Abstract 16691: Reduced Aortic Elasticity in Children With Connective Tissue Disorders Regardless of Aortic Dilatation
Introduction: Previous studies have demonstrated that reduced aortic elasticity in connective tissue disorders (CTDs) and the aortic stiffness could be an independent predictor of the progressive aortic dilatation. However, the evidence for aortic elasticity is less clear in children with CTDs, especially those who with non-dilated aortic root (AoR). The purpose of this study is to assess the aortic elasticity in children with CTDs who have dilated and non-dilated AoR.
Methods: A total of 31 children (age: 12±7 years) with CTDs including Marfan syndrome, Loeys-Dietz syndrome and aneurysms-osteoarthritis syndrome and 15 healthy age- and sex-matched children were studied. Aortic elasticity indexes including aortic distensibility, aortic strain were derived from echocardiography at the AoR and ascending aorta (AA). CTDs children with dilated AoR (z score ≧2.5, n=17), non-dilated AoR (z score <2.5, n=14) and controls were compared.
Results: Aortic distensibility and aortic strain at the AoR were decreased in both dilated and non-dilated groups compared to controls(aortic distensibility: 0.5±0.3х10-3 vs. 1.5±0.9х10-3 vs. 2.4±1.1х10-3, p<0.05, respectively. aortic strain: 2.5±1.8vs. 5.1±2.6vs. 10.4±3.8, p<0.001, respectively). Aortic strain at AA was also decreased in both CTDs groups compared to controls(6.0±4.6vs. 11.6±7.3vs. 21.5±12.4%, p<0.05, respectively). Z scores of AoR was significantly correlated with aortic distensibility(r=-0.63, p< 0.001), and aortic strain(r=-0.54, p=0.002) in CTDs group. Multivariate analysis revealed that aortic distensibility, aortic strain and the type of CTDs were independently relevant to AoR dilatation in CTDs group (p<0.05, respectively).
Conclusions: Aortic elasticity is impaired in children with CTDs. Moreover, CTDs children with non-dilated AoR showed impairment of aortic elastic properties. Measuring aortic elasticity may help to identify children with CTDs at greater risk for aortic complications.
Author Disclosures: Y. Akazawa: None. N. Motoki: None. A. Hachiya: None. D. Akagawa: None.
- © 2016 by American Heart Association, Inc.