Abstract

Background Aortic stiffness measures have been proposed as novel indicators of disease severity in patients with connective tissue disorders (CTD). However, these measures have not been studied extensively in children. Their relationship to adverse aortic outcomes in this population is also unknown. We studied CMR parameters of aortic stiffness in children and young adults with CTD and assessed their relationship with the rates of aortic dilation and surgical root replacement.

Highlights

  • Aortic stiffness measures have been proposed as novel indicators of disease severity in patients with connective tissue disorders (CTD)

  • We studied CMR parameters of aortic stiffness in children and young adults with CTD and assessed their relationship with the rates of aortic dilation and surgical root replacement

  • Higher aortic stiffness is associated with more rapid aortic root dilation and higher likelihood of aortic root replacement

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Summary

Background

Aortic stiffness measures have been proposed as novel indicators of disease severity in patients with connective tissue disorders (CTD). These measures have not been studied extensively in children. Their relationship to adverse aortic outcomes in this population is unknown. We studied CMR parameters of aortic stiffness in children and young adults with CTD and assessed their relationship with the rates of aortic dilation and surgical root replacement

Methods
Results
Conclusions
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