Advertisement
Canadian Journal of Cardiology

Pregnancy and Thoracic Aortic Disease: Managing the Risks

  • Shaynah Wanga
    Affiliations
    Department of Cardiology, Academic Medical Center, Amsterdam, The Netherlands

    Department of Medical Biochemistry, Academic Medical Center, Amsterdam, The Netherlands

    Interuniversity Cardiology Institute of The Netherlands, Utrecht, The Netherlands
    Search for articles by this author
  • Candice Silversides
    Affiliations
    Division of Cardiology, Department of Medicine, Mount Sinai Hospital and University Health Network, University of Toronto, Toronto, Ontario, Canada

    Department of Medicine, Montreal Heart Institute, Université de Montréal, Montreal, Quebec, Canada
    Search for articles by this author
  • Annie Dore
    Affiliations
    Department of Medicine, Montreal Heart Institute, Université de Montréal, Montreal, Quebec, Canada
    Search for articles by this author
  • Vivian de Waard
    Affiliations
    Department of Medical Biochemistry, Academic Medical Center, Amsterdam, The Netherlands
    Search for articles by this author
  • Barbara Mulder
    Correspondence
    Corresponding author: Dr Barbara Mulder, Academic Medical Center, Meibergdreef 15, 1105AZ Amsterdam, The Netherlands. Tel.: +020-566-2193; fax: +020-697-1385.
    Affiliations
    Department of Cardiology, Academic Medical Center, Amsterdam, The Netherlands

    Interuniversity Cardiology Institute of The Netherlands, Utrecht, The Netherlands
    Search for articles by this author
Published:September 17, 2015DOI:https://doi.org/10.1016/j.cjca.2015.09.003

      Abstract

      The most common aortopathies in women of childbearing age are bicuspid aortic valve, coarctation of the aorta, Marfan syndrome, Ehlers-Danlos syndrome, Loeys-Dietz syndrome, SMAD3 aortopathy, Turner syndrome, and familial thoracic aneurysm and dissection. The hemodynamic and hormonal changes of pregnancy increase the risk of progressive dilatation or dissection of the aorta in these women. The presence of hypertension increases the risk further. Therefore, appropriate preconception counselling is advised. For women who become pregnant, serial follow-up by a specialized multidisciplinary team throughout pregnancy and postpartum period is required. In this review we discuss risk assessment and management strategies for women with aortopathies.

      Résumé

      Les aortopathies les plus courantes chez les femmes en âge de procréer sont les suivantes : bicuspidie valvulaire aortique, coarctation de l’aorte, syndrome de Marfan, syndrome d’Ehlers-Danlos, syndrome de Loeys-Dietz, aortopathie associée au gène SMAD3, syndrome de Turner et formes familiales d’anévrisme et de dissection de l’aorte thoracique. Les changements hormonaux et hémodynamiques associés à une grossesse augmentent le risque d’une dilatation progressive ou d’une dissection de l’aorte chez ces femmes. Ce risque s’accroît en présence d’hypertension. Par conséquent, des conseils appropriés avant la conception sont de mise. Chez les femmes qui sont enceintes, un suivi périodique par une équipe multidisciplinaire spécialisée tout au long de la grossesse et durant la période postpartum est nécessaire. Dans cette analyse, nous discutons de l’évaluation du risque et des stratégies de prise en charge des femmes présentant une aortopathie.
      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'

      Subscribe:

      Subscribe to Canadian Journal of Cardiology
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      References

