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Current epoprostenol use in patients with severe idiopathic, heritable or anorexigen-associated pulmonary arterial hypertension: data from the French pulmonary hypertension registry.

AbstractOBJECTIVES:
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.
AuthorsEmmanuel Bergot, Olivier Sitbon, Vincent Cottin, Grégoire Prévot, Matthieu Canuet, Arnaud Bourdin, Pascal de Groote, Laurence Rottat, Virginie Gressin, Xavier Jaïs, Marc Humbert, Gérald Simonneau
JournalInternational journal of cardiology (Int J Cardiol) Vol. 172 Issue 3 Pg. 561-7 (Apr 01 2014) ISSN: 1874-1754 [Electronic] Netherlands
PMID24529947 (Publication Type: Journal Article, Multicenter Study, Observational Study, Research Support, Non-U.S. Gov't)
CopyrightCopyright © 2014 Elsevier Ireland Ltd. All rights reserved.
Chemical References
  • Antihypertensive Agents
  • Appetite Depressants
  • Epoprostenol
Topics
  • Adult
  • Antihypertensive Agents (administration & dosage)
  • Appetite Depressants (adverse effects)
  • Cardiac Catheterization
  • Dose-Response Relationship, Drug
  • Epoprostenol (administration & dosage)
  • Familial Primary Pulmonary Hypertension (drug therapy, etiology, physiopathology)
  • Female
  • Follow-Up Studies
  • Humans
  • Injections, Intravenous
  • Male
  • Middle Aged
  • Pulmonary Wedge Pressure (physiology)
  • Registries
  • Retrospective Studies
  • Severity of Illness Index
  • Treatment Outcome
  • Vascular Resistance

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