Cardiology in the Young

Review

Current insights in diagnosis and management of the cardiovascular complications of Marfan's syndrome

Gijs J. Nollena1, Maarten Groeninka1, Ernst E. van der Walla2 and Barbara J. M. Muldera1 c1

a1 Department of Cardiology, Academic Medical Center Amsterdam, Amsterdam, The Netherlands

a2 Department of Cardiology, Leiden University Medical Center, Leiden, The Netherlands

Abstract

Marfan's syndrome is an inherited disorder of connective tissue, caused by mutations in the fibrillin-1 gene located on chromosome 15. Diagnosis is still based on a combination of major and minor clinical features. Prognosis is mainly determined by the cardiovascular complications. Advances in surgical and medical treatment for these complications have dramatically improved the prognosis of the syndrome.

(Accepted March 01 2002)

Correspondence:

c1 Department of Cardiology, Room B2-240, Academic Medical Center Amsterdam (AMC), Meibergdreef 9, 1100 DD Amsterdam, The Netherlands. Tel: +31 20 5667731; Fax: +31 20 5666809; E-mail: b.j.mulder@amc.uva.nl