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Background: Aortic dilation is seen in pediatric/young adult patients with bicuspid aortic valve (BAV), and hemodynamic markers to predict aortic dilation are necessary for monitoring. Although promising hemodynamic metrics, such as abnormal wall shear stress (WSS) magnitude, have been proposed for adult BAV patients using four-dimensional (4D) flow cardiovascular magnetic resonance, those for pediatric BAV patients have less frequently been reported, partly due to scarcity of data to define normal WSS range. To circumvent this challenge, this study aims to investigate if a recently proposed 4D flow-based hemodynamic measurement, abnormal flow directionality, is associated with aortic dilation in pediatric/young adult BAV patients., Methods: 4D flow scans for BAV patients (<20 years old) and age-matched controls were retrospectively enrolled. Static segmentation for the aorta and pulmonary arteries was obtained to quantify peak systolic hemodynamics and diameters in the proximal aorta. In addition to peak velocity, WSS, vorticity, helicity, and viscous energy loss, direction of aortic velocity and WSS in BAV patients were compared with that of control atlas using registration technique; angle differences of >60 deg and >120 deg were defined as moderately and severely abnormal, respectively. The association between the obtained metrics and normalized diameters (Z-scores) was evaluated at the sinotubular junction, mid-ascending aorta, and distal ascending aorta., Results: Fifty-three BAV patients, including 18 with history of repaired aortic coarctation, and 17 controls were enrolled. Correlation between moderately abnormal velocity/WSS direction and aortic Z-scores was moderate to strong at the sinotubular junction and mid-ascending aorta (R = 0.62-0.81; p < 0.001) while conventional measurements exhibited weaker correlation (|R| = 0.003-0.47, p = 0.009-0.99) in all subdomains. Multivariable regression analysis found moderately abnormal velocity direction and existence of aortic regurgitation (only for isolated BAV group) were independently associated with mid-ascending aortic Z-scores., Conclusion: Abnormal velocity and WSS directionality in the proximal aorta were strongly associated with aortic Z-scores in pediatric/young adult BAV patients., Competing Interests: Declaration of competing interests The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

Purpose of Review: This review provides the summary of the appropriate use of these modalities when caring for patients with Kawasaki disease at diagnosis and for long-term management., Recent Findings: Kawasaki disease is an inflammatory syndrome of unknown etiology that can result in coronary artery dilations or aneurysms if left untreated in 25% of the patients and 3-5% in treated patients. In addition to coronary artery aneurysms, patients can have ventricular dysfunction, valvular regurgitation, aortic root dilation, and pericardial effusion due to inflammation of the myocardium. Noninvasive imaging modalities are important to these assessments. Echocardiography is the first-line noninvasive evaluation of coronaries and function. Cardiac magnetic resonance imaging is useful for functional assessment in long-term follow-up. Distal coronaries, thromboses, and stenoses are best evaluated by cardiac computed tomography. Future research should demonstrate the effectiveness of advanced functional imaging in patients with Kawasaki disease and decreased radiation from cardiac computed tomography., (© 2022. This is a U.S. Government work and not under copyright protection in the US; foreign copyright protection may apply.)