Outcome of pregnancy in women with Marfan's syndrome

Abstract

Objective To improve life expectancy and prevent premature mortality in women with Marfan's syndrome. Methods During the development of a regional genetic register for Marfan's Syndrome the outcome of 91 pregnancies in 36 women with this condition was established retrospectively and the cardiovascular and obstetric complications documented. Results No patient had a significant cardiovascular abnormality limiting function before her pregnancy. Of 36 women, four had an aortic dissection relating to pregnancy and two others required aortic surgery following delivery. Thirty women had uncomplicated gestational histories. The incidence of obstetric complications did not exceed expectation. Conclusions Women with Marfan's syndrome are at significant risk of aortic dissection in pregnancy even in the absence of preconceptional cardiovascular abnormality. Aortic root dilatation may be a predictor of risk but dissection may occur without significant dilatation. Guidelines for obstetric care are suggested and preconceptional assessment recommended.

Risk of Development of Abdominal Aortic Aneurysm and Dissection of Thoracic Aorta in a Postpartum Womanwith Marfans Syndrome

“…Therefore, pregnancy in Marfan's syndrome increases the risk of aortic root dilatation and aortic dissection and seriously threatens the life of the patient (3)(4)(5)(6)(7). We report the case of a young pregnant woman with Marfan's syndrome and mild dilatation of the aortic root, who developed postpartum abdominal aortic aneurysm followed by thoracic aortic dissection.…”

“…Pregnancy is known to be a crucial risk factor as it imposes substantial cardiovascular stress (4)(5)(6)(7) and accelerates the development of pathologic changes in the arterial wall (6), leading to the possibility of dilatation, dissection, and rupture of the aorta with progression of pregnancy (4)(5)(6)(7). Dissection and rupture of the aorta carry a high risk of maternal and fetal mortality (6); hence, women with Marfan's syndrome with dilatation of the aortic root…”

“…Pregnancy imposes substantial cardiovascular stresses such as pressure and volume overload (3)(4)(5)(6)(7). Therefore, pregnancy in Marfan's syndrome increases the risk of aortic root dilatation and aortic dissection and seriously threatens the life of the patient (3)(4)(5)(6)(7).…”

“…Prevalence of this disease is estimated to range from 1 in 10,000 to 1 in 20,000 (1)(2)(3)(4). The condition is characterized by musculoskeletal, cardiovascular, and ocular manifestations (1,2,4,5). Cardiovascular disorders in this condition include mitral valve prolapse, mitral regurgitation, and aortic root dilatation with aortic valve incompetence (1,2,5).…”

“…Cardiovascular disorders in this condition include mitral valve prolapse, mitral regurgitation, and aortic root dilatation with aortic valve incompetence (1,2,5). Aortic root dilatation and Stanford type A aortic dissection are the major causes of morbidity and mortality (5,6).…”