Study objective: To obtain information about the diagnosis and management of primary pulmonary hypertension (PPH), especially about the use of epoprostenol (Glaxo-Wellcome; Research Triangle Park, NC) in this patient population.
Background: Long-term IV epoprostenol therapy was approved recently for use in patients with PPH who are unresponsive to conventional therapy. Although epoprostenol represents a major advance in the treatment of PPH, there is no published consensus regarding the optimal use of this therapy.
Methods: A five-page survey was mailed to 23 investigators at medical centers treating five or more patients with PPH with long-term epoprostenol therapy.
Results: Nineteen of 23 investigators responded to the survey. During the initial hemodynamic evaluation, 11 investigators used changes in pulmonary vascular resistance (PVR), pulmonary artery pressure (PAP), and cardiac output, 5 investigators considered PVR and PAP only, and 2 investigators analyzed PVR alone to define a short-term vasodilator response. During long-term therapy, two thirds of the investigators increased the dose at scheduled intervals, while all investigators increased the dose in response to worsening symptoms. Epoprostenol doses were reported to range from 0.5 to 270 ng/kg/min. Nine investigators routinely repeated right heart catheterization an average of 7.5+/-3.8 months after starting epoprostenol, and the mean decrease in pulmonary artery pressure was between 15 and 25%.
Conclusion: This survey indicates that there is wide variation in the evaluation of patients with PPH and in the use of epoprostenol therapy. The lack of consensus suggests the need for multicenter collaborative studies in order to optimize the use of epoprostenol therapy for PPH.