Pediatric Cardiology

, Volume 36, Issue 5, pp 887–895 | Cite as

Aortic Involvement in Pediatric Marfan syndrome: A Review

Review Article
First Online:
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Accepted:

Abstract

Outlining specific protocols for the management of pediatric patients with Marfan syndrome has been challenging. This is mostly due to a dearth of clinical studies performed in pediatric patients. In Marfan syndrome, the major sources of morbidity and mortality relate to the cardiovascular system. In this review, we focus on aortic involvement seen in pediatric patients with Marfan syndrome, ranging from aortic dilatation to aortic rupture and heart failure. We discuss the histological, morphological, and pathogenetic basis of the cardiac manifestations seen in pediatric Marfan syndrome and use a specific case to depict our experienced range of cardiovascular manifestations. The survival for patients with Marfan syndrome may approach the expected survival for non-affected patients, with optimal management. With this potentiality in mind, we explore possible and actual management considerations for pediatric Marfan syndrome, examining both medical and surgical therapy modalities that can make the possibility of improved survival a reality.

Keywords

Marfan syndrome Pediatric Marfan syndrome Transforming growth factor-β Aortic root dilation Valve-sparing aortic root repair Bentall procedure 
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Copyright information

© Springer Science+Business Media New York 2015

Authors and Affiliations

  1. 1.Department of PediatricsJohn H. Stroger HospitalChicagoUSA
  2. 2.Department of Pediatric CardiologyJohn H. Stroger HospitalChicagoUSA

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