Abstract | INTRODUCTION:
Marfan syndrome is a multisystemic connective tissue disorder caused mainly by mutations in the fibrillin-1 gene. The entire cardiovascular system is affected in patients with Marfan syndrome. Aortic root dilatation, aortic valve regurgitation or - the most feared and life-threatening symptom - aortic root dissection are the most common manifestations. Therapeutic strategies, such as prophylactic aortic root surgery and pharmacological therapy, focus on the prevention of aortic dissection. Currently, the standard medicinal treatments targeting aortic dilatation and dissection consist of agents generally used to lower blood pressure and/or the inotropic state of the heart. By these means, the cyclic repetitive forces exerted on the aortic wall are diminished and thus the onset of aortic dilatation is potentially prevented. Although these pharmacological agents may offer some benefit in reduction of aortic aneurysm expansion rate, they do not target the underlying cause of the progressive aortic degradation. AREAS COVERED: This review discusses the effectiveness of frequently prescribed medications used to prevent and delay aortic complications in Marfan syndrome. New insights on the biochemical pathways leading to aortic disease are also discussed to highlight new targets for pharmacological therapy. EXPERT OPINION:
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Authors | Alexander W Hartog, Romy Franken, Aeilko H Zwinderman, Maarten Groenink, Barbara J M Mulder |
Journal | Expert opinion on pharmacotherapy
(Expert Opin Pharmacother)
Vol. 13
Issue 5
Pg. 647-62
(Apr 2012)
ISSN: 1744-7666 [Electronic] England |
PMID | 22397493
(Publication Type: Journal Article, Review)
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Topics |
- Animals
- Aortic Diseases
(drug therapy, etiology, physiopathology)
- Humans
- Marfan Syndrome
(complications, drug therapy, physiopathology)
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