Preview

Obstetrics, Gynecology and Reproduction

Advanced search

PREGNANCY AND DELIVERY IN PATIENTS WITH MESENHYMAL DYSPLASIAS (MARFAN SYNDROME, EHLERS-DANLOS SYNDROME, HEREDITARY HEMORRHAGIC TELANGIECTASIA)

https://doi.org/10.17749/2313-7347.2015.10.1.100-110

Full Text:

Abstract

Aim. To study the pregnancy course and delivery outcome in patients with mesenchymal dysplasias (Marfan syndrome, Ehlers-Danlos syndrome (EDS), Hereditary hemorrhagic telangiectasia (HHT).

Material and Methods. 56 pregnant women with mesenchymal dysplasias (23 with Marfan syndrome, 22 with Ehlers-Danlos syndrome, 11 with hereditary hemorrhagic telangictasia (HHT) were examined in specialized cardiological maternity house by using physical examination, routine laboratory tests, hemostasiological tests, ECG, echocardiography, MRI, radiology, pulse oximetry.

Results. All patients had hemorrhagic complications various localizations; 18 women with Marfan syndrome developed increasing of mitral regurgitation, 10 – aortic insufficiency, 13 – mitral valve insufficiency. 1 patient with Marfan syndrome was died due to aortic dissection in 45 day after delivery. Pulmonary arteriovenous malformations were visualized in 10 patients with HHT; the symptoms of hypoxemia developed in 6 HHT patients and were result of right-to-left. One patient with HHT developed the symptoms of compression of vena cava inferior due to aneurism of truncus brachiocephalicus. 40 patients had abnormal hemostasiological tests (platelet dysfunction – in 38 patients; DIC syndrome – in 31). Cesarean section was performed in 53 patients. Vaginal delivery (3 patients with EDS) was complicated by massive postpartum hemorrhage and deep vaginal tears.

Conclusions. Patients with mesenchymal dysplasias have a great risk of different complications, during pregnancy and delivery and require the multidisciplinary care and repeated hemostasiological testing. Cesarean section is the preferred method of delivery in such patients.

About the Authors

L. S. Radetskaya
First Moscow State Medical Sechenov University of the Ministry of Health Russian Federation
Russian Federation

MD, docent of the Department of Obstetrics and Gynecology, Faculty of Medical and Preventive Medicine First Moscow Medical Sechenov University. Adress: ul. Trubetskaya, 8, str. 2, Moscow, Russia, 119048



A. D. Makatsariya
First Moscow State Medical Sechenov University of the Ministry of Health Russian Federation
Russian Federation

MD, corresponding member of the Russian Academy of Sciences, Professor, Head of the Department of Obstetrics and Gynecology, Faculty of Medical and Preventive, First Moscow State Medical Sechenov University. Address: ul. Trubetskaya, 8, str. 2, Moskva, Russia, 119048. Tel.: +7 (495) 788-58-40



References

1. Kamoeva C.V., Savchenko T.N., Ivanova A.V., Abaeva Kh.A. Akusherstvo, ginekologiya i reproduktsiya / Obstetrics, gynecology and reproduction. 2013; 1: 16-21.

2. Kudinova E.G., Momot A.P., Trukhacheva N.V. FARMAKOEKONOMIKA. Sovremennaya farmakoekonomika i farmakoepidemiologiya / PHARMACOECONOMICS. Modern pharmacoeconomics and pharmacoepidemiology. 2014; 7 (2): 14-18.

3. Radetskaya L.S. Akusherstvo, ginekologiya i reproduktsiya / Obstetrics, gynecology and reproduction. 2015; 4: 74-85.

4. Abdalla S.A., Geithoff U.W., Bonneau D., Plauchu H. et al. Visceral manifestations in hereditary hemorrhagic telangiectasia type 2. J Med Genet. 2003; 40: 494-502.

5. Borck G., Beighton P., Wilhelm C., Kohlhase J., Kubisch C. Arterial rupture in classic Ehlers- Danlos syndrome with COL5A1 mutation. Am J Med Genet A. 2010 Aug; 152A (8): 2090-3.

6. Combeer E.L., Combeer A.D. A rare cause of maternal death: liver and inferior vena cava rupture due to previously undiagnosed Ehlers- Danlos Syndrome type IV. Eur J Anaesthesiol. 2008 Sep; 25 (9): 765-7.

7. Coulon C. Thoracic aortic aneurysms and pregnancy. Presse Med. 2015 Nov; 44 (11): 1126-35.

8. 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.

9. 2014 Oct; 290 (4): 797-802.

10. Emmanuel Y., Thorne S.A. Heart disease in pregnancy. Best Pract Res Clin Obstet Gynaecol. 2015 Jul; 29 (5): 579-97.

11. Erez Y., Ezra Y., Rojansky N. Ehlers-Danlos type IV in pregnancy. A case report and a literature review. Fetal Diagn Ther. 2008; 23 (1): 7-9.

12. Figueiredo S., Martins E., Lima M.R., Alvares S. Cardiovascular manifestations in Marfan syndrome. Rev Port Cardiol. 2001 Dec; 20 (12): 1203-18.

13. 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 Nov; 148 (5): 1333-45.

14. de Gussem E.M., Lausman A.Y., Beder A.J., Edwards C.P., Blanker M.H., Terbrugge K.G., Mager J.J., Faughnan M.E. Outcomes of pregnancy in women with hereditary hemorrhagic telangiectasia. Obstet Gynecol. 2014 Mar; 123 (3): 514-20.

15. Hassan N., Patenaude V., Oddy L., Abenhaim H.A. Pregnancy outcomes in Marfan syndrome: a retrospective cohort study. Am J Perinatol. 2015 Feb; 30 (2): 123-30.

