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 ar...

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Bibliographic Details
Published in:International journal of cardiology 2011-09, Vol.151 (3), p.268-272
Main Authors: Bryant, Timothy, Ahmad, Zaheer, Millward-Sadler, Harry, Burney, Kashif, Stedman, Brian, Kendall, Tim, Vettukattil, Joseph, Haw, Marcus, Salmon, Anthony P, Cope, Richard, Hacking, Nigel, Breen, David, Sheron, Nick, Veldtman, Gruschen R
Format: Article
Language:English
Subjects:
Adolescent
Adult
Biological and medical sciences
Budd-Chiari Syndrome - diagnosis
Budd-Chiari Syndrome - etiology
Cardiology. Vascular system
Cardiovascular
Female
Focal nodular hyperplasia
Fontan
Fontan Procedure - adverse effects
Hepatic nodules
Humans
Liver - blood supply
Liver - injuries
Liver - pathology
Liver Circulation - physiology
Male
Medical sciences
Young Adult
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Summary: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.
ISSN:0167-5273
1874-1754
DOI:10.1016/j.ijcard.2010.05.047