MARFAN SYNDROME AND PREGNANCY
DOI:
https://doi.org/10.11603/24116-4944.2020.1.11492Keywords:
Marfan syndrome, pregnancy, cardiac surgeryAbstract
The aim of the study – to review current issues and clinical guidelines in medical care for pregnant women with Marfan syndrome, as well as the experience of the national multidisciplinary team to reduce maternal and perinatal losses.
Materials and Methods. Our pregnancy heart team from National Amosov Institute of Сardio-vascular Surgery took care on 16 pregnant women and parturients with aortic aneurysms from January 2014 to June 2020. Hereditary diseases of the thoracic aorta with a typical phenotype (Marfan syndrome) were observed in 3 pregnant women. Among them – two were with AAD in the III trimester and in postpartum. One patient was observed by a multidisciplinary team from 14 weeks of gestation up to the delivery. Preventive cardiac surgery in 19 weeks of gestation with fetus in utero was performed.
Results and Discussion. In the group of pregnant women and parturients with thoracic aortic pathology, one maternal death was observed on the 7th day after cardiac surgery (parturient with MS and AAD at 40 weeks of gestation after emergency cesarean section and urgent Bentall de Bone intervention). The cause of death was spontaneous acute dissection of the thoracic aorta type B with ruptures of the descending thoracic aorta. Long-term results were observed in both other mothers – there were not maternal mortality or "near-miss". There were no perinatal losses. In the article we discuss pathomorphological and pathoanatomical factors that affect the increased risk of AAD in pregnant women with MS. Current views on medical care for pregnant women with MS, the principles of primary and secondary prevention of major cardiac events, including a life-threatening complication – acute aortic dissection. Obstetric and cardiac surgery tactics in pregnant patients with MS, including AAD, are discussed. We talked over recommendations for delivery methods in patients with MS, indications for preventive aortic surgery, a clinical case of preventive surgery in pregnant women with MS from the experience of national pregnancy heart team.
Conclusions. The presence of Marfan syndrome significantly increases maternal and perinatal risks and requires careful multidisciplinary support of the pregnant woman and parturient. The development of AAD in patients with MS during pregnancy is a life-threatening complication, the prevention of which requires medical and sometimes preventive cardiac surgery for pregnant women.
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