A systematic review of the pharmacological management of aortic root dilation in Marfan syndrome.
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.
Division of Cardiology, The Hospital for Sick Children, Toronto, Canada.
Varsha
V
Thakur
Kathryn N
KN
Rankin
Lisa
L
Hartling
Andrew S
AS
Mackie
D000319:Adrenergic beta-Antagonists / Q000627:therapeutic use*; D057911:Angiotensin Receptor Antagonists / Q000627:therapeutic use*; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use*; D001014:Aortic Aneurysm / Q000188:drug therapy* / Q000209:etiology; D001018:Aortic Diseases / Q000188:drug therapy; D004108:Dilatation, Pathologic / Q000188:drug therapy; D006801:Humans; D008382:Marfan Syndrome / Q000150:complications / Q000188:drug therapy*; D016896:Treatment Outcome
D000319:Adrenergic beta-Antagonists; D057911:Angiotensin Receptor Antagonists; D000806:Angiotensin-Converting Enzyme Inhibitors