Noncirrhotic portal hypertension

Abstract

Portal hypertension is characterized by an increase in portal pressure (> 10 mmHg) and could be a result of cirrhosis of the liver or of noncirrhotic diseases. When portal hypertension occurs in the absence of liver cirrhosis, noncirrhotic portal hypertension (NCPH) must be considered. The prognosis of this disease is much better than that of cirrhosis. Noncirrhotic diseases are the common cause of portal hypertension in developing countries, especially in Asia. NCPH is a heterogeneous group of diseases that is due to intrahepatic or extrahepatic etiologies. In general, the lesions in NCPH are vascular in nature and can be classified based on the site of resistance to blood flow. In most cases, these disorders can be explained by endothelial cell lesions, intimal thickening, thrombotic obliterations, or scarring of the intrahepatic portal or hepatic venous circulation. Many different conditions can determine NCPH through the association of these various lesions in various degrees. Many clinical manifestations of NCPH result from the secondary effects of portal hypertension. Patients with NCPH present with upper gastrointestinal bleeding, splenomegaly, ascites after gastrointestinal bleeding, features of hypersplenism, growth retardation, and jaundice due to portal hypertensive biliopathy. Other sequelae include hyperdynamic circulation, pulmonary complications, and other effects of portosystemic collateral circulation like portosystemic encephalopathy. At present, pharmacologic and endoscopic treatments are the treatments of choice for portal hypertension. The therapy of all disorders causing NCPH involves the reduction of portal pressure by pharmacotherapy or portosystemic shunting, apart from prevention and treatment of complications of portal hypertension.

Keywords: ADPKD, autosomal-dominant polycystic kidney disease; ARPKD, autosomal-recessive polycystic kidney disease; BCS, Budd-Chiari syndrome; Budd-Chiari syndrome; CHF, congenital hepatic fibrosis; CTGF, connective tissue growth factor; DSRS, distal splenorenal Shunt; EHPVO, extrahepatic portal vein obstruction; ERCP, endoscopic retrograde cholangio pancreatography; EST, endoscopic sclerotherapy; EVL, endoscopic variceal ligation; FHF, fulminant hepatic failure; GI, Gastrointestinal; GVHD, graft versus cells host disease; HLA, human lymphocyte antigen; HVPG, hepatic vanous pressure gradient; IPH, idiopathic portal hypertension; IVC, inferior vena cava; MRCP, magnetic resonance cholangio pancreatography; NCPF, noncirrhotic portal hypertension; NCPH, noncirrhotic portal hypertension; NRH, nodular regenerative hyperplasia; PVT, portal vein thrombosis; SCT, stem-cell transplantation; TIPS, transjugular intrahepatic portosystemic shunt placement; TIPSS, transjugular intrahepatic portosystemic shunt; VOD, veno-occlusive disease; congenital hepatic fibrosis; extra-hepatic portal venous obstruction; nodular regenerative hyperplasia; noncirrhotic intrahepatic portal hypertension; portal vein thrombosis; portosystemic shunting; schistosomiasis; veno-occlusive disease.