        • la Chapelle CF
        • Schutte J.M.
        • Schuitemaker N.W.
        • et al.
        Maternal mortality attributable to vascular dissection and rupture in the Netherlands: a nationwide confidential enquiry.
        BJOG. 2012; 119: 86-93
        • Huisman C.M.
        • Zwart J.J.
        • Roos-Hesselink J.W.
        • Duvekot J.J.
        • van Roosmalen J.
        Incidence and predictors of maternal cardiovascular mortality and severe morbidity in the Netherlands: a prospective cohort study.
        PLoS One. 2013; 8: 1-10
        • Hytten F.
        Blood volume changes in normal pregnancy.
        Clin Haematol. 1985; 14: 601-612
        • Thornburg K.L.
        • Jacobson S.L.
        • Giraud G.D.
        • Morton M.J.
        Hemodynamic changes in pregnancy.
        Semin Perinatol. 2000; 24: 11-14
        • Manalo-Estrella P.
        • Barker A.E.
        Histopathologic findings in human aortic media associated with pregnancy.
        Arch Pathol. 1967; 83: 336-341
        • Nolte J.E.
        Arterial dissections associated with pregnancy.
        J Vasc Surg. 1995; 21: 515-520
        • Wolinsky H.
        Effects of estrogen and progestogen treatment on the response of the aorta of male rats to hypertension. Morphological and chemical studies.
        Circ Res. 1972; 30: 341-349
        • Easterling T.R.
        • Benedetti T.J.
        • Schmucker B.C.
        • Carlson K.
        • Millard S.P.
        Maternal hemodynamics and aortic diameter in normal and hypertensive pregnancies.
        Obstet Gynecol. 1991; 78: 1073-1077
        • Meijboom L.J.
        • Timmermans J.
        • Zwinderman A.H.
        • Engelfriet P.M.
        • Mulder B.J.
        Aortic root growth in men and women with the Marfan’s syndrome.
        Am J Cardiol. 2005; 96: 1441-1444
        • Donnelly R.T.
        • Pinto N.M.
        • Kocolas I.
        • Yetman A.T.
        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: 224-229
        • Meijboom L.J.
        • Vos F.E.
        • Timmermans J.
        • et al.
        Pregnancy and aortic root growth in the Marfan syndrome: a prospective study.
        Eur Heart J. 2005; 26: 914-920
        • Rossiter J.P.
        • Repke J.T.
        • Morales A.J.
        • Murphy E.A.
        • Pyeritz R.E.
        A prospective longitudinal evaluation of pregnancy in the Marfan syndrome.
        Am J Obstet Gynecol. 1995; 173: 1599-1606
        • McKellar S.H.
        • MacDonald R.J.
        • Michelena H.I.
        • Connolly H.M.
        • Sundt T.M.
        Frequency of cardiovascular events in women with a congenitally bicuspid aortic valve in a single community and effect of pregnancy on events.
        Am J Cardiol. 2011; 107: 96-99
        • Howard D.P.
        • Banerjee A.
        • Fairhead J.F.
        • et al.
        Population-based study of incidence and outcome of acute aortic dissection and premorbid risk factor control: 10-year results from the Oxford Vascular Study.
        Circulation. 2013; 127: 2031-2037
        • Nienaber C.A.
        • Fattori R.
        • Mehta R.H.
        • et al.
        Gender-related differences in acute aortic dissection.
        Circulation. 2004; 109: 3014-3021
        • Michelena H.I.
        • Khanna A.D.
        • Mahoney D.
        • et al.
        Incidence of aortic complications in patients with bicuspid aortic valves.
        JAMA. 2011; 306: 1104-1112
        • Gravholt C.H.
        • Landin-Wilhelmsen K.
        • Stochholm K.
        • et al.
        Clinical and epidemiological description of aortic dissection in Turner’s syndrome.
        Cardiol Young. 2006; 16: 430-436
        • Matura L.A.
