Cardiovascular disease in Turner syndrome
Authors:
E. Klásková 1; Z. Tudos 2; J. Wiedermann 1; M. Šnajderová 3; J. Zapletalová 1
Authors place of work:
Dětská klinika, FN a LF UP, Olomouc, přednosta prof. MUDr. V. Mihál, CSc.
1; Radiologická klinika, FN a LF UP, Olomouc, přednosta prof. MUDr. M. Heřman, Ph. D.
2; Pediatrická klinika UK 2. LF a FN Motol, Praha, přednosta prof. MUDr. J. Lebl, CSc.
3
Published in the journal:
Čes-slov Pediat 2012; 67 (2): 103-111.
Category:
Review
Summary
Turner syndrome (TS) is relatively common disorder with typical features of short stature, gonadal dysgenesis and congenital defects of cardiovascular or uropoetic system.
Congenital cardiovascular disease affects approximately 50% of TS individuals and is the major cause of premature mortality due to the aortic dissection. Risk of acute aortic dissection is increased by more than 100-fold in young and middle-aged women with Turner syndrome when compare with the general population. Aortic dissection is usually associated with additional risk factors including bicuspid aortic valve or other abnormalities of the aortic valve, coarctation of aorta or dilatation of the ascending 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. Because of this fact, the Practice Committee of the American Society for Reproductive Medicine recommended in 2005 a careful screening for cardiovascular abnormalities in all TS patients before any planned attempt at pregnancy. Women with any risk factors for aortic dissection should not undertake pregnancy.
The review describes the spectrum of cardiovascular defects with particular attention to identifying risk factor for aortic dissection. Authors suggest that all individuals with Turner syndrome require continued cardiovascular monitoring throughout the lifespan including new methods such as magnetic resonance imaging of the heart and great vessels.
Key words:
Turner syndrome, congenital heart defects, aortic coarctation, bicuspid aortic valve, ascending aorta dilatation, aortic size index, aortic dissection, arterial hypertension, pregnancy
Zdroje
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Štítky
Neonatology Paediatrics General practitioner for children and adolescentsČlánok vyšiel v časopise
Czech-Slovak Pediatrics
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