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Management of the pregnant woman with Marfan syndrome complicated by ascending aorta dilation

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Abstract

Introduction

Marfan syndrome is a disorder of connective tissue associated with progressive dilation of the aorta and potential risk for aortic dissection. Women with Marfan syndrome who are, or wish to become, pregnant represent a unique and challenging patient population due to a risk for accelerated aortic growth and aortic dissection during pregnancy. Risk for aortic complications during pregnancy is related to the dimensions of the ascending aorta. Women with an aortic diameter ≥4.5 cm at the start of pregnancy are at higher risk for aortic dissection, and an aortic dimension >4.0 cm is considered a relative contraindication to pregnancy in the setting of Marfan syndrome.

Conclusion

Multidisciplinary care involving specialists familiar with Marfan syndrome should be emphasized before, during, and after pregnancy with the involvement of Maternal Fetal Medicine, Genetics, Cardiology, Cardiothoracic Surgery, Anesthesia, and other specialties on a case-by-case basis. We review the important aspects of the evaluation and management of pregnant women with Marfan syndrome.

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References

  1. Loeys BL, Dietz HC, Braverman AC et al (2010) The revised Ghent nosology for the Marfan syndrome. J Med Genet 47(7):476–485. doi:10.1136/jmg.2009.072785

    Article  CAS  PubMed  Google Scholar 

  2. Kainulainen K, Pulkkinen L, Savolainen A, Kaitila I, Peltonen L (1990) Location on chromosome 15 of the gene defect causing Marfan syndrome. N Engl J Med 323(14):935–939. doi:10.1056/jejm199010043231402

    Article  CAS  PubMed  Google Scholar 

  3. Dietz HC, Cutting GR, Pyeritz RE et al (1991) Marfan syndrome caused by a recurrent de novo missense mutation in the fibrillin gene. Nature 352(6333):337–339. doi:10.1038/352337a0

    Article  CAS  PubMed  Google Scholar 

  4. Kielty CM, Shuttleworth CA (1995) Fibrillin-containing microfibrils: structure and function in health and disease. Int J Biochem Cell Biol 27(8):747–760. doi:10.1016/1357-2725(95)-00028-N

    Article  CAS  PubMed  Google Scholar 

  5. Hollister DW, Godfrey M, Sakai LY, Pyeritz RE (1990) Immunohistologic abnormalities of the microfibrillar-fiber system in the Marfan syndrome. N Engl J Med 323(3):152–159. doi:10.1056/nejm199007193230303

    Article  CAS  PubMed  Google Scholar 

  6. Adams JA, Trent RJ (1998) Aortic complications of Marfan’s syndrome. Lancet 352(9142):1722–1723. doi:10.1016/S0140-6736(05)79822-6

    Article  CAS  PubMed  Google Scholar 

  7. Finkbohner R, Johnston D, Crawford ES, Coselli J, Milewicz DM (1995) Marfan syndrome. long-term survival and complications after aortic aneurysm repair. Circulation 91(3):728–733. doi:10.1161/01.cir.91.3.728

    Article  CAS  PubMed  Google Scholar 

  8. Silverman DI, Burton KJ, Gray J, Bosner MS, Kouchoukos NT, Roman MJ, Boxer M, Devereux RB, Tsipouras P (1995) Life expectancy in the Marfan syndrome. Am J Cardiol 75(2):157–160. doi:10.1016/S0002-9149(00)80066-1

    Article  CAS  PubMed  Google Scholar 

  9. Rossiter JP, Repke JT, Morales AJ, Murphy EA, Pyeritz RE (1995) A prospective longitudinal evaluation of pregnancy in the Marfan syndrome. Am J Obstet Gynecol 173(5):1599–1606. doi:10.1016/0002-9378(95)90655-x

    Article  CAS  PubMed  Google Scholar 

  10. Elkayam U, Ostrzega E, Shotan A, Mehra A (1995) Cardiovascular problems in pregnant women with Marfan syndrome. Ann Intern Med 123(2):117–122

    Article  CAS  PubMed  Google Scholar 

  11. Lipscomb KJ, Smith JC, Clarke B et al (1997) Outcome of pregnancy in women with Marfan’s syndrome. Br J Obstet Gynaecol 104(2):201–206