        • Ho V.B.
        • Rosing D.R.
        • Bondy C.A.
        Aortic dilatation and dissection in Turner syndrome.
        Circulation. 2007; 116: 1663-1670
        • Jondeau G.
        • Detaint D.
        • Tubach F.
        • et al.
        Aortic event rate in the Marfan population: a cohort study.
        Circulation. 2012; 125: 226-232
        • Svensson L.G.
        • Kim K.H.
        • Lytle B.W.
        • Cosgrove D.M.
        Relationship of aortic cross-sectional area to height ratio and the risk of aortic dissection in patients with bicuspid aortic valves.
        J Thorac Cardiovasc Surg. 2003; 126: 892-893
        • Maccarrick G.
        • Black J.H.
        • Bowdin S.
        • et al.
        Loeys-Dietz syndrome: a primer for diagnosis and management.
        Genet Med. 2014; 16: 1-12
        • Germain D.P.
        • Herrera-Guzman Y.
        Vascular Ehlers-Danlos syndrome.
        Ann Génétique. 2004; 47: 1-9
        • Cozijnsen L.
        • Braam R.L.
        • Waalewijn R.A.
        • et al.
        What is new in dilatation of the ascending aorta? Review of current literature and practical advice for the cardiologist.
        Circulation. 2011; 123: 924-928
        • Erbel R.
        • Eggebrecht H.
        Aortic dimensions and the risk of dissection.
        Heart. 2006; 92: 137-142
        • Legget M.E.
        • Unger T.A.
        • O’Sullivan C.K.
        • et al.
        Aortic root complications in Marfan’s syndrome: identification of a lower risk group.
        Heart. 1996; 75: 389-395
        • Pacini L.
        • Digne F.
        • Boumendil A.
        • et al.
        Maternal complication of pregnancy in Marfan syndrome.
        Int J Cardiol. 2009; 136: 156-161
        • Lind J.
        • Wallenburg H.C.
        The Marfan syndrome and pregnancy: a retrospective study in a Dutch population.
        Eur J Obstet Gynecol Reprod Biol. 2001; 98: 28-35
        • Meijboom L.J.
        • Drenthen W.
        • Pieper P.G.
        • et al.
        Obstetric complications in Marfan syndrome.
        Int J Cardiol. 2006; 110: 53-59
        • Curry R.A.
        • Gelson E.
        • Swan L.
        • et al.
        Marfan syndrome and pregnancy: maternal and neonatal outcomes.
        BJOG. 2014; 121: 610-617
        • Hassan N.
        • Patenaude V.
        • Oddy L.
        • Abenhaim H.A.
        Pregnancy outcomes in Marfan syndrome: a retrospective cohort study.
        Am J Perinatol. 2015; 30: 123-130
        • Pyeritz R.E.
        Maternal and fetal complications of pregnancy in the Marfan syndrome.
        Am J Med. 1981; 71: 784-790
        • Lipscomb K.J.
        • Smith J.C.
        • Clarke B.
        • Donnai P.
        • Harris R.
        Outcome of pregnancy in women with Marfan’s syndrome.
        Br J Obstet Gynaecol. 1997; 104: 201-206
        • Omnes S.
        • Jondeau G.
        • Detaint D.
        • et al.
        Pregnancy outcomes among women with Marfan syndrome.
        Int J Gynaecol Obstet. 2013; 122: 219-223
        • Loeys B.L.
        • Schwarze U.
        • Holm T.
        • et al.
        Aneurysm syndromes caused by mutations in the TGF-beta receptor.
        N Engl J Med. 2006; 355: 788-798
        • Tran-Fadulu V.
        • Pannu H.
        • Kim D.H.
        • et al.
        Analysis of multigenerational families with thoracic aortic aneurysms and dissections due to TGFBR1 or TGFBR2 mutations.
        J Med Genet. 2009; 46: 607-613
        • Van de Laar I.M.
        • van der Linde D.
        • Oei E.H.
        • et al.
        Phenotypic spectrum of the SMAD3-related aneurysms-osteoarthritis syndrome.
        J Med Genet. 2012; 49: 47-57
