The online version of this article (doi:10.1186/1471-2261-14-47) contains supplementary material, which is available to authorized users.
The authors declare that they have no competing interests.
BÁ carried out the collection of the samples, participated in the analysis and interpretation of clinical data, drafted the manuscript and performed the statistical analysis. KB carried out the measurements, participated in the analysis and interpretation of clinical data, drafted the manuscript and performed the statistical analysis. BSZ carried out the collection of the samples, participated in the analysis of the molecular biological data. MP participated in the collection of the data, drafted the manuscript and revised the intellectual content. LD participated in the collection of the data, drafted the manuscript and performed the statistical analysis. BO participated in pulmonological clinical examinations. ZSN participated in the ELISA process and in RT-PCR measurements. FT participated in the design of the study and revised the intellectual content. BM revised the manuscript and provided professional advices and institutional background. ZSZ conceived of the study, participated in the coordination of the patients and in the study design, and helped to draft the manuscript. All authors read and approved the final manuscript.
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.
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.
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.
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.
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- Possible extracardiac predictors of aortic dissection in Marfan syndrome
Zsolt B Nagy
- BioMed Central
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