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European Heart Journal Advance Access originally published online on September 23, 2005
European Heart Journal 2005 26(21):2346-2347; doi:10.1093/eurheartj/ehi548
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© The European Society of Cardiology 2005. All rights reserved. For Permissions, please e-mail: journals.permissions@oxfordjournals.org

Comment on pregnancy and aortic root growth in the Marfan syndrome: reply

Barbara J.M. Mulder

Department of Cardiology
Rm B2-240
Academic Medical Center
Meibergdreef 9
1105 AZ Amsterdam
The Netherlands
Tel: +31 20 5662193
Fax: +31 20 5666809
E-mail address: b.j.mulder{at}amc.uva.nl

Lilian J. Meijboom

Department of Cardiology
Rm B2-240
Academic Medical Center
Meibergdreef 9
1105 AZ Amsterdam
The Netherlands

Thank you for the opportunity to respond to the comment from Hager and co-workers. It is an immense tragedy when a young woman dies during pregnancy. We agree with Hager and co-workers that there is no definite safe aortic root diameter for women with Marfan syndrome to get pregnant. Dissections may occur at normal aortic diameters in patients with Marfan syndrome. Should we therefore advise all women with Marfan syndrome against pregnancy? During recent years, a panel of international experts has reached consensus that pregnancy can be tolerated in women with Marfan syndrome with a slightly dilated aortic root.1 This expert consensus is being validated by our findings, which indicate that pregnancy is relatively safe in women with Marfan syndrome and an aortic root diameter up to 45 mm.2 However, women with a previous dissection should not get pregnant. We agree with Hager et al. that all patients with Marfan syndrome deserve close and careful monitoring before, during, and after pregnancy. Before pregnancy, all women should undergo a magnetic resonance angiogram to investigate if there is dilatation in other parts of the aorta. Also, frequent echocardiographic imaging should be performed throughout pregnancy and the postpartum period to check for progressive aortic dilatation. In the future other risk factors for aortic dissection, such as aortic elasticity, might become available to identify high-risk patients for aortic dissection during pregnancy.3

References

  1. Therrien J, Gatzoulis M, Graham T, Bink-Boelkens M, Connelly M, Niwa K, Mulder B, Pyeritz R, Perloff J, Somerville J, Webb GD. Canadian Cardiovascular Society Consensus Conference 2001 update: Recommendations for the Management of Adults with Congenital Heart Disease—Part II. Can J Cardiol 2001;17(10):1029–1050.[Web of Science][Medline]
  2. Meijboom LJ, Vos FE, Timmermans J, Boers GH, Zwinderman AH, Mulder BJM. Pregnancy and aortic root growth in the Marfan syndrome: a prospective study. Eur Heart J 2005;26:914–920.[Abstract/Free Full Text]
  3. Nollen GJ, Groenink M, Tijssen JGP, Van der Wall EE, Mulder BJM. Aortic stiffness and diameter predict progressive aortic dilatation in patients with Marfan syndrome. Eur Heart J 2004;25:1146–1152.[Abstract/Free Full Text]

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This Article
Right arrow FREE Full Text (PDF) Freely available
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26/21/2346-a    most recent
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