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

Study Questions:

What is the rate of aortic growth and associated clinical outcomes during and after pregnancy among women with Marfan syndrome?

Methods:

All patients were seen in a tertiary cardiology aortopathy clinic. Patients were included if they fulfilled Ghent criteria for a diagnosis of Marfan syndrome either with or without confirmatory genetic testing, were a female 18 years or older, had attained final height, and had had a clinic visit within the past 18 months. Subjects were grouped as having had a previously documented pregnancy (yes, no). Women followed prospectively had a confirmed diagnosis of Marfan syndrome prior to pregnancy, underwent echocardiography at a minimum of once within the 12 months prior to pregnancy, at 12-16 weeks’ gestation, 24-26 weeks’ gestation, 35-39 weeks’ gestation, 4-16 weeks postpartum, and then annually per clinical protocol. Nulliparous females and females not receiving prospective cardiac care during pregnancy had a minimum of annual echocardiograms from the time of diagnosis until last follow-up. The primary outcomes were a composite adverse outcome (defined as death, aortic dissection, severe symptomatic aortic insufficiency, or need for urgent surgery), and the need for elective aortic surgery.

Results:

A total of 98 women were included, of which 69 experienced 199 pregnancies (170 were live births). The median number of pregnancies per women was 3 (interquartile range: 1-12). 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. 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 compared to women with no pregnancies. Risk factors for adverse cardiac outcomes 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:

The investigators concluded that there is a low incidence of aortic complications during pregnancy among women with Marfan syndrome and an aortic diameter <4.5 cm. However, pregnancy does increase the long-term risk of aortic complications.

Perspective:

These data help clinicians discuss the short- and long-term implications of pregnancy with women who carry a diagnosis of Marfan syndrome. Continued follow-up and collaboration with other centers caring for such patients will assist in the management of Marfan syndrome in women.

Clinical Topics: Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Invasive Cardiovascular Angiography and Intervention, Noninvasive Imaging, Cardiac Surgery and CHD & Pediatrics, Congenital Heart Disease, CHD & Pediatrics and Imaging, CHD & Pediatrics and Interventions, CHD & Pediatrics and Quality Improvement, Interventions and Imaging, Interventions and Structural Heart Disease, Echocardiography/Ultrasound

Keywords: Follow-Up Studies, Postpartum Period, Live Birth, Risk Factors, Genetic Testing, Prevalence, Cardiovascular Diseases, Marfan Syndrome, Gravidity, Cardiac Surgical Procedures, Pregnancy Complications, Pregnancy, Echocardiography


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