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Hepatic changes in the failing Fontan circulation

Hepatic changes in the failing Fontan circulation
Hepatic changes in the failing Fontan circulation
Background: The failing Fontan circulation is associated with hepatic impairment. The nature of this liver injury is poorly defined.
Objective: To establish the gross and histological liver changes of patients with Fontan circulation relative to clinical, biochemical and haemodynamic findings.
Methods: Patients were retrospectively assessed for extracardiac Fontan conversion between September 2003 and June 2005, according to an established clinical protocol. Twelve patients, mean age 24.6 (range 15.8–43.4) years were identified. The mean duration since the initial Fontan procedure was 14.1 (range 6.9–26.4) years.
Results: Zonal enhancement of the liver (4/12) on CT was more common in patients with lower hepatic vein pressures (p = 0.007), and in those with absent cardiac cirrhosis on histological examination (p = 0.033). Gastro-oesophageal varices (4/12) were more common in patients with higher hepatic vein pressure (21 (6.3) vs 12.2 (2.2) mm Hg, p = 0.013) and associated with more advanced cirrhosis (p = 0.037). The extent of cirrhosis (7/12) was positively correlated with the hepatic vein pressure (r = 0.83, p = 0.003). A significant positive correlation was found between the Fontan duration and the degree of hepatic fibrosis (r = 0.75, p = 0.013), as well as presence of broad scars (r = 0.71, p = 0.021). Protein-losing enteropathy (5/12) occurred more frequently in patients with longer Fontan duration (11.7 (3.2) vs 17.9 (6.1) years, p = 0.038).
Conclusions: Liver injury, which can be extensive in this patient group, is related to Fontan duration and hepatic vein pressures. CT scan assists non-invasive assessment. Cardiac cirrhosis with the risk of developing gastro-oesophageal varices and regenerative liver nodules, a precursor to hepatocellular carcinoma, is common in this patient group.
579-584
Kiesewetter, Christoph H.
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Sheron, Nick
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Vettukattill, Joseph J.
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Hacking, Nigel
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Stedman, Brian
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Millward-Sadler, Harry
db60d76b-22ce-4da2-85b7-40ddd5734378
Haw, Marcus
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Cope, Richard
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Salmon, Anthony P.
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Sivaprakasam, Muthukumaran C.
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Kendall, Tim
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Keeton, Barry R.
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Iredale, John P.
607673ce-77b2-4418-b317-2aa778110ee2
Veldtman, Gruschen R.
d9b03375-5c07-46b8-9d85-8ac5b0f8e08a
Kiesewetter, Christoph H.
2c781566-a16f-457f-a7cf-2aa4f8f5178e
Sheron, Nick
cbf852e3-cfaa-43b2-ab99-a954d96069f1
Vettukattill, Joseph J.
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Hacking, Nigel
fdc70f9c-e9d6-485c-a53d-e6988fa75cb0
Stedman, Brian
180a4644-f670-4da1-84db-9d5640e4ec1d
Millward-Sadler, Harry
db60d76b-22ce-4da2-85b7-40ddd5734378
Haw, Marcus
9568da9d-30da-4d2e-887c-d9565bdf2e5f
Cope, Richard
12e7ce3c-2de9-4d17-aeee-056e8aa7b1b4
Salmon, Anthony P.
fef1b05b-8bb8-45ab-8183-73f422c1955c
Sivaprakasam, Muthukumaran C.
82b71a00-96db-4bc0-8678-1239f2186c77
Kendall, Tim
46abfa7a-8d31-48e9-8824-6096bf7f4213
Keeton, Barry R.
614aecb7-3e20-4e24-b4ca-91d1a13484fa
Iredale, John P.
607673ce-77b2-4418-b317-2aa778110ee2
Veldtman, Gruschen R.
d9b03375-5c07-46b8-9d85-8ac5b0f8e08a

Kiesewetter, Christoph H., Sheron, Nick, Vettukattill, Joseph J., Hacking, Nigel, Stedman, Brian, Millward-Sadler, Harry, Haw, Marcus, Cope, Richard, Salmon, Anthony P., Sivaprakasam, Muthukumaran C., Kendall, Tim, Keeton, Barry R., Iredale, John P. and Veldtman, Gruschen R. (2006) Hepatic changes in the failing Fontan circulation. Heart, 93 (5), 579-584. (doi:10.1136/hrt.2006.094516).

Record type: Article

Abstract

Background: The failing Fontan circulation is associated with hepatic impairment. The nature of this liver injury is poorly defined.
Objective: To establish the gross and histological liver changes of patients with Fontan circulation relative to clinical, biochemical and haemodynamic findings.
Methods: Patients were retrospectively assessed for extracardiac Fontan conversion between September 2003 and June 2005, according to an established clinical protocol. Twelve patients, mean age 24.6 (range 15.8–43.4) years were identified. The mean duration since the initial Fontan procedure was 14.1 (range 6.9–26.4) years.
Results: Zonal enhancement of the liver (4/12) on CT was more common in patients with lower hepatic vein pressures (p = 0.007), and in those with absent cardiac cirrhosis on histological examination (p = 0.033). Gastro-oesophageal varices (4/12) were more common in patients with higher hepatic vein pressure (21 (6.3) vs 12.2 (2.2) mm Hg, p = 0.013) and associated with more advanced cirrhosis (p = 0.037). The extent of cirrhosis (7/12) was positively correlated with the hepatic vein pressure (r = 0.83, p = 0.003). A significant positive correlation was found between the Fontan duration and the degree of hepatic fibrosis (r = 0.75, p = 0.013), as well as presence of broad scars (r = 0.71, p = 0.021). Protein-losing enteropathy (5/12) occurred more frequently in patients with longer Fontan duration (11.7 (3.2) vs 17.9 (6.1) years, p = 0.038).
Conclusions: Liver injury, which can be extensive in this patient group, is related to Fontan duration and hepatic vein pressures. CT scan assists non-invasive assessment. Cardiac cirrhosis with the risk of developing gastro-oesophageal varices and regenerative liver nodules, a precursor to hepatocellular carcinoma, is common in this patient group.

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Published date: 27 September 2006

Identifiers

Local EPrints ID: 59349
URI: http://eprints.soton.ac.uk/id/eprint/59349
PURE UUID: 59bc96be-176e-41c6-82eb-aaa877bf0e94
ORCID for Nick Sheron: ORCID iD orcid.org/0000-0001-5232-8292

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Date deposited: 03 Sep 2008
Last modified: 15 Mar 2024 11:15

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Contributors

Author: Christoph H. Kiesewetter
Author: Nick Sheron ORCID iD
Author: Joseph J. Vettukattill
Author: Nigel Hacking
Author: Brian Stedman
Author: Harry Millward-Sadler
Author: Marcus Haw
Author: Richard Cope
Author: Anthony P. Salmon
Author: Muthukumaran C. Sivaprakasam
Author: Tim Kendall
Author: Barry R. Keeton
Author: John P. Iredale
Author: Gruschen R. Veldtman

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