A new on-line method for predicting aortic root dilatation during two-dimensional echocardiography in pediatric patients with Marfan syndrome using the sinus of valsalva to annulus ratio.

The objective of this study was to determine if calculation of the sinus of Valsalva to annulus ratio (SOV/Ann) effectively screens for root dilatation (ARD) during two-dimensional (2D) echocardiography in young patients (1 month-15 years) with suspected or confirmed Marfan syndrome. The aortic root was imaged using 2D echocardiography (parasternal long-axis view) and the SOV/Ann ratio calculated/analyzed in 27 Marfan patients and 45 age-matched controls. All controls had a SOV/Ann ratio <1.45, and 82% of Marfan's with ARD had a SOV/Ann ratio 1.45. Five of the Marfan patients with ARD had a SOV/Ann ratio < 1.45 due to normalization of the SOV/Ann ratio because of pathologic annular enlargement. Determination of the SOV/Ann ratio allows rapid on-line screening for aortic root dilatation during 2D echocardiography. A SOV/Ann ratio 1.45 predicted ARD in young Marfan patients with a high degree of accuracy (sensitivity = 0.82, specificity = 1.00). Screening for ARD using the SOV/Ann ratio fails when annular dilatation has progressed to the degree that the SOV/Ann ratio normalizes (SOV/Ann < 1.45). In this subgroup of patients, determination of aortic root dilatation will have to be evaluated by standard methods.