“…Clinical trials of tadalafil and ambrisentan have shown differences in outcomes in patients receiving placebo that were not improved by subsequent treatment during the time frame examined. (5,6) Although treatment guidelines summarize current knowledge of therapeutic approaches to PAH, based on scientific rigor and evidence from available clinical trials (7,8), the optimal treatment of patients or patient groups is not known. True evidence based recommendations and guidelines could really only be derived from head to head comparison of agents and sequences of treatment, unlikely given the rare nature of the disease.…”