Open Access Highly Accessed Research article

Possible extracardiac predictors of aortic dissection in Marfan syndrome

Bence Ágg14, Kálmán Benke14*, Bálint Szilveszter1, Miklós Pólos1, László Daróczi1, Balázs Odler2, Zsolt B Nagy3, Ferenc Tarr1, Béla Merkely1 and Zoltán Szabolcs14

Author Affiliations

1 Heart and Vascular Center, Semmelweis University, Városmajor str. 68, 1122 Budapest, Hungary

2 Department of Pulmonology, Semmelweis University, Budapest, Hungary

3 Association of Genetics for Health, Budapest, Hungary

4 Hungarian Marfan Foundation, Budapest, Hungary

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BMC Cardiovascular Disorders 2014, 14:47  doi:10.1186/1471-2261-14-47

Published: 11 April 2014

Abstract

Background

According to previous studies, aortic diameter alone seems to be insufficient to predict the event of aortic dissection in Marfan syndrome (MFS). Determining the optimal schedule for preventive aortic root replacement (ARR) aortic growth rate is of importance, as well as family history, however, none of them appear to be decisive. Thus, the aim of this study was to search for potential predictors of aortic dissection in MFS.

Methods

A Marfan Biobank consisting of 79 MFS patients was established. Thirty-nine MFS patients who underwent ARR were assigned into three groups based on the indication for surgery (dissection, annuloaortic ectasia and prophylactic surgery). The prophylactic surgery group was excluded from the study. Transforming growth factor-β (TGF-β) serum levels were measured by ELISA, relative expression of c-Fos, matrix metalloproteinase 3 and 9 (MMP-3 and −9) were assessed by RT-PCR. Clinical parameters, including anthropometric variables - based on the original Ghent criteria were also analyzed.

Results

Among patients with aortic dissection, TGF-β serum level was elevated (43.78 ± 6.51 vs. 31.64 ± 4.99 ng/l, p < 0.0001), MMP-3 was up-regulated (Ln2α = 1.87, p = 0.062) and striae atrophicae were more common (92% vs. 41% p = 0.027) compared to the annuloaortic ectasia group.

Conclusions

We found three easily measurable parameters (striae atrophicae, TGF-β serum level, MMP-3) that may help to predict the risk of aortic dissection in MFS. Based on these findings a new classification of MFS, that is benign or malignant is also proposed, which could be taken into consideration in determining the timing of prophylactic ARR.

Keywords:
Marfan syndrome; Aortic dissection; Predictors; Cardiac surgery