Portopulmonary hypertension (PPHTN) refers to pulmonary arterial hypertension that is associated with portal hypertension; it is a well recognized complication of chronic liver disease [1-3]. In this topic review, the definition, classification, epidemiology, and pathogenesis of PPHTN are discussed. In addition, its clinical presentation, diagnosis, treatment, and prognosis are reviewed. Hepatopulmonary syndrome, a separate but related condition characterized by hypoxemic respiratory insufficiency due to increased intrapulmonary shunting, is discussed separately [1,4,5]. (See "Hepatopulmonary syndrome: Prevalence, causes, clinical manifestations, and diagnosis".)
PPHTN is considered present when pulmonary arterial hypertension (PAH) exists in a patient who has coexisting portal hypertension, and no alternative cause of the PAH exists (eg, collagen vascular disease, congenital heart disease, or certain drugs) .
PAH is defined according to right heart catheterization :
●Mean pulmonary artery pressure (mPAP) >25 mmHg at rest
●Pulmonary capillary wedge pressure (PCWP) <15 mmHg