ArticleClinical research in cardiology : official journal of the German Cardiac Society2017
Aortic events in a nationwide Marfan syndrome cohort.
Article in Clinical research in cardiology : official journal of the German Cardiac Society, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 34 papers, 1 of them a synthesis that pooled it.
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Who cites it
34 citing papers in PubMed, 1 synthesis or guideline pooled it, 71 citations in OpenAlex.
- Consideration of Sex Differences in Design and Reporting of Experimental Arterial Pathology Studies-Statement From ATVB Council.Arteriosclerosis, thrombosis, and vascular biology · 2018Guideline
- Single-Cell Proteomics Reveals Novel Cell Phenotypes in Marfan Mouse Aneurysm.Molecular & cellular proteomics : MCP · 2026Article
- Occurrence of Acute Aortic Dissection Twice During the Same Pregnancy.Brazilian journal of cardiovascular surgery · 2025Article
- Management of aortic disease in children with FBN1-related Marfan syndrome.European heart journal · 2024Review
- β-Aminopropionitrile Induces Distinct Pathologies in the Ascending and Descending Thoracic Aortic Regions of Mice.Arteriosclerosis, thrombosis, and vascular biology · 2024Article
- β-aminopropionitrile Induces Distinct Pathologies in the Ascending and Descending Thoracic Aortic Regions of Mice.bioRxiv : the preprint server for biology · 2024Article
- Association of NOTCH3 With Elastic Fiber Dispersion in the Infrarenal Abdominal Aorta of Cynomolgus Monkeys.Arteriosclerosis, thrombosis, and vascular biology · 2023Article
- 17 β-Estradiol Impedes Aortic Root Dilation and Rupture in Male Marfan Mice.International journal of molecular sciences · 2023Article
- 17 β-estradiol impedes aortic root dilation and rupture in male Marfan mice.bioRxiv : the preprint server for biology · 2023Article
- The Natural History of Bicuspid Aortic Valve Disease.Structural heart : the journal of the Heart Team · 2023Review
- Sex and Gender Differences in Aortic Disease.US cardiology · 2023Review
- Impact of pathogenicJournal of medical genetics · 2023Article
- Molecular characterization and investigation of the role of genetic variation in phenotypic variability and response to treatment in a large pediatric Marfan syndrome cohort.Genetics in medicine : official journal of the American College of Medical Genetics · 2022Article
- Musculoskeletal diseases in Marfan syndrome: a nationwide registry study.Orphanet journal of rare diseases · 2022Article
- Long-term outcomes in heritable thoracic aortic disease.Frontiers in cardiovascular medicine · 2022Article
- β-Aminopropionitrile-induced aortic aneurysm and dissection in mice.JVS-vascular science · 2022Review
- Long-term outcomes after aortic root replacement for patients with Marfan syndrome.Journal of thoracic disease · 2021Article
- Non-aortic cardiovascular disease in Marfan syndrome: a nationwide epidemiological study.Clinical research in cardiology : official journal of the German Cardiac Society · 2021Article
- Acute Type A Dissection during Pregnancy with Marfan's Syndrome.The Thoracic and cardiovascular surgeon reports · 2021Article
- Recurrent infective endocarditis versus first-time infective endocarditis after heart valve surgery.Clinical research in cardiology : official journal of the German Cardiac Society · 2020Article
Corrections and comments
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Authors and funding
9 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundMarfan syndrome is associated with morbidity and mortality due to aortic dilatation and dissection. Preventive aortic root replacement has been the standard treatment in Marfan syndrome patients with aortic dilatation. In this study, we present aortic event data from a nationwide Marfan syndrome cohort.
methodThe nationwide cohort of Danish Marfan syndrome patients was established from the Danish National Patient Registry and the Cause of Death Register, where we retrieved information about aortic surgery and dissections. We associated aortic events with age, sex, and Marfan syndrome diagnosis prior or after the first aortic event.
resultsFrom the total cohort of 412 patients, 150 (36.4 %) had an aortic event. Fifty percent were event free at age 49.6. Eighty patients (53.3 %) had prophylactic surgery and seventy patients (46.7 %) a dissection. The yearly event rate was 0.02 events/year/patient in the period 1994-2014. Male patients had a significant higher risk of an aortic event at a younger age with a hazard ratio of 1.75 (CI 1.26-2.42, p = 0.001) compared with women. Fifty-three patients (12.9 %) were diagnosed with MFS after their first aortic event which primarily was aortic dissection [n = 44 (83.0 %)].
conclusionMore than a third of MFS patients experienced an aortic event and male patients had significantly more aortic events than females. More than half of the total number of dissections was in patients undiagnosed with MFS at the time of their event. This emphasizes that diagnosing MFS is lifesaving and improves mortality risk by reducing the risk of aorta dissection.
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