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Rossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics)

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Severe cardiovascular system involvement in a patient with Marfan’s syndrome

Abstract

The given clinical case of a 14-year-old child with Marfan’s syndrome demonstrates the early severe cardiovascular system diseases, such as dilated cardiomyopathy and aortic root dilatation, that required surgical treatment when he was 9 years old, and grades 2 and 3 insufficiency of the mitral and aortic valves, respectively. The paper presents the modern view of the involvement of the heart and vessels in Marfan’s syndrome (cardiomyopathy as a new sign of the disease, arterial dissection). It shows new possibilities for treating cardiovascular disorders in children with this disease, such as pathogenetic therapy with losartan, anti-transforming growth factor-β antibodies. 

About the Authors

O. S. Groznova
Research Clinical Institute of Pediatrics, N.I. Pirogov Russian National Research Medical University, Moscow
Russian Federation


E. P. Kalachanova
Research Clinical Institute of Pediatrics, N.I. Pirogov Russian National Research Medical University, Moscow
Russian Federation


I. V. Leontyeva
Research Clinical Institute of Pediatrics, N.I. Pirogov Russian National Research Medical University, Moscow
Russian Federation


P. E. Rzhevskaya
Research Clinical Institute of Pediatrics, N.I. Pirogov Russian National Research Medical University, Moscow
Russian Federation


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Review

For citations:


Groznova O.S., Kalachanova E.P., Leontyeva I.V., Rzhevskaya P.E. Severe cardiovascular system involvement in a patient with Marfan’s syndrome. Rossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics). 2015;60(2):95-99.

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ISSN 1027-4065 (Print)
ISSN 2500-2228 (Online)