| Background & Aims : Idiopathic non-cirrhotic portal hypertension(INCPH) is a heterogenous liver disease characterized by intrahepatic portal hypertension of unknown etiology and in the absence of cirrhosis. Variceal bleeding(VB) is a major and lethal complication of INCPH. However, due to limited data, current therapeutic strategies have not been addressed in consensus, mostly following the guidelines regarding cirrhotic portal hypertension. Transjugular intrahepatic portosystemic shunt(TIPS) has gained wide acceptance as a treatment for the management of VB in cirrhosis. But its role in INCPH patients with VB is largely unknown. We aimed at investigating the outcome of TIPS forVB in such patients, in comparison to those with cirrhotic portal hypertension(CPH) with VB.Methods: Between December 2001 and March 2015, seventy consecutive INCPH patients with VB successfully treated by TIPS were evaluated. One hundred and forty patients with CPH receiving TIPS for VB, matched for age, sex, indication, Child-Pugh score, and type and size of stent, served as controls. The patients were followed up from their date of TIPS placement until death or to the end of the study(September 1st, 2015).Comparisons, utilizing the Kaplan–Meier method, between both groups were made for incidence of rebleeding, shunt patency, HE and survival.Results: Of the 70 INCPH patients, the mean age was 41.6±19.22 years(range 9-76 years) and 53% was male. The liver function was Child A in 48 and Child B in 22. Active variceal bleeding was present with 8/70 and 12/140 patients in INCPH and CPH at admission, respectively. There were no significant differences in baseline characteristics between two groups. During a median follow-up of 30 months, the cumulative probability of rebleeding(20% vs. 21.4%; p=0.363) and shunt dysfunction(18.5% vs. 12.1%; p=0.435) were not significantly different between INCPH and CPH groups. Moreover, comparing to CPH, INCPH patients had significantly lower cumulative 1-, 5-year rate of HE(10.3% vs. 32.3%; 21.9% vs. 40.4%, respectively; p=0.018), and higher 1-, 5-year survival rate(95.7% vs. 85.3%; 89.1% vs. 52.8%, respectively; p=0.0013). On multivariate analysis, post-TIPS portosystemic pressure gradient(PSG)(HR 1.209, 95% CI1.052-1.388) and development of shunt dysfunction(HR 23.119, 95% CI6.215-85.99) were independent predictors of rebleeding. And rebleeding was significantly associated with death(HR 10.094, 95%CI 1.409- 72.295).Conclusions: Compared with CPH, TIPS prevents variceal rebleeding effectively without increasing HE occurrence in INCPH patients, as well as a longer survival. A sufficient reduction of post-TIPS PSG and good long-term shunt patency are necessary to decrease rebleeding in INCPH patients. Further prospective studies on TIPS vs. endoscopic treatments are warranted so as to elucidate whether it should be considered as an alternative option for VB in this population. |
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