        • Rudd N.L.
        • Nimrod C.
        • Holbrook K.A.
        • Byers P.H.
        Pregnancy complications in type IV Ehlers-Danlos Syndrome.
        Lancet. 1983; 1: 50-53
        • Pepin M.
        • Schwarze U.
        • Superti-Furga A.
        • Byers P.H.
        Clinical and genetic features of Ehlers-Danlos syndrome type IV, the vascular type.
        N Engl J Med. 2000; 342: 673-680
        • Murray M.L.
        • Pepin M.
        • Peterson S.
        • Byers P.H.
        Pregnancy-related deaths and complications in women with vascular Ehlers-Danlos syndrome.
        Genet Med. 2014; 16: 874-880
        • Karnis M.F.
        • Zimon A.E.
        • Lalwani S.I.
        • et al.
        Risk of death in pregnancy achieved through oocyte donation in patients with Turner syndrome: a national survey.
        Fertil Steril. 2003; 80: 498-501
        • Beauchesne L.M.
        • Connolly H.M.
        • Ammash N.M.
        • Warnes C.A.
        Coarctation of the aorta: outcome of pregnancy.
        J Am Coll Cardiol. 2001; 38: 1728-1733
        • Regalado E.S.
        • Guo D.C.
        • Estrera A.L.
        • Buja L.M.
        • Milewicz D.M.
        Acute aortic dissections with pregnancy in women with ACTA2 mutations.
        Am J Med Genet A. 2014; 164: 106-112
        • Yuan S.M.
        Bicuspid aortic valve in pregnancy.
        Taiwan J Obstet Gynecol. 2014; 53: 467-480
        • Sybert V.P.
        • McCauley E.
        Turner’s syndrome.
        N Engl J Med. 2004; 351: 1227-1238
        • Plunkett M.D.
        • Bond L.M.
        Staged repair of acute type I aortic dissection and coarctation in pregnancy.
        Ann Thorac Surg. 2000; 69: 1945-1947
        • Radhi J.M.
        • McKay R.
        • Tyrrell M.J.
        Fibromuscular dysplasia of the aorta presenting as multiple recurrent thoracic aneurysms.
        Int J Angiol. 1998; 7: 215-218
        • Ciura V.A.
        • Bromley A.
        • Wong J.
        A case of type A aortic dissection with underlying fibromuscular dysplasia.
        J Radiol Case Rep. 2011; 5: 22-28
        • Dawley B.
        • Ritchie A.
        Carotid and vertebral arterial fibromuscular dysplasia masquerading as severe preeclampsia: a case report.
        W V Med J. 2011; 107: 12-14
        • Cohen D.L.
        • Townsend R.R.
        • Clark T.W.
        Renal artery stenosis due to fibromuscular dysplasia in an 18-week pregnant woman.
        Obstet Gynecol. 2005; 105: 1232-1235
        • Lind J.
        The Marfan and Ehlers-Danlos syndromes and pregnancy.
        Erasmus MC: University Medical Center Rotterdam/Dissertation, 2000 (Available at:) (Accessed May 30, 2015)
        • Goland S.
        • Elkayam U.
        Cardiovascular problems in pregnant women with Marfan syndrome.
        Circulation. 2009; 119: 619-623
        • Immer F.F.
        • Bansi A.G.
        • Immer-Bansi A.S.
        • et al.
        Aortic dissection in pregnancy: analysis of risk factors and outcome.
        Ann Thorac Surg. 2003; 76: 309-314
        • Oyelese Y.
        • Ananth C.V.
        Postpartum hemorrhage: epidemiology, risk factors, and causes.
        Clin Obstet Gynecol. 2010; 53: 147-156
        • Zwart J.J.
        • Richters J.M.
        • Öry F.
        • et al.
        Severe maternal morbidity during pregnancy, delivery and puerperium in the Netherlands: a nationwide population-based study of 371 000 pregnancies.
        BJOG. 2008; 115: 842-850
        • Sorokin Y.
        • Johnson M.P.
        • Rogowski N.
        • Richardson D.A.
        • Evans M.I.
        Obstetric and gynecologic dysfunction in the Ehlers-Danlos syndrome.
