Stretched

Released on 19/11/2014

Key Learning Points

  • Aortic Root Dilation - when to perform surgery?
  • Clinical Features of Marfan Syndrome
  • Diagnosis of Marfan Syndrome
  • Fibrillin‐1 (FBN1) testing
  • Marfan Syndrome
  • Marfan Syndrome - mode of delivery
  • Pregnancy and Marfan Syndrome
  • Pregnancy and Marfan Syndrome - potential risks to the mother and Fetus.
  • Prenatal dIagnosis of Marfan Syndrome
  • Slowing down aortic root dilation - drugs and teratogenicity
  • Transmission of Marfan Syndrome to the fetus
  • Which beta blocker to use?

References

1. YUAN SM, JING H. Marfan's syndrome: an overview. Sao Paulo Med J [online] 2010 Dec, 128(6):360-6 [viewed 17 November 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/21308160
2. ROBINSON PN, ARTEAGA-SOLIS E, BALDOCK C, COLLOD-BéROUD G, BOOMS P, DE PAEPE A, DIETZ HC, GUO G, HANDFORD PA, JUDGE DP, KIELTY CM, LOEYS B, MILEWICZ DM, NEY A, RAMIREZ F, REINHARDT DP, TIEDEMANN K, WHITEMAN P, GODFREY M. The molecular genetics of Marfan syndrome and related disorders. J Med Genet [online] 2006 Oct, 43(10):769-87 [viewed 17 November 2014] Available from: doi:10.1136/jmg.2005.039669
3. JUDGE DP, DIETZ HC. Marfan's syndrome Lancet [online] 2005 Dec 3, 366(9501):1965-1976 [viewed 17 November 2014] Available from: doi:10.1016/S0140-6736(05)67789-6
4. CURRY RA, GELSON E, SWAN L, DOB D, BABU-NARAYAN SV, GATZOULIS MA, STEER PJ, JOHNSON MR. Marfan syndrome and pregnancy: maternal and neonatal outcomes. BJOG [online] 2014 Apr, 121(5):610-7 [viewed 17 November 2014] Available from: doi:10.1111/1471-0528.12515
5. KIM SW, KIM D, HONG JM. Acute aortic dissection in pregnancy with the marfan syndrome. Korean J Thorac Cardiovasc Surg [online] 2014 Jun, 47(3):291-3 [viewed 17 November 2014] Available from: doi:10.5090/kjtcs.2014.47.3.291 GOLAND S., ELKAYAM U.. Cardiovascular Problems in Pregnant Women With Marfan Syndrome. Circulation [online] 2009 January, 119(4):619-623 [viewed 17 November 2014] Available from: doi:10.1161/CIRCULATIONAHA.104.493569
6. BURT C. C., DURBRIDGE J.. Management of cardiac disease in pregnancy. Continuing Education in Anaesthesia, Critical Care & Pain [online] 2009 March, 9(2):44-47 [viewed 17 November 2014] Available from: doi:10.1093/bjaceaccp/mkp005
7. DORMAND H, MOHIADDIN RH. Cardiovascular magnetic resonance in Marfan syndrome. J Cardiovasc Magn Reson [online] 2013 Apr 15:33 [viewed 17 November 2014] Available from: doi:10.1186/1532-429X-15-33
8. DE OLIVEIRA NC, DAVID TE, IVANOV J, ARMSTRONG S, ERIKSSON MJ, RAKOWSKI H, WEBB G. Results of surgery for aortic root aneurysm in patients with Marfan syndrome. J Thorac Cardiovasc Surg [online] 2003 Apr, 125(4):789-96 [viewed 17 November 2014] Available from: doi:10.1067/mtc.2003.57
9. BROWN O. R., DEMOTS H., KLOSTER F. E., ROBERTS A., MENASHE V. D., BEALS R. K.. Aortic root dilatation and mitral valve prolapse in Marfan's syndrome: an ECHOCARDIOgraphic study. Circulation [online] 1975 October, 52(4):651-657 [viewed 17 November 2014] Available from: doi:10.1161/01.CIR.52.4.651