Preoperative Assessment and Management of Liver Transplant Candidates With Portopulmonary Hypertension

Michael J. Krowka, MD Professor of Medicine Division of Pulmonary and Critical Care Medicine Mayo Clinic Rochester, MN Pulmonary artery hypertension (PAH) that occurs as a consequence of portal hypertension is termed portopulmonary hypertension (POPH) and is associated with significant morbidity and mortality. Among liver transplant (LT) candidates, reported incidence rates of POPH range from 4.5% to 8.5%. The severity of POPH is unrelated to the severity of portal hypertension or the liver disease. In LT patients, intraoperative death and immediate post-LT mortality are feared clinical events when transplantation is attempted in the setting of untreated, moderate to severe POPH. Specific pulmonary artery vasodilator medications (PAH-specific therapy) appear effective in reducing pulmonary artery pressures, improving right ventricular (RV) function and survival. Thus, screening for and accurately diagnosing POPH prior to LT has become a standard of care. The post-LT course of patients with moderate POPH is unpredictable, but most patients can be weaned from PAH-specific therapy over time. In this article, we present an overview of the preoperative assessment of POPH with an emphasis on risk assessment for transplant and the most recent medical treatment options.