ArticleOrphanet journal of rare diseases2020
Increased visceral arterial tortuosity in Marfan syndrome.
Article in Orphanet journal of rare diseases, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 19 papers.
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Who cites it
19 citing papers in PubMed, 22 citations in OpenAlex.
- Diving MCA and First-Pass Effect: A Subgroup Analysis From the ARISE II Study.Stroke (Hoboken, N.J.) · 2026Article
- Patient-Specific Fluid-Structure Interaction Simulations Suggest Wall-Shear-Stress-Related Biomarkers in Type B Dissection Associated with Marfan Syndrome.Annals of biomedical engineering · 2026Article
- Topology-preserving, diameter-specific framework for conjunctival vessel segmentation and tortuosity analysis in diabetes.Scientific reports · 2026Article
- Peripheral arterial lesions detected by vascular ultrasound and their association with aortic events in heritable thoracic aortic diseases.International journal of cardiology. Heart & vasculature · 2026Article
- Article
- Marfan syndrome cardiomyocytes show excess of titin isoform N2BA and extended sarcomeric M-band.The Journal of general physiology · 2025Article
- Growth Rate Assessed by Vascular Deformation Mapping Predicts Type B Aortic Dissection in Marfan Syndrome.Journal of the American Heart Association · 2025Article
- Case Report: Challenges in the surgical treatment of Marfan-associated aortic aneurysms: a literature review starting from a clinical case.Frontiers in surgery · 2025Article
- Growth Rate Assessed by Vascular Deformation Mapping predicts Type B Aortic Dissection in Marfan Syndrome.medRxiv : the preprint server for health sciences · 2024Article
- Tortuosity in non-atherosclerotic vascular diseases is associated with age, arterial aneurysms, and hypertension.Orphanet journal of rare diseases · 2024Article
- Review
- Nanoscale Structural Comparison of Fibrillin-1 Microfibrils Isolated from Marfan and Non-Marfan Syndrome Human Aorta.International journal of molecular sciences · 2023Article
- A Workflow for Three-Dimensional Reconstruction and Quantification of the Monkey Optic Nerve Head Vascular Network.Journal of biomechanical engineering · 2022Article
- Splenic Injury After a Colonoscopy: Threading the Scope Carefully in Heritable Connective Tissue Disorders.Cureus · 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
- The Molecular Genetics of Marfan Syndrome.International journal of medical sciences · 2021Review
- Case Report: Morphological Characterization and Long-Term Observation of Bilateral Sequential Internal Mammary Artery Aneurysms in a Patient With Confirmed FBN1 Mutation.Frontiers in cardiovascular medicine · 2021Article
- Detection of 3D Arterial Centerline Extraction in Spiral CT Coronary Angiography.Journal of healthcare engineering · 2021Article
- Optimising the mutation screening strategy in Marfan syndrome and identifying genotypes with more severe aortic involvement.Orphanet journal of rare diseases · 2020Article
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Authors and funding
12 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundClinical evidence suggests that the currently recommended approach to estimate the risk of aortic dissection in Marfan syndrome (MFS) is not reliable enough. Therefore, we investigated the possible role of visceral arterial tortuosity in the risk stratification. METHODS AND
resultsSplenic and renal arteries of 37 MFS patients and 74 age and gender matched control subjects were segmented using CT angiography imaging. To measure tortuosity, distance metric (DM), sum of angles metric (SOAM), inflection count metric (ICM), and the ratio of ICM and SOAM (ICM/SOAM) were calculated. DM of the splenic, right and left renal artery was significantly higher in MFS patients than in controls (2.44 [1.92-2.80] vs. 1.75 [1.57-2.18] p < 0.001; 1.16 [1.10-1.28] vs. 1.11 [1.07-1.15] p = 0.011; 1.40 [1.29-1.70] vs. 1.13 [1.09-1.23] p < 0.001, respectively). A similar tendency for ICM and an opposite tendency for SOAM were observed. ICM/SOAM was significantly higher in the MFS group compared to controls in case of all three arteries (73.35 [62.26-93.63] vs. 50.91 [43.19-65.62] p < 0.001; 26.52 [20.69-30.24] vs. 19.95 [16.47-22.95] p < 0.001; 22.81 [18.64-30.96] vs. 18.38 [15.29-21.46] p < 0.001, respectively). MFS patients who underwent aortic root replacement had increased right and left renal DM and ICM/SOAM compared to MFS patients without surgery.
conclusionTo our knowledge this is the first demonstration of increased arterial tortuosity in MFS on visceral arteries. Visceral arterial tortuosity, dominated by curves of lower frequency but higher amplitude according to the observed opposite tendency between the DM and SOAM metrics, could be a possible new predictor of serious manifestations of MFS.
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