| Augmentation index and the evolution of aortic disease in marfan-like syndromes. | |
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MedLine Citation:
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PMID: 20395939 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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Kai Mortensen; Johannes Baulmann; Meike Rybczynski; Sara Sheikhzadeh; Muhammet A Aydin; Henrik Treede; Ellen Dombrowski; Kristin Kühne; Philipp Peitsmeier; Christian R Habermann; Peter N Robinson; Manfred Stuhrmann; Jürgen Berger; Thomas Meinertz; Yskert von Kodolitsch |
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Publication Detail:
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Type: Journal Article Date: 2010-04-15 |
Journal Detail:
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Title: American journal of hypertension Volume: 23 ISSN: 1941-7225 ISO Abbreviation: Am. J. Hypertens. Publication Date: 2010 Jul |
Date Detail:
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Created Date: 2010-06-18 Completed Date: 2010-09-23 Revised Date: 2011-06-30 |
Medline Journal Info:
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Nlm Unique ID: 8803676 Medline TA: Am J Hypertens Country: United States |
Other Details:
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Languages: eng Pagination: 716-24 Citation Subset: IM |
Affiliation:
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Centre of Cardiology and Cardiovascular Surgery, University Hospital Eppendorf, Hamburg, Germany. |
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| MeSH Terms | |
Descriptor/Qualifier:
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Adult Aneurysm, Dissecting / physiopathology Aortic Aneurysm, Thoracic / physiopathology Aortic Diseases / pathology, physiopathology* Blood Pressure* Blood Pressure Determination Disease Progression Ectopia Lentis / physiopathology Female Heart Rate* Hemodynamics* Humans Loeys-Dietz Syndrome / physiopathology Male Marfan Syndrome / physiopathology* Mitral Valve Prolapse / physiopathology Young Adult |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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