        J Reprod Med. 1994; 39: 281-284
        • Lind J.
        • Wallenburg H.C.
        Pregnancy and the Ehlers-Danlos syndrome: a retrospective study in a Dutch population.
        Acta Obstet Gynecol Scand. 2002; 81: 293-300
        • Castori M.
        • Morlino S.
        • Dordoni C.
        • et al.
        Gynecologic and obstetric implications of the joint hypermobility syndrome (a.k.a. Ehlers-Danlos syndrome hypermobility type) in 82 Italian patients.
        Am J Med Genet A. 2012; 158: 2176-2182
        • Romo A.
        • Carceller R.
        • Tobajas J.
        Intrauterine growth retardation (IUGR): epidemiology and etiology.
        Pediatr Endocrinol Rev. 2009; 6: 332-336
        • Allen S.
        Epidemiology of premature rupture of the mebranes.
        Clin Obstet Gynecol. 1991; 34: 685-693
        • Goldenberg R.L.
        • Culhane J.F.
        • Iams J.D.
        • Romero R.
        Epidemiology and causes of preterm birth.
        Lancet. 2008; 371: 75-84
        • Regan L.
        • Rai R.
        Epidemiology and the medical causes of miscarriage.
        Baillieres Best Pract Res Clin Obstet Gynaecol. 2000; 14: 839-854
        • Ersbøll A.S.
        • Hedegaard M.
        • Søndergaard L.
        • Ersbøll M.
        • Johansen M.
        Treatment with oral beta-blockers during pregnancy complicated by maternal heart disease increases the risk of fetal growth restriction.
        BJOG. 2014; 121: 618-626
        • Xie R.H.
        • Guo Y.
        • Krewski D.
        • et al.
        Beta-blockers increase the risk of being born small for gestational age or of being institutionalised during infancy.
        BJOG. 2014; 121: 1090-1096
        • Meidahl Petersen K.
        • Jimenez-Solem E.
        • Andersen J.T.
        • et al.
        β-Blocker treatment during pregnancy and adverse pregnancy outcomes: a nationwide population-based cohort study.
        BMJ Open. 2012; 2 (pii e001185)
        • Barabas A.P.
        Ehlers-Danlos syndrome: associated with prematurity and premature rupture of foetal membranes; possible increase in incidence.
        BMJ. 1966; 2: 682-684
        • Parry S.
        • Strauss 3rd, J.F.
        Premature rupture of the fetal membranes.
        N Engl J Med. 1998; 338: 663-670
        • Regitz-Zagrosek V.
        • Blomstrom Lundqvist C.
        • Borghi C.
        • et al.
        ESC Guidelines on the management of cardiovascular diseases during pregnancy.
        Eur Heart J. 2011; 32: 3147-3197
        • Finkbohner R.
        • Johnston D.
        • Crawford E.S.
        • Coselli J.
        • Dianna M.
        Marfan syndrome. Long-term survival and complications after aortic aneurysm repair.
        Circulation. 1995; 91: 728-733
        • Judge D.P.
        • Dietz H.C.
        Marfan’s syndrome.
        Lancet. 2005; 366: 1965-1976
        • Warnes C.A.
        Bicuspid aortic valve and coarctation: two villains part of a diffuse problem.
        Heart. 2003; 89: 965-966
        • Tarani L.
        • Lampariello S.
        • Raguso G.
        • et al.
        Pregnancy in patients with Turner’s syndrome: six new cases and review of literature.
        Gynecol Endocrinol. 1998; 12: 83-87
        • Mujezinovic F.A.
        Procedure-related complications of amniocentesis and chorionic villous sampling: a systematic review.
        Obstet Gynecol. 2007; 110: 687-694
        • Lledó B.
        • Ten J.
        • Galán F.M.
        • Bernabeu R.
        Preimplantation genetic diagnosis of Marfan syndrome using multiple displacement amplification.
        Fertil Steril. 2006; 86: 949-955
        • Delvigne A.
        • Rozenberg S.
