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SHR Neuro Cancer Cardio Lipid Metab Microb
Mortensen, K; Baulmann, J; Rybczynski, M; Sheikhzadeh, S; Aydin, MA; Treede, H; Dombrowski, E; Kühne, K; Peitsmeier, P; Habermann, CR; Robinson, PN; Stuhrmann, M; Berger, J; Meinertz, T; von Kodolitsch, Y.
Augmentation index and the evolution of aortic disease in marfan-like syndromes.
Am J Hypertens. 2010; 23(7):716-724
Doi: 10.1038/ajh.2010.78
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- Baulmann Johannes
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- Abstract:
- 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. 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. 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. 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.
- Find related publications in this database (using NLM MeSH Indexing)
- Adult -
- Aneurysm, Dissecting - physiopathology
- Aortic Aneurysm, Thoracic - physiopathology
- Aortic Diseases - pathology
- Aortic Diseases - 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 -
- Find related publications in this database (Keywords)
- applanation tonometry
- augmentation index
- bicuspid aortic valve
- blood pressure
- hypertension
- Loeys-Dietz syndrome