The present-day view on the pathogenesis and prevention of life-threatening complications of pregnancy and delivery in patients with mesenchymal dysplasias (Marfan syndrome, Ehlers-Danlos syndrome, Rendu-Weber-Osler syndrome)
Objective. To systematize the current medical knowledge and experience in the management of pregnancy and delivery in patients with mesenchymal dysplasias (Marfan syndrome, Ehlers-Danlos syndrome, Rendu-Osler syndrome).Makatsaria A.D., Radetskaya L.S.
Material and methods. The paper includes the data of foreign investigations and medical guidelines for this problem, which have been published in the past 5 years.
Results. The paper describes the molecular bases of each syndrome, diagnostic criteria, and their clinical manifestations in various organs and systems: musculoskeletal and cardiovascular systems, the organ of vision, etc. Such patients during pregnancy are at extremely high risk for life-threatening complications.
Conclusion. A successful outcome of pregnancy requires early diagnosis and a multidisciplinary approach throughout during pregnancy and delivery.
Keywords
mesenchymal dysplasias
congenital connective tissue diseases
Marfan syndrome
Ehlers-Danlos syndrome. Rendu-Weber-Osler
pregnancy
aortic dissection in pregnant women
cesarean section
bleeding
Supplementary Materials
- Table 1. Basic diagnostic criteria of Marphan syndrome
- Table 2. Classification and diagnostic criteria of Ehlers-Danlo syndrome
References
1. Hassan N., Patenaude V., Oddy L., Abenhaim H.A. Pregnancy outcomes in Marfan syndrome: a retrospective cohort study. J. Perinatol. 2015; 30(2):123-30.
2. Emmanuel Y., Thorne S.A. Heart disease in pregnancy. Best Pract. Res. Clin. Obstet. Gynaecol. 2015; 29(5): 579-97.
3. Naud K., Horne G., Van den Hof M. A woman with Marfan syndrome in pregnancy: managing high vascular risk with multidisciplinary care. J. Obstet. Gynaecol. Can. 2015; 37(8): 724-7.
4. Omnes S., Jondeau G., Detaint D., Dumont A., Yazbeck C., Guglielminotti J. et al. Pregnancy outcomes among women with Marfan syndrome. Int. J. Gynaecol. Obstet. 2013; 122(3): 219-23.
5. Westhoff-Bleck M., Hilfiker-Kleiner D. Marfan syndrome and pregnancy: monitoring and management. Eur. Heart J. 2015; 36(18): 1066-7.
6. Coulon C. Thoracic aortic aneurysms and pregnancy. Presse Med. 2015; 44(11): 1126-35.
7. Cox D.A., Ginde S., Kuhlmann R.S., Earing M.G. Management of the pregnant woman with Marfan syndrome complicated by ascending aorta dilation. Arch. Gynecol. Obstet. 2014; 290(4): 797-802.
8. Kim S.W., Kim D., Hong J.M. Acute aortic dissection in pregnancy with the marfan syndrome. Korean J. Thorac. Cardiovasc. Surg. 2014; 47(3): 291-3.
9. Kim W.H., Bae J., Choi S.W., Lee J.H., Kim C.S., Cho H.S., Lee S.M. Stanford type A aortic dissection in a patient with Marfan syndrome during pregnancy: a case report. Korean J. Anesthesiol. 2016;69(1): 76-9.
10. Master M., Day G. Acute aortic dissection in pregnancy in a woman with undiagnosed marfan syndrome. Case Rep. Obstet. Gynecol. 2012; 2012: 490169.
11. Muiño Mosquera L., De Backer J. Managing aortic aneurysms and dissections during pregnancy. Expert Rev. Cardiovasc. Ther. 2015;13(6): 703-14.
12. Smok D.A. Aortopathy in pregnancy. Semin. Perinatol. 2014; 38(5): 295-303.
13. Wanga S., Silversides C., Dore A., de Waard V., Mulder B. Pregnancy and thoracic aortic disease: managing the risks. Can. J. Cardiol. 2016; 32(1): 78-85.
14. Yang Z., Yang S., Wang F., Wang C. Acute aortic dissection in pregnant women. Gen. Thorac. Cardiovasc. Surg. 2016; 64(5): 283-5.
15. Yang G., Peng W., Zhao Q., Peng J., Xiang X., Chai X. Aortic dissection in women during the course of pregnancy or puerperium: a report of 11 cases in central south China. Int. J. Clin. Exp. Med. 2015; 8(7): 11607-12.
16. Murray M.L., Pepin M., Peterson S., Byers P.H. Pregnancy-related deaths and complications in women with vascular Ehlers-Danlos syndrome. Genet. Med. 2014; 16(12): 874-80.
17. de Gussem E.M., Lausman A.Y., Beder A.J., Edwards C.P., Blanker M.H., Terbrugge K.G. et al. Outcomes of pregnancy in women with hereditary hemorrhagic telangiectasia. Obstet. Gynecol. 2014; 123(3): 514-20.
18. Inocêncio G., Braga A., Lima T., Buchner G. Osler-Weber-Rendu syndrome during pregnancy. BMJ Case Rep. 2013 Jun 25; 2013. pii: bcr2013009792.
19. Takahashi H., Matsubara S., Saito K., Bando M. Good obstetric outcome after embolisation of pulmonary arteriovenous malformation in hereditary haemorrhagic telangiectasia: planned pregnancy may also be important in this condition. Aust. N. Z. J. Obstet. Gynaecol. 2014;54(2): 191.
20. Guntupalli K.K., Karnad D.R., Bandi V., Hall N., Belfort M. Critical illness in pregnancy: Part II: Common medical conditions complicating pregnancy and puerperium. Chest. 2015; 148(5): 1333-45.
Received 07.04.2017
Accepted 28.04.2017
About the Authors
Aleksandr D. Makatsariya, RAS corresponding member, DM, Professor, Head of obstetrics and gynecology department, Head of hemostasis pathology laboratory,I.M. Sechenov First Moscow State Medical University. 119992, Russia, Moscow, Malaya Trubetskaya str. 8 bld. 2. E-mail: gemostasis@mail.ru
Lyudmila S. Radetskaya, Candidate of Medical Science, Associate Professor, Senior Researcher, Obstetrics and Gynecology Department,
I.M. Sechenov First Moscow State Medical University. 119992, Russia, Moscow, Malaya Trubetskaya str. 8 bld. 2. Tel.: +79265313988. E-mail: udaeva@gmail.com
For citations: Makatsaria A.D., Radetskaya L.S. The present-day view on the pathogenesis and prevention of life-threatening complications of pregnancy and delivery in patients with mesenchymal dysplasias (Marfan syndrome, Ehlers-Danlos syndrome, Rendu-Weber-Osler syndrome). Akusherstvo i Ginekologiya/Obstetrics and Gynecology. 2018; (1): 127-34. (in Russian)
https://dx.doi.org/10.18565/aig.2018.1.127-134
Similar Articles
