Clin Res Cardiol 96: Suppl 1 (2007)

V103 - Long-Term Survival With End-Stage Idiopathic Pulmonary Arterial Hypertension (IPAH): Impact of Prostanoid and Endothelin-Receptor Antagonist Treatment After Patients’ Listing for Transplantation
M. Dandel1, H. B. Lehmkuhl1, S. Mulahasanovic1, Y. Weng1, O. Grauhan1, C. Knosalla1, R. Hetzer1
1Klinik für Herz-, Thorax- und Gefäßchirurgie, Deutsches Herzzentrum Berlin, Berlin;
Background: Without specific medical therapy, survival after IPAH diagnosis is often shorter than the waiting time for a graft. We investigated the impact of iloprost and bosentan therapy on IPAH patients’ outcome after listing for transplantation (Tx) with the goal to answer the following questions:
1. Is Tx still most promising for long-term survival with IPAH once the criteria for Tx listing are fulfilled, or is there evidence that prostanoids and endothelin-receptor antagonists can improve survival to such an extent that it will exceed post-Tx survival ?
2. How efficient is this treatment for mortality reduction after patients’ listing for transplantation ?
3. Would this treatment provide survival benefits also for those who do not respond to vasodilator testing (i.v. prostacyclin and/or nitric oxide inhalation) ?
Methods: We analyzed results of vasodilator testing, kind and duration of medical treatment, clinical improvement and survival in IPAH patients listed for Tx between 1996-2006. 
Results: Among 59 listed patients, 24 (40.7%) died before Tx after 2.9 months (median). Without iloprost or bosentan therapy mortality on Tx lists reached 64.3%, whereas with iloprost (aerosolized or intravenous) and/or oral bosentan treatment it was only 33.3%. Patients with iloprost and/or bosentan therapy showed similar survival, regardless whether or not they responded to vasodilator testing. Survival after listing was better for patients who were transplanted than for those who received iloprost and/or bosentan but not Tx (p=0.017). Only 1 patient could be safely withdrawn from the Tx list . 
Conclusions: Iloprost and bosentan allow highly effective bridge-to-transplant in end-stage IPAH, regardless of pulmonary vascular responsiveness during acute vasodilator testing, and therefore there is no reason for not applying this treatment to all IPAH patients on Tx waiting lists.  However, in our patients survival benefit from this treatment after listing was lower than from Tx. Thus, for the majority of end-stage IPAH patients, Tx remains the most efficient therapy and therefore, to avoid or delay transplantation iloprost and/or bosentan treatment should be started in the earlier stages of the disease.