Log in to MyACC
PEARS to Prevent Aortic Root Dilatation in Marfan
Study Questions:
What is the effectiveness of personalized external aortic root support (PEARS) for prevention of aortic root dilatation in Marfan patients?
Methods:
The investigators prospectively monitored a total of 24 consecutive Marfan patients, operated on from 2004-2012, with magnetic resonance imaging. Following a pre-defined protocol, baseline and follow-up aorta measurements were made in a blinded random sequence. Aortic measurements before the operation and at the latest follow-up were compared by paired t-test. The presence or absence of aortic regurgitation before and after the surgery as a binary variable was compared by the McNemar test.
Results:
The mean age of the patients was 33 ± 13.3 years (range: 16-58 years), and the mean aortic root diameter was 45 ± 2.8 mm (range: 41-52 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:
The authors concluded that PEARS is effective in stabilizing the aortic root and preventing its dilatation.
Perspective:
This study reports that PEARS keeps the aortic root size stable and prevents dilatation in Marfan patients. However, the unsupported segments, the aortic arch and the descending aorta, remain prone to dilatation over time, and hence, close follow-up is mandatory for the continued risk of type B dissection, as is the case after Bentall and valve-sparing root replacement (VSRR) operations. It appears that three-dimensional printing may allow a personalized approach to external aortic root support for prevention of aortic root dilatation and aortic dissection in patients with Marfan syndrome. Longer-term follow-up studies with an appropriate control group are necessary to establish the effectiveness of personalized external aortic root support to prevent aortic dissection and dilatation.
Clinical Topics: Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Invasive Cardiovascular Angiography and Intervention, Noninvasive Imaging, Prevention, Valvular Heart Disease, Vascular Medicine, Aortic Surgery, Cardiac Surgery and Arrhythmias, Cardiac Surgery and CHD and Pediatrics, Cardiac Surgery and VHD, Congenital Heart Disease, CHD and Pediatrics and Arrhythmias, CHD and Pediatrics and Imaging, CHD and Pediatrics and Interventions, Interventions and Imaging, Interventions and Structural Heart Disease, Interventions and Vascular Medicine, Magnetic Resonance Imaging
Keywords: Aneurysm, Dissecting, Aorta, Thoracic, Aortic Aneurysm, Aortic Valve Insufficiency, Cardiac Surgical Procedures, Dilatation, Dilatation, Pathologic, Heart Defects, Congenital, Magnetic Resonance Imaging, Marfan Syndrome, Primary Prevention, Sinus of Valsalva, Vascular Diseases
< Back to Listings
