Open Access

ARTICLE

Serial cardiac MRIs in adult Fontan patients detect progressive hepatic enlargement and congestion

Matthew J. Lewis¹, Elizabeth Hecht², Jonathan Ginns¹, Joshua Benton², Martin Prince², Marlon S. Rosenbaum¹

1 Division of Cardiology, Department of Medicine, Schneeweiss Adult Congenital Heart Center, Columbia University Medical Center, New York, New York, USA
2 Division of Abdominal Imaging, Department of Radiology, Columbia University Medical Center, New York, New York, USA

* Corresponding Author: Matthew Lewis, MD MPH, Herbert Irving Pavilion, 161 Fort Washington Ave Suite 627, New York, NY 10032, USA. Email:

Congenital Heart Disease 2017, 12(2), 153-158. https://doi.org/10.1111/chd.12422

Abstract

Background: 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.
Methods and Results: 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).
Conclusions: 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.

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