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Extrathoracic Aneurysms in Marfan Syndrome: A Systematic Review of the Literature

  • Lucía Requejo-García
    Affiliations
    Angiology and Vascular Surgery Department, Hospital Universitario de La Ribera, Alzira, Valencia, Spain
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  • Rafael Martínez-López
    Affiliations
    Angiology and Vascular Surgery Department, Hospital Universitario y Politécnico La Fe, Valencia, Spain
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  • Emma Plana-Andani
    Affiliations
    Angiology and Vascular Surgery Department, Hospital Universitario y Politécnico La Fe, Valencia, Spain

    Research Group on hemostasis, Thrombosis, Arteriosclerosis and Vascular Biology, Instituto de Investigación Sanitara-Hospital La Fe, Valencia, Spain
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  • Pilar Medina-Badenes
    Affiliations
    Research Group on hemostasis, Thrombosis, Arteriosclerosis and Vascular Biology, Instituto de Investigación Sanitara-Hospital La Fe, Valencia, Spain
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  • Amparo Hernándiz-Martínez
    Affiliations
    Research Group on Regeneration and Heart Transplantation, Instituto de Investigación Sanitara-Hospital La Fe, Valencia, Spain
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  • Alvaro Torres-Blanco
    Affiliations
    Angiology and Vascular Surgery Department, Hospital Universitario y Politécnico La Fe, Valencia, Spain
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  • Manuel Miralles-Hernández
    Correspondence
    Correspondence to: Manuel Miralles-Hernández, Angiology and Vascular Surgery Department, Hospital Universitari i Politècnic La Fe, Avda Fernando Abril Martorell 106, 46026 Valencia, Spain
    Affiliations
    Angiology and Vascular Surgery Department, Hospital Universitario y Politécnico La Fe, Valencia, Spain

    Research Group on hemostasis, Thrombosis, Arteriosclerosis and Vascular Biology, Instituto de Investigación Sanitara-Hospital La Fe, Valencia, Spain

    Surgery Department, Faculty of Medicine, University of Valencia (UV), Valencia, Spain
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Published:August 24, 2022DOI:https://doi.org/10.1016/j.avsg.2022.08.005

      Background

      Marfan syndrome (MS) most often shows as thoracic aortic aneurysm (TAA) or aortic dissection, but it may also involve other vascular territories. This study aimed to identify those extrathoracic vascular manifestations most frequently associated with MS.

      Methods

      A systematic review of the literature with Preferred Reporting Items for Systematic Reviews and Meta-Analyses criteria was carried out. The following databases were included: MEDLINE, Embase, Web of Science, Cumulative Index of Nursing and Health Sciences Literature (CINHAL); Spanish database MEDESY Cochrane Central Register of Controlled Trials (CENTRAL).

      Results

      A total of 10,008 articles were identified, leaving 155 for the first stage of data analysis (total incidence of aneurysms) and 83 for the second (descriptive data analysis). Overall, 518 aneurysms were identified: 149 in the head and neck, 94 in the extremities, and 275 in the aortic, iliac, and visceral sectors. Mostly, they were simultaneously discovered during studies of the TAA. In the abdominal aorta, the presentation with rupture in 11 of 32 patients stands out. Resection and bypass were the most frequently used methods for repair in the treated cases.

      Conclusions

      Although its frequency in the general population is unknown, this systematic review suggests that extrathoracic aneurysmal arterial involvement in the MS may be more frequent than expected. We believe screening for aneurysms in other vascular sectors may be advisable, especially in patients with MS and TAA.
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