SavedAdd your journal

Journals / GORM:Gynecology Obstetrics & Reproductive Medicine / 2010 / Cilt: 16 - Sayı: 3

Marfan sendromu ve gebelik olgu sunumu ve güncel derleme

Marfan Syndrome and pregnancy -A case report and review of the literature

Özgür ÖZYÜNCÜ
Sertaç ESİN
Adam GYIMADU
Pages
186–189
DOI
—
🏛️Bibliographic metadata and full text access for this article are provided via DergiPark.
DergiPark Record ↗
✨

AI Summary & Key Takeaways

AI Insight
⚡ TL;DR(Too Long; Didn't Read)

Therefore, the aim of this study is to review the literature over a case with a successful management during pregnancy.

📌 3 Key Takeaways

🎯
Objective & Hypothesis:Marfan syndrome is an autosomal dominant disorder characterized by abnormalities of skeletal, cardiovascular and ocular systems.
📊
Key Findings & Data:Pregnant women may suffer from hemodynamic stress resulting from increased cardiac output and structural changes in the aortic wall leading dilatation and dissection of the aorta.
💡
Conclusion & Impact:Therefore, the aim of this study is to review the literature over a case with a successful management during pregnancy.
🔬Design:Review & Systematic Survey
📍Scope:Comprehensive Sample
⚙️Variables:Domain Parameters

Abstract

Marfan syndrome is an autosomal dominant disorder characterized by abnormalities of skeletal, cardiovascular and ocular systems. Pregnant women may suffer from hemodynamic stress resulting from increased cardiac output and structural changes in the aortic wall leading dilatation and dissection of the aorta. It is usually advised to avoid pregnancy to women with Marfan Syndrome however with advances in medicine, successful pregnancies with Marfan Syndrome are reported. Therefore, the aim of this study is to review the literature over a case with a successful management during pregnancy.

Özet

Marfan Sendromu iskelet, kardiyovasküler oküler system anomalileri ile seyreden otozomal dominant geçişli kalıtsal bir hastalıktır. Gebelikte kardiyak yükün artması ve aort duvarında olan genişleme hemodinamik strese neden olup aorta dilatasyonuna ve hatta diseksiyonuna neden olabilir. Marfan Send - romu olan kadınlara genelde gebelikten kaçınılması önerilse de tıpta olan gelişmeler sayesinde Marfan sendromu ile birlikte başarılı olarak sonuçlanan gebelikler de bildirilmiştir. Bu çalışmanın amacı, Marfan Sendromu olan bir gebeliğin başarılı bir şekilde yönetimi üzerinden litertürdeki son durumun derlenmesidir.

🔖

Cite This Article

ÖZYÜNCÜ, Ö., ESİN, S., GYIMADU, A. (2010). Marfan sendromu ve gebelik olgu sunumu ve güncel derleme. GORM:Gynecology Obstetrics & Reproductive Medicine, Cilt: 16 - Sayı: 3, 186–189. https://ajindex.com/articles/227066

📄 Full Text PDF Viewer

📄

PDF görüntüleyici hazırlanıyor...

References (14)

  1. Gray JR, Bridges AB, West RR, et al. Life expectancy in British Marfan syndrome populations. Clin Genet 1998; 54:124-8
  2. Anderson RA, Fineron PW. Aortic dissection in pregnancy: importance of pregnancy-induced changes in the vessel wall and bicuspid aortic valve in pathogenesis. Br J Obstet Gynaecol. 1994;101(12):1085-8
  3. Meijboom LJ, Drenthen W, Pieper PG, Groenink M, van der Post JA, Timmermans J, Voors AA, Roos-Hesselink JW, van Veldhuisen DJ, Mulder BJ; On behalf of ZAHARA investigators. Obstetric complications in Marfan syndrome. Int J Cardiol. 2006; 110(1):53-9
  4. De Paepe A, Devereux RB, Dietz HC, Hannekam RC, Pyeritz RE. Revised diagnostic criteria for the Marfan syndrome. Am J Med Genet 1996; 62:417-26
  5. Robinson P, Booms P, Katzke S, Ladewig M, Neumann L, Palz M, et al. Mutations of FBN1 and genotype-phenotype correlations in Marfan syndrome and related fibrillinopathies. Hum Mutat 2002; 20:153-61
  6. McKusick VA. Heritable disorders of connective tissue. St Louis:CV Mosby, 1972
  7. Buffolo E, Palma JH, Gomes WJ, Vega H, Born D, Moron AF, Carvalho AC. Succesful use of deep hypothermic circulatory arrest in pregnancy. Ann Thorac Surg. 1994; 58(5):1532-4
  8. Kalu G, Toplis P. Marfan syndrome and pregnancy-a district hospital perspective. J Obstet Gynaec 1999;19 (6):594-7 9. Murdoch JL,Walker BA, Halpern BL, Kuzma JW, McKusick VA. Life expectancy and causes of death in the Marfan syndrome. N Engl J Med 1972; 286:804-8
  9. Silverman DI, Burton KJ, Gray J, et al. Life expectancy in the Marfan syndrome. Am J Cardiol 1995; 75:157-60
  10. Dean JCS. Management of Marfan syndrome. Heart 2002; 88:97-103
  11. Lind J, Wallenburg HCS. The Marfan syndrome and pregnancy: a retrospective study in a dutch population. Eur J Obstet Rep Bio 1998;2001:28-35 13. Lalchandani S, Wingfield M. Pregnancy in women with Marfan’s syndrome. Eur J. Obstet Gynecol Reprod Bio 2003;110:125-30
  12. Groenink M, Lohuis TA, Tijssen JG et al. 1999. Survival and complication free survival in Marfan’s syndrome: implications of current guidelines. Heart 82:499-504 15. Roman MJ, Rosen SE, Kramer-Fox R et al. 1993. Prognostic significance of the pattern of aortic root dilatation in Marfan syndrome. J Am Col Cardio 22:1470-6
  13. Chow SL: Acute aortic dissection in a patient with Marfan’s syndrome complicated by gestational hypertension. Med J Aust 1993;159:760-2
  14. Pacini L, Digne F, Boumendil A, Muti C, Detaint D, Boileau C, Jondeau G. Maternal complication of pregnancy in Marf