BACKGROUND: Liver cirrhosis causes over one million deaths annually worldwide, but its prognosis varies depending on the presence of complications and decompensating events. Reduction of portal pressure is associated with a reduced risk of mortality in cirrhotic patients. Statin therapy has successfully reduced portal pressure in previous studies, but its effects on overall mortality are unclear. This report aims to determine whether statin therapy significantly affects mortality in patients with liver cirrhosis.
METHODS: A comprehensive literature search was conducted using five electronic databases: PubMed, Scopus, Embase, Ovid MEDLINE, and Web of Science. Meta-analyses, randomized controlled trials (RCTs), and cohort studies were selected based on pre-set inclusion and exclusion criteria. The quality of selected studies was evaluated using critical appraisal tools developed by the Center for Evidence-Based Medicine.
RESULTS: One meta-analysis, one RCT, and one retrospective cohort study were included in this report. The meta-analysis and cohort study were of good quality and reported significantly reduced mortality with statin therapy in cirrhosis patients. However, the RCT had poor validity and did not report a statistically significant difference in mortality between the intervention and control groups. The survival benefits of statins may be limited to Child-Pugh A and B patients only, but this requires confirmation in a larger population of Child-Pugh C patients.
CONCLUSIONS: Statins potentially reduce mortality in patients with liver cirrhosis, but more evidence is required before they can be widely recommended in clinical practice for this indication.

BACKGROUND: Portal hypertensive biliopathy (PHB) was caused by anatomical and functional abnormalities in the intrahepatic and extrahepatic bile ducts secondary to portal hypertension. Currently, there is no consensus regarding to the optimal treatment for PHB. Transjugular intrahepatic portosystemic shunt (TIPS) is the treatment choice for the management of symptomatic PHB, however, it could be very difficult in patients with PHB and cavernous transformation of portal vein.
METHODS: We report a case of PHB, successfully managed with TIPS. A 23-year-old man with liver cirrhosis presented with jaundice. Magnetic resonance cholangiopancreatography (MRCP) showed multiple tortuous hepatopetal collateral vessels compressing the common bile duct (CBD) and leading to the dilated proximal bile duct. He was diagnosed with PHB and treated with TIPS. A guidewire was inserted into the appropriate collateral vessel through transsplenic approach to guide intrahepatic puncture and TIPS was performed successfully. After the operation, portal vein pressure decreased and the symptoms of biliary obstruction were relieved significantly. In addition, the patient showed no jaundice at a follow-up of one year.
CONCLUSIONS: For PHB patients presenting for cavernous transformation of the portal vein, which precludes the technical feasibility of TIPS, a combined transjugular/transsplenic approach could be an alternative option.

BACKGROUND: Congenital portosystemic shunts (CPSS) are rare vascular malformations and can be classified into extrahepatic and intrahepatic shunts. Extrahepatic CPSS, also termed Abernethy malformations are associated with severe long-term complications including portopulmonary hypertension, liver atrophy, hyperammoniemia and hepatic encephalopathy. We report a hitherto undescribed variant of Abernethy malformation requiring an innovative approach for interventional treatment.
METHODS: We describe a 31-year-old patient following surgical repair of atrioventricular septal defect at the age of 6 years. In the long-term follow-up he showed persistent pulmonary hypertension which deteriorated despite dual pulmonary vasodilative treatment. When he developed arterial desaturation and symptomatic hyperammoniemia detailed reassessment revealed as underlying cause a hitherto undescribed variant of Abernethy malformation connecting the portal vein with the right lower pulmonary vein. Following interdisciplinary discussions we opted for an interventional approach. Since the malformation was un-accessible to interventional closure via antegrade venous or retrograde arterial access, a transhepatic percutaneous puncture of the portal vein was performed. Temporary balloon occlusion of the malformation revealed only a slight increase in portal venous pressure. Interventional occlusion of the large vascular connection was achieved via this transhepatic approach by successive implantation of two large vascular occluding devices. The postinterventional course was unremarkable and both ammonia levels and arterial saturation normalized at follow-up of 12 months.
CONCLUSIONS: Portal vein anomalies should be included in the differential diagnoses of pulmonary hypertension or pulmonary arterio-venous malformations. Based on careful assessment of the anatomy and testing of portal vein hemodynamics interventional therapy of complex Abernethy malformations can be performed successfully in specialized centers.

Transjugular intrahepatic portosystemic shunt (TIPS) is well established as an effective treatment tool for portal hypertension. However, the effects of TIPS in patients with liver cirrhosis and portal hypertension have not been adequately verified in clinical trials.
To evaluate the effects of TIPS in patients with liver cirrhosis and portal hypertension with or without portal vein thrombosis (PVT).
A total of 55 patients with liver cirrhosis and portal hypertension received TIPS treatment from December 2014 to April 2018 were enrolled. Clinical data, including portal pressure, Child-Pugh score, and relevant complications were recorded.
TIPS was successfully performed in 54 patients. The overall technical success rate was 98.19% without serious technical complications. After TIPS treatment, portal pressure was significantly reduced from 38.13 ± 4.00 cmH2O to 24.14 ± 3.84 cmH2O (P < 0.05). In addition, symptoms including gastrointestinal bleeding and ascites were improved after TIPS treatment. During the 6 to 21-month follow up, hepatic encephalopathy in 15 patients (27.8%), shunt dysfunction in 5 patients (9.3%), rebleeding in 12 patients (22.2%) and deterioration of liver function in 2 patients (3.7%) were recorded. Moreover, there were no significant differences in the rates of rebleeding and hepatic encephalopathy between patients with PVT and the non-PVT group, whereas the occurrence rate of TIPS dysfunction was higher in the PVT group.
TIPS treatment could alleviate the symptoms of liver cirrhosis and portal hypertension in individuals with or without PVT. However, complications during follow-up should be appropriately noted and addressed with corresponding treatments.

