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          Institute: MPI für molekulare Genetik     Collection: Research Group Development and Disease     Display Documents



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ID: 539740.0, MPI für molekulare Genetik / Research Group Development and Disease
Augmentation index and the evolution of aortic disease in marfan-like syndromes.
Authors:Mortensen, K.; Baulmann, J.; Rybczynski, M.; Sheikhzadeh, S.; Aydin, M. A.; Treede, H.; Dombrowski, E.; Kühne, K.; Peitsmeier, P.; Habermann, C. R.; Robinson, P. N.; Stuhrmann, M.; Berger, J.; Meinertz, T.; von Kodolitsch, Y.
Language:English
Date of Publication (YYYY-MM-DD):2010-04-15
Title of Journal:American Journal of Hypertension
Journal Abbrev.:Am J Hypertens
Volume:23
Issue / Number:7
Start Page:716
End Page:724
Copyright:© 2011 American Journal of Hypertension, Ltd.
Review Status:not specified
Audience:Experts Only
Abstract / Description:BACKGROUND: The augmentation index at a heart rate of 75 beats/min (AIx@HR75) and central pulse pressure (CPP) can be measured noninvasively with applanation tonometry (APT). In this observational study, we investigated the relationship between AIx@HR75, CPP and aortic disease in patients with Marfan-like syndromes. METHODS: We performed APT in 78 consecutive patients in whom classic Marfan syndrome (MFS) had been excluded (46 men and 32 women aged 34 +/- 13 years). These patients comprised 9 persons with MFS-like habitus, 6 with a bicuspid aortic valve (BAV), 5 with MASS phenotype, 3 with vascular type of Ehlers-Danlos syndrome (EDS), 3 with familial thoracic aortic aneurysm, 2 with Loeys-Dietz syndrome (LDS), 1 with mitral valve prolapse syndrome, 1 with familial ectopia lentis, and 48 persons with Marfan-like features but no defined syndrome. During 20 +/- 18 months after APT, we observed progression of aortic diameters in 15 patients, and aortic surgery or aortic dissection in 3 individuals. RESULTS: All 11 patients with Marfan-like syndromes and progression of aortic disease exhibited AIx@HR75 > or =11%, including 8 individuals with aortic diameters < or =95th percentile of normal at baseline. Similarly, all 7 individuals without any defined syndrome but progression of aortic diameters exhibited AIx@HR75 >11%, including 6 individuals with aortic diameters < or =95th percentile at the time of APT. Aortic disease did not evolve at AIx@HR75 <11%. CPP is also related to aortic disease progression. CONCLUSIONS: Aortic disease evolution relates to AIx@HR75 and CPP in Marfan like syndromes. Larger studies with comprehensive clinical and echocardiographic follow-up over long time intervals will be required to establish APT for prediction of aortic disease evolution in Marfan-like syndromes.
Free Keywords:applanation tonometry;
augmentation index;
bicuspid aortic valve;
blood pressure;
hypertension;
Loeys–Dietz syndrome
Comment of the Author/Creator:Correspondence author: Yskert von Kodolitsch, (kodolitsch@uke.de)
External Publication Status:published
Document Type:Article
Communicated by:Stefan Mundlos
Affiliations:MPI für molekulare Genetik
External Affiliations:1.Centre of Cardiology and Cardiovascular Surgery, University Hospital Eppendorf, Hamburg, Germany;
2.Department of Cardiology, Medical Clinic and Policlinic, University of Wuerzburg, Würzburg, Germany:
3.Department of Diagnostic and Interventional Radiology, University Hospital Eppendorf, Hamburg, Germany;
4.Institute of Medical Genetics, Charité Universitätsmedizin Berlin, Berlin, Germany;
5.Institute of Human Genetics, Medical School, Hannover, Germany;
6.Department of Medical Biometry and Epidemiology, University Hospital Eppendorf, Hamburg, Germany.
Identifiers:ISSN:0895-7061 [ID No:1]
URL:http://www.ncbi.nlm.nih.gov/pubmed/20395939 [ID No:2]
DOI:10.1038/ajh.2010.78 [ID No:3]
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