Copyright © 2004 by the European Society of Cardiology.
Clinical research
Aortic stiffness and diameter predict progressive aortic dilatation in patients with Marfan syndrome
a Department of Cardiology, Academic Medical Center, Meibergdreef 9, Amsterdam 1105 AZ, The Netherlands
b Department of Cardiology, Leiden University Medical Center, Leiden, The Netherlands
* Corresponding author. Tel.: +31-20-5-662-193; fax: +31-205-666-809
E-mail address: b.j.mulder{at}amc.uva.nl
Received 2 January 2004; revised 20 April 2004; accepted 29 April 2004
Abstract
Aim Patients with Marfan syndrome may develop dissection due to progressive dilatation in the entire aorta, which is not always predictable by mere anatomic assessment of the aortic diameter, especially of the descending aorta. The aim of this study was to identify the predictive value of aortic stiffness on the occurrence of dissection and progressive aortic dilatation.
Methods and results In 78 non-operated patients with Marfan syndrome (mean age 31±8 years, mean aortic root diameter 43±6 mm, range 31–55 mm) aortic stiffness and diameters were assessed by magnetic resonance imaging (MRI) at multiple levels. After a median follow-up of 71 months (25–75%: 68–72 months) a second MRI was performed and the incidence of aortic dissection and progressive aortic dilatation, defined as mean aortic diameter increase 
1 mm/year was determined.
During follow-up, 4 (5%) of 78 patients developed an aortic dissection (1 type A, 2 type B, and 1 infra-renal dissection). Twenty (26%) of the 78 patients had progressive aortic root dilatation. There were 5 (6%) patients with progressive descending thoracic aortic dilatation and 6 (7%) with progressive abdominal aortic dilatation. Multivariate logistic regression analysis revealed that local distensibility was an independent predictor of progressive thoracic descending aortic dilatation (OR=4.14, 95% CI, 1.02–16.7). For progressive aortic root and abdominal aortic dilatation local initial diameter appeared to be the major predictor (OR=1.37, 95% CI, 1.16–1.62; OR=1.36, 95% CI, 1.09–1.69, respectively).
Conclusion In patients with Marfan syndrome both aortic diameter and aortic distensibility are independent predictors of progressive aortic dilatation. For optimal risk assessment and monitoring of patients with Marfan syndrome, both aortic stiffness and diameter should be assessed at least annually.
Key Words: Aorta • Elasticity • Magnetic resonance imaging • Aneurysm
CiteULike 
Connotea 
Del.icio.us What's this?
This article has been cited by other articles:
|
|
 |
|
  S. Nistri, J. Grande-Allen, M. Noale, C. Basso, P. Siviero, S. Maggi, G. Crepaldi, and G. Thiene Aortic elasticity and size in bicuspid aortic valve syndrome Eur. Heart J., February 2, 2008; 29(4): 472 - 479. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 H Senzaki, Y Iwamoto, H Ishido, T Matsunaga, M Taketazu, T Kobayashi, H Asano, T Katogi, and S Kyo Arterial haemodynamics in patients after repair of tetralogy of Fallot: influence on left ventricular after load and aortic dilatation Heart, January 1, 2008; 94(1): 70 - 74. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 H. B. Grotenhuis, J. Ottenkamp, J. J.M. Westenberg, J. J. Bax, L. J.M. Kroft, and A. de Roos Reduced aortic elasticity and dilatation are associated with aortic regurgitation and left ventricular hypertrophy in nonstenotic bicuspid aortic valve patients. J. Am. Coll. Cardiol., April 17, 2007; 49(15): 1660 - 1665. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 A. Graham Stuart and A. Williams Marfan's syndrome and the heart Arch. Dis. Child., April 1, 2007; 92(4): 351 - 356. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
Y F Cheung, X Ou, and S J Wong Central and peripheral arterial stiffness in patients after surgical repair of tetralogy of Fallot: implications for aortic root dilatation Heart, December 1, 2006; 92(12): 1827 - 1830. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 D. Baumgartner, C. Baumgartner, E. Schermer, G. Engl, U. Schweigmann, G. Matyas, B. Steinmann, and J. I. Stein Different patterns of aortic wall elasticity in patients with Marfan syndrome: A noninvasive follow-up study J. Thorac. Cardiovasc. Surg., October 1, 2006; 132(4): 811 - 819. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
P M Engelfriet, E Boersma, J G P Tijssen, B J Bouma, and B J M Mulder Beyond the root: dilatation of the distal aorta in Marfan's syndrome Heart, September 1, 2006; 92(9): 1238 - 1243. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J De Backer, G J Nollen, D Devos, G Pals, P Coucke, K Verstraete, E E van der Wall, A De Paepe, and B J M Mulder Variability of aortic stiffness is not associated with the fibrillin 1 genotype in patients with Marfan's syndrome. Heart, July 1, 2006; 92(7): 977 - 978. [Full Text] [PDF]
|
|
|
|
|
|
B. J.M. Mulder and L. J. Meijboom Comment on pregnancy and aortic root growth in the Marfan syndrome: reply Eur. Heart J., November 1, 2005; 26(21): 2346 - 2347. [Full Text] [PDF]
|
|
|
|
 |
|
 P. K. Van Kien, F. Mathieu, L. Zhu, A. Lalande, C. Betard, M. Lathrop, F. Brunotte, J.-E. Wolf, and X. Jeunemaitre Mapping of Familial Thoracic Aortic Aneurysm/Dissection With Patent Ductus Arteriosus to 16p12.2-p13.13 Circulation, July 12, 2005; 112(2): 200 - 206. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
E. Kasikcioglu Aortic elastic properties in athletes with Marfanoid habitus: the need for early and accurate diagnostic methods Eur. Heart J., January 1, 2005; 26(1): 100 - 100. [Full Text] [PDF]
|
|