Non-cirrhotic Extra-Hepatic Porto-Systemic Shunt Causing Adult-Onset Encephalopathy Treated with Endovascular Closure.

INTRODUCTION: Encephalopathy secondary to hyperammonemia due to Congenital Extra-hepatic Porto-systemic shunt (CEPS) in the absence of liver cirrhosis is an exceptionally unusual condition. We describe the case of a 54-year-old woman admitted to the Emergency Department complaining of recurrent episodes of confusion and worsening cognitive impairment. At admission, the patient displayed slowing cognitive-motor skills with marked static ataxia and impaired gait. Hyperammonemia was detected in the serum. An abdominal computed tomography (CT) excluded portal hypertension and liver cirrhosis, detecting a congenital extra-hepatic porto-systemic shunt which is a highly unusual vascular malformation. The patient was treated by interventional radiologists with a successful endovascular closure. AREAS COVERED: We have performed a review of the last three decades of the literature, starting from the introduction of CT scanning in common clinical practice. Eighteen studies (case reports) described 29 patients with encephalopathy secondary to hyperammonemia due to CEPS in the absence of liver cirrhosis: They underwent treatment similar to our case report of CEPS. EXPERT COMMENTARY: Encephalopathy secondary to hyperammonemia in the absence of hepatic dysfunction is an important diagnostic dilemma to many clinicians. An interventional radiologic approach is currently preferred.

Coronary artery plaque formation at coronary CT angiography: morphological analysis and relationship to hemodynamics.

We aimed to demonstrate that coronary CT angiography (cCTA) can be used to non-invasively study the effect of hemodynamic factors in the pathophysiology of plaque formation. cCTA data of 73 patients were analyzed. All detected plaques were classified according to location (bifurcation, non-branching segment), configuration (eccentric, concentric), orientation (myocardial, lateral, epicardial side of the vessel wall), and composition (calcified, mixed, non-calcified). Bifurcation lesions were further characterized using the Medina classification. Of 382 plaques, 8.1% were in the LM, 46.3% in the LAD, 18.3% in the LCx, and 25.9% in the RCA. Also, 25.1% were completely calcified, 72.3% were mixed, and 2.6% were purely non-calcified. Of the plaques, 51.3% were bifurcation lesions. The most frequent (40%) Medina pattern was 1.1.0 (lesion starts before, extends beyond bifurcation, sparing the side branch). Eighty percent of plaques were eccentric. A significant (p < 0.01) majority (55%) were on the myocardial side, while 17.3% were lateral, and 27.7% epicardial. Of all non-calcified and mixed plaques, 45.1% (p < 0.01) were myocardial, whereas only 14.3% were lateral, 20.6% epicardial, and 19.9% concentric. We conclude that cCTA can non-invasively study the effect of vascular hemodynamics, such as turbulent flow (bifurcations) and low shear stress (myocardial vessel wall), on the distribution and composition of atherosclerotic plaque deposition.