Abstract 17982: Serial Cardiac MRIs Show Progressive Hepatic Disease in the Adult Fontan Patient
Background: The progression of hepatic disease in adult patients with a Fontan is not well understood. MRI allows for the delineation of liver dimensions and the evaluation of hepatic congestion. By using cardiovascular magnetic resonance imaging (CMR), we sought to assess the hypothesis that adult Fontan patients have progressive hepatic enlargement and congestion.
Methods: We retrospectively reviewed all adult patients with a Fontan at our Center who underwent serial CMRs. Liver function tests (LFTs) and pre-specified clinical data were collected for each patient. Hepatic dimensions, right hepatic vein (RHV) size and spleen diameter were evaluated using coronal CMR images. Hepatic congestion was evaluated by a radiologist with expertise in hepatic imaging using post-gadolinium images.
Results: Twenty-two patients (54% male) met inclusion criteria. Mean age at time of first CMR was 26 years and the mean time between CMRs was 5.1 years. Nine patients (41%) had a classic RA-PA Fontan and 13 (59%) patients had a lateral tunnel Fontan.
There was a significant increase in the mean lateral to medial linear hepatic dimension from the first CMR to the second CMR (190 cm vs. 204 cm, p=0.0001). There was a significant increase in the mean RHV diameter (10.9 cm vs. 13.4 cm, p=0.002) and spleen diameter (9.35 cm vs. 11.1 cm, p=0.003). There was no significant difference in the rate or absolute value of linear hepatic dimension increase, RHV diameter increase, or spleen diameter increase between types of Fontan, gender, or in patients with abnormal LFTs.
Serial post gadolinium images were available in 19 (86%) patients. Four of the 19 patients (21%) showed progressive congestion across serial studies. There was a significant association between patients with abnormal bilirubin and those that developed increased hepatic congestion (p=0.001).
Conclusion: Adult Fontan patients displayed progressive enlargement of the liver over a median follow-up of 5 years. While patients with elevated bilirubin were more likely to have progressive hepatic congestion on CMR, no specific variable was associated with degree or rate of enlargement. This suggests that clinically silent, progressive liver abnormalities may be common in this population and warrant further investigation.
- © 2013 by American Heart Association, Inc.