Prediction of Fontan-Associated Liver Disease Using a Novel Cine Magnetic Resonance Imaging “Vortex Flow Map” in the Right Atrium

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Background:Long-term hepatic dysfunction is an increasingly recognized complication of the Fontan operation for univentricular hearts. The purpose of this study was to determine whether Fontan-associated liver disease (FALD) could be predicted by flow dynamics in the right atrium (RA) of Fontan circulation.

Methods and Results:Cardiac MRI and the serum levels of total bilirubin (TBil) and hyaluronic acid (HA) were analyzed in 36 patients who underwent an atriopulmonary connection type of Fontan operation. The mean follow-up period was 53 months. Three views (axial, coronal, and sagittal) of the cine images were scanned for the maximum cross-section of the RA obtained with1.5-Tesla scanner. We developed a “vortex flow map” to demonstrate the ratio of the circumferential voxel movement in each phase to the total movement throughout a cardiac cycle towards the center of the RA. The maximum ratio was used as the magnitude of vortex flow (MVF%) in the 3 views of the RA cine imaging. Patients with coronal MVF ≥13.6% had significantly lower free rates of TBil ≥1.8 mg/dL than those with coronal MVF <13.6% (log-rank value=4.50; P<0.05; hazard ratio=4.54). Patients with sagittal MVF ≥14.0% had significantly lower free rates of HA ≥50 ng/mL than those with coronal MVF <14.0% (log-rank value=4.40; P<0.05; hazard ratio=4.12).

Conclusions:A reduced vortex flow in the RA during the late phase of the Fontan operation was associated with the development of FALD. MVF can be used as an imaging biomarker to predict FALD.