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Background Patients with hereditary hemorrhagic telangiectasia have liver vascular malformations that can cause high-output cardiac failure (HOCF). Known sequelae include pulmonary hypertension, tricuspid regurgitation, and atrial fibrillation. Methods and Results The objectives of this study were to describe the clinical, echocardiographic, and hemodynamic characteristics and prognosis of hereditary hemorrhagic telangiectasia patients with HOCF who were found to have a subaortic membrane (SAoM). A retrospective observational analysis comparing patients with and without SAoM was performed. Among a cohort of patients with HOCF, 9 were found to have a SAoM in the left ventricular outflow tract by echocardiography (all female, mean age 64.8±4.0 years). The SAoM was discrete and located in the left ventricular outflow tract 1.1±0.1 cm below the aortic annular plane. It caused turbulent flow, mild obstruction (peak velocity 2.8±0.2 m/s, peak gradient 32±4 mm Hg), and no more than mild aortic insufficiency. Patients with SAoM (n=9) had higher cardiac output (12.1±1.3 versus 9.3±0.7 L/min, P =0.04) and mean pulmonary artery pressures (36±3 versus 28±2 mm Hg, P =0.03) compared with those without SAoM (n=19) during right heart catheterization. Genetic analysis revealed activin receptor-like kinase 1 mutations in each of the 8 patients with SAoM who had available test results. The presence of a SAoM was associated with a trend towards higher 5-year mortality during follow-up. Conclusions SAoM with mild obstruction occurs in patients with hereditary hemorrhagic telangiectasia and HOCF. SAoM was associated with features of more advanced HOCF and poor outcomes.

This multidisciplinary update of the Society of Radiologists in Ultrasound consensus statement on liver elastography incorporates the large volume of new information available in the literature since the initial publication. The recommended procedure for acquiring stiffness measurements is reviewed. There has been substantial improvement in the acoustic radiation force impulse (ARFI) technology-most notably the addition of a quality assessment of the shear wave propagation. Due to the efforts of the Quantitative Imaging Biomarkers Alliance, or QIBA, the variability of liver stiffness measurements between systems had decreased. There are now effective treatments for hepatitis B and hepatitis C, and follow-up after effective treatment should be based on the use of the delta change of the value obtained at viral eradication or suppression. Because the detection of compensated advanced chronic liver disease (cACLD) is very important, the new guidelines are made based on the probability of cACLD for given stiffness values. The panel recommends a vendor-neutral rule of four for interpretation for ARFI techniques. This new method simplifies interpretation of liver stiffness results and is more clinically relevant., (© RSNA, 2020.)

Objectives: Measurements of liver stiffness and spleen stiffness are useful noninvasive ways to assess fibrosis and portal hypertension in patients with chronic liver disease. One method for assessing stiffness is by point shear wave elastography via acoustic radiation force impulse imaging (ARFI). Its advantage is that sites where stiffness is measured are visualized sonographically. However, its reliability has not been well established, and all studies done to date evaluating the use of ARFI in chronic liver disease have been performed outside the United States. We aimed to characterize the intraobserver and interobserver variability of ARFI-measured liver and spleen stiffness., Methods: Two hepatologists evaluated unselected hepatology outpatients with ARFI. Exclusions were hepatocellular carcinoma, ascites, a surgical shunt or transjugular intrahepatic portosystemic shunt, portal thrombosis, and cholestatic disease. Each operator obtained 20 measurements from the right liver lobe and spleen. Intraclass correlation coefficients (ICC) were calculated., Results: A total of 177 patients were included: median age, 61 years; 85% male; and 43% obese. Intraobserver ICCs were the same for both observers for liver stiffness (0.89; 95% confidence interval [CI], 0.85-0.92) and spleen stiffness (0.72; 95% CI, 0.61-0.80). Interobserver agreement was excellent for liver stiffness (ICC, 0.85; 95% CI, 0.76-0.90) but not as good for spleen stiffness (ICC, 0.73; 95% CI, 0.60-0.83). A body mass index of 30 kg/m 2 or greater, waist circumference of greater than 105 cm, and skin-to-capsule distance of 2 cm or greater negatively affected the ICC for liver stiffness; small spleen size negatively affected the ICC for spleen stiffness., Conclusions: To our knowledge, this article is the first report of ARFI findings in a US population with chronic liver disease. Liver stiffness reproducibility was excellent, particularly in nonobese patients. Spleen stiffness reproducibility was excellent in those with larger spleens and therefore may be most useful in patients with cirrhosis and portal hypertension., (© 2016 by the American Institute of Ultrasound in Medicine.)

