Patients with aortic root dilatation due to Marfan syndrome (MS), aortic ectasia (AE) and bicuspid aortic valve (BV) are at potential risk for progressive dilatation, dissection or rupture.

We performed a prospective study in children to identify aortic dilatation and establish a strategy for surgical intervention. Data were obtained via the unit database (Cardiobase) for the period June 1991- March 1997. Serial echocardiograms with full doppler and cross-sectional imaging were performed (Acuson 128 XP/10C). The aortic root was measured at 4 levels (aortic cusps, sinus of valsalva, supra-aortic ridge, ascending aorta) and plotted against body surface area. (BSA). Measurements were repeated at 6-12 month intervals. Children with aortic root measurements >97 centile received B blockers. Surgical repair was performed if the aortic root was >5cm at presentation or if the aortic root continued to dilate despite B blocker.

One hundred and thirty five children (<18yrs) were referred for assessment. Forty five fitted the diagnostic criteria for MS or AE and an additional 12 had probable MS. A further 40 children were diagnosed with BV. Overall age ranged from 1 month to 18 years (median 10 years, 91 (52%) male). In the probable or definite MS and AE group, 46 (80%) had aortic root dilatation >97th centile for BSA and 7 (15%) had aortic regurgitation. In the BV group, 11 (27%) had a dilated aortic root: 4 (36%) of these had valve stenosis and 5 (45%) had regurgitation. Over this period 5 patients underwent surgery which included: aortic root remodelling (1), aortic root replacement (ARR) with aortic homograft (1), ARR with pulmonary autograft (1), ARR with a composite graft (2). In addition 2 patients required replacement of the aortic arch. Their age ranged between 6 and 16 (mean 14) years. At a mean follow up of 20 (4-46) months, they are asymptomatic. None of the children followed up has dissected or died.

Aortic root dilatation is common in children with MS and BV and tends to progress. Detailed echocardiography follow-up relating aortic root size to somatic growth allows optimal timing of surgery.