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

Abstract

BackgroundPersonalized 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. ObjectivesThe aim of this study was to assess effectiveness of PEARS for prevention of aortic root dilatation in Marfan patients. MethodsA 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. ResultsThe 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). ConclusionsPEARS 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.