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Marfan's Disease

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Marfan's syndrome is an autosomal dominant inherited abnormality which has been localized to a mutation in Chromosome 15. Cardiovascular features include mitral valve prolapse, mitral regurgitation, aortic insufficiency, and aortic root dilation which can progress or be complicated by dissection. The mean age for cardio- vascular events is 32 years.

Small retrospective studies suggest an increased risk of aortic dissection in women with Marfan's syndrome who become pregnant. In women with preconception aortic dilation, greater risk is due to the changes of cardiac output and plasma volume that occur by the 6th week of pregnancy and the increased stress during labor. Vaginal delivery is acceptable in patients with Marfan's and epidural anesthesia may be helpful in lowering the blood pressure at delivery. However if the aortic root is greater than 4 cm a cesarean section should be considered.


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