Epidemiology, physiopathology and diagnosis of pulmonary hypertension in hepatic (PH) cirrhosis

Liver cirrhosis is a complex and progressive disease associated with high mortality. In developing countries, alcoholic liver disease is the most common form of liver cirrhosis, followed by chronic viral disease, especially hepatitis C virus infection. Cirrhosis is associated with systemic and splanchnic hemodynamic abnormalities, including increased vascular volume, decreased systemic vascular resistance, and increased cardiac output. At the splanchnic vascular bed, increases in portal flow and intrahepatic resistance have been described, inducing portal hypertension. Pulmonary arterial hypertension is a progressive disease of pulmonary circulation, without left ventricle and valvular heart disease; it is closely related with structural changes in pulmonary arteries. Idiopathic pulmonary arterial hypertension is related to abnormalities in cellular signals, inducing arterial hypertrophy and increased vascular tone. Porto-pulmonary hypertension includes simultaneous portal and pulmonary arterial hypertension. To confirm disease, it is important to exclude concomitant heart disease. Porto-pulmonary hypertension requires important components: portal hypertension, shear vascular stress, and cellular activation with pulmonary arterial hypertrophy. In this short manuscript, the epidemiology patho-physiology, and diagnostic criteria of the disease are reviewed to optimize early diagnosis and treatment.