TY - JOUR
T1 - Aortic stiffness and diameter predict progressive aortic dilatation in patients with Marfan syndrome
AU - Nollen, Gijs J.
AU - Groenink, Maarten
AU - Tijssen, Jan G. P.
AU - van der Wall, Ernst E.
AU - Mulder, Barbara J. M.
PY - 2004
Y1 - 2004
N2 - 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 distensibitity was an independent predictor of progressive thoracic descending aortic dilatation (OR=4.14, 95% Cl, 1.02-16.7). For progressive aortic root and abdominal aortic ditatation local initial diameter appeared to be the major predictor (OR= 1. 37, 95% Cl, 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. (C) 2004 The European Society of Cardiology. Published by Elsevier Ltd. All rights reserved
AB - 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 distensibitity was an independent predictor of progressive thoracic descending aortic dilatation (OR=4.14, 95% Cl, 1.02-16.7). For progressive aortic root and abdominal aortic ditatation local initial diameter appeared to be the major predictor (OR= 1. 37, 95% Cl, 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. (C) 2004 The European Society of Cardiology. Published by Elsevier Ltd. All rights reserved
U2 - https://doi.org/10.1016/j.ehj.2004.04.033
DO - https://doi.org/10.1016/j.ehj.2004.04.033
M3 - Article
C2 - 15231373
SN - 0195-668X
VL - 25
SP - 1146
EP - 1152
JO - European Heart journal
JF - European Heart journal
IS - 13
ER -