European Heart Journal Advance Access originally published online on September 23, 2005
European Heart Journal 2005 26(21):2346; doi:10.1093/eurheartj/ehi547
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Comment on pregnancy and aortic root growth in the Marfan syndrome
Klinik für Kinderkardiologie und
angeborene Herzfehler
Deutsches Herzzentrum München
Technische Universität München
Lazarettstr. 36
D-80636 München
Germany
Tel: +49 89 1218 1650
Fax: +49 89 1218 3013
E-mail address: a-hager{at}web.de
Klinik für Kinderkardiologie und
angeborene Herzfehler
Deutsches Herzzentrum München
Klinik an der Technischen
Universität München
München
Germany
Klinik für Kinderkardiologie und
angeborene Herzfehler
Deutsches Herzzentrum München
Klinik an der Technischen
Universität München
München
Germany
Meijboom and coworkers1 reported on the aortic root growth rate of women with Marfan syndrome during pregnancy. They could not find a significant increase in the aortic root diameter in 31 pregnancies of 23 patients and concluded that ‘Pregnancy in women with Marfan syndrome seems to be relatively safe up to an aortic root diameter of 45 mm’.
We believe that it is too early to draw such a conclusion. All statistical tests performed in this study were aimed to find any growth. These tests failed, but a power analysis to determine the case number necessary to find any differences was not performed. The authors even reported on one woman with an aortic dissection during pregnancy and on an increased growth of the aortic root during long-term follow-up in those patients with an aortic root diameter >40 mm at baseline in a subgroup analysis.
We recently lost one of our patients, a 36-year-old woman with aortic coarctation and bicuspid aortic valve. These patients usually have structural abnormalities in the aortic medial wall predisposing to dilatation, aneurysm, and rupture, which are similar but less pronounced than those described in Marfan syndrome.2,3 This woman died from aortic rupture at the 36th week of her second pregnancy. Her ascending aorta measured 40–41 mm and did not show any progression of diameter assessed several times by echocardiography as well as by helical CT prior to her second pregnancy. Unfortunately, the patient was not seen in our centre during pregnancy, and no consecutive imaging was performed.
Summarizing, this study did not provide real evidence for the conclusion that ‘Pregnancy in women with Marfan syndrome seems to be relatively safe up to an aortic root diameter of 45 mm’. We should recommend to monitor all pregnant women with Marfan syndrome very carefully and closely, as suggested in many previous studies,4,5 because aortic dissection does not only depend on aortic diameter progression and may also occur in Marfan patients with a normal aortic diameter.5
References
- Meijboom LJ, Vos FE, Timmermans J, Boers GH, Zwinderman AH, Mulder BJ. Pregnancy and aortic root growth in the Marfan syndrome: a prospective study. Eur Heart J 2005;26:914–920.
[Abstract/Free Full Text] - Niwa K, Perloff JK, Bhuta SM, Laks H, Drinkwater DC, Child JS, Miner PD. Structural abnormalities of great arterial walls in congenital heart disease: light and electron microscopic analyses. Circulation 2001;103:393–400.
[Abstract/Free Full Text] - Isner JM, Donaldson RF, Fulton D, Bhan I, Payne DD, Cleveland RJ. Cystic medial necrosis in coarctation of the aorta: a potential factor contributing to adverse consequences observed after percutaneous balloon angioplasty of coarctation sites. Circulation 1987;75:689–695.
[Abstract/Free Full Text] - Immer FF, Bansi AG, Immer-Bansi AS, McDougall J, Zehr KJ, Schaff HV, Carrel TP. Aortic dissection in pregnancy: analysis of risk factors and outcome. Ann Thorac Surg 2003;76:309–314.
[Abstract/Free Full Text] - Lipscomb KJ, Smith JC, Clarke B, Donnai P, Harris R. Outcome of pregnancy in women with Marfan's syndrome. Br J Obstet Gynaecol 1997;104:201–206.[Web of Science][Medline]
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