Pulmonary arterial hypertension associated with portal hypertension occurs in about 5% of patients being evaluated for liver transplantation. Treatment is required to facilitate safe transplantation, and oral pulmonary vasodilators have yet to be prospectively evaluated for this disease. The objective of this study was to determine the hemodynamic outcome in a consecutive cohort of patients offered sildenafil as first-line treatment for portopulmonary hypertension. We identified consecutive patients at the University of Rochester referred by the liver transplant team. All had catheter-confirmed disease and were treated with sildenafil. Patients were excluded from analysis if they did not have follow-up catheterizations. The change in pulmonary vascular resistance at the time of first follow-up was the primary outcome. Eleven patients began sildenafil, and 9 had follow-up right heart catheterizations during a 3-year period. Pulmonary vascular resistance dropped in each patient; as a group, the mean dropped from 575 to 375 dynes/second/cm(5). Four of 9 patients achieved a mean pulmonary artery pressure </= 35 mm Hg at the time of first recatheterization, and 1 patient received a successful cadaveric transplant. Four of 6 patients with more than 1 follow-up catheterization had sustained hemodynamic benefit. One patient initially responded to therapy with favorable hemodynamics but was found in the operating room to have recrudescent disease (on therapy) that precluded safe transplantation. In conclusion, sildenafil was associated with improved hemodynamics in this small, uncontrolled cohort. A multicenter, prospective evaluation is warranted in this group uniformly excluded from phase III clinical trials.
Copyright 2008 AASLD.