title: Fontan-associated liver disease: Spectrum of US findings.
abstract: OBJECTIVE
  To describe ultrasonography (US) findings of Fontan-associated liver disease (FALD) and to determine whether screening US examinations can identify FALD before biochemical hepatic dysfunction.
  
  
  METHODS
  This retrospective study included 55 patients who underwent Fontan procedure over a 20-year period. Hepatobiliary US findings (n=55), CT or MRI findings (n=19), biochemical hepatic function tests (n=49), and histopathological results (n=4) were analyzed. Images were reviewed focusing on the hepatic parenchymal changes, presence of focal lesions, and signs of portal hypertension.
  
  
  RESULTS
  Hepatic parenchymal changes (either heterogeneous echotexture or surface nodularity) evident on US were present in 67% (37/55) and showed positive correlation with the Fontan duration. Hyper-echoic lesions were noted in 35% (19/55) and showed a predilection for multiplicity, small size, right lobe location, and irregular margin on high-frequency transducer. These lesions were not demonstrated by CT or MRI or by low-frequency transducer. Histopathological results of targeted biopsy for hyper-echoic lesions revealed lesser degree of patchy sinusoidal and portal fibrosis than seen in cases with surface nodularity. Abnormal parenchymal enhancement was commonly seen with CT or MRI in 63% (12/19) and hypervascular nodules in 21% (4/19). Most patients (82%, 40/49) showed normal biochemical hepatic function tests, despite the presence of hepatic parenchymal changes on imaging.
  
  
  CONCLUSIONS
  The common US findings of FALD included heterogeneous parenchymal echotexture, surface nodularity, and hyper-echoic lesions. We suggest that hyper-echoic lesions without surface nodularity detected by high-frequency transducer may represent the early stage of fibrosis. US examination may be useful for identifying the progression of FALD before biochemical hepatic dysfunction.
authors.affiliation: Department of Radiology and Center for Imaging Science, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul 135-710, South Korea.
authors.forename: Jung Min
authors.initials: JM
authors.lastname: Bae
authors.affiliation: Department of Radiology and Center for Imaging Science, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul 135-710, South Korea. Electronic address: hathor97.jeon@samsung.com.
authors.forename: Tae Yeon
authors.initials: TY
authors.lastname: Jeon
authors.affiliation: Department of Pathology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul 135-710, South Korea.
authors.forename: Jung Sun
authors.initials: JS
authors.lastname: Kim
authors.affiliation: Department of Pathology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul 135-710, South Korea.
authors.forename: Seokhwi
authors.initials: S
authors.lastname: Kim
authors.affiliation: Department of Radiology and Center for Imaging Science, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul 135-710, South Korea.
authors.forename: Sook Min
authors.initials: SM
authors.lastname: Hwang
authors.affiliation: Department of Radiology and Center for Imaging Science, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul 135-710, South Korea.
authors.forename: So-Young
authors.initials: SY
authors.lastname: Yoo
authors.affiliation: Department of Radiology and Center for Imaging Science, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul 135-710, South Korea.
authors.forename: Ji Hye
authors.initials: JH
authors.lastname: Kim
mesh_terms: D000293:Adolescent; D001653:Bile Ducts, Intrahepatic / Q000000981:diagnostic imaging / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D003287:Contrast Media; D018450:Disease Progression; D005260:Female; D005355:Fibrosis; D005500:Follow-Up Studies; D018729:Fontan Procedure / Q000009:adverse effects*; D006801:Humans; D006975:Hypertension, Portal / Q000000981:diagnostic imaging / Q000209:etiology / Q000473:pathology; D061705:Image-Guided Biopsy / Q000379:methods; D008107:Liver Diseases / Q000000981:diagnostic imaging* / Q000209:etiology / Q000473:pathology; D008111:Liver Function Tests; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D061330:Multidetector Computed Tomography / Q000379:methods; D011168:Portal System / Q000000981:diagnostic imaging / Q000473:pathology; D012189:Retrospective Studies; D014159:Transducers; D018084:Ultrasonography, Interventional / Q000379:methods; D055815:Young Adult
chemical_list: D003287:Contrast Media
keywords: Fontan-associated liver disease; Hepatic fibrosis; US