Management of severe idiopathic pulmonary hypertension in a patient with acute liver failure treated with liver transplantation

Abstract Pulmonary arterial hypertension (PAH) is a rare, progressive disorder that may develop in association with portal hypertension [1] (porto-pulmonary hypertension, PoPH). When liver transplantation (LT) is performed in patients with PoPH, the risks of cardiogenic shock and death during and early after the surgery [ 1 , 2 ] are increased, as a consequence of abrupt haemodynamic changes occurring during the procedure. Indeed, these risks could be higher in patients with multi-organ failure (MOF). However, little is known about the management of LT in patients with other causes of PAH such as the idiopathic form (iPAH), representing roughly 40% of all cases of PAH [3] . Here we describe the management of a patient presenting the unusual combination of severe iPAH developing MOF due to acute liver failure, successfully treated with LT.