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Safety and Efficacy of Ambrisentan for the Therapy of Portopulmonary Hypertension.
Chest. 2010 Aug 12;
Authors: Cartin-Ceba R, Swanson K, Iyer V, Wiesner RH, Krowka MJ
ABSTRACT BACKGROUND: Ambrisentan is a selective endothelin receptor antagonist (ERA) that is FDA-approved to treat pulmonary artery hypertension. We describe hemodynamic responses and clinical outcomes of patients with portopulmonary hypertension (POPH) treated with ambrisentan. METHODS: In this observational study, we prospectively identified and followed consecutive adult POPH patients from January 2007 until December 2009 that received monotherapy with ambrisentan up to 10 mg daily. Liver enzymes were assessed monthly. Pulmonary hemodynamics were assessed by echocardiograms and right heart catheterizations. RESULTS: We identified 13 patients (7 males) with POPH and began monotherapy with ambrisentan. The median age (IQR) was 57(52-60). Patients were followed for a median (IQR) of 613 days (385-1011). The median MELD score was 10(8.5-15); eight patients had Child's A classification. Median time on ambrisentan therapy was 390 days (363-611). Two patients died, one of advanced hepatocellular carcinoma, and one of septic shock following pneumonia. The mean pulmonary artery pressure decreased from a baseline median (IQR) of 58 mmHg (37-63) to 41 mmHg (27-48) (p=0.004). Pulmonary vascular resistance median (IQR) was reduced from 445 (329-834) to 174 dyne.sec/cm(5) (121-361) (p=0.008). There was no difference in the longitudinal analysis of liver function tests (AST, ALT, Total bilirubin and INR) after 12 months of therapy. One patient underwent successful liver transplantation and normalized pulmonary hemodynamics after transplantation. CONCLUSION: In this small cohort of patients with moderate to severe pulmonary hypertension in the setting of POPH, we have shown that ambrisentan monotherapy can significantly improve pulmonary hemodynamics without adverse effect on hepatic function.
PMID: 20705798 [PubMed - as supplied by publisher]