    Article  CAS  PubMed  Google Scholar 

  12. Lind J, Wallenburg HC (2001) The Marfan syndrome and pregnancy: a retrospective study in a Dutch population. Eur J Obstet Gynecol Reprod Biol 98(1):28–35

    Article  CAS  PubMed  Google Scholar 

  13. Goland S, Barakat M, Khatri N, Elkayam U (2009) Pregnancy in Marfan syndrome: maternal and fetal risk and recommendations for patient assessment and management. Cardiol Rev 17(6):253–262

    Article  PubMed  Google Scholar 

  14. Pacini L, Digne F et al (2009) Maternal complication of pregnancy in Marfan syndrome. Int J Cardiol 136(2):156–161

    Article  PubMed  Google Scholar 

  15. Donnelly RT, Pinto NM, Kocolas I, Yetman AT (2012) The immediate and long-term impact of pregnancy on aortic growth rate and mortality in women with Marfan syndrome. J Am Coll Cardiol 60(3):224–229

    Article  PubMed  Google Scholar 

  16. Meijboom LJ, Vos FE, Timmermans J et al (2005) Pregnancy and aortic root growth in the Marfan syndrome: a prospective study. Eur Heart J 26(9):914–920

    Article  PubMed  Google Scholar 

  17. Earing MG, Webb GD (2005) Congenital heart disease and pregnancy: maternal and fetal risks. Clin Perinatol 32(4):913–919

    Article  PubMed  Google Scholar 

  18. Barrett JM, Van Hooydonk JE, Boehm FH (1982) Pregnancy-related rupture of arterial aneurysms. Obstet Gynecol Surv 37(9):557–566

    Article  CAS  PubMed  Google Scholar 

  19. Manalo-Estrella P, Barker AE (1967) Histopathology findings in human aortic media associated with pregnancy. Arch Pathol 83(4):336–341

    CAS  PubMed  Google Scholar 

  20. Slangen BF, van Ingen Schenau DS, van Gorp AW et al (1997) Aortic distensibility and compliance in conscious pregnant rats. Am J Physiol 272(3):H1260–H1265

    CAS  PubMed  Google Scholar 

  21. Easterling TR, Benedetti TJ, Schumucker BC, Carlson K, Millard SP (1991) Maternal hemodynamics and aortic diameter in normal and hypertensive pregnancies. Obstet Gynecol 78(6):1073–1077

    CAS  PubMed  Google Scholar 

  22. Regitz-Zagrosek V, Lundqvist CB, Borghi C et al (2011) Task Force on the Management of Cardiovascular Diseases during Pregnancy of the European Society of Cardiology-Expert consensus document on management of cardiovascular diseases during pregnancy. Eur Heart J 32(24):3147–3197

  23. Hiratzka LF, Bakris GL, Beckman JA et al (2010) ACCF/AHA Guidelines for the diagnosis and management of patients with thoracic aortic disease. Circulation 121(13):e266–e369

    Article  PubMed  Google Scholar 

  24. Meijboom LJ, Drenthen W, Pieper PG et al (2006) Obstetric complications in Marfan syndrome. Int J Cardiol 110(1):53–59

    Article  PubMed  Google Scholar 

  25. Silversides CK, Marelli A, Beauchesne L et al (2010) Canadian cardiovascular society 2009 consensus conference on the management of adults with congenital heart disease: executive summary. Can J Cardiol 26(3):143–150

    Article  PubMed Central  PubMed  Google Scholar 

  26. Shores J, Berger KR, Murphy EA, Pyeritz RE (1994) Progression of aortic dilatation and the benefit of long-term beta-adrenergic blockade in Marfan’s syndrome. N Engl J Med 330(19):1335–1341

    Article  CAS  PubMed  Google Scholar 

  27. Keane MG, Pyeritz RE (2008) Medical management of Marfan syndrome. Circulation 117(21):2802–2813

    Article  PubMed  Google Scholar 

  28. Immer FF, Bansi AG, Immer-Bansi AS et al (2003) Aortic dissection in pregnancy: analysis of risk factors and outcome. Ann Thorac Surg 76(1):309–314

