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Cardiovascular disease in Turner syndrome, cardiovascular risks associated with pregnancy


Authors: E. Klásková;  J. Zapletalová;  A. Sobek;  D. Horák;  J. Wiedermann;  A. Sobek jr.
Authors‘ workplace: Dětská klinika FN a LF UP, Olomouc 1;  FERTIMED, Centrum pro léčbu neplodnosti, Olomouc 2;  Radiologická klinika FN a LF UP, Olomouc 3
Published in: Prakt Gyn 2010; 14(3): 142-146
Category: Review Article

Overview

Turner syndrome (TS), or monosomy X, is characterised by short stature, premature ovarian failure and congenital cardiovascular defects in phenotypic females. Congenital cardiovascular disease affects approximately 50% of individuals and is the major cause of premature mortality in adults with TS. Dissection of the aorta occurs in TS patients at a remarkably young age (mean 36 years). Aortic dissection is usually associated with additional risk factors including bicuspid aortic valve or other abnormalities of the aortic valve, coarctation or dilatation of the aorta, and systemic arterial hypertension. In recent years, increasing numbers of women with TS have become pregnant via assisted reproduction. The risk for aortic dissection or rupture during pregnancy is 2% or higher and the risk of death is increased as much as 100-fold. Because of this fact, the Practice Committee of the American Society for Reproductive Medicine recommended in 2006 a careful screening for cardiovascular abnormalities including echocardiography and cardiovascular magnetic resonance imaging in all TS patients before any planned attempt at pregnancy. Women with any risk factors for aortic dissection should not undertake pregnancy.

Key words:
Turner syndrome – congenital heart disease – aortic dissection – systemic arterial hypertension – pregnancy


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Labels
Paediatric gynaecology Gynaecology and obstetrics Reproduction medicine
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