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Possible extracardiac predictors of aortic dissection in Marfan syndrome

Bence Agg, Kálmán Benke1, Bálint Szilveszter

  • 1Heart and Vascular Center, Semmelweis University, Városmajor str, 68, 1122 Budapest, Hungary. kalman.benke@gmail.com.

BMC Cardiovascular Disorders
|April 12, 2014
PubMed
Abstract

Insights

Predicting aortic dissection in Marfan syndrome (MFS) is crucial. Easily measurable factors like TGF-β serum levels, MMP-3, and striae atrophicae may help identify high-risk MFS patients for timely aortic root replacement.

Area of Science:

  • Cardiovascular Research
  • Genetics and Genomics
  • Biomarker Discovery

Background:

  • Aortic diameter alone is insufficient for predicting aortic dissection in Marfan syndrome (MFS).
  • Optimal timing for preventive aortic root replacement (ARR) in MFS is challenging.
  • Family history and aortic growth rate are not decisive predictors for MFS complications.

Purpose of the Study:

  • To identify novel predictors of aortic dissection in Marfan syndrome.
  • To investigate the role of TGF-β serum levels and MMP expression in MFS aortic dissection.
  • To explore clinical parameters for risk stratification in MFS patients.

Main Methods:

  • Established a Marfan Biobank with 79 MFS patients.
  • Analyzed 39 MFS patients who underwent ARR, categorized by surgical indication.
  • Measured TGF-β serum levels (ELISA) and MMP-3/MMP-9 expression (RT-PCR); assessed clinical parameters.

Main Results:

  • Elevated TGF-β serum levels (p < 0.0001) and increased MMP-3 expression (p = 0.062) in patients with aortic dissection compared to the annuloaortic ectasia group.
  • Striae atrophicae were significantly more common in the dissection group (92% vs. 41%, p = 0.027).
  • TGF-β serum level, MMP-3, and striae atrophicae emerged as potential predictors.

Conclusions:

  • Striae atrophicae, TGF-β serum level, and MMP-3 are easily measurable parameters that may predict aortic dissection risk in MFS.
  • Proposed a new classification of MFS as benign or malignant based on these predictors.
  • Findings could aid in determining the optimal timing for prophylactic ARR in MFS patients.

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