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Pregnancy and delivery in patients with Marfan syndrome (Сase study)

HEALTH OF WOMAN. 2016.6(112):62–65 

Pregnancy and delivery in patients with Marfan syndrome (Сase study) 

Golyanovskyy O. V., Rubinshtein A. M., Strashevych K. V.

Shupyk National Medical Academy of Postgraduate Education, Kiev 

The article is devoted to the problem of pregnancy, delivery and the postpartum period in patients with Marfan syndrome – the system genetically determined pathology of the connective tissue. This disease is often accompanied by disorders of the vascular wall with the formation and subsequent stratification of aneurysms. Today the basic diagnostic method for vascular aneurysms remains echocardiography, which allows estimating the degree of vascular dilation and determining the tactics for such patients.

The article presents the clinical case of pregnancy, delivery and the postpartum period management in patient with Marfan syndrome, portal hypertension, subhepatic form, compensated by the essential porto-caval anastomosis, aneurysm of the vena cava inferior.

Key words: Marfan syndrome in pregnancy, abdominal aortic aneurysm, portal hypertension syndrome.


1. Agg B, Benke K, Szilveszter B et al. 2014, Apr 11. Possible extracardiac predictors of aortic dissection in Marfan syndrome. BMC Cardiovasc Disord. 14:47. http://dx.doi.org/10.1186/1471-2261-14-47; PMid:24720641 PMCid:PMC4021409

2. 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 60:224. http://dx.doi.org/10.1016/j.jacc.2012.03.051; PMid:22789886

3. European Society of Gynecology (ESG), Association for European Paediatric Cardiology (AEPC), German Society for Gender Medicine (DGesGM), et al. ESC Guidelines on the management of cardiovascular diseases during pregnancy: the Task Force on the Management of Cardiovascular Diseases during Pregnancy of the European Society of Cardiology (ESC). Eur Heart J 32:3147. 2011. http://dx.doi.org/10.1093/eurheartj/ehr218; PMid:21873418

4. Goland S, Elkayam U. 2009. Cardiovascular problems in pregnant women with marfan syndrome. Circulation 119:619–623. http://dx.doi.org/10.1161/CIRCULATIONAHA.104.493569; PMid:19188522

5. Hecht F, Beals RK. 1972. «New» syndrome of congenital contractural arachnodactyly originally described by Marfan in 1896. Pediatrics 49:574–579. PMid:4552107

6. Naud K, Horne G, van den Hof M. 2015. A woman with Marfan syndrome in pregnancy: managing high vascular risk with multidisciplinary care. J Obstet Gynaecol Can 37(8):724–727. http://dx.doi.org/10.1016/S1701-2163(15)30177-8

7. von Kodolitsch Y, De Backer J, Schuler H et al. 2015, Jun 16. Perspectives on the revised Ghent criteria for the diagnosis of Marfan syndrome. Appl Clin Genet. 8:137-55. doi: 10. 2147/ TACG. S60472. eCollection 2015.

8. Marfan AB. 1896. Un cas de deformation congenital des quatre membres plus prononcee aux extremities caracterisee par l’allongement des os avec un certain degre d’amincissement. Bull Mem Soc Med Hip (Paris) 13:220–226.

9. McKusick VA. 1955. The cardiovascular aspects of Marfan’s syndrome: A heritable disorder of connective tissue. Circulation 11:321–341. http://dx.doi.org/10.1161/01.CIR.11.3.321; PMid:14352380

10. Silversides CK, Kiess M, Beauchesne L et al. 2010. Canadian Cardiovascular Society 2009 Consensus Conference on the management of adults with congenital heart disease: Outflow tract obstrustion, coarctation of the aorta, tetralogy of Fallot, Ebstein anomaly and Marfan’s Syndrome. Can J Cardiol. 26(3):e80–e97. http://dx.doi.org/10.1016/S0828-282X(10)70355-Xhttp://dx.doi.org/10.1016/S0828-282X(10)70354-8http://dx.doi.org/10.1016/S0828-282X(10)70356-1http://dx.doi.org/10.1016/S0828-282X(10)70352-4

11. Yeh YC, Lin TF, Chang HC et al. 2009. Combination of low-dose nalbuphine and morphine in patient-controlled analgesia decreases incidence of opioid-related side effects. J. Formos. Med. Assoc. 108(7):548–553. http://dx.doi.org/10.1016/S0929-6646(09)60372-7