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Relation of aortic root dilatation and age in Marfan's syndrome

Aburawi, Elhadi LU and O'Sullivan, John (2007) In European Heart Journal 28(3). p.376-379
Abstract
Aims The main aim of this study was to describe the age at which pathological aortic root dilation occurs in patients with Marfan's syndrome (MFS). Methods and results A total of 160 patients with MFS attending a regional cardiac centre were reviewed retrospectively. Dilation of the ascending aorta was diagnosed by comparing the maximum aortic sinus measurement with control data from the literature. We employed a Kaplan-Meier survival curve to estimate the age at which dilatation occurs. The mean age of the total group at presentation was 15.5 years (range 1.5-40 years). Skeletal abnormalities were present in 95%. Eye involvement was found in 18%. In the 115/160 patients with an abnormal aortic root, 78/115 (68%) developed aortic root... (More)
Aims The main aim of this study was to describe the age at which pathological aortic root dilation occurs in patients with Marfan's syndrome (MFS). Methods and results A total of 160 patients with MFS attending a regional cardiac centre were reviewed retrospectively. Dilation of the ascending aorta was diagnosed by comparing the maximum aortic sinus measurement with control data from the literature. We employed a Kaplan-Meier survival curve to estimate the age at which dilatation occurs. The mean age of the total group at presentation was 15.5 years (range 1.5-40 years). Skeletal abnormalities were present in 95%. Eye involvement was found in 18%. In the 115/160 patients with an abnormal aortic root, 78/115 (68%) developed aortic root dilatation before 19 years of age. From the Kaplan-Meier curve, it can be estimated that about 35% of the patients have aortic root dilatation already at the age of 5 years and 70% before the age of 20 years, and at least 80% by 40 years. There were 31 patients with normal aortic root when first seen but 24/31 (77%) developed aortic root dilatation before the age of 19 years and 7/31 (22.6%) after 19 years of age. Of those (seven patients) who developed new pathological aortic root dilatation after age 19 years, the age range was between 21 and 40 years with a mean of 27 years. Overall, 13 patients (8%) had surgery for aortic root replacement. Conclusion Aortic root dilatation develops early in MFS and was present in 35% by the age of 5 years and 68% by 19 years. Even though new aortic root dilation is relatively rare, it is not possible to safely discharge patients with MFS as about one-third of the patients in our series who developed new pathological aortic root dilation did so after the age of 19 years. (Less)
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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
cohort study, puberty, Marfan's syndrome, aorta root
in
European Heart Journal
volume
28
issue
3
pages
376 - 379
publisher
Oxford University Press
external identifiers
  • wos:000244259800019
  • scopus:33846994068
ISSN
1522-9645
DOI
10.1093/eurheartj/ehl457
language
English
LU publication?
yes
id
540b68b8-a0ae-442a-9b13-06df5f543c6f (old id 674218)
date added to LUP
2007-12-11 09:17:02
date last changed
2017-03-05 04:07:47
@article{540b68b8-a0ae-442a-9b13-06df5f543c6f,
  abstract     = {Aims The main aim of this study was to describe the age at which pathological aortic root dilation occurs in patients with Marfan's syndrome (MFS). Methods and results A total of 160 patients with MFS attending a regional cardiac centre were reviewed retrospectively. Dilation of the ascending aorta was diagnosed by comparing the maximum aortic sinus measurement with control data from the literature. We employed a Kaplan-Meier survival curve to estimate the age at which dilatation occurs. The mean age of the total group at presentation was 15.5 years (range 1.5-40 years). Skeletal abnormalities were present in 95%. Eye involvement was found in 18%. In the 115/160 patients with an abnormal aortic root, 78/115 (68%) developed aortic root dilatation before 19 years of age. From the Kaplan-Meier curve, it can be estimated that about 35% of the patients have aortic root dilatation already at the age of 5 years and 70% before the age of 20 years, and at least 80% by 40 years. There were 31 patients with normal aortic root when first seen but 24/31 (77%) developed aortic root dilatation before the age of 19 years and 7/31 (22.6%) after 19 years of age. Of those (seven patients) who developed new pathological aortic root dilatation after age 19 years, the age range was between 21 and 40 years with a mean of 27 years. Overall, 13 patients (8%) had surgery for aortic root replacement. Conclusion Aortic root dilatation develops early in MFS and was present in 35% by the age of 5 years and 68% by 19 years. Even though new aortic root dilation is relatively rare, it is not possible to safely discharge patients with MFS as about one-third of the patients in our series who developed new pathological aortic root dilation did so after the age of 19 years.},
  author       = {Aburawi, Elhadi and O'Sullivan, John},
  issn         = {1522-9645},
  keyword      = {cohort study,puberty,Marfan's syndrome,aorta root},
  language     = {eng},
  number       = {3},
  pages        = {376--379},
  publisher    = {Oxford University Press},
  series       = {European Heart Journal},
  title        = {Relation of aortic root dilatation and age in Marfan's syndrome},
  url          = {http://dx.doi.org/10.1093/eurheartj/ehl457},
  volume       = {28},
  year         = {2007},
}