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Aortic Involvement in Pediatric Marfan syndrome: A Review

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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.

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Authors and Affiliations

  1. Department of Pediatrics, John H. Stroger Hospital, 1901 W. Harrison Street, Chicago, IL, USA

    Omonigho Ekhomu

  2. Department of Pediatric Cardiology, John H. Stroger Hospital, 1901 W. Harrison Street, Room 2864, Chicago, IL, 60612, USA

    Zahra J. Naheed

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  1. Omonigho Ekhomu
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  2. Zahra J. Naheed
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Correspondence to Zahra J. Naheed.

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Ekhomu, O., Naheed, Z.J. Aortic Involvement in Pediatric Marfan syndrome: A Review. Pediatr Cardiol 36, 887–895 (2015). https://doi.org/10.1007/s00246-015-1101-0

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  • DOI: https://doi.org/10.1007/s00246-015-1101-0

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