CC BY-NC-ND 4.0 · AJP Rep 2018; 08(04): e234-e240
DOI: 10.1055/s-0038-1675347
Case Report
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Pregnancy after Aortic Root Replacement in Marfan's Syndrome: A Case Series and Review of the Literature

Dominique Williams
1   Cardiovascular Division, Department of Medicine, Washington University School of Medicine, St. Louis, Missouri
,
Kathryn J. Lindley
1   Cardiovascular Division, Department of Medicine, Washington University School of Medicine, St. Louis, Missouri
,
Melissa Russo
2   Department of Obstetrics and Gynecology, Warren Alpert Medical School at Brown University, Providence, Rhode Island
,
Jennifer Habashi
3   Johns Hopkins University School of Medicine, Howard Hughes Medical Institute, Baltimore, Maryland
,
Harry C. Dietz
3   Johns Hopkins University School of Medicine, Howard Hughes Medical Institute, Baltimore, Maryland
,
Alan C. Braverman
1   Cardiovascular Division, Department of Medicine, Washington University School of Medicine, St. Louis, Missouri
› Author Affiliations
Further Information

Publication History

12 June 2018

30 August 2018

Publication Date:
18 October 2018 (online)

Abstract

Objectives We sought to characterize pregnancy-related aortic complications in women with Marfan's syndrome who had prior aortic root replacement.

Study Design This is a retrospective case series study and literature review of women with Marfan's syndrome with pregnancy after aortic root replacement. We surveyed women with Marfan's syndrome who had successful pregnancy after aortic root replacement using the Marfan Foundation Website and from two large tertiary care Marfan's clinics. Clinical data, counseling information, and details of pregnancy-related aortic complications were compiled. A literature review was performed assessing aortic outcomes in women with Marfan's syndrome with pregnancy after aortic surgery.

Results Fourteen women with 20 pregnancies were identified. Two women had three pregnancies following root replacement for aortic dissection. There were no aortic dissections during the 20 pregnancies. In contrast, aortic dissection was frequently reported in the literature.

Conclusions Women with Marfan's syndrome who become pregnant following aortic root replacement remain at risk for distal aortic dissection related to pregnancy. The exact risk is difficult to quantify but is not zero and women should be counseled accordingly.

