The progression of hepatic disease in adult Fontan patients is not well understood. They reviewed the experience with serial cardiac MRIs (CMR) in adult Fontan patients to determine if hepatic anatomic markers of prolonged Fontan exposure were present and if clinical predictors of progressive hepatic congestion could be identified.A retrospective cohort study of all adult Fontan patients who had undergone at least two CMRs was performed. Hepatic dimensions, inferior vena cava (IVC) size, right hepatic vein (RHV) size and spleen diameter were determined from images acquired at the time of clinically guided CMR. Two radiologists with expertise in hepatic imaging graded congestion and liver size independently using post-gadolinium contrast sequences. Twenty-seven patients met inclusion criteria. Over a mean time of 5.1 years between CMRs, there was a significant increase in mean lateral-medial hepatic dimension (P = .005), mean RHV diameter (P = .004), and mean splenic diameter (P = .001). Serial post-gadolinium imaging was available in 25/27 (93%) patients of which 15/27 (55%) showed evidence of progressive hepatic congestion across serial studies. Progressive hepatic congestion was associated with single ventricle ejection fraction (SVEF) less than 50% (P = .008), and larger indexed end-diastolic (EDVI) and end-systolic volume (ESVI). RHV diameter was the only anatomic variable significantly correlated with time from Fontan completion (P = .004).Serial CMRs detected progressive liver and hepatic vein enlargement in our cohort of adult Fontan patients over a mean time of 5.2 years. Progressive hepatic congestion occurs in a significant number of adult Fontan patients and may be associated with ventricular enlargement and decreased ventricular function by CMR.
