Survival of Fontan patients after HT has significantly improved in the current era. Currently, expected post-HT survival for Fontan patients is on par with other CHD patients. Fontan patients should not be excluded from consideration for HT solely on a history of Fontan.
Background
Liver cirrhosis is recognized with long-term follow-up of patients
after the Fontan procedure. The effect of liver cirrhosis on the use of
heart transplant (HT) and on post-HT outcomes is unknown.
Methods
We reviewed Fontan patients evaluated for HT from 2004 to 2012 with
hepatic computed tomography (CT) imaging, classified as normal,
non-cirrhotic changes, or cirrhosis. The primary outcome was 1-year
all-cause mortality, and the secondary outcome was differences in serial
post-HT liver evaluation.
Results
CT imaging in 32 Fontan patients evaluated for HT revealed 20
(63%) with evidence of liver disease, including 13 (41%)
with cirrhosis. Twenty underwent HT, including 5 non-cirrhotic and 7
cirrhosis patients. Characteristics at listing between normal or
non-cirrhotic (n = 13) and cirrhosis
(n = 7) groups were similar, except cirrhosis
patients were older (median 17.6 vs 9.6 years, p =
0.002) and further from Fontan (median 180 vs 50 months, p
< 0.05). Serial liver evaluation was similar, including aspartate
aminotransferase, alanine aminotransferase, bilirubin, albumin, and
tacrolimus dose at 1, 3, 6, 9, and 12 months. Overall patient survival was
80% at 1 year, with no difference between cirrhosis and
non-cirrhosis patients (86% vs 77%, p
= 0.681). Liver biopsies were performed in 7 patients before HT, and
all specimens showed architectural changes with bridging fibrosis.
Conclusions
Most patients evaluated for HT had abnormal liver findings by CT,
with cirrhosis in 41%. One-year mortality and serial liver
evaluation were similar between groups after HT. Liver cirrhosis identified
by CT imaging may not be an absolute contraindication to HT alone in this
population.
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