This Article Full Text Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Request Permissions Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Goland, S. Articles by Elkayam, U. Search for Related Content PubMed PubMed Citation Articles by Goland, S. Articles by Elkayam, U. Related Collections Valvular heart disease Echocardiography Aneurysm, AVM, hematoma Genetics of cardiovascular disease

(Circulation. 2009;119:619-623.)
© 2009 American Heart Association, Inc.

Clinician Update

Cardiovascular Problems in Pregnant Women With Marfan Syndrome

Sorel Goland, MD; Uri Elkayam, MD

From the Department of Medicine and Obstetrics and Gynecology, University of Southern California Keck School of Medicine, Los Angeles (U.E.), and Kaplan Medical Center, Rehovot, Israel (S.G.).

Correspondence to Uri Elkayam, MD, Department of Medicine and Obstetrics and Gynecology, LAC+USC Medical Center, 1200 N State St, Los Angeles, CA 90033. E-mail Elkayam@usc.edu

An extract of the first 250 words of the full text is provided, because this article has no abstract.

	Introduction

 
Case presentation: A 34-year-old Korean woman with Marfan syndrome (MFS) was referred for cardiac evaluation in the 24th week of her first pregnancy. Although she was diagnosed with MFS at the age of 7 years, the patient did not have cardiac evaluations before her pregnancy. An echocardiogram demonstrated mildly dilated left ventricle with normal systolic function, moderately dilated left atrium, severe dilation of the aortic root with maximum diameter of 64 mm, and moderate aortic regurgitation. Magnetic resonance imaging showed a 62x54-mm aortic root aneurysm with no evidence of aortic dissection. The patient was started on metoprolol 50 mg BID, which was increased to 100 mg BID, and she was advised to have an elective surgical aortic repair. She decided to delay her surgery to allow fetal maturity. The patient was hospitalized for 2 weeks for close monitoring and underwent a successful cesarean section at 28 weeks’ gestation followed by a successful Bentall procedure to repair her aortic aneurysm and replace her aortic valve.

	Diagnosis of MFS

 
The diagnosis is based on the Ghent criteria (Table) and relies on history, physical examination, and a molecular genetic test.¹ MFS often remains undiagnosed before pregnancy and is recognized only after the development of complications. Because pregnancy-associated complications may be life threatening, health providers caring for women at childbearing ages should be familiar with the diagnostic criteria of MFS. The syndrome involves multiple organ systems, and the diagnosis is at times challenging² and requires a multidisciplinary approach by specialists in cardiology, ophthalmology, . . . [Full Text of this Article]