Cardiology in the Young

Original Articles

A systematic review of the pharmacological management of aortic root dilation in Marfan syndrome

Varsha Thakura1, Kathryn N. Rankina2a3, Lisa Hartlinga3a4 and Andrew S. Mackiea2a3 c1

a1 Division of Cardiology, The Hospital for Sick Children, Toronto, Canada

a2 Division of Cardiology, Stollery Children's Hospital, Edmonton, Canada

a3 Department of Pediatrics, University of Alberta, Edmonton, Canada

a4 Alberta Research Centre for Health Evidence, University of Alberta, Edmonton, Canada


Background Marfan syndrome causes aortic dilation leading to dissection and death. This systematic review examined the use of beta-blockers, angiotensin-converting enzyme inhibitors, and angiotensin II receptor blockers in the management of aortic dilation in this disease.

Methods We searched four databases – Medline, EMBASE, Web of Science, and The Cochrane Central Register of Controlled Trials – two conference proceedings, references of retrieved articles, and a web-based trial registry. The primary outcome was mortality. The secondary outcomes were aortic dissection, need for elective surgical repair, change in aortic dilation, and adverse events. Two reviewers selected studies, abstracted data, and assessed study quality. Meta-analyses were not performed because of study heterogeneity.

Results A total of 18 studies were included – 12 completed and six in progress. Of the completed studies, three before-and-after treatment, one prospective cohort, three retrospective cohorts, and two randomised control trials examined beta-blockers; one randomised and one non-randomised trial examined angiotensin-converting enzyme inhibitors; and one retrospective cohort study examined angiotensin II receptor blockers. Studies in progress are all randomised trials. Mortality was not impacted by drug therapy, although studies were underpowered with respect to this outcome. All drug classes were associated with a decrease in the rate of aortic dilation (angiotensin-converting enzyme inhibitors or angiotensin II receptor blockers >beta-blockers); none had an impact on other secondary outcomes.

Conclusions On the basis of existing evidence, beta-blockers, angiotensin-converting enzyme inhibitors, and angiotensin II receptor blockers slow the progression of aortic dilation in Marfan syndrome. Angiotensin-converting enzyme inhibitors and angiotensin II receptor blockers may have more effect than beta-blockers; however, more methodologically rigorous studies currently in progress are needed to evaluate the impact of drug therapy on clinical outcomes.

(Received April 22 2012)

(Accepted August 31 2012)

(Online publication October 19 2012)


  • Beta-blockers;
  • angiotensin-converting enzyme inhibitors;
  • angiotensin receptor blockers


c1 Correspondence to: Dr A. S. Mackie, MD, SM, Division of Cardiology, Stollery Children's Hospital, 8440-112th Street NW, Edmonton, AB, Canada T6G 2B7. Tel: +1 (780) 407 8361; Fax: +1 (780) 407 3954; E-mail: