title: Fontan circulation causes early, severe liver damage. Should we offer patients a tailored strategy?
abstract: BACKGROUND
  In patients with Fontan circulation, the liver is profoundly affected by chronic venous stasis. Little is known about early hepatic changes in this population.
  
  
  METHODS
  We performed echocardiography, abdominal ultrasound, liver elastography, cardiac catheterization, esophago-gastro-duodenoscopy and calculated MELD-XI score in 64 Fontan patients (69% minors), at an interval of 1-15years since Fontan.
  
  
  RESULTS
  Cardiac output remained stable in the first 5years after Fontan, then significantly decreased (r=-0.45, p(r=0)=0.003). NYHA class significantly increased after Fontan. Patients in NYHA class II/III (n=21, 14 minors) had significantly higher hepatic pressures, but normal ventricular function and pulmonary vascular resistances (PVR). Patients with pulmonary arterial pressure (PAP) ≥15mmHg (n=12, 6 minors) and those with PVR≥2WU*m(2) (n=27, 25 minors), had higher hepatic pressures (p<0.0001), a higher incidence of liver collaterals and/or esophageal varices (p<0.0001) and splenomegaly (p<0.02). Liver stiffness (LS) was elevated in most patients (median, 25th-75th percentile:17.3KPa, 14.1-21.4). It rapidly increased during the first 5-years after Fontan, compared to the following 5-years (from 12.2KPa, 9.8-14.1 to 17.5KPa, 14.3-24.5, p=0.007), then remained stable (19.1KPa, 16.9-22.6, p=0.60). MELD-XI score increased linearly with the time interval since Fontan (r=0.31, p(r=0)=0.01). For patients above 12years we found a linear correlation between LS and MELD-XI score in the 6-15years period after Fontan (r=0.40. p(r=0)=0.04). The overall incidence of established liver cirrhosis was 22%.
  
  
  CONCLUSIONS
  This is the largest study showing that Fontan circulation prompts early, progressive and eventually irreversible liver damage. Precautions should be taken immediately after Fontan, to protect this fragile population.
authors.affiliation: Division of Pediatric Cardiology, Citta' della Salute e della Scienza, Turin, Italy. Electronic address: gagnoletti@cittadellasalute.to.it.
authors.forename: Gabriella
authors.initials: G
authors.lastname: Agnoletti
authors.affiliation: Division of Pediatric Cardiology, Citta' della Salute e della Scienza, Turin, Italy.
authors.forename: Gaetana
authors.initials: G
authors.lastname: Ferraro
authors.affiliation: Division of Pediatric Cardiology, Citta' della Salute e della Scienza, Turin, Italy.
authors.forename: Roberto
authors.initials: R
authors.lastname: Bordese
authors.affiliation: Division of Pediatric Cardiology, Citta' della Salute e della Scienza, Turin, Italy.
authors.forename: Davide
authors.initials: D
authors.lastname: Marini
authors.affiliation: Division of Pediatric Cardiology, Citta' della Salute e della Scienza, Turin, Italy.
authors.forename: Simona
authors.initials: S
authors.lastname: Gala
authors.affiliation: Division of Statistics, Citta' della Salute e della Scienza, Turin, Italy.
authors.forename: Laura
authors.initials: L
authors.lastname: Bergamasco
authors.affiliation: Division of Pediatric Cardiology, Citta' della Salute e della Scienza, Turin, Italy.
authors.forename: Francesca
authors.initials: F
authors.lastname: Ferroni
authors.affiliation: Division of Gastroenterology, Citta' della Salute e della Scienza, Turin, Italy.
authors.forename: Pier Luigi
authors.initials: PL
authors.lastname: Calvo
authors.affiliation: Division of Gastroenterology, Citta' della Salute e della Scienza, Turin, Italy.
authors.forename: Claudio
authors.initials: C
authors.lastname: Barletti
authors.affiliation: Division of Gastroenterology, Citta' della Salute e della Scienza, Turin, Italy.
authors.forename: Fabio
authors.initials: F
authors.lastname: Cisarò
authors.affiliation: Department of Cinical and Biological Sciences, University of Turin, Italy.
authors.forename: Filomena
authors.initials: F
authors.lastname: Longo
authors.affiliation: Division of Pediatric Cardiology Cardiac Surgery, Citta' della Salute e della Scienza, Turin, Italy.
authors.forename: Carlo
authors.initials: C
authors.lastname: Pace Napoleone
mesh_terms: D000293:Adolescent; D002648:Child; D054459:Elasticity Imaging Techniques; D005260:Female; D018729:Fontan Procedure / Q000009:adverse effects*; D006801:Humans; D008107:Liver Diseases / Q000000981:diagnostic imaging* / Q000209:etiology*; D008297:Male; D012307:Risk Factors; D012720:Severity of Illness Index; D014463:Ultrasonography
keywords: Congenital heart disease; Fontan; Liver