ESPE Abstracts (2014) 82 P-D-2-3-614

Turner Syndrome

Aortic Dilation in a Large Cohort of Paediatric and Young Adult Patients with Turner Syndrome

Nadia Geniuk, Elisa Vaiani, Ana Rizzi, Gabriela Guercio, Monica Warman, Diego Michelli, Marco Rivarola & Alicia Belgorosky

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Hospital de Pediatria Garrahan, Buenos Aires, Argentina


Background: Aortic dilation (AD) occurs in Turner syndrome (TS) increasing the risk of aortic dissection at all ages. There are no current guidelines on what specific aortic diameter measurement should provoke concern in pediatric TS. Because of their small stature, an aortic size index (ASI) has been proposed to normalize the ascending aorta (AA) to body size in young adults’ with TS. However, a more reliable index has been also proposed: the ratio of vascular diameter to thoracic vertebra (TV) diameter, which is constant through age in normal children (AVI).

Objective and hypotheses: To evaluate AD using these two different methods.

Method: TS patients (n=87) were studied. Ages ranged from 3.2 to 25.7 years. According to chronological age they were divided in three groups (Gr). Gr1 (n: 11): 1–7.9 years, Gr2 (n: 42): 8–15.9 years, and Gr3 (n: 34): ≥16 years. Simple chest computed tomography were done in all patients. AA and TV diameters were measured. AD was defined as ASI >2 cm/m2 and AVI >2 SDS according to published reference values.

Results: AD was significantly greater (P<0.01) in all groups. Gr1=81 and 18%, Gr2=47 and 2.3%, and Gr3=23 and 2.9% of patients, using ASI and AVI respectively. All patients with severe AD (ASI >2.5 cm/m2) presented AVI >2 SDS.

Conclusion: We found a high prevalence of AD in our population of TS. ASI overestimates the risk of AD in all Grs, particularly in younger patients. AVI seems to be a more useful tool to asses AD in the pediatric population. Additional follow up is necessary to evaluate the long time consequences of these findings.

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