Assessment and management of patients with portopulmonary hypertension undergoing liver transplantation

Portopulmonary hypertension (PoPH) is defined as the presence of otherwise unexplained pre-capillary pulmonary hypertension in patients with portal hypertension of cirrhotic or non-cirrhotic aetiology. PoPH occurs in at least 5–8.5% of patients being worked up for a liver transplant (LT) and its prevalence is thought to be increasing. Uncontrolled PoPH prior to LT is associated with high perioperative morbidity and mortality, with severe PoPH being considered a contraindication to LT. Early recognition and appropriate management of PoPH in patients being considered for LT is therefore imperative to achieve optimal outcomes. This review provides a detailed overview of: the epidemiology, prognosis and pathophysiology of PoPH; clinical assessment, screening and diagnostic approach; and pre-, peri‑ and post-transplant management of PoPH in patients undergoing LT. The current evidence base in this area is limited. This review particularly focuses on the evidence both supporting and challenging current practices and highlights areas for future research.