XXXX therapy is associated with improved hemodynamics in liver transplantation candidates with pulmonary arterial hypertension.

@entity1 @entity4557 @entity7855 @entity101
Pulmonary arterial @entity101 associated with portal @entity101 occurs in about 5% of @entity1 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 @entity1 offered @entity7855 as first-line treatment for @entity101 . We identified consecutive @entity1 at the University of Rochester referred by the liver transplant team. All had catheter-confirmed disease and were treated with @entity7855 . @entity1 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 @entity1 began @entity7855 , and 9 had follow-up right heart catheterizations during a 3-year period. Pulmonary vascular resistance dropped in each @entity1 ; as a group, the mean dropped from 575 to 375 dynes/second/cm(5). Four of 9 @entity1 achieved a mean pulmonary artery pressure </= 35 mm Hg at the time of first recatheterization, and 1 @entity1 received a successful cadaveric transplant. Four of 6 @entity1 with more than 1 follow-up catheterization had sustained hemodynamic benefit. One @entity1 initially responded to therapy with favorable hemodynamics but was found in the operating room to have @entity4557 (on therapy) that precluded safe transplantation. In conclusion, @entity7855 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.
