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

Izgi, C; Newsome, S; Alpendurada, F; Nyktari, E; Boutsikou, M; Pepper, J; Treasure, T; (2018) External Aortic Root Support to Prevent Aortic Dilatation in Patients With Marfan Syndrome. Journal of the American College of Cardiology , 72 (10) pp. 1095-1105. 10.1016/j.jacc.2018.06.053. Green open access

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Abstract

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.

Type: Article
Title: External Aortic Root Support to Prevent Aortic Dilatation in Patients With Marfan Syndrome
Open access status: An open access version is available from UCL Discovery
DOI: 10.1016/j.jacc.2018.06.053
Publisher version: https://doi.org/10.1016/j.jacc.2018.06.053
Language: English
Additional information: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions.
Keywords: aneurysm; aorta; dissection; magnetic resonance; Marfan syndrome
UCL classification: UCL
UCL > Provost and Vice Provost Offices > UCL BEAMS
UCL > Provost and Vice Provost Offices > UCL BEAMS > Faculty of Maths and Physical Sciences
UCL > Provost and Vice Provost Offices > UCL BEAMS > Faculty of Maths and Physical Sciences > Dept of Mathematics
UCL > Provost and Vice Provost Offices > UCL BEAMS > Faculty of Maths and Physical Sciences > Dept of Mathematics > Clinical Operational Research Unit
URI: https://discovery-pp.ucl.ac.uk/id/eprint/10125640
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