16. Inocêncio G., Braga A., Lima T., Buchner G. Osler-Weber-Rendu syndrome during pregnancy. BMJ Case Rep. 2013 Jun 25; 2013.

17. Jakobi P., Weiner Z., Best L., Itskovitz-Eldor J. Hereditary hemorrhagic telangiectasia with pulmonary arteriovenous malformations. Obstet Gynecol. 2001 May; 97 (5 Pt 2): 813-4.

18. Jondeau G., Nataf P., Belarbi A., Farcot J.C., Iung B., Delorme G., Gandjbakhch I., Bourdarias J.P. Aortic dissection at 6 months gestation in a women with Marfan's syndrome. Masui. 2002 Aug; 51 (8): 916-20.

19. Khurshid I., Downie G.H. Pulmonary arteriovenous malformation. Postgrad Med J. 2002; 78: 191-197.

20. Kim S.W., Kim D., Hong J.M. Acute aortic dissection in pregnancy with the marfan syndrome. Korean J Thorac Cardiovasc Surg. 2014 Jun; 47 (3): 291-3.

21. 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 Feb; 69 (1): 76-9.

22. Koh M.B., Hunt B.J. The management of perioperative bleeding. Blood Rev. 2003 Sep; 17 (3): 179-85.

23. Kohler F., Fotuhi P., Baumann G. Pregnancy and congenital heart defects. Z Kardiol. 2001; 90 (4): 30-5.

24. Lambaudie E., Depret-Mosser S., Occelli B., Papageorgiou T., Dognin A., Bertrand M., de Martinville B., Codaccioni X., Monnier J.C. Marfan syndrome and pregnancy. Apropos of 4 cases. Gynecol Obstet Fertil. 2002 Jul-Aug; 30 (7-8): 567-75.

25. Master M., Day G. Acute aortic dissection in pregnancy in a woman with undiagnosed marfan syndrome. Case Rep Obstet Gynecol. 2012; 2012: 490169.

26. Mobeen I., Rossoff L.J. Pulmonary arteriovenous malformations: a clinical review. Postgrad Med J. 2000; 76: 390-394.

27. 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 Dec; 16 (12): 874-80.

28. Muiño Mosquera L., De Backer J. Managing aortic aneurysms and dissections during

29. pregnancy. Expert Rev Cardiovasc Ther. 2015 Jun; 13 (6): 703-14.

30. 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 Aug; 37 (8): 724-7.

31. Omnes S., Jondeau G., Detaint D., Dumont A., Yazbeck C., Guglielminotti J., Luton D., Azria E. Pregnancy outcomes among women with Marfan syndrome. Int J Gynaecol Obstet. 2013 Sep; 122 (3): 219-23.

32. Palmquist M., Pappas J.G., Petrikovsky B., Blakemore K., Roshan D. Successful pregnancy outcome in Ehlers-Danlos syndrome, vascular type. J Matern Fetal Neonatal Med. 2009 Oct; 22 (10): 924-7.

33. Preiss M., Hosli I., Holzgreve W., Zerkowski H.R. Aortic dissection in pregnancy in Marfan syndrome-case report and treatment concept. Z Geburtshilfe Neonatol. 2001 May-Jun; 205 (3): 110-3.

34. Rahman J., Rahman F.Z., Rahman W., al-Suleiman S.A., Rahman M.S. Obstetric and gynecologic complications in women with Marfan syndrome. J Reprod Med. 2003 Sep; 48 (9): 723-8.

35. Shovlin CL.Pulmonary arteriovenous malformations. Am J Respir Crit Care Med. 2014 Dec 1; 190 (11): 1217-28.

36. Smok D.A. Aortopathy in pregnancy. Semin Perinatol. 2014 Aug; 38 (5): 295-303.

37. Swietlik E., Doboszynska A. Recurrence of arterio-venous malformations with life-threatening complications in a pregnant woman with hereditary teleangiectasia. J Physiol Pharmacol. 2008 Dec; 59 (6): 683-8.

38. 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 Apr; 54 (2): 191.

39. Uchida T., Ogino H., Ando M., Okita Y., Yagihara T., Kitamura S. Aortic dissection in pregnant woman with the Marfan syndrome. Kyobu Geka. 2002 Jul; 55 (8): 693-6.

40. Wallace G.M., Shovlin C.L. A hereditary haemorrhagic telangiectasia family with pulmonary involvement is unlinked to the known HHT genes, endoglin and ALK-1. Thorax. 2000 Aug; 55 (8): 685-90.

41. Wanga S., Silversides C., Dore A., de Waard V., Mulder B. Pregnancy and Thoracic Aortic Disease: Managing the Risks. Can J Cardiol. 2016 Jan; 32 (1): 78-85.

42. Westhoff-Bleck M., Hilfiker-Kleiner D. Marfan syndrome and pregnancy:monitoring and management. Eur Heart J. 2015 May 7; 36 (18): 1066-7.

43. 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 Jul 15; 8 (7): 11607-12.

44. Yang Z., Yang S., Wang F., Wang C. Acute aortic dissection in pregnant women. Gen Thorac Cardiovasc Surg. 2014 Aug 2.


For citation:


Radetskaya L.S., Makatsariya A.D. PREGNANCY AND DELIVERY IN PATIENTS WITH MESENHYMAL DYSPLASIAS (MARFAN SYNDROME, EHLERS-DANLOS SYNDROME, HEREDITARY HEMORRHAGIC TELANGIECTASIA). Obstetrics, Gynecology and Reproduction. 2016;10(1):100-110. (In Russ.) https://doi.org/10.17749/2313-7347.2015.10.1.100-110

Views: 207


ISSN 2313-7347 (Print)
ISSN 2500-3194 (Online)