Clinical Features Differ Substantially Between Caucasian and Asian Populations of Marfan Syndrome

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  • Franken Romy
    Department of Cardiology, Academic Medical Center Interuniversity Cardiology Institute of the Netherlands
  • den Hartog Alexander W.
    Department of Cardiology, Academic Medical Center Interuniversity Cardiology Institute of the Netherlands
  • van de Riet Liz
    Department of Cardiology, Academic Medical Center
  • Timmermans Janneke
    Department of Cardiology, Radboud University Nijmegen Medical Center
  • Scholte Arthur J.
    Department of Cardiology, Leiden University Medical Center
  • van den Berg Maarten P.
    Department of Cardiology, University Medical Center Groningen
  • de Waard Vivian
    Department of Medical Biochemistry, Academic Medical Center
  • Zwinderman Aeilko H.
    Department of Clinical Epidemiology and Biostatistics, Academic Medical Center
  • Groenink Maarten
    Department of Cardiology, Academic Medical Center Department of Radiology, Academic Medical Center Interuniversity Cardiology Institute of the Netherlands
  • Yip James W.
    Department of Cardiology, National University Hospital
  • Mulder Barbara J.M.
    Department of Cardiology, Academic Medical Center Interuniversity Cardiology Institute of the Netherlands

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Background: Prevention of aortic dissection and sudden death in patients with Marfan syndrome (MFS) requires accurate diagnosis. MFS is diagnosed by the Ghent criteria, which are primarily based on clinical features of Caucasian MFS populations. We determined whether the Ghent criteria apply to Asian MFS populations. Methods and Results: In this multicenter study, we included 255 adult MFS patients according to the Ghent criteria of 2010. Patients were excluded if they were neither Caucasian nor Asian. The Asian MFS population (n=49) had a smaller body surface area (BSA: 1.8m² vs. 2.0m², P<0.001), a more severely affected aortic root (absolute aortic diameter: 42.9mm vs. 43.3mm, P=0.802; corrected for BSA: 24.9mm vs. 21.7mm, P<0.001; Z-score: 4.5 vs. 3.6, P=0.013), and more often a positive systemic score (75.5% vs. 60.0%, P=0.045), but less frequently ectopia lentis (24.5% vs. 48.1%, P=0.004) compared with the Caucasian population (n=206). Conclusions: The Ghent criteria do not necessarily apply to Asian MFS populations, resulting in a more severely affected cardiovascular system. This may be related to under diagnosis of MFS by multiple factors, including the use of Z-score, and genetic and racial differences. The Ghent criteria should be adapted for Asian populations in order to accurately diagnose MFS.  (Circ J 2013; 77: 2793–2798)<br>

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  • Circulation Journal

    Circulation Journal 77 (11), 2793-2798, 2013

    一般社団法人 日本循環器学会

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