Liver involvement in diabetes is well recognized in the form of steatohepatitis and glycogenic hepatopathy. More recently, sinusoidal fibrosis, even in the absence of steatosis, has also been suggested to be associated with diabetes (diabetic hepatosclerosis); however, case-control studies are lacking. In addition, microangiopathy (hyaline arteriolosclerosis), a well-known complication of diabetes, has not been well studied in liver. Therefore, we undertook a cross-sectional blinded study with the specific aim of evaluating the association between hepatic sinusoidal fibrosis and hepatic arteriolosclerosis (HA) with diabetes. Liver biopsy findings from 89 diabetic patients obtained between January 2006 and December 2009 were compared with those of 89 nondiabetic patients matched by age and hepatitis C virus infection status. Patients with cirrhosis, liver mass, right heart failure, significant alcohol use, or insufficient available clinical information were excluded. Medical records were reviewed for the presence of diabetes, body mass index, diabetes treatment, and comorbidities at the time of biopsy (eg, underlying liver disease, hypertension, dyslipidemia). Liver biopsies were evaluated blinded to all clinical data (including presence or absence of diabetes) for a variety of histologic features, especially patterns of fibrosis and HA. Diabetic patients had a higher average body mass index (33 vs. 30 m/kg, P=0.0039), prevalence of hypertension (78% vs. 33%, P<0.0001), and dyslipidemia (52% vs. 20%, P<0.0001). Among diabetic patients, 87% had type 2 diabetes, and 57% used insulin. Whereas sinusoidal fibrosis, with or without steatosis, was not significantly associated with the presence of diabetes, HA was significantly more prevalent among diabetic patients compared with controls: 45% versus 29% (P=0.0298). The presence of both diabetes and hypertension had a significant odds for HA: with an adjusted odds ratio of 2.632 (95% confidence interval, 1.178-5.878; P=0.0183). Biliary changes were associated with HA in some cases (10.6%).In this study, we describe the histopathologic entity of HA for the first time. It is a small-vessel complication (microangiopathy) of the liver observed mainly in patients with diabetes who also have arterial hypertension. The clinical and prognostic implications of this finding, particularly regarding liver injury, remain to be further investigated.

Unlabelled: We have previously shown, in a semiquantitative analysis of liver biopsies showing cirrhosis, that thickness of fibrous septa separating cirrhotic nodules and small size of cirrhotic nodules correlated independently with portal pressure (as determined by the hepatic venous pressure gradient; HVPG) and were independent predictors of the presence of clinically significant portal hypertension (PH). This study aimed to confirm these results using quantitative analysis of these biopsies using digital image analysis. Biopsies of 42 patients with cirrhosis and HVPG measurements within 6 months of the biopsy were included in the study. The following parameters were scored quantitatively and without knowledge of HVPG results: total fibrosis area, septal thickness, nodule size, and number of nodules per millimeter of length of liver biopsy. Fibrosis area was the only parameter that independently correlated with HVPG (r = 0.606; P < 0.0001). Correlation was significant, even among patients with clinically significant PH (r = 0.636; P < 0.005). Fibrosis area and nodule size were both independently predictive of the presence of clinically significant PH (r = 0.57; P = 0.003)., Conclusions: On quantitative analysis, fibrosis area was the parameter that correlated best with HVPG and the presence of clinically significant PH. Beyond pathophysiological implications, this also has methodological implications that are discussed in this article., (Copyright © 2011 American Association for the Study of Liver Diseases.)

The modern paradigm considers cirrhosis as a dynamic and potentially reversible disease. It consists of two different entities, compensated and decompensated cirrhosis, each with a distinct prognosis and different predictors of survival. The development of portal hypertension is a hallmark in the history of cirrhosis, and its progression parallels that of the disease. In consequence, portal pressure measurement by means of HVPG allows stratifying cirrhosis in stages with defined outcomes, prognosis, and management strategies.

Background & Aims: Over-the-counter analgesics have been proposed to lead to decompensation of compensated cirrhosis or to further decompensation of an already decompensated patient. We performed a prospective, case-control study to investigate the effects of analgesics on acute hepatic decompensation., Methods: Data from consecutive cirrhotic patients hospitalized at 2 tertiary care hospitals for decompensation of cirrhosis (cases, n = 91) were compared with that from consecutive patients with compensated cirrhosis that were followed in the liver clinic (n = 153) and with randomly selected noncirrhotic patients concurrently hospitalized with the cases (n = 89). All patients were given a structured questionnaire to collect information on recent use of acetaminophen, nonsteroidal anti-inflammatory drugs and alcohol., Results: Only 32 (35%) of the cirrhotic patients used over-the-counter analgesics (19% acetaminophen, 16% nonsteroidal anti-inflammatory drugs), compared with 80 of the cirrhotic controls (52%; 25% acetaminophen, 31% nonsteroidal anti-inflammatory drugs), and 62 (70%) of the noncirrhotic controls. Acetaminophen use did not differ between groups, even for those with recent alcohol use. The doses and days of nonsteroidal anti-inflammatory drug use were higher among cirrhotic patients, compared with controls. Alcohol ingestion was significantly greater among patients with alcoholic cirrhosis, compared with controls., Conclusions: In patients with cirrhosis, acetaminophen use at doses lower than those recommended is not associated with acute hepatic decompensation, even in patients with recent alcohol ingestion. Nonsteroidal anti-inflammatory drugs might be associated with deleterious effects on cirrhosis. Alcohol ingestion is associated with decompensation in patients with alcoholic cirrhosis.

