Abstract 13627: Hepatic Venous Return to Lungs in Fontan is Dependent on Age-Dependent Caval Flow Ratio

Introduction:The Fontan procedure is performed between 2-4 years of age and is a palliative technique for patients born with single ventricle defects. The Fontan physiology is the result of a total cavopulmonary connection between superior and inferior vena cava (SVC and IVC) and the hepatic veins to the pulmonary arteries (PA). Maldistribution of hepatic blood flow to the lungs can result in the formation of pulmonary arteriovenous malformations (PAVM) and resulting cyanosis. Hepatic blood flow distribution (HFD) to right and left lungs is highly dependent on the local flow dynamics. We sought to investigate the impact of SVC-to-IVC flow ratio and age on HFD.Methods:SVC and IVC flow data measured by cardiac MRI (CMR) from 81 Fontan patients were collected to establish the relationship between SVC: IVC flow ratio vs. age. 3D Patient-specific computational fluid dynamics (CFD) modeling was performed for 5 patients who underwent Fontan procedure with an extracardiac conduit to quantify the HFD to the right and left PAs. We evaluated the dependency of HFD on SVC: IVC flow ratio by quantifying HFD to different SVC: IVC flow ratios (2, 1.0, and 0.5) for a given Fontan anatomy.Results:SVC: IVC flow ratio inverted at ~10 years of age (n=81, Figure A) indicating a clear shift to lower body predominance in overall caval flow. CFD analysis (n=5) showed that the shift in SVC-IVC flow had a significant effect on the HFD (Fig. B-F). Change in HFD was more substantial as SVC: IVC flow ratio shifted from 2 to 1 [~5-10 years] with an average % change of 21±6 compared to the HFD change that occurred for a shift of 1 to 0.5 [9±11 for ~10-20 years] (Fig. B-F).Conclusion:Well-balanced distribution of HFD is essential to prevent PAVMs and to ensure long-term efficacy of a Fontan. This study highlights how SVC: IVC flow ratios change over time and can impact HFD to PAs. Interventions to address HFD maldistribution should not be considered in isolation and should incorporate predictions of SVC: IVC changes over time.