Pulmonary hypertension in the setting of cirrhosis and portal hypertension is known as portopulmonary hypertension (PPHTN). Moderate or severe PPHTN is uncommon, but has a poor prognosis and is considered to be a contraindication to liver transplantation. We assessed the impact of vasodilation therapy on pulmonary hemodynamics and outcome after liver transplant in these patients. Eighty-six patients evaluated for liver transplant between 1997 and 2005 had an estimated right ventricular systolic pressure >40 mm Hg or a clinical suspicion of PPHTN. Right heart catheterization confirmed PPHTN in 30 patients (ten mild, eight moderate, and 12 severe). Sixteen of the 20 with moderate-to-severe pulmonary hypertension (mPAP >or= 35) were otherwise considered suitable liver transplant candidates and were treated with vasodilation therapy. mPAP fell to less than 35 mm Hg in 12 patients (75%) and 11 of them then underwent orthotopic liver transplantation. One- and five-year survivals in the transplanted patients were 91% and 67%, respectively. Nine of 11 were off vasodilator therapy after a median of 9.2 months following transplantation. None of the patients who failed vasodilator therapy survived (median survival, 8 months). Effective pharmacologic control of PPHTN before liver transplant is associated with excellent posttransplant survival that is similar to patients transplanted for other indications.