We report here a case of portopulmonary hypertension following transjugular intrahepatic portosystemic shunt.

To explore the candidates, efficacy and safety of interventional therapies in the treatment of portal vein occlusion (PVO).
In our study, 13 patients diagnosed with PVO were included. Of all 13 patients, two received percutaneous portal vein recanalization (PVR), 10 received PVR and transjugular intrahepatic portosystemic shunt (PVR-TIPS), and one underwent intrahepatic portal branch-large collateral vessel shunt.
Interventional approaches were completed in all patients, and the technical success rate was 100%. The portal pressure gradient of patients treated with PVR-TIPS fell from 31 ± 4 to 12 ± 3 mmHg. During the procedures, no life-threatening complications occurred. All the clinical symptoms were effectively controlled after the interventional therapies and all the patients survived during the follow-up, with no rebleeding or overt hepatic encephalopathy. But stent thrombosis occurred in one patient, the cumulative rate of stent patency was 92%.
Interventional therapy was proved to be a well tolerated and effective strategy for PVO. For PVO patients without high intrahepatic resistance, if the patient is equipped with available portal inflow tract (superior mesenteric vein or splenic vein) and outflow tract (intrahepatic portal branches), PVR is the first choice; if the outflow tract is completely blocked with only available inflow tract, PVR-TIPS can be considered. For PVO patients with high intrahepatic resistance, as long as there is an available portal inflow tract, PVR-TIPS can be adopted.

Idiopathic non-cirrhotic portal hypertension is a rare diagnosis caused by an unknown etiology with elevated intrahepatic portal pressures in the absence of underlying liver disease. We present a unique case of a 57-year-old male with a left ventricular assist device and preserved right ventricular function that was found to have an elevated hepatic venous pressure gradient and sequelae of portal hypertension without underlying liver disease. There is limited treatment available as management is primarily aimed toward preventing complications of the disease. This case highlights the need for further investigative research of this disease entity and its pathogenesis.

Portopulmonary hypertension (PoPH) is a well-recognized complication of portal hypertension. This study reports a case of PoPH that was secondarily caused by post-traumatic mesenteric arteriovenous fistula. A 38-year-old man with a history of knife stabbing wounds in the abdomen in 2003 was admitted to the hospital with exertional shortness of breath and a mechanic murmur over the umbilical region. Computed tomography indicated signs of PoPH and mesenteric arteriovenous fistula. Percutaneous catheter-directed embolization was first performed but failed. Subsequently, the patient was successfully treated with fistula resection and partial enterectomy. The patient had been postoperatively followed regularly, and chief symptoms had been alleviated significantly and pulmonary pressure had successfully decreased to normal range. We believe that this is the first case of PoPH caused by mesenteric arteriovenous fistula.

Regarding the treatment for a portosystemic shunt, surgical or interventional radiological closure of the shunt was established. Interventional radiology including balloon-occluded retrograde transvenous obliteration can worsen portal hypertension and create a large thrombus close to the major venous system in the case of a huge portosystemic shunt. In contrast, it is also difficult to treat some cases through surgery alone when huge complicated shunts exist very deep in the body. Herein, we report a successful case of surgical shunt ligation for portosystemic encephalopathy in a hybrid operation room that enabled intraoperative angiography and computed tomography. A 62-year-old woman with chronic hepatitis C was referred to our hospital due to high levels of serum ammonia and hepatic encephalopathy. She had a massive, complicated portosystemic shunt from the inferior mesenteric vein to the left renal vein but did not have esophageal or gastric varices. It was difficult to occlude the portosystemic shunt by interventional radiologic techniques because the shunt had an extremely large amount of blood flow and many collateral routes. We performed the shunt ligation in the hybrid operation room. Intraoperative angiography provided detailed information about the portosystemic shunt, such as direction or volume of blood flow and collateral routes in real time. Her encephalopathy disappeared completely and she remains healthy with improved liver functional reserve to date. In conclusion, this is a successful case of a hybrid operation for an extremely large and complicated portosystemic shunt, providing for intraoperative angiography as a safe and reliable surgical treatment for portosystemic encephalopathy in patients with liver cirrhosis.

In adult liver transplantation, renoportal anastomosis (RPA) has been introduced as a useful technique for patients with grade 4 portal vein thrombosis and a splenorenal shunt. Here, we report a pediatric case in which RPA allowed a left lateral lobe living donor liver transplantation (LDLT) despite portal vein thrombosis and a large splenorenal shunt. At 36 days old, the patient underwent a Kasai operation for biliary atresia. At 17 months old, she underwent LDLT because of repetitive cholangitis. Pretransplant examinations revealed a large splenorenal shunt and portal vein thrombosis. Simple end-to-end portal reconstruction and clamping of the collateral route after removing the thrombosis were unsuccessful. Thus, RPA was performed using a donor superficial femoral vein as an interpositional graft. The portal vein pressure was 20 mm Hg after arterial reperfusion. Ligation of the splenic artery reduced the portal vein pressure. Although she developed severe acute cellular rejection and chylous ascites, there were no signs of portal vein complications. She was discharged 73 days after transplantation without any signs of renal dysfunction. The patient\'s condition was good at her last follow-up, 22 months after transplantation. To our knowledge, this is the youngest case of RPA in pediatric left lateral lobe LDLT. Additionally, this is the first case of RPA with splenic artery ligation and using the donor\'s superficial femoral vein as the venous graft for RPA. Although long-term follow-up is necessary, RPA could be a salvage option in LDLT in infants if other methods are unsuccessful.