External Aortic Root Support to Prevent Aortic Dilatation in Patients With Marfan Syndrome

Background: Personalized external aortic root support (PEARS) was introduced in 2004 for prevention of aortic root dilatation in Marfan patients. The individual's aortic root is replicated by 3-dimensional printing. A polymer mesh sleeve is manufactured, which is implanted with the aim to support and stabilize the aortic wall.

Objectives: The aim of this study was to assess effectiveness of PEARS for prevention of aortic root dilatation in Marfan patients.

Methods: A total of 24 consecutive Marfan patients operated 2004 to 2012 were prospectively monitored with magnetic resonance imaging. Following a pre-defined protocol, baseline and follow-up aorta measurements were made in a blinded random sequence.

Results: The mean age of the patients was 33 ± 13.3 years (range: 16 to 58 years), and the mean aortic root diameter was 45 ± 2.8 mm (range: 41 to 52 mm). Follow-up was 6.3 ± 2.6 years. There was no increase in the aortic root and ascending aorta diameters, but there was a tendency toward reduction: annulus diameter 28.9 ± 2.3 mm to 28.5 ± 2.4 mm (change -0.39 mm, 95% confidence interval [CI]: -1.05 to 0.27 mm), sinus of Valsalva diameter 44.9 ± 2.9 mm to 44.5 ± 3.0 mm (change -0.37 mm, 95% CI: -1.23 to 0.51 mm), and ascending aorta diameter 32.4 ± 3.6 mm to 32.3 ± 3.7 mm (change -0.10 mm, 95% CI: -0.92 to 0.74 mm). In the same period, the descending aorta diameter increased from 22.9 ± 2.4 mm to 24.2 ± 3.0 mm (change 1.32 mm, 95% CI: 0.70 to 1.94 mm; p < 0.001) with a tendency toward increase in aortic arch diameter 24.1 ± 2.0 mm to 24.5 ± 2.8 mm (change 0.41 mm, 95% CI: -0.56 to 1.37 mm).

Conclusions: PEARS is effective in stabilizing the aortic root and preventing its dilatation. It is a viable alternative for prevention of aortic root dissection in Marfan patients.