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Augmentation index and the evolution of aortic disease in marfan-like syndromes.

AbstractBACKGROUND:
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.
AuthorsKai 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
JournalAmerican journal of hypertension (Am J Hypertens) Vol. 23 Issue 7 Pg. 716-24 (Jul 2010) ISSN: 1941-7225 [Electronic] United States
PMID20395939 (Publication Type: Journal Article)
Topics
  • Adult
  • Aortic Dissection (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

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