Dr. Krowka discusses a recent presentation on Portopulmonary hypertension.
ABSTRACT
Background: Ambrisentan is a newer endothelin receptor antagonist (ERA) with potential advantages over Bosentan for the therapy of POPH: selective ERA antagonism, once-daily dosing and minimizes elevation in liver enzymes. We describe hemodynamic responses and clinical outcomes of patients with POPH treated with Ambrisentan at Mayo Clinic, Rochester, MN.
Methods: We prospectively identified consecutive adult POPH patients evaluated at Mayo Clinic from January 2007 until May 2009 deemed candidates for oral pulmonary hypertension therapy. In this open label study, patients received 5 mg of Ambrisentan daily for 4 weeks with dose increase to 10 mg daily thereafter. Liver enzymes were assessed monthly. Baseline and follow-up echocardiograms and right heart catheterizations were accomplished.
Results: We identified 12 patients with POPH started on single therapy with Ambrisentan. The median age (IQR, interquartile range) was 55(52-60). Seven patients were male and 11 were Caucasian. Patients were followed for a median of 613 days (334-1011). The main etiology of portal hypertension was cirrhosis (10 cases).The most common etiology of cirrhosis was alcohol (5 cases), followed by hepatitis C (3 cases). Cirrhotic patients had a median MELD score of 10(7-12), and the majority of patients were in Child’s A classification (8 cases). Median time on Ambrisentan therapy was 390 days (206-611). Two patients died, one of advanced hepatocellular carcinoma, and one of septic shock following pneumonia. Mean pulmonary artery pressure and pulmonary vascular resistance significantly decreased after therapy with Ambrisentan, with associated improvement in functional class. Transaminases and total bilirubin levels remained stable after initiation of Ambrisentan.
Conclusion: In this prospective cohort of patients with POPH, Ambrisentan proved to be efficacious with significant improvement in hemodynamics and functional class. In addition, Ambrisentan was safe with no significant elevation in transaminases or total bilirubin.
AUTHORS
Rodrigo Cartin-Ceba, Karen Swanson, Michael J. Krowka from Mayo Clinic.
