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Current Vascular Pharmacology

Editor-in-Chief

ISSN (Print): 1570-1611
ISSN (Online): 1875-6212

Systematic Review Article

Management and Outcomes of Aortic Dissection in Pregnancy with Marfan Syndrome: A Systematic Review

Author(s): Hassan Al-Thani, Ahammed Mekkodathil and Ayman El-Menyar*

Volume 18, