Irefin, Samuel A.

Background: Portopulmonary hypertension (POPH) is defined as pulmonary arterial hypertension (PAH) in the context of portal hypertension. Severe POPH has been considered an absolute contraindication of orthotopic liver transplantation (OLT).Objective: Since there are no definitive guidelines for the immediate preoperative, intraoperative, and postoperative evaluation and treatment of POPH patients, we have used published literature along with our experience to review current knowledge in this area.Summary: Moderate-to-severe POPH has important consequences in the perioperative management of candidates for OLT. Adequate right ventricular function is critical to survive the hemodynamic burden of OLT. Immediate preoperative assessment of hemodynamics; careful intraoperative monitoring and management of volume status, pressure changes, and ventricular function; and postoperative transitions of PAH-specific therapy are key components to successful OLT. We emphasize the advantages of the echocardiogram during all of these phases and stress the importance of a team approach to plan care and respond to the multiple challenges of OLT in POPH.