    Article  PubMed  Google Scholar 

  29. Ueland K (1978) Cardiovascular diseases complicating pregnancy. Clin Obstet Gynecol 21(2):429–442

    Article  CAS  PubMed  Google Scholar 

  30. Volach V, Elami A, Gilon D et al (2006) Pregnancy in Marfan syndrome after aortic root replacement: a case report and review of the literature. Cong Heart Dis 1(4):184–188

    Article  Google Scholar 

  31. Simpson LL, Athanassious AM, D’Alton ME (1997) Marfan syndrome in pregnancy. Curr Opin Obstet Gynecol 9(5):337–341

    Article  CAS  PubMed  Google Scholar 

  32. Lacassie HJ, Millar S, Leithe LG et al (2005) Dural ectasia: a likely cause of inadequate spinal anesthesia in two parturients with Marfan’s syndrome. Br J Anaesth 94(4):500–504

    Article  CAS  PubMed  Google Scholar 

  33. Singh SI, Brooks C, Dobkowski W (2008) General anesthesia using remifentanil for cesarean delivery in a parturient with Marfan’s syndrome. Can J Anesth 55(8):526–531

    Article  PubMed  Google Scholar 

  34. Ngan Kee WD, Khaw KS, Ma KC, Wong AS, Lee BB, Ng FF (2006) Maternal and neonatal effects of remifentanil at induction of general anesthesia for cesarean delivery: a randomized, double-blind, controlled trial. Anesthesiol 104(1):14–20

    Article  Google Scholar 

  35. Goland S, Elkayam U (2009) Cardiovascular problems in pregnant women with Marfan syndrome. Circulation 119(4):619–623

    Article  PubMed  Google Scholar 

  36. Rutherford RB, Nolte JE (1995) Aortic and other arterial dissections associated with pregnancy. Semin Vasc Surg 8(4):299–305

    CAS  PubMed  Google Scholar 

  37. Konishi Y, Tatuta N, Kimada K (1980) Dissecting aneurysm during pregnancy and the puerperium. Jpn Circ J 44(9):726–733

    Article  CAS  PubMed  Google Scholar 

  38. Pomini F, Mercogliano D, Cavalletti C et al (1996) Cardiopulmonary bypass in pregnancy. Ann Thorac Surg 61(1):259–268

    Article  CAS  PubMed  Google Scholar 

  39. Arnoni RT, Arnoni AS, Bonini RC et al (2003) Risk factors associated with cardiac surgery during pregnancy. Ann Thorac Surg 76(5):1605–1608

    Article  PubMed  Google Scholar 

  40. Parry AJ, Westaby S (1996) Cardiopulmonary bypass during pregnancy. Ann of Thorac Surg. 61(6):1865–1869

    Article  CAS  Google Scholar 

  41. Tang GH, Malekan R, Lansman SL, Spielvogel D (2013) Aortic valve-sparing reimplantation and mitral repair in a pregnant, second trimester Marfan patient: surgical decision. Ann Thorac Surg 95(2):701–703

    Article  PubMed  Google Scholar 

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Conflict of interest

The authors report no conflicts of interest.

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Authors and Affiliations

  1. Division of Cardiology, Department of Pediatrics, Medical College of Wisconsin/Children’s Hospital of Wisconsin, 9000 W. Wisconsin Avenue, Milwaukee, WI, 53226, USA

    Daniel A. Cox, Salil Ginde & Michael G. Earing

  2. Division of Maternal Fetal Medicine, Department of Obstetrics and Gynecology, Medical College of Wisconsin, 9000 W. Wisconsin Avenue, Milwaukee, WI, 53226, USA

    Randall S. Kuhlmann

  3. Division of Adult Cardiovascular Medicine, Department of Internal Medicine, Medical College of Wisconsin, 9000 W. Wisconsin Avenue, Milwaukee, WI, 53226, USA

    Michael G. Earing

Authors
  1. Daniel A. Cox
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  2. Salil Ginde
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  4. Michael G. Earing
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Correspondence to Daniel A. Cox.

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Cox, D.A., Ginde, S., Kuhlmann, R.S. et al. Management of the pregnant woman with Marfan syndrome complicated by ascending aorta dilation. Arch Gynecol Obstet 290, 797–802 (2014). https://doi.org/10.1007/s00404-014-3307-4

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  • DOI: https://doi.org/10.1007/s00404-014-3307-4

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