L'hypertension porto-pulmonaire.

Portopulmonary hypertension (PoPH) is a type of pulmonary arterial hypertension (PAH) occurring in patients with portal hypertension. PoPH affects 5% to 6% of patients with advanced liver disease and accounts for 5% to 15% of PAH cases. The underlying mechanisms for the development of PoPH remain poorly understood but result from complex pathophysiological interactions between the portal and pulmonary circulation. Although portal hypertension is required for the pathogenesis of PoPH, the severity of PoPH does not correlate with the severity of liver disease or portal hypertension. Compared with idiopathic PAH, PoPH is associated with significantly worse survival. Because symptoms of PoPH are nonspecific and because the diagnosis of PoPH may compromise eligibility for liver transplantation (LT) or for a transjugular intrahepatic portosystemic shunt (TIPS) insertion, it is recommended to screen all LT or TIPS candidates for PAH using echocardiography. Specific treatments targeting PAH improve pulmonary haemodynamics allowing patients to receive MELD exception points for a safe LT and to avoid a potential deterioration of PAH while awaiting LT. The complexity of managing PoPH requires a multidisciplinary team approach and those patients should be referred to an expert center. [ABSTRACT FROM AUTHOR]