Turner syndrome Flashcards
phenotype of Turner’s syndrome
short stature and primary amenorrhea
characteristics of variable penetrance in Turner’s
skeletal, renal and cardiovascular malformations
prevalence of Turners
1 in 2,000
1 in 40 die by _________ by age 86
aortic dissection
risk of death in perinatal period for aortic dissection or rupture in Turners
2%
risk of mosaic vs pure Turners in pregnancy outcomes
no difference
risk factors for aortic dissection during pregnancy in Turners
aortic root dilation, bicuspid aortic valve, coarctation of the aorta and hypertension
imaging modalities for aortic root diameter measurement
MRI or transesophageal echocardiography
aortic size measured by MRI should be adjusted for
body surface area
An ASI greater than ________ identifies patients at risk for dissection
> 2.0cm/m2
percentage of women with ASI > 2.5cm/m2 experienced dissection
33%
percentage of dissections that occur in the third trimester or postpartum
50%
recommendations for surveillance of pregnancy
1) treatment of hypertension
2) periodic echo or MRI
3) consultation with cardiologist
delivery plan for women with ASI < 2cm/m2
may attempt vaginal delivery under epidural anesthesia
delivery plan for women with ASI > 2cm/m2
election cesarean before onset of labor
