Medical management of aortic disease in children with Marfan syndrome.

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.