Cardiac anomalies, particularly left sided lesions, are common in patients with Turner syndrome (TS). Accordingly, all TS patients should undergo initial echocardiography. Also, reports of catastrophic aortic dissection and rupture in patients thought to have no cardiac lesion have raised concerns about undetected aortic dilatation as TS patients mature. The primary goal of this study was to analyze longitudinal changes in diastolic aortic diameter (AoD) in a pediatric TS population. Echocardiography (two dimensional sector scan and cardiac Doppler) was performed in 67 TS patients; to date 24 have undergone a second echo at least 2 years after the initial study. A mean interval of 6.0 years (range 2.1-9.8 yr) elapsed between studies, with a mean age of 15.0 years at the second echo. 20/24 patients received hGH and 14/24 took estrogen in the interval. AoD Z scores were calculated for body surface area (BSA) using published normative data. As shown in the table, mean AoD Z score was elevated relative to normal controls at both initial measure (0.80 SD) and follow-up (0.96 SD) but did not change significantly between studies. Individual changes in AoD Z score ranged from -1.3 to +3.1, and the largest increase in AoD was 10 mm. Four patients had both baseline and follow-up AoD Z scores >2 SD. In two other patients, AoD increased from <1 SD initially to >3 SD at repeat. Thus, 25% of the study population (6/24) had an AoD >2 SD at the second exam, and 2 subjects(8%) had a worrisome interval increase in AoD. The results suggest a need for routine longitudinal cardiac surveillance in TS, given the prevalence of AoD>2 SD compared to size-matched normal females.

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