TY - JOUR T1 - Ambrisentan for therapy of portopulmonary hypertension (POPH): Update on safety and efficacy JF - European Respiratory Journal JO - Eur Respir J VL - 40 IS - Suppl 56 SP - P949 AU - Rodrigo Cartin-Ceba AU - Karen Swanson AU - Michael Krowka Y1 - 2012/09/01 UR - http://erj.ersjournals.com/content/40/Suppl_56/P949.abstract N2 - Aim: To present an update of the long-term hemodynamic response and clinical outcomes of POPH patients treated with ambrisentan.Methods: Observational study of POPH patients from 01/2007 to 12/2011 treated with ambrisentan. Clinical data, baseline and follow up transthoracic echocardiograms (TTE) and right heart catheterizations (RHC) were accomplished and compared.Results: A total of 27 patients with POPH were started on ambrisentan(female=15). Median age (IQR) was 56 (53-60). Median follow up 874 days (472-1548). Median time on ambrisentan 391 days (259-839). Ten patients underwent liver transplantation successfully. Nine out of 27 patients died, 7 deaths due to complications of chronic liver disease, one patient of sepsis, and one died of an acute coronary syndrome. Follow up RHC data were available in 20 patients. Mean pulmonary artery pressure (mPAP) improved from 42 mmHg (35-57) to 38.5 (28-43.5), p= 0.001; pulmonary vascular resistance (PVR) improved from 434 dynes*s/cm−5 (311-611) to 228 (154-361), p=0.001; and cardiac output increased from 6 L/min (5-7.4) to 7.9 (6.4-9.2), p=0.005. TTE data showed that RV size and function improved in 19 and 18 patients respectively. Ambrisentan was well tolerated in all but one patient who developed severe edema and required discontinuation after 2 weeks of initiation. No significant elevation of transaminases requiring discontinuation of the medication was identified.Conclusion: In this cohort of patients with POPH, ambrisentan proved to be safe and efficacious for the therapy of POPH patients. Ambrisentan resulted in significant improvement in hemodynamics and normalization of RV size, function and PVR in the majority of the patients. ER -