Response to Letters Regarding Article “Segmental Aortic Stiffness in Children and Young Adults With Connective Tissue Disorders: Relationships With Age, Aortic Size, Rate of Dilation, and Surgical Root Replacement”

Abstract

We thank Murakami et al for their interest in our article.1 They propose that the aortic dilatation observed in patients with connective tissue disorders is a compensatory response to increased aortic stiffness to preserve coronary blood flow. Although our study was not designed to test this interesting hypothesis, we agree that future investigations designed specifically to address this theory are warranted. Weismann et al question the use of aortic strain in our study and suggest that its relationship with adverse aortic outcomes may be driven by aortic size alone. However, their comments fail to take into account that, in addition to aortic size, several other factors …