Idiopathic portal hypertension: Natural history and long-term outcome

Abstract

Idiopathic portal hypertension (IPH) is a rare cause of intrahepatic portal hypertension. Data on natural history and prognosis of IPH are limited. We sought to describe the complications and long-tem outcome of IPH by retrospectively studying 69 biopsyproven cases of IPH. Mean duration of follow-up was 6.7 6 4.6 years. All patients had evidence of portal hypertension (PH) at diagnosis, and 42% were symptomatic. Variceal bleeding (VB) was the most common manifestation. In those without bleeding at diagnosis, 74% had varices at first endoscopy. In those with large varices, the 1-year probability of first bleeding despite primary prophylaxis was 9%. The 1-year probability of rebleeding was 22%. Ascites and hepatic encephalopathy was documented in 26% and 7% of patients, respectively, at least once during the clinical course. The 1-year probability of developing portal vein thrombosis (PVT) was 9%, and 53% of patients receiving anticoagulation achieved recanalization. Human immunodeficiency virus (HIV) infection and VB at diagnosis were the independent predictors of PVT. Seven patients died (6 as a result of an IPH-related cause) and 2 were transplanted. Probability of liver transplantation-free survival was 82% at 10 years. Presence of a severe associated disorder and ascites as a presenting symptom were associated with poor survival. Conclusion: Variceal bleeding is a major complication of IPH. Using, in IPH patients, the same management approach for PH as in cirrhosis is safe and maintains a low incidence of first bleeding and rebleeding in IPH patients. PVT is a frequent complication, particularly in those with HIV infection. Despite several complications, overall survival of patients with IPH is considerably good. (HEPATOLOGY 2014;59:2276-2285

Consensus conference on TIPS management: Techniques, indications, contraindications

“…Liver function impairment occurs mainly in the context of intercurrent conditions [194]. In 69 biopsy proven cases of INCPH with a mean follow-up of 6.7 ± 4.6 years, all had evidence of portal hypertension at diagnosis, and 42% were symptomatic [195]. Amongst 41 patients with NCIPH treated with TIPS for portal hypertensive complications 11 died: serum creatinine, ascites as indication for TIPS and the presence of significant comorbidities at the time of the procedure emerged as death-related risk factors [193].…”

Novel rat model of repetitive portal venous embolization mimicking human non-cirrhotic idiopathic portal hypertension

“…NCIPH is a rare disorder and causes substantial complications such as gastro-abdominal bleeding [7]. Causes and pathophysiology of NCIPH are still unknown [4]. However, various possible associations, e.g.…”

“…In the absence of cirrhosis, other synonyms are non-cirrhotic portal fibrosis (NCPF), or hepatoportal sclerosis and obliterative venopathy in Eastern countries [1]. Importantly, this condition occurs in the absence of other well characterized causes of portal hypertension such as chronic liver disease and splanchnic venous thrombosis [2][3][4][5]. The prognosis of this disease is considered as benign with a 5 year survival of almost 100%, but long term outcomes are still unknown [4,6,7].…”

“…Importantly, this condition occurs in the absence of other well characterized causes of portal hypertension such as chronic liver disease and splanchnic venous thrombosis [2][3][4][5]. The prognosis of this disease is considered as benign with a 5 year survival of almost 100%, but long term outcomes are still unknown [4,6,7]. Since the prevalence of NCIPH in the Western world is low, recently published literature is rare [8].…”

“…Gastrooesophageal varices and bleeding complications remain a major burden for NCIPH-patients. Therefore, prophylaxis and treatment of variceal bleeding are of clinical relevance [4,7].…”