Congenital Cardiovascular Disease in Turner Syndrome
Identifieur interne : 006E92 ( Main/Curation ); précédent : 006E91; suivant : 006E93Congenital Cardiovascular Disease in Turner Syndrome
Auteurs : Carolyn A. Bondy [États-Unis]Source :
- Congenital Heart Disease [ 1747-079X ] ; 2008-01.
Abstract
Turner syndrome (TS), or monosomy X, occurs in ∼1/2000 live born females. Intelligence is normal and short stature is the most obvious and consistent feature of the syndrome. Congenital cardiovascular disease affects ∼50% of individuals and is the major cause of premature mortality in adults. Unfortunately, this most important aspect of the syndrome has received little attention outside of pediatric medicine, and adult cardiological follow‐up is seriously lacking. This review describes the spectrum of cardiovascular defects with particular attention to identifying risk factors for aortic dissection/rupture. X‐chromosome genetic pathways implicated in Turner cardiovascular disease, including premature coronary artery disease, are discussed. Recent guidelines for diagnosis and treatment of girls and women with TS are reviewed.
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DOI: 10.1111/j.1747-0803.2007.00163.x
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