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant vascular disorder that can involve the liver diffusely in the form of vascular malformations ranging from small telangiectases to discrete arteriovenous malformations. Anatomically, three different patterns of abnormal vascular communications can occur in liver: portal vein to hepatic vein (portovenous), hepatic artery to hepatic vein (arteriovenous) and hepatic artery to portal vein (arterioportal), with the most common being arteriovenous. Only 5 to 8% of patients with these vascular malformations are symptomatic. When symptomatic, patients present with high-output cardiac failure, biliary ischemia (which, when severe, can progress to biliary and hepatic necrosis and lead to acute liver failure), or portal hypertension. Other less common presentations include portosystemic encephalopathy and abdominal angina. Diagnosis is confirmed by Doppler ultrasonography or multidetector computed tomography. The hallmark findings are intrahepatic hypervascularization and an enlarged common hepatic artery. Focal nodular hyperplasia and nodular regenerative hyperplasia are common findings. Symptomatic patients are treated with intensive medical treatment aimed at the predominant clinical presentation. Patients who fail aggressive medical therapy and those with acute biliary/hepatic necrosis should be considered for liver transplantation.

Liver involvement in hereditary hemorrhagic telangiectasia (HHT) consists of extensive intrahepatic vascular malformations associated with blood shunting (arteriovenous, arterioportal and/or portovenous). It is a rare disorder that nevertheless can result in significant systemic and hepatobiliary abnormalities. Although hepatic vascular malformations are present in a majority of patients with HHT, symptoms occur in a only a minority with a clear predominance for the female gender. Symptoms from liver vascular malformations are often misdiagnosed and this can lead to potentially harmful interventions. In this review article, clinical findings of liver involvement in HHT and their pathophysiology are discussed as well as diagnostic methodologies, therapies used and their outcome. Data presented is based on a review of the literature performed in October 2006 using the following MEDLINE search terms: (hereditary hemorrhagic telangiectasia [ALL] OR Rendu-Osler-Weber [ALL]) AND (liver OR hepatic [ALL]). Papers were considered if they were published in English and if they included specific cases that were sufficiently described.

Background/aims: While the definitive diagnosis of cirrhosis is histological, it is the degree of portal hypertension, as determined by the hepatic venous pressure gradient (HVPG), that is an important determinant of the severity of cirrhosis. An HVPG > or =10 mmHg (termed clinically significant portal hypertension or CSPH) is predictive of the development of complications of cirrhosis, including death. This study aimed to determine the relationship between specific histological parameters and HVPG in cirrhosis., Methods: Forty-three patients with biopsy-proven cirrhosis and HVPG measurements within 6 months of the biopsy were included in the study. The following parameters were scored semiquantitatively and without knowledge of HVPG results: sinusoidal fibrosis, septal thickness, loss of portal tracts and central veins, nodule size, inflammation, steatosis, and iron., Results: Septal thickness (p=0.03), small nodularity (p=0.003), loss of portal tracts (p=0.01), inflammation (p=0.04) and alcoholic etiology (p=0.01) correlated with the presence of CSPH. However, small nodularity and septal thickness were the only parameters independently predictive of CSPH (r=0.658, p<0.05)., Conclusions: We describe a subclassification of histological cirrhosis based on the severity of portal hypertension that consists of a combination of nodule size and septal thickness, with small nodularity and thick septa being independent predictors of the presence of CSPH.

Two methods are used to estimate ultrasound attenuation in liver. These were based on amplitude change and frequency change as a result of depth dependent attenuation. Evaluation of the two methods against a family of calibrated phantoms yielded correlation coefficients of 0.98 and 0.99, respectively. Liver attenuation in 26 control subjects was 0.50 and 0.52 dB/MHz/cm, respectively. Liver attenuation was estimated in 50 patients who later underwent liver biopsy. Comparison with quantitative histologic results showed that the presence of fat alone accounted for the increased attenuation associated with cirrhosis. Similar high attenuation values were found in patients with fatty infiltration. Fibrosis alone did not result in elevated liver attenuation. Cirrhotics without fatty infiltration had attenuation similar to that of the controls. Mechanisms of action are discussed.