Augmentation index and the evolution of aortic disease in marfan-like syndromes.
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.
Mortensen
Kai
K
Centre of Cardiology and Cardiovascular Surgery, University Hospital Eppendorf, Hamburg, Germany.
Baulmann
Johannes
J
Rybczynski
Meike
M
Sheikhzadeh
Sara
S
Aydin
Muhammet A
MA
Treede
Henrik
H
Dombrowski
Ellen
E
Kühne
Kristin
K
Peitsmeier
Philipp
P
Habermann
Christian R
CR
Robinson
Peter N
PN
Stuhrmann
Manfred
M
Berger
Jürgen
J
Meinertz
Thomas
T
von Kodolitsch
Yskert
Y
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