Supplementary Material

 
  • References

  • 1 Finkbohner R, Johnston D, Crawford ES, Coselli J, Milewicz DM. Marfan syndrome. Long-term survival and complications after aortic aneurysm repair. Circulation 1995; 91 (03) 728-733
  • 2 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 (02) 201-206
  • 3 Pacini L, Digne F, Boumendil A. , et al. Maternal complication of pregnancy in Marfan syndrome. Int J Cardiol 2009; 136 (02) 156-161
  • 4 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 (05) 1599-1606
  • 5 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. 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. . J Am Coll Cardiol 2010; 55 (14) e27-e129
  • 6 Regitz-Zagrosek V, Blomstrom Lundqvist C, Borghi C. , et al; European Society of Gynecology (ESG); Association for European Paediatric Cardiology (AEPC); German Society for Gender Medicine (DGesGM); ESC Committee for Practice Guidelines. 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 2011; 32 (24) 3147-3197
  • 7 McDermott CD, Sermer M, Siu SC, David TE, Colman JM. Aortic dissection complicating pregnancy following prophylactic aortic root replacement in a woman with Marfan syndrome. Int J Cardiol 2007; 120 (03) 427-430
  • 8 Meijboom LJ, Drenthen W, Pieper PG. , et al; ZAHARA investigators Obstetric complications in Marfan syndrome. Int J Cardiol 2006; 110 (01) 53-59
  • 9 Chavanon O, Rama A, Leprince P. , et al. Valve-sparing operation in a young woman with Marfan syndrome: a word of caution. J Thorac Cardiovasc Surg 2006; 132 (03) 683-684
  • 10 Sayama S, Takeda N, Iriyama T. , et al. Peripartum type B aortic dissection in patients with Marfan syndrome who underwent aortic root replacement: a case series study. BJOG 2018; 125 (04) 487-493
  • 11 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 (03) 224-229
  • 12 Tutarel O, Lotz J, Roentgen P, Drexler H, Meyer GP, Westhoff-Bleck M. Pregnancy in a Marfan patient with pre-existing aortic dissection. Int J Cardiol 2007; 114 (02) E36-E37
  • 13 Williams A, Child A, Rowntree J, Johnson P, Donnai P. Marfan's syndrome: successful pregnancy after aortic root and arch replacement. BJOG 2002; 109 (10) 1187-1188
  • 14 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 (02) 392-398
  • 15 Omnes S, Jondeau G, Detaint D. , et al. Pregnancy outcomes among women with Marfan syndrome. Int J Gynaecol Obstet 2013; 122 (03) 219-223
  • 16 Curry RA, Gelson E, Swan L. , et al. Marfan syndrome and pregnancy: maternal and neonatal outcomes. BJOG 2014; 121 (05) 610-617
  • 17 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 (04) 184-188
  • 18 Loeys BL, Dietz HC, Braverman AC. , et al. The revised Ghent nosology for the Marfan syndrome. J Med Genet 2010; 47 (07) 476-485
  • 19 Roman MJ, Pugh NL, Hendershot TP. , et al; GenTAC InvestigatorsDietzHarry C.HabashiJenniferPrakashSiddharth K.MaslenCheryl L.SongHoward K.BavariaJoseph E.MilewskiKariannaWeinsaftJonathan W.McDonnellNazliAschFederico M.TolunayH. EserDesvigne-NickensPatriceTsengHungKronerBarbara L. Aortic complications associated with pregnancy in Marfan syndrome: the NHLBI National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC). J Am Heart Assoc 2016; 5 (08) e004052
  • 20 Pyeritz RE. Maternal and fetal complications of pregnancy in the Marfan syndrome. Am J Med 1981; 71 (05) 784-790
  • 21 Abbas AE, Lester SJ, Connolly H. Pregnancy and the cardiovascular system. Int J Cardiol 2005; 98 (02) 179-189
  • 22 Goland S, Barakat M, Khatri N, Elkayam U. Pregnancy in Marfan syndrome: maternal and fetal risk and recommendations for patient assessment and management. Cardiol Rev 2009; 17 (06) 253-262
  • 23 Loeys BL, Schwarze U, Holm T. , et al. Aneurysm syndromes caused by mutations in the TGF-beta receptor. N Engl J Med 2006; 355 (08) 788-798
  • 24 den Hartog AW, Franken R, Zwinderman AH. , et al. The risk for type B aortic dissection in Marfan syndrome. J Am Coll Cardiol 2015; 65 (03) 246-254
  • 25 Song HK, Kindem M, Bavaria JE. , et al; Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions Consortium. Long-term implications of emergency versus elective proximal aortic surgery in patients with Marfan syndrome in the Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions Consortium Registry. J Thorac Cardiovasc Surg 2012; 143 (02) 282-286
  • 26 Groenink M, den Hartog AW, Franken R. , et al. Losartan reduces aortic dilatation rate in adults with Marfan syndrome: a randomized controlled trial. Eur Heart J 2013; 34 (45) 3491-3500
  • 27 Lacro RV, Dietz HC, Sleeper LA. , et al; Pediatric Heart Network Investigators. Atenolol versus losartan in children and young adults with Marfan's syndrome. N Engl J Med 2014; 371 (22) 2061-2071
  • 28 Jondeau G, Ropers J, Regalado E. , et al; Montalcino Aortic Consortium. International registry of patients carrying TGFBR1 or TGFBR2 mutations: results of the MAC (Montalcino Aortic Consortium). Circ Cardiovasc Genet 2016; 9 (06) 548-558
  • 29 Braverman AC, Moon MR, Geraghty P, Willing M, Bach C, Kouchoukos NT. Pregnancy after aortic root replacement in Loeys–Dietz syndrome: high risk of aortic dissection. Am J Med Genet A 2016; 170 (08) 2177-2180