The authors provide further insight into the phenotype of AOS with SMAD3 mutations, and present recommendations for a clinical work-up.

Background— Recent transcatheter aortic valve replacement studies have raised concerns about adverse cerebrovascular events. The etiopathology of the embolized material is currently unknown. Methods and Results— A total of 40 patients underwent transcatheter aortic valve replacement with the use of a dual filter–based embolic protection device (Montage Dual Filter System, Claret Medical, Inc). Macroscopic material liberated during the transcatheter aortic valve replacement procedure was captured in the device filter baskets in 30 (75%) patients and sent for histopathologic analysis. The captured material varied in size from 0.15 to 4.0 mm. Amorphous calcified material (size, 0.55–1.8 mm) was identified in 5 patients (17%). In 8 patients (27%), the captured material (size, 0.25–4.0 mm) contained valve tissue composed of loose connective tissue (collagen and elastic fibers) with focal areas of myxoid stroma, with or without coverage by endothelial cells and intermixed with fibrin. In another 13 (43%) patients, collagenous tissue, which may represent elements of vessel wall and valvelike structures, was identified. In 9 patients (30%), thrombotic material was intermixed with neutrophils (size, 0.15–2.0 mm). Overall, thrombotic material was found in 52% of patients, and tissue fragments compatible with aortic valve leaflet or aortic wall origin were found in 52% (21/40) of patients. Conclusions— Embolic debris traveling to the brain was captured in 75% of transcatheter aortic valve replacement procedures where a filter-based embolic protection device was used. The debris consisted of fibrin, or amorphous calcium and connective tissue derived most likely from either the native aortic valve leaflets or aortic wall.

Chronic mesenteric ischaemia is a severe and incapacitating disease, causing complaints of post-prandial pain, fear of eating and weight loss. Even though chronic mesenteric ischaemia may progress to acute mesenteric ischaemia, chronic mesenteric ischaemia remains an underappreciated and undertreated disease entity. Probable explanations are the lack of knowledge and awareness among physicians and the lack of a gold standard diagnostic test. The underappreciation of this disease results in diagnostic delays, underdiagnosis and undertreating of patients with chronic mesenteric ischaemia, potentially resulting in fatal acute mesenteric ischaemia. This guideline provides a comprehensive overview and repository of the current evidence and multidisciplinary expert agreement on pertinent issues regarding diagnosis and treatment, and provides guidance in the multidisciplinary field of chronic mesenteric ischaemia.

Gastroesophageal variceal bleeding in patients with cirrhosis is associated with significant morbidity and mortality, as well as a high rebleeding risk. Limited data are available on the role of transjugular intrahepatic portosystemic shunt (TIPS) with covered stents in patients receiving standard endoscopic, vasoactive, and antibiotic treatment. In this multicenter randomized trial, long-term endoscopic variceal ligation (EVL) or glue injection 1 b-blocker treatment was compared with TIPS placement in 72 patients with a first or second episode of gastric and/or esophageal variceal bleeding, after hemodynamic stabilization upon endoscopic, vasoactive, and antibiotic treatment. Randomization was stratified according to Child-Pugh score. Kaplan-Meier (event-free) survival estimates were used for the endpoints rebleeding, death, treatment failure, and hepatic encephalopathy. During a median follow-up of 23 months, 10 (29%) of 35 patients in the endoscopy 1 b-blocker group, as compared to 0 of 37 (0%) patients in the TIPS group, developed variceal rebleeding (P 5 0.001). Mortality (TIPS 32% vs. endoscopy 26%; P 5 0.418) and treatment failure (TIPS 38% vs. endoscopy 34%; P 5 0.685) did not differ between groups. Early hepatic encephalopathy (within 1 year) was significantly more frequent in the TIPS group (35% vs. 14%; P 5 0.035), but during longterm follow-up this difference diminished (38% vs. 23%; P 5 0.121). Conclusions: In unselected patients with cirrhosis, who underwent successful endoscopic hemostasis for variceal bleeding, covered TIPS was superior to EVL 1 b-blocker for reduction of variceal rebleeding, but did not improve survival. TIPS was associated with higher rates of early hepatic encephalopathy. (HEPATOLOGY 2016;63:581-589) G astroesophageal variceal bleeding (GEVB) is a severe complication of portal hypertension. Rebleeding is associated with significant morbidity and mortality. Hospitalization costs for rebleeding range between $6,600 and $23,000 in the United States.For these reasons, management should be directed at its prevention.1,2 Secondary prevention is first achieved by endoscopic treatment (endoscopic variceal ligation [EVL] for esophageal varices and N-butyl cyanoacrylate injection for gastric varices) in combination with bAbbreviations: CI, confidence interval; EVL, endoscopic variceal ligation; GEVB, gastroesophageal variceal bleeding; HCC, hepatocellular carcinoma; HR, hazard ratio; IQR, interquartile range; MELD, Model for End-Stage Liver Disease; PTFE, polytetrafluorethylene; TIPS, transjugular intrahepatic portosystemic shunt.From the Departments of

Eligibility varied substantially depending on the sizing criterion. In clinical practice both under- and oversizing were common. Industry guidelines should recognize the oval shape of the aortic annulus.