Role of hepatic vein catheterisation and transient elastography in the diagnosis of idiopathic portal hypertension

2012 Digestive & Liver Disease 44;10 (855-860)

BACKGROUND: Idiopathic portal hypertension is a rare cause of portal hypertension, frequently misdiagnosed as cryptogenic cirrhosis. This study evaluates specific findings at hepatic vein catheterisation or liver stiffness in idiopathic portal hypertension. METHODS: 39 cases of idiopathic portal hypertension patients were retrospectively reviewed. Hepatic vein catheterisation and liver stiffness measurements were compared to matched patients with cirrhosis and portal hypertension, and non-cirrhotic portal vein thrombosis, included as controls. RESULTS: Hepatic vein-to-vein communications were found in 49% idiopathic portal hypertension patients precluding adequate hepatic venous pressure gradient measurements in 12. In the remaining 27 patients, mean hepatic venous pressure gradient (HVPG) was 7.1 +/- 3.1 mm Hg. Only 5 patients had HVPG>/=10mmHg. HVPG was markedly lower than in cirrhosis (17 +/- 3 mm Hg, p13.6 kPa. CONCLUSIONS: Patients with idiopathic portal hypertension frequently have hepatic vein-to-vein communications and, despite unequivocal signs of portal hypertension, HVPG and liver stiffness values much lower than the cut-off for clinical significant portal hypertension in cirrhosis. These findings oblige to formally rule-out idiopathic portal hypertension in the presence of signs of portal hypertension.

Pubmed : 22721839