Systemic connective tissue involvement and cardiac involvement: the 2010 revised Gent nosology in the Marfan syndrome diagnostics
https://doi.org/10.15829/1560-4071-2013-1-7-13
Abstract
The author presents his view on the problem of Marfan syndrome-related hereditary connective tissue disorders, in particular, primary mitral valve prolapse and Marfanoid habitus, as the most prevalent manifestations. The changes in the assessment of external and visceral manifestations of Marfan syndrome, according to the originalGentnosology and its 2010 revision, are discussed. The importance of the evaluation of systemic connective tissue involvement, with the systemic score calculation and cardiac extracellular matrix assessment, is emphasised. The author presents the evidence justifying the need for a wider use of the cardiac involvement concept, in order to assess the severity of structural and functional disturbances of cardiac extracellular matrix in patients with primary mitral valve prolapse and Marfanoid habitus. The cardiac involvement assessment should be based on the systemic score, minor cardiac criteria, the number and type of minor cardiac abnormalities, the results of clinical examination, and the serum levels of transforming growth factors (TGF)
About the Author
E. V. Zemtsovsky
V. A. Almazov Federal Centre of Heart, Blood, and Endocrinology; St. Petersburg State Pediatric Medical University
Russian Federation
Russian Federation
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Review
For citations:
Zemtsovsky E.V. Systemic connective tissue involvement and cardiac involvement: the 2010 revised Gent nosology in the Marfan syndrome diagnostics. Russian Journal of Cardiology. 2013;(1):7-13. (In Russ.) https://doi.org/10.15829/1560-4071-2013-1-7-13
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