ArticleHeart and vessels2016
Histopathology of aortic complications in bicuspid aortic valve versus Marfan syndrome: relevance for therapy?
Article in Heart and vessels, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 28 papers, 1 of them a synthesis that pooled it.
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Who cites it
28 citing papers in PubMed, 1 synthesis or guideline pooled it, 48 citations in OpenAlex.
- The American Association for Thoracic Surgery consensus guidelines on bicuspid aortic valve-related aortopathy: Full online-only version.The Journal of thoracic and cardiovascular surgery · 2018Guideline
- Towards a regional and national cardiothoracic biobank in India: translating a Dutch model to address a poorly characterised disease burden.The Lancet regional health. Southeast Asia · 2026Review
- Article
- Article
- Circulating Matrix Metalloproteinases for Prediction of Aortic Dilatation in Children with Bicuspid Aortic Valve: A Single-Center, Observational Study.International journal of molecular sciences · 2024Observational
- Is the intimal thickness a key contributor to thoracic aortopathy?Indian journal of thoracic and cardiovascular surgery · 2023Article
- The role of transforming growth factor beta in bicuspid aortic valve aortopathy.Indian journal of thoracic and cardiovascular surgery · 2023Article
- The Importance of Aortic Valve Bicuspid Phenotype in Valvular Evolution in Pediatric Patients: A Case Report and Literature Mini-Review.International journal of molecular sciences · 2023Review
- Adventitial adaptive immune cells are associated with ascending aortic dilatation in patients with a bicuspid aortic valve.Frontiers in cardiovascular medicine · 2023Article
- Risk for acquired coronary artery disease in genetic vs. congenital thoracic aortopathy.Frontiers in cardiovascular medicine · 2022Article
- Monocyte-to-HDL-cholesterol ratio is associated with Ascending Aorta Dilatation in Patients with Bicuspid Aortic Valve.African health sciences · 2021Article
- A Systematic Histopathologic Evaluation of Type-A Aortic Dissections Implies a Uniform Multiple-Hit Causation.Journal of cardiovascular development and disease · 2021Article
- Retinal and Choroidal Vasculature in Patients with Marfan Syndrome.Translational vision science & technology · 2020Observational
- Marfan Syndrome Versus Bicuspid Aortic Valve Disease: Comparative Analysis of Obstetric Outcome and Pregnancy-Associated Immediate and Long-Term Aortic Complications.Journal of clinical medicine · 2020Article
- The Development of the Ascending Aortic Wall in Tricuspid and Bicuspid Aortic Valve: A Process from Maturation to Degeneration.Journal of clinical medicine · 2020Article
- Aortic root surgery in Marfan syndrome.Indian journal of thoracic and cardiovascular surgery · 2019Review
- Seno-destructive smooth muscle cells in the ascending aorta of patients with bicuspid aortic valve disease.EBioMedicine · 2019Article
- The Potential Beneficial Effects of Resveratrol on Cardiovascular Complications in Marfan Syndrome Patients⁻Insights from Rodent-Based Animal Studies.International journal of molecular sciences · 2019Review
- Endothelial/Epithelial Mesenchymal Transition in Ascending Aortas of Patients With Bicuspid Aortic Valve.Frontiers in cardiovascular medicine · 2019Review
- Aortic Wall Elastic Properties in Case of Bicuspid Aortic Valve.Frontiers in physiology · 2019Review
Corrections and comments
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Authors and funding
11 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Patients with bicuspid aortic valve (BAV) and patients with Marfan syndrome (MFS) are more prone to develop aortic dilation and dissection compared to persons with a tricuspid aortic valve (TAV). To elucidate potential common and distinct pathways of clinical relevance, we compared the histopathological substrates of aortopathy. Ascending aortic wall biopsies were divided in five groups: BAV (n = 36) and TAV (n = 23) without and with dilation and non-dilated MFS (n = 8). General histologic features, apoptosis, the expression of markers for vascular smooth muscle cell (VSMC) maturation, markers predictive for ascending aortic dilation in BAV, and expression of fibrillin-1 were investigated. Both MFS and BAV showed an altered distribution and decreased fibrillin-1 expression in the aorta and a significantly lower level of differentiated VSMC markers. Interestingly, markers predictive for aortic dilation in BAV were not expressed in the MFS aorta. The aorta in MFS was similar to the aorta in dilated TAV with regard to the presence of medial degeneration and apoptosis, while other markers for degeneration and aging like inflammation and progerin expression were low in MFS, comparable to BAV. Both MFS and BAV aortas have immature VSMCs, while MFS and TAV patients have a similar increased rate of medial degeneration. However, the mechanism leading to apoptosis is expected to be different, being fibrillin-1 mutation induced increased angiotensin-receptor-pathway signaling in MFS and cardiovascular aging and increased progerin in TAV. Our findings could explain why angiotensin inhibition is successful in MFS and less effective in TAV and BAV patients.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.