A case of portopulmonary hypertension characterized by repeated syncope was retrospectively analyzed. Intrahepatic or extrahepatic factor-induced portal hypertension complicated with metabolic disorder of vasoactive substances, vascular pressure, inflammation, etc. may result in systolic and diastolic dysfunction of pulmonary arteries and systemic hyperdynamic circulation, the long-term effect of which can induce vascular remodeling and consequently, pulmonary hypertension. The pathogenic process is rather insidious. Pulmonary hypertension is clinically characterized by the raised average pulmonary artery pressure, normal pulmonary capillary wedge pressure and high pulmonary vascular resistance. Currently available therapeutic approaches include drug therapy targeting on pulmonary hypertension and liver transplantation.
Blood Pressure ; Humans ; Hypertension, Portal ; complications ; diagnosis ; Hypertension, Pulmonary ; complications ; diagnosis ; Liver Transplantation ; Syncope ; complications ; diagnosis

Humans ; Hypertension, Portal ; diagnosis ; etiology ; therapy ; Pancreatic Diseases ; complications

No abstract available.
*Elasticity Imaging Techniques ; Female ; Humans ; Hypertension, Portal/*complications/*diagnosis ; Liver Cirrhosis/*complications/*diagnosis ; Male

Portal vein thrombosis (PVT) is an uncommon cause of presinusoidal portal hypertension. Among various hepatoportal disorders, noncirrhotic portal hypertension conditions such as idiopathic portal hypertension (IPH) are considered to have a close relation with PVT. PVT is known to have several predisposing conditions, including infection, malignancies, and coagulation disorders. There is growing interest and recognition that deficiencies in proteins C and S are associated with a hypercoagulable state. These deficiencies are regarded as key factors of systemic hypercoagulability and recurrent venous thromboembolism. We report the case of a 19-year-old male diagnosed as IPH with PVT and combined deficiencies in proteins C and S.
Humans ; Hypertension, Portal/complications/*diagnosis/pathology ; Male ; *Portal Vein ; Protein C Deficiency/*complications ; Protein S Deficiency/*complications ; Tomography, X-Ray Computed ; Venous Thrombosis/complications/*diagnosis/pathology ; Young Adult

Portal hypertension is a severe consequence of chronic liver diseases and is responsible for the main clinical complications of liver cirrhosis. Hepatic venous pressure gradient (HVPG) measurement is the best available method to evaluate the presence and severity of portal hypertension. Clinically significant portal hypertension is defined as an increase in HVPG to >10 mmHg. In this condition, the complications of portal hypertension might begin to appear. HVPG measurement is increasingly used in the clinical fields, and the HVPG is a robust surrogate marker in many clinical applications such as diagnosis, risk stratification, identification of patients with hepatocellular carcinoma who are candidates for liver resection, monitoring of the efficacy of medical treatment, and assessment of progression of portal hypertension. Patients who had a reduction in HVPG of > or =20% or to < or =12 mmHg in response to drug therapy are defined as responders. Responders have a markedly decreased risk of bleeding/rebleeding, ascites, and spontaneous bacterial peritonitis, which results in improved survival. This review provides clinical use of HVPG measurement in the field of liver disease.
Chronic Disease ; Hemodynamics ; Hemorrhage/etiology ; Hepatic Veins/physiology ; Humans ; Hypertension, Portal/complications ; Liver Cirrhosis/diagnosis ; Liver Diseases/complications/*physiopathology ; Portal Pressure

<0.01). The cumulative 3-year recurrence-free survival rate showed no statistical difference between the two groups. However, the cumulative 3-year survival rate was significantly higher in the non-portal hypertension group (82.8% vs. 53%, respectively, P=0.014). CONCLUSION: Indirectly diagnosed portal hypertension is correlated with the development of complications and poor prognosis after the surgical resection of HCC.
Adult ; Aged ; Carcinoma, Hepatocellular/complications/*surgery ; Female ; Humans ; Hypertension, Portal/*diagnosis/etiology ; Liver Neoplasms/complications/*surgery ; Male ; Middle Aged ; Postoperative Complications/*diagnosis ; Prognosis ; Survival Rate

Owing to the high prevalence of various chronic liver diseases, cirrhosis is one of the leading causes of morbidity and mortality worldwide. In recent years, the development of non-invasive tests of fibrosis allows accurate diagnosis of cirrhosis and reduces the need for liver biopsy. In this review, we discuss the application of these non-invasive tests beyond the diagnosis of cirrhosis. In particular, their role in the selection of patients for hepatocellular carcinoma surveillance and varices screening is highlighted.
Biomarkers/blood ; Carcinoma, Hepatocellular/diagnosis ; *Elasticity Imaging Techniques ; Endoscopy, Digestive System ; Humans ; Hypertension, Portal/complications ; Liver Cirrhosis/complications/*diagnosis ; Liver Neoplasms/diagnosis ; Risk Factors

