Augmentation Index Relates to Progression of Aortic Disease in Adults With Marfan Syndrome

Abstract

We provide evidence that APT parameters relate to aortic disease progression in medically treated patients with MFS. We believe that APT has a potential to improve risk stratification in the clinical management of MFS patients.

Central pulse pressure and augmentation index in asymptomatic bicuspid aortic valve disease

“…Central hemodynamics add new functional information to standard imaging procedures by identifying impaired arterial vessel function parameters irrespective of aortic diameter size or ≤moderate aortic valve dysfunction. Interestingly, our young adults with a bicuspid aortic valve presented with increased CPP and AIx@HR75 but normal pulse wave velocities, which were also found in Marfan and Marfan-like syndromes [1,2]. We found that despite betablockers or angiotensin antagonists, presence of a bicuspid aortic valve was associated with increased CPP and AIx@HR75 measurements.…”

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Increased central pulse pressure (CPP) and augmentation index (AIx) relate to cardiovascular events in healthy individuals and were shown to be increased in persons with heritable aortic disease such as Marfan syndrome or Marfan-like syndromes [1,2]. Bicuspid aortic valve disease has a prevalence of ≤2% in the general population, and affected persons may be at risk for cardiovascular complications including aortic aneurysm and aortic dissection.

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“…Peripheral blood pressures were similar in both groups, with lower diastolic pressures in the study patients (P b .001). We performed applanation tonometry as described previously [1], and comparison of control individuals and study patients yielded significantly increased CPP (P b .001) and AIx adjusted to a heart rate of 75 beats/min (AIx@HR75; P = .01; Table 1; Fig. 1).…”

“…Of 113 consecutive out-patients whom we saw at annual or twoyear intervals for follow-up of an asymptomatic, isolated bicuspid aortic valve we excluded 41 individuals for previous aortic valve intervention, 12 for Marfan or other syndromes, 5 for complex cardiovascular malformations, 5 for cardiac symptoms, 3 for inadequate tonometric measurements, 2 for rejecting to participate in the study, and 5 for other criteria [1]. The remaining 40 individuals constituted our study group that comprised 30 men and 10 women at a mean age of 44 ± 16 years.…”

Augmenting the Assessment of Marfan Syndrome?

“…5 This study is the largest to date in MFS patients using this methodology, and examines an extremely well characterized population. The technology is also clinically relevant, due to its wide availability and ease of use.…”