title: Arterialised hepatic nodules in the Fontan circulation: hepatico-cardiac interactions.
abstract: Hypervascular nodules occur commonly when there is hepatic venous outlet obstruction. Their nature and determinants in the Fontan circulation is poorly understood. We reviewed the records of 27 consecutive Fontan patients who had computerized tomography scan (CT) over a 4 year period for arterialised nodules and alterations in hepatic flow patterns during contrast enhanced CT scans and related these findings to cardiac characteristics. Mean patient age was 24 ± 5.8 years, (range 16.7-39.8) and mean Fontan duration was 16.8 ± 4.8 years (range 7.3-28.7). Twenty-two patients demonstrated a reticular pattern of enhancement, 4 a zonal pattern and only 1 demonstrated normal enhancement pattern. Seven (26%) patients had a median of 4 (range 1-22) arterialised nodules, mean size 1.8 cm (range 0.5 to 3.2 cm). All nodules were located in the liver periphery, their outer aspect lying within 2 cm of the liver margin. Patients with nodules had higher mean RA pressures (18 mmHg ± 5.6 vs. 13 mmHg ± 4, p=0.025), whereas their mixed venous saturation and aortic saturation was not significantly different (70% ± 11 vs. 67% ± 9 and 92% ± 10 vs. 94% ± 4, p>0.05). Post-mortem histology suggests focal nodular hyperplasia is the underlying pathology. ConclusionsAbnormalities of hepatic blood flow and the presence of arterialised nodules are common in the failing Fontan circulation. They occur especially when central venous pressures are high, and very likely indicate arterialisation of hepatic blood flow and reciprocal portal venous deprivation. The underlying pathology is most likely focal nodular hyperplasia.
authors.affiliation: Department of Radiology, Southampton General Hospital, UK.
authors.forename: Timothy
authors.initials: T
authors.lastname: Bryant
authors.forename: Zaheer
authors.initials: Z
authors.lastname: Ahmad
authors.forename: Harry
authors.initials: H
authors.lastname: Millward-Sadler
authors.forename: Kashif
authors.initials: K
authors.lastname: Burney
authors.forename: Brian
authors.initials: B
authors.lastname: Stedman
authors.forename: Tim
authors.initials: T
authors.lastname: Kendall
authors.forename: Joseph
authors.initials: J
authors.lastname: Vettukattil
authors.forename: Marcus
authors.initials: M
authors.lastname: Haw
authors.forename: Anthony P
authors.initials: AP
authors.lastname: Salmon
authors.forename: Richard
authors.initials: R
authors.lastname: Cope
authors.forename: Nigel
authors.initials: N
authors.lastname: Hacking
authors.forename: David
authors.initials: D
authors.lastname: Breen
authors.forename: Nick
authors.initials: N
authors.lastname: Sheron
authors.forename: Gruschen R
authors.initials: GR
authors.lastname: Veldtman
mesh_terms: D000293:Adolescent; D000328:Adult; D006502:Budd-Chiari Syndrome / Q000175:diagnosis* / Q000209:etiology; D005260:Female; D018729:Fontan Procedure / Q000009:adverse effects*; D006801:Humans; D008099:Liver / Q000098:blood supply / Q000293:injuries* / Q000473:pathology*; D008102:Liver Circulation / Q000502:physiology; D008297:Male; D055815:Young Adult