Hepatoportal sclerosis (HPS) is defined as sclerosis of portal areas in the absence of cirrhosis. There is little information about HPS in children in the literature. The aim of this study was to describe the clinical presentation, associated disorders, laboratory characteristics and outcome of children who were diagnosed as HPS. This study included 12 children diagnosed as HPS by the Pathology Department between 2005 and 2011. Data were collected from the gastroenterology clinic charts retrospectively, including demographics, presentation characteristics, laboratory data and recent status of patients. Twelve patients were enrolled (6 girls, 6 boys). The median age of patients was 13.5 yr. Median age at the time of biopsy was 11 yr. Four patients had splenomegaly, 3 had esophageal varices, one had hepatopulmonary syndrome and had been transplanted. Smooth muscle antibody was found positive in 4 patients, without autoimmune hepatitis findings in liver biopsy. One patient had celiac disease and another patient had positive celiac disease serology but pathology findings. Another patient had Turner's syndrome. Mean follow-up time was 39 months (3.3 yr) after biopsy. Hepatoportal sclerosis does not necessarily present with portal hypertension in children.
Adolescent ; Child ; Child, Preschool ; Female ; Follow-Up Studies ; Gastroenterology ; Humans ; Hypertension, Portal/complications/*diagnosis/pathology ; Liver/pathology ; Liver Diseases/complications/*diagnosis/pathology ; Male ; Portal Vein/pathology ; Retrospective Studies ; Sclerosis/*diagnosis/*pathology

OBJECTIVETo evaluate the predictive value of portal vein flow rate preoperative for portal vein thrombosis (PVT) after periesophagastric devascularization in hepatitis B cirrhosis-related portal hypertension.

METHODSFrom January 2007 to July 2008, 45 patients with portal hypertension caused by hepatitis B cirrhosis were performed splenectomy with peri-esophagogastric devascularization in the same medical group in West China Hospital of Sichuan University. The portal vein flow rate and the diameter of portal vein were measured with doppler sonography respectively before and after the operation. At the same time, the level of PT and PLT were detected. The weight of spleens were measured after operation.

RESULTSThirteen cases suffered from PVT postoperatively. Portal vein flow rate was significantly lower in patients with PVT postoperation than that in patients without PVT (P < 0.01). In patients with PVT (n = 13) postoperation, the preoperative portal vein flow rate was (19.5 +/- 5.3) cm/s. Among the 13 cases, there were 12 cases whose flow rate were lower than 25 cm/s, and 1 case whose flow rate was 32. 3 cm/s; In patients without PVT (n = 32), the preoperative portal vein flow rate was (9.6 +/- 8.0) cm/s. In patients with lower rate (n = 17), the incidence rate of PVT was 70.6%; in patients with higher rate (n = 28), the incidence rate of PVT was 3.6%. The incidence rate of PVT in patients with lower rate was significantly lower than patients with higher rate (P < 0.01). The diameter of portal vein in patients with PVT was significantly wider than patients without PVT. The diameter of portal vein was negative correlative with the portal vein flow rate. The value 25 cm/s was of diagnostic efficiency, the sensitivity was 92.3%, and specificity was 70.6%.

CONCLUSIONSThe portal vein flow rate preoperative can be used as an early predictor of portal vein thrombosis after periesophagastric devascularization in hepatitis B cirrhosis-related portal hypertension to give a guide to clinical work.

Adult ; Aged ; Blood Flow Velocity ; Female ; Humans ; Hypertension, Portal ; etiology ; physiopathology ; surgery ; Liver Cirrhosis ; complications ; Male ; Middle Aged ; Portal Vein ; diagnostic imaging ; physiopathology ; Postoperative Complications ; diagnosis ; etiology ; Preoperative Care ; Risk Factors ; Splenectomy ; Ultrasonography ; Venous Thrombosis ; diagnosis ; etiology

Ascites, hepatic encephalopathy and variceal hemorrhage are three major complications of portal hypertension. The diagnostic evaluation of ascites involves an assessment of its etiology by determining the serum-ascites albumin gradient and the exclusion of spontaneous bacterial peritonitis. Ascites is primarily related to an inability to excrete an adequate amount of sodium into urine, leading to a positive sodium balance. Sodium restriction and diuretic therapy are keys of ascites control. But, with the case of refractory ascites, large volume paracentesis and transjugular portosystemic shunts are required. In hepatorenal syndrome, splanchnic vasodilatation with reduction in effective arterial volume causes intense renal vasoconstriction. Splanchnic and/or peripheral vasoconstrictors with albumin infusion, and renal replacement therapy are only bridging therapy. Liver transplantation is the only definitive modality of improving the long term prognosis.
Anti-Bacterial Agents/therapeutic use ; Ascites/complications/*diagnosis/therapy ; Bacterial Infections/*diagnosis ; Hepatic Encephalopathy/complications ; Hepatorenal Syndrome/complications/*diagnosis/therapy ; Humans ; Hypertension, Portal/*complications ; Liver Transplantation ; Peritonitis/*diagnosis/drug therapy/etiology ; Serum Albumin/administration & dosage