        Review of clinical course and treatment of ovarian hyperstimulation syndrome (OHSS).
        Hum Reprod Update. 2003; 9: 77-96
        • Hiratzka L.F.
        • Bakris G.L.
        • Beckman J.A.
        • et al.
        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.
        Circulation. 2010; 121: 1544-1579
        • Stadié R.
        • Geipel A.
        • Breuer J.
        • et al.
        Prenatal diagnosis of an isolated aneurysm of the ascending aorta.
        Ultrasound Obstet Gynecol. 2006; 27: 233-234
        • López-Ramón M.
        • García de la Calzada M.D.
        • Salazar-Mena J.
        Prenatal diagnosis of ascending aorta aneurysm.
        Rev Esp Cardiol. 2010; 63: 1194
        • Gersony D.R.
        • McClaughlin M.A.
        • Jin Z.
        • Gersony W.M.
        The effect of beta-blocker therapy on clinical outcome in patients with Marfan’s syndrome: a meta-analysis.
        Int J Cardiol. 2007; 114: 303-308
        • Gao L.
        • Mao Q.
        • Wen D.
        • et al.
        The effect of beta-blocker therapy on progressive aortic dilatation in children and adolescents with Marfan’s syndrome: a meta-analysis.
        Acta Paediatr. 2011; 100: e101-e105
        • Ong K.T.
        • Perdu J.
        • De Backer J.
        • et al.
        Effect of celiprolol on prevention of cardiovascular events in vascular Ehlers-Danlos syndrome: a prospective randomised, open, blinded-endpoints trial.
        Lancet. 2010; 376: 1476-1484
        • Alwan S.
        • Polifka J.E.
        • Friedman J.M.
        Angiotensin II receptor antagonist treatment during pregnancy.
        Birth Defects Res A Clin Mol Teratol. 2005; 73: 123-130
        • Prisant L.M.
        • Mawulawde K.
        • Kapoor D.
        • Joe C.
        Coarctation of the aorta: a secondary cause of hypertension.
        J Clin Hypertens (Greenwich). 2004; 6 (352): 347-350
        • Parlakgumus H.A.
        • Haydardedeoglu B.
        • Alkan O.
        Aortic dissection accompanied by preeclampsia and preterm labor.
        J Obstet Gynaecol Res. 2010; 36: 1121-1124
        • Huang J.
        • Liu H.
        • Ding Y.L.
        Two cases of acute aortic dissection following preeclampsia in non-Marfan patients.
        Chin Med J (Engl). 2012; 125: 2073-2075
        • Silversides C.K.
        • Kiess M.
        • Beauchesne L.
        • et al.
        Canadian Cardiovascular Society 2009 Consensus Conference on the management of adults with congenital heart disease: outflow tract obstruction, coarctation of the aorta, tetralogy of Fallot, Ebstein anomaly, and Marfan’s syndrome.
        Can J Cardiol. 2010; 26: e80-97
        • Boodhwani M.
        • Andelfinger G.
        • Leipsic J.
        • et al.
        Canadian Cardiovascular Society position statement on the management of thoracic aortic disease.
        Can J Cardiol. 2014; 30: 577-589
        • Reitman E.
        • Flood P.
        Anaesthetic considerations for non-obstetric surgery during pregnancy.
        Br J Anaesth. 2011; 107: i72-i78
        • John A.S.
        • Gurley F.
        • Schaff H.V.
        • et al.
        Cardiopulmonary bypass during pregnancy.
        Ann Thorac Surg. 2011; 91: 1191-1196
        • Arnoni R.T.
        • Arnoni A.S.
        • Bonini R.C.
        • et al.
        Risk factors associated with cardiac surgery during pregnancy.
        Ann Thorac Surg. 2003; 76: 1605-1608
        • Lee S.W.
        • Khaw K.S.
        • Ngan Kee W.D.
        • et al.
        Haemodynamic effects from aortocaval compression at different angles of lateral tilt in non-labouring term pregnant women.
        Br J Anaesth. 2012; 109: 950-956