The Immediate and Long-Term Impact of Pregnancy on Aortic Growth Rate and Mortality in Women With Marfan Syndrome

• Published in: Journal of the American College of Cardiology Vol. 60; no. 3; pp. 224 - 229
• Main Authors: Donnelly, Ryan T., MD, Pinto, Nelangi M., MD, Kocolas, Irene, MD, Yetman, Anji T., MD
• Format: Journal Article
• Language: English
• Published: New York, NY Elsevier Inc 17.07.2012; Elsevier; Elsevier Limited
• Subjects: Adolescent; Adult; Age; aorta; Aorta - physiopathology; Biological and medical sciences; Blood pressure; Cardiology; Cardiology. Vascular system; Cardiovascular; Clinical outcomes; Confidence intervals; Dissection; Echocardiography; Family medical history; Female; Females; Gender; Growth rate; Humans; Internal Medicine; Marfan syndrome; Marfan Syndrome - diagnostic imaging; Marfan Syndrome - mortality; Marfan Syndrome - physiopathology; Medical sciences; Mortality; Mutation; Patients; Postpartum period; Pregnancy; Pregnancy Complications, Cardiovascular - diagnostic imaging; Pregnancy Complications, Cardiovascular - physiopathology; Prospective Studies; Retrospective Studies; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; Studies; Surgery; Ultrasonic imaging; Utah - epidemiology; Womens health; Young Adult; aorta; MFS; Marfan syndrome; pregnancy; Human; Connective tissue disease; Skin disease; Prognosis; Elastic tissue disease; Growth rate; Mortality; Long term; Genetic disease; Term pregnancy; Systemic disease; Aorta; Female; Circulatory system; Cardiology; Woman
• ISSN: 0735-1097; 1558-3597
• DOI: 10.1016/j.jacc.2012.03.051

Objectives The study sought to assess the impact of pregnancy on the rate of aortic growth as well as on short- and long-term clinical outcomes in women with Marfan syndrome. Background There is a paucity of data on peripartum and long-term clinical outcomes in women with Marfan syndrome who are followed prospectively during pregnancy. Methods Echocardiographic, demographic, and surgical data review of all adult females with a confirmed diagnosis of Marfan syndrome was performed. Results Of the 98 women identified, 69 (72%) experienced a total of 199 pregnancies resulting in 170 (86%) live births. The median number of pregnancies per women was 3 (interquartile range: 1 to 12). Obstetrical complications occurred in 17 (10%) and adverse fetal outcomes in 22 (13%). No woman experienced aortic dissection or required cardiac surgery during pregnancy. Aortic growth rate increased during pregnancy and did not return to baseline following pregnancy completion. Despite the lack of catastrophic peripartum complications, the prevalence of both aortic dissection and elective aortic surgery during long-term follow-up was higher in those women who had a prior pregnancy. Risk factors for adverse cardiac outcome included greater aortic diameter, greater rate of aortic growth during pregnancy, increased number of pregnancies, lack of beta-blocker use during pregnancy, and lack of prospective pregnancy follow-up. Conclusions There is a low incidence of aortic complications during pregnancy in women with Marfan syndrome and an aortic diameter <4.5 cm. However, pregnancy does increase the risk of aortic complications in the long-term in this group of patients.