Poor outcome following aborted orthotopic liver transplantation due to severe porto-pulmonary hypertension

Background/purpose: The diagnosis of porto-pulmonary hypertension (PPHN) in patients with end-stage liver disease at the time of surgery is not an uncommon occurrence. The decision to proceed with orthotopic liver transplantation (OLT) in patients with severe PPHN is associated with high perioperative mortality. We sought to determine the progression of PPHN and patient outcome following aborted OLT. Methods: A retrospective analysis of 2150 OLT cases was performed. Results: The analysis revealed that six cases (0.3%) were cancelled due to severe PPHN at the time of surgery. The progression to severe PPHN occurred on median over a period of 82 days (range 10-229 days). Following aborted OLT, three patients (50%) expired shortly after. Conclusions: Cancellation of OLT due to severe pulmonary hypertension was also associated with high mortality. Moreover, this preliminary study reveals that once diagnosed, PPHN can progress to a more severe form over a short period of time. Therefore, we recommend more frequent monitoring of PPHN in this population of patients.