• Michal Schäfer, Adel Younoszai, Uyen Truong, Lorna P Browne, Max B Mitchell, James Jaggers, David N Campbell, Kendall S Hunter, D Dunbar Ivy, and Michael V Di Maria.
• Division of Cardiology, Heart Institute, Children's Hospital Colorado, University of Colorado Denver Anschutz Medical Campus, Denver, Colo; Department of Bioengineering, College of Engineering and Applied Sciences, University of Colorado Denver Anschutz Medical Campus, Denver, Colo. Electronic address: michal.schafer@ucdenver.edu.
• J. Thorac. Cardiovasc. Surg. 2019 Feb 1; 157 (2): 699-707.

ObjectiveElastic properties of the thoracic aorta are responsible for buffering systemic afterload, and may be particularly important in patients with Fontan circulation, in whom heart failure is a major source of attrition. The purpose of this study was to characterize regional stiffness in the ascending and descending aorta in patients with hypoplastic left heart syndrome and single left ventricle morphology after Fontan operation by cardiac magnetic resonance imaging, and to assess whether changes in aortic stiffness are associated with the ventricular function.MethodsPhase-contrast-derived pulse-wave velocity (PWV) and relative-area change (RAC) were measured in the ascending and descending aorta of patients with hypoplastic left heart syndrome (n = 9), patients with single left ventricle circulation (n = 18), and normal controls (n = 8) by magnetic resonance imaging. Stiffness metrics were then correlated with the ventricular volumetric and functional indices.ResultsPatients with hypoplastic left heart syndrome had elevated ascending aortic PWV along with reduced RAC when compared with controls (both P values < .001). Patients with a single left ventricle presented no change in PWV but had reduced RAC in comparison to controls (P < .01). There were no differences in PWV and RAC between all considered groups in the descending aorta. PWV and RAC measured in the ascending aorta correlated with end-systolic and end-diastolic volume indices, ventricular ejection fraction, and ventricular-vascular coupling ratio.ConclusionsAortic stiffness is most elevated in patients with hypoplastic left heart syndrome, yet patients with single left ventricle morphology show signs of abnormal stiffness as well in the form of reduced aortic strain. Stiffness indices measured in the ascending aorta were associated with overall ventricular function and measures of aortoventricular coupling in both patient populations.Copyright © 2018 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.

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