title: Augmentation index and the evolution of aortic disease in marfan-like syndromes.
abstract: 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.
authors.affiliation: Centre of Cardiology and Cardiovascular Surgery, University Hospital Eppendorf, Hamburg, Germany.
authors.forename: Kai
authors.initials: K
authors.lastname: Mortensen
authors.forename: Johannes
authors.initials: J
authors.lastname: Baulmann
authors.forename: Meike
authors.initials: M
authors.lastname: Rybczynski
authors.forename: Sara
authors.initials: S
authors.lastname: Sheikhzadeh
authors.forename: Muhammet A
authors.initials: MA
authors.lastname: Aydin
authors.forename: Henrik
authors.initials: H
authors.lastname: Treede
authors.forename: Ellen
authors.initials: E
authors.lastname: Dombrowski
authors.forename: Kristin
authors.initials: K
authors.lastname: Kühne
authors.forename: Philipp
authors.initials: P
authors.lastname: Peitsmeier
authors.forename: Christian R
authors.initials: CR
authors.lastname: Habermann
authors.forename: Peter N
authors.initials: PN
authors.lastname: Robinson
authors.forename: Manfred
authors.initials: M
authors.lastname: Stuhrmann
authors.forename: Jürgen
authors.initials: J
authors.lastname: Berger
authors.forename: Thomas
authors.initials: T
authors.lastname: Meinertz
authors.forename: Yskert
authors.initials: Y
authors.lastname: von Kodolitsch
mesh_terms: D000328:Adult; D000784:Aortic Dissection / Q000503:physiopathology; D017545:Aortic Aneurysm, Thoracic / Q000503:physiopathology; D001018:Aortic Diseases / Q000473:pathology / Q000503:physiopathology*; D001794:Blood Pressure; D001795:Blood Pressure Determination; D018450:Disease Progression; D004479:Ectopia Lentis / Q000503:physiopathology; D005260:Female; D006339:Heart Rate; D006439:Hemodynamics; D006801:Humans; D055947:Loeys-Dietz Syndrome / Q000503:physiopathology; D008297:Male; D008382:Marfan Syndrome / Q000503:physiopathology*; D008945:Mitral Valve Prolapse / Q000503:physiopathology; D055815:Young Adult