Jennifer Romanowicz, Sungho Park, Jenifer Bunn, Roni M. Jacobsen, Brian Fonseca, Jenny E. Zablah, Erin K. Englund, Alex J. Barker, Jesse A. Davidson
Children’s Hospital Colorado and University of Colorado Anschutz Medical Campus.
United States
Journal of Cardiovascular Magnetic Resonance
J Cardiovasc Magn Reson 2025;
DOI: 10.1016/j.jocmr.2025.101907
Abstract
Background: Accumulation of progressive extracardiac disease is nearly universal for patients with single ventricle heart disease palliated to a Fontan circulation; however, etiologies are poorly understood. Limited flow reserve in the Fontan circulation may underlie extracardiac disease found in Fontan physiology through reduced oxygen and nutrient delivery to the tissues. This study aimed to determine regional flow volumes and oxygen delivery to key organ systems in children and adolescents with a Fontan circulation.
Methods: In 17 Fontan subjects and 14 biventricular controls, regional arterial flow volumes to the carotid, celiac, superior mesenteric, renal, and iliac arteries were quantified with MRI. Arterial oxygen content was calculated using subject hemoglobin level and pulse oximetry, and regional oxygen delivery was calculated using regional flow volume and oxygen content for the above listed arteries. Cardiac output was measured from ascending aorta flow, systemic blood flow from the caval veins, and aorto-pulmonary collateral flow was calculated as the difference between the two. Flows were compared between groups (t-test) and associations were analyzed between flows and with maximal exercise performance on clinical cardiopulmonary exercise testing (Pearson correlation).
Results: On average, renal and iliac arterial flows were lower in the Fontan group, compared to controls. Carotid, celiac, and superior mesenteric arterial flows were preserved in the Fontan group. Arterial oxygen content was equivalent between groups, and thus, regional oxygen delivery followed the same pattern as regional flows. Cardiac output was no different between groups, but systemic blood flow was lower in Fontans due to loss of flow to aorto-pulmonary collaterals. Systemic blood flow correlated with iliac flow such that those with the lowest systemic flow had the least amount of iliac flow. Celiac arterial flow correlated with percent-predicted peak oxygen consumption (VO2) on exercise testing.
Conclusions: Our results are consistent with a limited flow reserve in the Fontan circulation with sacrifice of iliac arterial flow as global systemic blood flow decreases. Importantly, these data were measured with subjects supine and at rest. Future work requires the addition of exercise to determine how flow to specific organs is affected by increasing metabolic demand from the extremities.
Category
Abnormal Systemic to Pulmonary Arterial Collaterals or Connections
Diagnostic Testing for Pulmonary Vascular Disease. Non-invasive Testing
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Filed (PHiled). Greater than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes