Natural history of idiopathic noncirrhotic portal hypertension in the long term follow-up

Natural History of idiopathic noncirrhotic portal hypertension in the long term follow-up Background and Aim: Idiopathic noncirrhotic portal hypertension (INCPH) is a rare cause of portal hypertension. Limited reports were published about the prognosis of INCPH. We aimed to evaluate the clinical course of patients with INCPH during the long term follow-up. Material and Methods: Between 1990-2015,we retrospectively evaluated INCPH patients diagnosed by liver biopsy/laparoscopy, portomesenteric venous system imaging and biochemical results. All other etiologies of chronic liver diseases and portal hypertension were excluded. Clinical follow-up details were recorded in whole group. Results: Thirty-four patients (mean age 45.7±13.8 years, 64.7% female) were included. Mean duration of follow-up was 8.2 ± 6.2 years (1-24 years). Thirty-eight percent of patients were presented with variceal bleeding and treated with band ligation. Prophylactic band ligation was performed in seven patients. Portal vein thrombosis (PVT) and ascites were developed in 38% (n=13) and 23.5% (n=8) of cases during the follow-up, respectively. Prothrombotic states were evaluated in all cases. Prothrombotic factors were found in four cases developedPVT during the follow-up (prothrombin 200210A, MTHFR C677T heterozygote mutation and essential thrombocytosis). Factor V Leiden heterozygote mutation was detected in one patient. Ascites was observed only 4/21(19%) INCPH patients, when we excluded patients with PVT. Baseline and end of follow-up mean serum albumin, total bilirubin and INR levels were 4.05±0.4 vs 4.2±0.4 g/dl, 1.2±0.9 vs 1.3±0.72 mg/dl, 1.3±0.5 vs 1.2±0.2, respectively (p=0.05). There were no deaths associated with liver complications. Only one case died another reason. Conclusion: Idiopathic noncirrhotic portal hypertension cases have a good survival. During the follow-up period, portal venous system should be evaluated carefully. In the long term follow up period, liver dysfunction may develop in a quaternary of cases.