title: Serial cardiac MRIs in adult Fontan patients detect progressive hepatic enlargement and congestion.
abstract: BACKGROUND
  The progression of hepatic disease in adult Fontan patients is not well understood. They reviewed the experience with serial cardiac MRIs (CMR) in adult Fontan patients to determine if hepatic anatomic markers of prolonged Fontan exposure were present and if clinical predictors of progressive hepatic congestion could be identified.
  
  
  RESULTS
  A retrospective cohort study of all adult Fontan patients who had undergone at least two CMRs was performed. Hepatic dimensions, inferior vena cava (IVC) size, right hepatic vein (RHV) size and spleen diameter were determined from images acquired at the time of clinically guided CMR. Two radiologists with expertise in hepatic imaging graded congestion and liver size independently using post-gadolinium contrast sequences. Twenty-seven patients met inclusion criteria. Over a mean time of 5.1 years between CMRs, there was a significant increase in mean lateral-medial hepatic dimension (P = .005), mean RHV diameter (P = .004), and mean splenic diameter (P = .001). Serial post-gadolinium imaging was available in 25/27 (93%) patients of which 15/27 (55%) showed evidence of progressive hepatic congestion across serial studies. Progressive hepatic congestion was associated with single ventricle ejection fraction (SVEF) less than 50% (P = .008), and larger indexed end-diastolic (EDVI) and end-systolic volume (ESVI). RHV diameter was the only anatomic variable significantly correlated with time from Fontan completion (P = .004).
  
  
  CONCLUSIONS
  Serial CMRs detected progressive liver and hepatic vein enlargement in our cohort of adult Fontan patients over a mean time of 5.2 years. Progressive hepatic congestion occurs in a significant number of adult Fontan patients and may be associated with ventricular enlargement and decreased ventricular function by CMR.
authors.affiliation: Division of Cardiology, Department of Medicine, Schneeweiss Adult Congenital Heart Center, Columbia University Medical Center, New York, New York, USA.
authors.forename: Matthew J
authors.initials: MJ
authors.lastname: Lewis
authors.affiliation: Division of Abdominal Imaging, Department of Radiology, Columbia University Medical Center, New York, New York, USA.
authors.forename: Elizabeth
authors.initials: E
authors.lastname: Hecht
authors.affiliation: Division of Cardiology, Department of Medicine, Schneeweiss Adult Congenital Heart Center, Columbia University Medical Center, New York, New York, USA.
authors.forename: Jonathan
authors.initials: J
authors.lastname: Ginns
authors.affiliation: Division of Abdominal Imaging, Department of Radiology, Columbia University Medical Center, New York, New York, USA.
authors.forename: Joshua
authors.initials: J
authors.lastname: Benton
authors.affiliation: Division of Abdominal Imaging, Department of Radiology, Columbia University Medical Center, New York, New York, USA.
authors.forename: Martin
authors.initials: M
authors.lastname: Prince
authors.affiliation: Division of Cardiology, Department of Medicine, Schneeweiss Adult Congenital Heart Center, Columbia University Medical Center, New York, New York, USA.
authors.forename: Marlon S
authors.initials: MS
authors.lastname: Rosenbaum
mesh_terms: D000328:Adult; D003287:Contrast Media / Q000008:administration & dosage; D018450:Disease Progression; D005260:Female; D018729:Fontan Procedure / Q000009:adverse effects*; D005682:Gadolinium / Q000008:administration & dosage; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000503:physiopathology / Q000601:surgery*; D006503:Hepatic Veins / Q000000981:diagnostic imaging* / Q000503:physiopathology; D006529:Hepatomegaly / Q000000981:diagnostic imaging* / Q000209:etiology / Q000503:physiopathology; D006571:Heterocyclic Compounds / Q000008:administration & dosage; D006801:Humans; D008102:Liver Circulation; D008279:Magnetic Resonance Imaging; D008297:Male; D009942:Organometallic Compounds / Q000008:administration & dosage; D011237:Predictive Value of Tests; D012189:Retrospective Studies; D013154:Spleen / Q000000981:diagnostic imaging; D013318:Stroke Volume; D013997:Time Factors; D016896:Treatment Outcome; D014682:Vena Cava, Inferior / Q000000981:diagnostic imaging; D055815:Young Adult
chemical_list: D003287:Contrast Media; D006571:Heterocyclic Compounds; D009942:Organometallic Compounds; C062402:gadoteridol; D005682:Gadolinium
keywords: adult Fontan; cardiac MRI; hepatic disease