Author + information
- Nancy McCabe,
- Jenny Mas-Moya,
- Alton B. Farris,
- Robert Elder,
- Brian Kogon,
- Michael McConnell and
- Wendy Book
Liver fibrosis is a common complication after Fontan palliation for single ventricle heart defects, but the clinical utility of liver biopsies is unclear. The purpose of this study was to determine if degree of liver fibrosis on biopsy correlates with clinical findings.
A chart review of adults with Fontan palliation and a liver biopsy at Emory's Adult Congenital Heart Center was performed (2001-2011). Core liver biopsies were examined using digitally scanned whole slide images of trichrome-stained sections. Portal and lobular fibrosis were quantitated using a positive pixel count algorithm tuned to detect fibrosis. Logistic regression and bivariate correlations were used to examine the relationship between fibrosis and ventricular dysfunction, IVC diameter, clinical features of portal hypertension (defined as ≥2 of the following: platelets <150K, splenomegaly, varices, ascites), and adverse outcomes (death, transplant, or hepatocellular carcinoma [HCC]).
Ten adults were identified (40% female, mean age 31±7 years). Mean time since Fontan was 18.6 years (range: 6-29), 7 had atriopulmonary connection of whom 4 underwent revision, 8 had a systemic left ventricle, 1 had ventricular dysfunction, and mean IVC diameter was 2.1 ± .23 cm. On biopsy, mean portal area was 1.4 × 105 ± 8.7 × 104 um2 and portal % fibrosis was 66% ± 16%. Mean lobular area was 5.9 × 105 ± 2.4 × 105 um2 and lobular % fibrosis was 20% ± 19%. Features of portal hypertension were present in 7, and 3 had an adverse outcome (death=1, transplant=1, HCC=1). No relationship was found between fibrosis and ventricular dysfunction (p=.26-.59), features of portal hypertension (p=.39-.98), or clinical outcomes (p=.14-.98). A strong positive correlation was found between mean portal area and time since Fontan (r= .82, p=.003) and a moderate trending correlation between portal % fibrosis and IVC diameter (r= .59, p=.07).
In adult patients following Fontan palliation, quantitative portal fibrosis by liver biopsy increases with time since surgery but does not correlate with clinical outcomes. Further evaluation in a larger population is needed to confirm these findings.
Poster Sessions, Expo North
Saturday, March 09, 2013, 10:00 a.m.-10:45 a.m.
Session Title: Congenital Cardiology Solutions: Fontan Physiology in the Adult – Liver, Pregnancy and Survival
Abstract Category: 12. Congenital Cardiology Solutions: Adult
Presentation Number: 1117-118
- 2013 American College of Cardiology Foundation