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Medical management of aortic disease in children with Marfan syndrome

Lindsay, Mark E.a,b,c

doi: 10.1097/MOP.0000000000000671
CARDIOVASCULAR MEDICINE: Edited by Daniel Bernstein

Purpose of review Marfan syndrome (MFS) is the most common cause of aortic aneurysm in children and is associated with premature mortality. Whereas surgical therapy remains the primary treatment to prevent aortic complications, medical therapy may slow or prevent aortic growth. Pediatricians and pediatric cardiologists should become familiar with the diagnosis and management of MFS. This review focuses on the medical management for the most important life-threatening manifestation of MFS, thoracic aortic disease.

Recent findings Several large-scale clinical trials and one meta-analysis have been reported comparing angiotensin receptors blockers with the standard therapy, beta blockers.

Summary From the assembled evidence described in this review, beta blockers and angiotensin receptors blockers show clinical equivalence and either class of agent may be an appropriate choice for the medical management of aortic growth in children with MFS.

aMassachusetts General Hospital Thoracic Aortic Center

bDivision of Cardiology

cPediatric Cardiology, Departments of Medicine and Pediatrics, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, USA

Correspondence to Mark E. Lindsay, MD, PhD, Richard B. Simches Research Building, Room 3224, 185 Cambridge St., Boston, MA 02114, USA. Tel: +1 617 643 3458; fax: +1 617 643 3451; e-mail:

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