Non-cirrhotic portal hypertension - diagnosis and management

J Hepatol. 2014 Feb;60(2):421-41. doi: 10.1016/j.jhep.2013.08.013. Epub 2013 Aug 23.


NCPH is a heterogeneous group of liver disorders of vascular origin, leading to PHT with near normal HVPG. NCPF/IPH is a disorder of young adults or middle aged women, whereas EHPVO is a disorder of childhood. Early age acute or recurrent infections in an individual with thrombotic predisposition constitute the likely pathogenesis. Both disorders present with clinically significant PHT with preserved liver functions. Diagnosis is easy and can often be made clinically with support from imaging modalities. Management centers on control and prophylaxis of variceal bleeding. In EHPVO, there are additional concerns of growth faltering, portal biliopathy, MHE and parenchymal dysfunction. Surgical shunts are indicated in patients with failure of endotherapy, bleeding from sites not amenable to endotherapy, symptomatic hypersplenism or symptomatic biliopathy. Persistent growth failure, symptomatic and recurrent hepatic encephalopathy, impaired quality of life or massive splenomegaly that interferes with daily activities are other surgical indications. Rex-shunt or MLPVB is the recommended shunt for EHPVO, but needs proper pre-operative radiological assessment and surgical expertise. Both disorders have otherwise a fairly good prognosis, but need regular and careful surveillance. Hepatic schistosomiasis, CHF and NRH have similar presentation and comparable prognosis.

Keywords: APASL; Asia Pacific Association for the Study of the Liver; CHF; CSRS, PSRS, DSRS; EHPVO; ERCP; EST; EVL; Extrahepatic portal venous obstruction; HIV; HPS; HVPG; INCPH; IPH; LTx; MHE; MLPVB; MRCP; NCPF; NCPH; NRH; Non cirrhotic portal fibrosis; PHT; PV; PVT; Portal biliopathy; Portal hypertension; QoL; Shunt surgery; UVC; central, proximal, and distal splenorenal shunt; congenital hepatic fibrosis; endoscopic retrograde cholangiopancreatography; endoscopic sclerotherapy; endoscopic variceal ligation; extrahepatic portal venous obstruction; hepatic venous pressure gradient; hepatopulmonary syndrome; human immunodeficiency virus; idiopathic non-cirrhotic portal hypertension; idiopathic portal hypertension; liver transplantation; magnetic resonance cholangiopancreatography; mesenterico left portal vein bypass or Rex shunt; minimal hepatic encephalopathy; nodular regenerative hyperplasia; non-cirrhotic portal fibrosis; non-cirrhotic portal hypertension; portal hypertension; portal vein; portal vein thrombosis; quality of life; umbilical vein catheterization.

Publication types

  • Review

MeSH terms

  • Animals
  • Disease Models, Animal
  • Esophageal and Gastric Varices / diagnosis
  • Esophageal and Gastric Varices / etiology
  • Esophageal and Gastric Varices / therapy
  • Female
  • Hemodynamics
  • Humans
  • Hypertension, Portal / diagnosis*
  • Hypertension, Portal / etiology
  • Hypertension, Portal / therapy*
  • Idiopathic Noncirrhotic Portal Hypertension
  • Liver Cirrhosis / diagnosis
  • Liver Cirrhosis / etiology
  • Liver Cirrhosis / therapy
  • Male
  • Pancytopenia / diagnosis
  • Pancytopenia / etiology
  • Pancytopenia / therapy
  • Portal Vein / physiopathology
  • Splenomegaly / diagnosis
  • Splenomegaly / etiology
  • Splenomegaly / therapy