title: Direct measurement of porto-systemic gradient in a failing Fontan circulation.
abstract: We describe the case history of a 42-year-old man with cardiac cirrhosis, portal hypertension, and life-threatening variceal bleeding after Fontan revision surgery. Direct pressure measurements in the portal vein, though high, demonstrated only a modest portosystemic gradient (PSG), 9 mm Hg. A transjugular intrahepatic portosystemic shunt procedure was performed. This reduced the PSG (3 mm Hg). His bleeding was controlled. The patient's histopathological findings were identical to that previously documented in Fontan patients, raising the question of whether these subdiaphragmatic hemodynamics are representative of the broader failing Fontan population.
authors.affiliation: Department of Adult Congenital Heart Disease, Cardiothoracic Division, Southampton University Hospital, Southampton, UK.
authors.forename: Madhavi
authors.initials: M
authors.lastname: Velpula
authors.forename: Nick
authors.initials: N
authors.lastname: Sheron
authors.forename: Neill
authors.initials: N
authors.lastname: Guha
authors.forename: Tony
authors.initials: T
authors.lastname: Salmon
authors.forename: Nigel
authors.initials: N
authors.lastname: Hacking
authors.forename: Gruschen R
authors.initials: GR
authors.lastname: Veldtman
mesh_terms: D000328:Adult; D001795:Blood Pressure Determination; D004932:Esophageal and Gastric Varices / Q000209:etiology; D017809:Fatal Outcome; D018729:Fontan Procedure / Q000009:adverse effects*; D006471:Gastrointestinal Hemorrhage / Q000209:etiology; D006330:Heart Defects, Congenital / Q000503:physiopathology / Q000601:surgery*; D006801:Humans; D006975:Hypertension, Portal / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology* / Q000601:surgery; D008297:Male; D017082:Portal Pressure; D019168:Portasystemic Shunt, Transjugular Intrahepatic; D012086:Reoperation; D017211:Treatment Failure