ArticleBMC cardiovascular disorders2014
Possible extracardiac predictors of aortic dissection in Marfan syndrome.
Article in BMC cardiovascular disorders, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers.
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Who cites it
20 citing papers in PubMed, 41 citations in OpenAlex.
- Circulating interleukin-6 (IL-6) levels are associated with aortic dimensions in genetic aortic conditions.PloS one · 2019Trial
- Genotype-associated structural and nanomechanical alterations of aortic fibrillin-1 microfibrils in Marfan syndrome.Matrix biology plus · 2026Article
- Phenotypic Associations of Early Cardiovascular Surgery in Pediatric Marfan Syndrome.Children (Basel, Switzerland) · 2026Article
- Extracardiac Manifestations Fail to Predict the Severity of Cardiac Phenotype in Children and Young Adults with Marfan Syndrome.Pediatric cardiology · 2025Article
- Low Molecular Weight Hyaluronic Acid Added to Six Specific Amino Acids in the Treatment of Striae Alba (SA): An Observational Study.Aesthetic plastic surgery · 2024Observational
- Bullous striae distensae in a young patient with cardiomyopathy.Dermatology reports · 2023Article
- Nanoscale Structural Comparison of Fibrillin-1 Microfibrils Isolated from Marfan and Non-Marfan Syndrome Human Aorta.International journal of molecular sciences · 2023Article
- Observational
- Amino Acid and Phospholipid Metabolism as an Indicator of Inflammation and Subtle Cardiomyopathy in Patients with Marfan Syndrome.Metabolites · 2021Article
- Potential predictors of severe cardiovascular involvement in Marfan syndrome: the emphasized role of genotype-phenotype correlations in improving risk stratification-a literature review.Orphanet journal of rare diseases · 2021Review
- Optimising the mutation screening strategy in Marfan syndrome and identifying genotypes with more severe aortic involvement.Orphanet journal of rare diseases · 2020Article
- Increased visceral arterial tortuosity in Marfan syndrome.Orphanet journal of rare diseases · 2020Article
- Rab7‑mediated autophagy regulates phenotypic transformation and behavior of smooth muscle cells via the Ras/Raf/MEK/ERK signaling pathway in human aortic dissection.Molecular medicine reports · 2019Article
- Article
- Hungarian Marfan family with largeJournal of thoracic disease · 2018Article
- The effects of acute and elective cardiac surgery on the anxiety traits of patients with Marfan syndrome.BMC psychiatry · 2017Article
- Bentall procedure: quarter century of clinical experiences of a single surgeon.Journal of cardiothoracic surgery · 2016Article
- Periosteal osteosarcoma and Marfan's syndrome: A case report and literature review.Oncology letters · 2016Article
- Blood Plasma TGF- β1 Concentration in Sporadic Dilatative Pathology of Ascending Aorta: More Questions than Answers.PloS one · 2015Article
- Striae Distensae Treatment Review and Update.Indian dermatology online journalReview
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Authors and funding
10 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundAccording 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.
methodsA 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.
resultsAmong 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.
conclusionsWe 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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