Portopulmonary hypertension: A consequence of portal hypertension

Portopulmonary hypertension (POPH) is an uncommon pulmonary vascular consequence of portal hypertension. It presents as exertional dyspnoea andmay lead to right heart failure and death if untreated. There is no relationship between the existence and degree of POPH and the severity of advanced liver disease. Due to the spectrum of pulmonary haemodynamic changes associated with hepatic dysfunction, screening by transthoracic echocardiography and confirmation by right heart catheterisation (RHC) is necessary for accurate diagnosis and therapeutic considerations. Despite the lack of controlled studies, pulmonary vasomodulation therapies in POPH can significantly improve pulmonary haemodynamics and right ventricular (RV) function. The potential to "cure" POPH, at least haemodynamically, with a combination of pulmonary vasomodulation therapy and liver transplantation appears to be an attainable goal in a cohort of POPH patients yet to be optimally characterised. Controlled, multicentre studies and long-term follow-up post-liver transplantation are needed.