Document Detail

Natural history of cardiovascular manifestations in Marfan syndrome.
MedLine Citation:
PMID:  11159287     Owner:  NLM     Status:  MEDLINE    
AIMS: To investigate the natural history of mitral valve and aortic abnormalities in patients with Marfan syndrome during childhood and adolescence. METHODS: Fifty 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. RESULTS: Mitral 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. CONCLUSIONS: During 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.
C D van Karnebeek; M S Naeff; B J Mulder; R C Hennekam; M Offringa
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Archives of disease in childhood     Volume:  84     ISSN:  1468-2044     ISO Abbreviation:  Arch. Dis. Child.     Publication Date:  2001 Feb 
Date Detail:
Created Date:  2001-02-22     Completed Date:  2001-04-05     Revised Date:  2009-11-18    
Medline Journal Info:
Nlm Unique ID:  0372434     Medline TA:  Arch Dis Child     Country:  England    
Other Details:
Languages:  eng     Pagination:  129-37     Citation Subset:  AIM; IM    
Department of Paediatrics H3-150, Emma Children's Hospital, Academic Medical Center, University of Amsterdam, Meibergdreef 9, 1105 AZ Amsterdam, Netherlands.
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MeSH Terms
Age of Onset
Aortic Valve Insufficiency / etiology,  surgery
Child, Preschool
Cohort Studies
Dilatation, Pathologic / etiology,  therapy
Disease Progression
Heart Valve Diseases / etiology*,  mortality
Marfan Syndrome / complications*,  mortality
Mitral Valve Insufficiency / etiology,  surgery
Mitral Valve Prolapse / etiology,  surgery
Retrospective Studies
Risk Factors
Sex Factors
Survival Analysis

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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