Aortic Involvement in Pediatric Marfan Syndrome: A Review

Overview

Journal Pediatr Cardiol

Specialties Cardiology & Vascular Diseases
Pediatrics

Date 2015 Feb 12

PMID 25669767

Citations 3

Authors

Omonigho Ekhomu

Zahra J Naheed

Affiliations

Soon will be listed here.

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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