Article
Natural history of cardiovascular manifestations in Marfan
syndrome
C D M van Karnebeeka, M S J Naeffa, B J M Mulderb, R C M Hennekama, M Offringaa
a Department of
Paediatrics H3-150, Emma Children's Hospital, Academic Medical Center,
University of Amsterdam, Meibergdreef 9, 1105 AZ Amsterdam, Netherlands, b Department of Cardiology,
Academic Medical Center
Correspondence to: Dr Offringa m.offringa{at}amc.uva.nl
Accepted 8 June 2000
AIMS
To investigate the natural
history of mitral valve and aortic abnormalities in patients with
Marfan syndrome during childhood and adolescence.
METHODSFifty two patients with
Marfan syndrome were followed for a mean of 7.9 years. Occurrence of
adverse cardiovascular outcomes was measured clinically and by
ultrasound examination.
RESULTSMitral valve prolapse (MVP)
was diagnosed in 46 patients at a mean age of 9.7 years, more than 80%
of whom presented as "silent MVP". Mitral regurgitation (MR)
occurred in 25 patients, aortic dilatation in 43, and aortic
regurgitation (AR) in 13. Both MVP and aortic dilatation developed at a
constant rate during the age period 5-20 years. In 23 patients MVP was
diagnosed before aortic dilatation, in 18 the reverse occurred, and in
11 patients the two abnormalities were diagnosed simultaneously. During
follow up, 21 patients showed progression of mitral valve dysfunction; progression of aortic abnormalities occurred in 13. Aortic surgery was
performed in 10; two died of subsequent complications. Mitral valve
surgery was performed in six. In sporadic female Marfan patients the
age at initial diagnosis of MVP, MR, aortic dilatation, and AR was
lowest, the grade of MR and AR most severe, the time lapse between the
occurrence of MVP and subsequent MR as well as between dilatation and
subsequent AR shortest, and the risk for cardiovascular associated
morbidity and mortality highest.
CONCLUSIONSDuring childhood and
adolescence in Marfan syndrome, mitral valve dysfunction as well as
aortic abnormalities develop and progress gradually, often without
symptoms, but may cause considerable morbidity and mortality by the end
of the second decade, especially in female sporadic patients.
Keywords: Marfan syndrome; cohort study; mitral valve; aorta
© 2001 by Archives of Disease in Childhood
CiteULike 
Complore 
Connotea 
Del.icio.us 
Digg 
Reddit 
Technorati What's this?
Relevant Article
-
Rapid responses
Arch. Dis. Child. 2001 84: 422.[Extract] [Full Text]
This article has been cited by other articles:
-
Kiotsekoglou, A, Sutherland, G R, Moggridge, J C, Nassiri, D K, Camm, A J, Child, A H
(2009). The unravelling of primary myocardial impairment in Marfan syndrome by modern echocardiography. Heart
95: 1561-1566
[Abstract] [Full Text] -
Everitt, M. D., Pinto, N., Hawkins, J. A., Mitchell, M. B., Kouretas, P. C., Yetman, A. T.
(2009). Cardiovascular surgery in children with Marfan syndrome or Loeys-Dietz syndrome.. J. Thorac. Cardiovasc. Surg.
137: 1327-1333
[Abstract] [Full Text] -
Faivre, L., Masurel-Paulet, A., Collod-Beroud, G., Callewaert, B. L., Child, A. H., Stheneur, C., Binquet, C., Gautier, E., Chevallier, B., Huet, F., Loeys, B. L., Arbustini, E., Mayer, K., Arslan-Kirchner, M., Kiotsekoglou, A., Comeglio, P., Grasso, M., Halliday, D. J., Beroud, C., Bonithon-Kopp, C., Claustres, M., Robinson, P. N., Ades, L., De Backer, J., Coucke, P., Francke, U., De Paepe, A., Boileau, C., Jondeau, G.
(2009). Clinical and Molecular Study of 320 Children With Marfan Syndrome and Related Type I Fibrillinopathies in a Series of 1009 Probands With Pathogenic FBN1 Mutations. Pediatrics
123: 391-398
[Abstract] [Full Text] -
van der Klaauw, A. A, Bax, J. J, Smit, J. W A, Holman, E. R, Delgado, V., Bleeker, G. B, Biermasz, N. R, Roelfsema, F., Romijn, J. A, Pereira, A. M
(2008). Increased aortic root diameters in patients with acromegaly. Eur J Endocrinol
159: 97-103
[Abstract] [Full Text] -
Aburawi, E. H., O'Sullivan, J.
(2007). Relation of aortic root dilatation and age in Marfan's syndrome. Eur Heart J
28: 376-379
[Abstract] [Full Text] -
CHAFFINS, J. A.
(2007). Marfan Syndrome. radtech
78: 222-236
[Abstract] [Full Text] -
Bhudia, S. K., Troughton, R., Lam, B.-K., Rajeswaran, J., Mills, W. R., Gillinov, A. M., Griffin, B. P., Blackstone, E. H., Lytle, B. W., Svensson, L. G.
(2006). Mitral Valve Surgery in the Adult Marfan Syndrome Patient. Ann. Thorac. Surg.
81: 843-848
[Abstract] [Full Text] -
Milewicz, D. M., Dietz, H. C., Miller, D. C.
(2005). Treatment of Aortic Disease in Patients With Marfan Syndrome. Circulation
111: e150-e157
[Full Text]
eLetters:
Read all eLetters
- Role of beta-adrenergic blocker therapy in children with Marfan syndrome and aortic root dilatation
- P Venugopalan
- ADC Online, 23 Feb 2001 [Full text]
- Role of beta-adrenergic blocker in Marfan syndrome with aortic root dilatation - Authors' response
- Clara DM van Karnebeek
- ADC Online, 14 Mar 2001 [Full text]
- Full Text
- Full Text (PDF)
- Submit a response
- Read responses to this article
- Alert me when this article is cited
- Alert me when eLetters are posted
- Alert me if a correction is posted
- Citation Map
Services
- Email this link to a friend
- Similar articles in ADC Online
- Similar articles in PubMed
- Add article to my folders
- Download to citation manager
- Request Permissions
Citing Articles
Google Scholar
PubMed
Topic Collections
-
Epidemiologic studies
-
Drugs: cardiovascular system
-
Rheumatology
-
Valvar diseases
-
Clinical diagnostic tests
- Relevant Article
Bookmark with
Register for free content
The full back archive is now available for all BMJ Journals. Institutional subscribers may access the entire archive as part of their subscription. Personal subscribers will also have access to all content when logged in. Non-subscribers who register have free access to all articles published before 2006 right back to volume 1 issue 1. Register here to access the free archive of all BMJ Journals.
Don't forget to sign up for content alerts so you keep up to date with all the articles as they are published.
