V
authors: Gressin
authors: University Paris-Sud, Le Kremlin-Bicêtre, France; APHP, Centre de Référence de l'Hypertension Pulmonaire Sévère, Le Kremlin-Bicêtre, France; Inserm U999, Le Plessis Robinson, France.
authors: Xavier
authors: X
authors: Jaïs
authors: University Paris-Sud, Le Kremlin-Bicêtre, France; APHP, Centre de Référence de l'Hypertension Pulmonaire Sévère, Le Kremlin-Bicêtre, France; Inserm U999, Le Plessis Robinson, France.
authors: Marc
authors: M
authors: Humbert
authors: University Paris-Sud, Le Kremlin-Bicêtre, France; APHP, Centre de Référence de l'Hypertension Pulmonaire Sévère, Le Kremlin-Bicêtre, France; Inserm U999, Le Plessis Robinson, France.
authors: Gérald
authors: G
authors: Simonneau
mesh_terms: D000328:Adult; D000959:Antihypertensive Agents / Q000008:administration & dosage; D001067:Appetite Depressants / Q000009:adverse effects*; D006328:Cardiac Catheterization; D004305:Dose-Response Relationship, Drug; D011464:Epoprostenol / Q000008:administration & dosage*; D065627:Familial Primary Pulmonary Hypertension / Q000188:drug therapy* / Q000209:etiology / Q000503:physiopathology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007275:Injections, Intravenous; D008297:Male; D008875:Middle Aged; D011669:Pulmonary Wedge Pressure / Q000502:physiology*; D012042:Registries; D012189:Retrospective Studies; D012720:Severity of Illness Index; D016896:Treatment Outcome; D014655:Vascular Resistance
chemical_list: D000959:Antihypertensive Agents; D001067:Appetite Depressants; D011464:Epoprostenol
keywords: Combination therapy; Epoprostenol; Pulmonary arterial hypertension; Survival