Characterization of the Rate of Aortic Dilation in Young Patients with Thoracic Aortic Aneurysm
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ORIGINAL ARTICLE
Characterization of the Rate of Aortic Dilation in Young Patients with Thoracic Aortic Aneurysm Adam P. Wheeler1 · Ziyi Yang2 · Timothy M. Cordes1 · Larry W. Markham1 · Benjamin J. Landis1 Received: 5 June 2020 / Accepted: 16 September 2020 © Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract Longitudinal changes in aortic diameters of young patients with thoracic aortic aneurysm (TAA) have not been completely described, particularly over long periods of follow-up. This retrospective study sought to characterize the rates of proximal aortic dilation in young patients, identify risk factors for TAA progression, and evaluate the predictive utility of early echocardiographic follow-up. Inclusion criteria were: (1) TAA or TAA-predisposing genetic diagnosis, (2) age 10 years at first echocardiogram were associated with increased rate of ascending aorta dilation. At the ascending aorta, over 25% of patients had categorical increase in TAA severity between first and last echocardiograms, and such patients demonstrated higher rate of dilation within their first 2 years of follow-up. These longitudinal findings highlight progressive ascending aorta dilation in young patients, which may worsen around adolescence. This may help determine timing of follow-up and target ages for clinical trials. Keywords Thoracic aortic aneurysm · Bicuspid aortic valve · Marfan syndrome · Aortopathy
Introduction Thoracic aortic aneurysm (TAA) is an aortopathy which predisposes to sudden, life-threatening aortic dissection or rupture [1]. Patients who progress to severe aortic dilation often require prophylactic aortic repair surgery, which frequently occurs in adulthood. Genetic conditions associated with TAA include Marfan syndrome (MFS) and Loeys–Dietz syndrome, and congenital bicuspid aortic valve Electronic supplementary material The online version of this article (https://doi.org/10.1007/s00246-020-02464-2) contains supplementary material, which is available to authorized users. * Benjamin J. Landis [email protected] 1
Division of Pediatric Cardiology, Department of Pediatrics, Indiana University School of Medicine, Indianapolis, IN, USA
Department of Biostatistics, Indiana University School of Medicine, Indianapolis, IN, USA
2
(BAV) predisposes to TAA. Identifying TAA or establishing a genetic diagnosis at a young age, and early in disease course, facilitates monitoring and clinical interventions aiming to halt progression. Ultimately, this may prevent an aortic dissection or the need for prophylactic aortic surgery [2]. Optimizing clinical decision-making for young patients depends upon a thorough understanding of the longitudinal characteristics of aortic dilation over time. TAA groups including BAV, MFS, and idiopathic TAA have been individually described longitudinally; however, prior studies were limited by having variable follow-up intervals and including patients with short periods of follow-up [3–5]. Previous cross-sectional studies of young patients with BAV have observe
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