ArticleNetherlands heart journal : monthly journal of the Netherlands Society of Cardiology and the Netherlands Heart Foundation2015
The revised role of TGF-β in aortic aneurysms in Marfan syndrome.
Article in Netherlands heart journal : monthly journal of the Netherlands Society of Cardiology and the Netherlands Heart Foundation, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 22 papers.
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Who cites it
22 citing papers in PubMed, 45 citations in OpenAlex.
- A diagnostic RNA sequencing assay for direct identification and interpretation of pathogenic variants in theFrontiers in molecular biosciences · 2025Article
- TGFβ level in healthy and children with Marfan syndrome-effective reduction under sartan therapy.Frontiers in pediatrics · 2024Article
- Article
- Can 10 000 Healthy Steps a Day Slow Aortic Root Dilation in Pediatric Patients With Marfan Syndrome?Journal of the American Heart Association · 2022Article
- Mechanism of Disease: Recessive ADAMTSL4 Mutations and Craniosynostosis with Ectopia Lentis.Case reports in genetics · 2022Article
- Association of TGF-β Canonical Signaling-Related Core Genes With Aortic Aneurysms and Aortic Dissections.Frontiers in pharmacology · 2022Review
- Transforming Growth Factor-β and the Renin-Angiotensin System in Syndromic Thoracic Aortic Aneurysms: Implications for Treatment.Cardiovascular drugs and therapy · 2021Review
- Blood pressure-independent inhibition of Marfan aortic root widening by the angiotensin II receptor blocker valsartan.Physiological reports · 2021Article
- The Molecular Genetics of Marfan Syndrome.International journal of medical sciences · 2021Review
- Localized Antileptin Therapy Prevents Aortic Root Dilatation and Preserves Left Ventricular Systolic Function in a Murine Model of Marfan Syndrome.Journal of the American Heart Association · 2020Article
- Fibrillin microfibrils and elastic fibre proteins: Functional interactions and extracellular regulation of growth factors.Seminars in cell & developmental biology · 2019Review
- Pharmacologic Management of Aneurysms.Circulation research · 2019Review
- Angiotensin, transforming growth factor β and aortic dilatation in Marfan syndrome: Of mice and humans.International journal of cardiology. Heart & vasculature · 2018Article
- Aortopathy in a Mouse Model of Marfan Syndrome Is Not Mediated by Altered Transforming Growth Factor β Signaling.Journal of the American Heart Association · 2017Article
- Pathogenic Mechanisms of Bicuspid Aortic Valve Aortopathy.Frontiers in physiology · 2017Review
- Vascular smooth muscle cells in Marfan syndrome aneurysm: the broken bricks in the aortic wall.Cellular and molecular life sciences : CMLS · 2017Review
- NVVC/NHJ Durrer prizes 2015.Netherlands heart journal : monthly journal of the Netherlands Society of Cardiology and the Netherlands Heart Foundation · 2016Article
- Marfan syndrome: current perspectives.The application of clinical genetics · 2016Review
- Coupling between MRI-assessed regional aortic pulse wave velocity and diameters in patients with thoracic aortic aneurysm: a feasibility study.Netherlands heart journal : monthly journal of the Netherlands Society of Cardiology and the Netherlands Heart Foundation · 2015Article
- Perspectives on the revised Ghent criteria for the diagnosis of Marfan syndrome.The application of clinical genetics · 2015Review
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Authors and funding
11 authors at 1 institution in 1 country.
Funding
Abstract
backgroundRecently, we demonstrated that losartan reduced the aortic root dilatation rate (AoDR) in adults with Marfan syndrome (MFS); however, responsiveness was diverse. The aim was to determine the role of transforming growth factor-β (TGF-β) as therapeutic biomarker for effectiveness of losartan on AoDR.
methodsBaseline plasma TGF-β levels of 22 healthy controls and 99 MFS patients, and TGF-β levels after 1 month of losartan treatment in 42 MFS patients were measured. AoDR was assessed by magnetic resonance imaging at baseline and after 3 years of follow-up.
resultsPatients with MFS had higher TGF-β levels compared with healthy controls (121 pg/ml versus 54 pg/mL, p = 0.006). After 1 month of therapy, losartan normalised the TGF-β level in 15 patients (36%); the other 27 patients (64%) showed a significant increase of TGF-β. After 3 years of losartan therapy, patients with a decrease in TGF-β had significantly higher AoDR compared with patients with increased TGF-β (1.5 mm/3 years versus 0.5 mm/3 years, p = 0.04). Patients showing a decrease in TGF-β after losartan therapy had significantly elevated baseline TGF-β levels compared with patients with increased TGF-β (189 pg/ml versus 94 pg/ml, p = 0.05).
conclusionPatients responding to losartan therapy with a reduction of the plasma TGF-β level had higher baseline TGF-β levels and a higher AoDR. Most likely, TGF-β levels may be considered to be a readout of the disease state of the aorta. We propose that increased angiotensin II is the initiator of aorta dilatation and is responsible for increased TGF-β levels in MFS. The concept of TGF-β as initiator of aortic dilatation in MFS patients should be nuanced.
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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.