Pregnancy Outcomes in Marfan Syndrome: A Retrospective Cohort Study
01 October 2013
31 March 2014
04 June 2014 (online)
Objective Marfan syndrome (MFS) is a rare connective tissue disease with significant risk for adverse cardiovascular outcomes. Our objective was to evaluate pregnancy and cardiovascular outcomes in pregnant women with MFS.
Study Design We conducted a population-based retrospective cohort study using the Healthcare Cost and Utilization Project Nationwide Inpatient Sample (HCUP NIS) database from 2003 to 2010. We used unconditional regression analyses to compare maternal and fetal outcomes among pregnancies in women with and without MFS.
Results Out of the 7,094,400 births in our cohort, 339 deliveries were to women with MFS. There was one maternal death and six aortic dissections among women with MFS. Births to women with MFS were more likely to be premature, odds ratio (OR) 2.15 (1.60–2.89), have intrauterine growth restricted and small for gestational age infants, OR 2.06 (1.24–3.43). Women with MFS were more likely to deliver by cesarean section, OR 1.91 (1.53–2.38) and were at higher risk of major morbidities including cardiac arrhythmias, OR 10.64 (5.49–20.61) and pneumothorax, OR 51.95 (6.18, 437.10).
Conclusion Pregnant women with MFS are at a particularly high risk of adverse pregnancy and cardiovascular events. Preconception counseling should take these risks into consideration and appropriate pregnancy care in tertiary centers should be considered.
The funding was obtained from the authors' institution.
- 1 Judge DP, Dietz HC. Marfan's syndrome. Lancet 2005; 366 (9501) 1965-1976
- 2 Brooke BS, Habashi JP, Judge DP, Patel N, Loeys B, Dietz III HC. Angiotensin II blockade and aortic-root dilation in Marfan's syndrome. N Engl J Med 2008; 358 (26) 2787-2795
- 3 Gelson E, Johnson M. Pregnancy outcomes: effect of maternal heart disease: effect of heart disease on pregnancy outcomes. Expert Rev Obstet Gynecol 2010; 5 (5) 605-617
- 4 Marnach ML, Ramin KD, Ramsey PS, Song SW, Stensland JJ, An KN. Characterization of the relationship between joint laxity and maternal hormones in pregnancy. Obstet Gynecol 2003; 101 (2) 331-335
- 5 Park JI, Chang CL, Hsu SY. New Insights into biological roles of relaxin and relaxin-related peptides. Rev Endocr Metab Disord 2005; 6 (4) 291-296
- 6 Donnelly RT, Pinto NM, Kocolas I, Yetman AT. The immediate and long-term impact of pregnancy on aortic growth rate and mortality in women with Marfan syndrome. J Am Coll Cardiol 2012; 60 (3) 224-229
- 7 Meijboom LJ, Drenthen W, Pieper PG , et al; ZAHARA investigators. Obstetric complications in Marfan syndrome. Int J Cardiol 2006; 110 (1) 53-59
- 8 Allyn J, Guglielminotti J, Omnes S , et al. Marfan's syndrome during pregnancy: anesthetic management of delivery in 16 consecutive patients. Anesth Analg 2013; 116 (2) 392-398
- 9 Guo C, Xu D, Wang C. Successful treatment for acute aortic dissection in pregnancy—Bentall procedure concomitant with cesarean section. J Cardiothorac Surg 2011; 6: 139
- 10 Houston L, Tuuli M, Macones G. Marfan syndrome and aortic dissection in pregnancy. Obstet Gynecol 2011; 117 (4) 956-960
- 11 Jondeau G, Nataf P, Belarbi A , et al. Aortic dissection at 6 months gestation in a women with Marfan's syndrome. Simultaneous Bentall intervention and cesarean section [article in French]. Arch Mal Coeur Vaiss 2000; 93 (2) 185-187
- 12 Kravchenko IM. Marfan syndrome and pregnancy [article in Ukrainian]. Lik Sprava 1999; (4) 108-111
- 13 Simsek Y, Colak C, Yilmaz E, Celik E, Erdil N, Celik O. Successful management of spontaneous aortic dissection type B in the third trimester of pregnancy. Cardiovasc J Afr 2012; 23 (10) e7-e9
- 14 Wang H, Zhuang J, Chen J, Song J, Liang YP, Zheng M. Marfan's Syndrome: Successful term pregnancy after repair of a dissecting thoracic aortic aneurysm at 21 weeks gestation. Pak J Med Sci 2011; 27 (3) 696-698
- 15 Lind J, Wallenburg HC. The Marfan syndrome and pregnancy: a retrospective study in a Dutch population. Eur J Obstet Gynecol Reprod Biol 2001; 98 (1) 28-35
- 16 Lipscomb KJ, Smith JC, Clarke B, Donnai P, Harris R. Outcome of pregnancy in women with Marfan's syndrome. Br J Obstet Gynaecol 1997; 104 (2) 201-206
- 17 Pacini L, Digne F, Boumendil A , et al. Maternal complication of pregnancy in Marfan syndrome. Int J Cardiol 2009; 136 (2) 156-161
- 18 Rossiter JP, Repke JT, Morales AJ, Murphy EA, Pyeritz RE. A prospective longitudinal evaluation of pregnancy in the Marfan syndrome. Am J Obstet Gynecol 1995; 173 (5) 1599-1606
- 19 Goland S, Elkayam U. Cardiovascular problems in pregnant women with Marfan syndrome. Circulation 2009; 119 (4) 619-623
- 20 Lalchandani S, Wingfield M. Pregnancy in women with Marfan's Syndrome. Eur J Obstet Gynecol Reprod Biol 2003; 110 (2) 125-130
- 21 Anum EA, Hill LD, Pandya A, Strauss III JF. Connective tissue and related disorders and preterm birth: clues to genes contributing to prematurity. Placenta 2009; 30 (3) 207-215
- 22 Hiratzka LF, Bakris GL, Beckman JA , et al; American College of Cardiology Foundation; American Heart Association Task Force on Practice Guidelines; American Association for Thoracic Surgery; American College of Radiology; American Stroke Association; Society of Cardiovascular Anesthesiologists; Society for Cardiovascular Angiography and Interventions; Society of Interventional Radiology; Society of Thoracic Surgeons; Society for Vascular Medicine. 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with thoracic aortic disease: executive summary. A report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine. Catheter Cardiovasc Interv 2010; 76 (2) E43-E86
- 23 Michaelson-Cohen R, Elstein D, Ioscovich A , et al. Severe heart disease complicating pregnancy does not preclude a favourable pregnancy outcome: 15 years' experience in a single centre. J Obstet Gynaecol 2011; 31 (7) 597-602
- 24 Rahman J, Rahman FZ, Rahman W, al-Suleiman SA, Rahman MS. Obstetric and gynecologic complications in women with Marfan syndrome. J Reprod Med 2003; 48 (9) 723-728
- 25 Volach V, Elami A, Gilon D, Pollak A, Ginosar Y, Ezra Y. Pregnancy in Marfan syndrome after aortic root replacement: a case report and review of the literature. Congenit Heart Dis 2006; 1 (4) 184-188
- 26 American College of Obstetricians and Gynecologists. Thromboembolism in pregnancy. Obstet Gynecol 2011; 118: 718-729