Screening for aortic root dilation in marfan syndrome using the ratio of the aortic root to descending aortic diameters in children.

BACKGROUND

The aim of this study was to develop a simpler echocardiographic screening tool for aortic root dilation in pediatric patients with Marfan syndrome. Aortic root dilation represents the most common cause of morbidity and mortality in patients with Marfan syndrome. It is traditionally defined by nomograms, relating aortic root dimensions to height or body surface area. In this study, the descending aorta (DAo) was used as an internal reference, thereby eliminating the need for nomograms when screening for aortic root dilation.

METHODS

The diameters of the aortic root at the sinus of Valsalva and the DAo were measured in 35 patients with Marfan syndrome and 52 normal controls in the parasternal long-axis echocardiographic view. The root/DAo ratio was calculated.

RESULTS

A root/DAo ratio > or = 2 provided optimal sensitivity for root dilation (100%), whereas a root/DAo ratio > or = 2.3 provided optimal specificity (100%).

CONCLUSION

The root/DAo ratio provides a simple, rapid, and valuable screening test for aortic root dilation, independent of nomograms and body size.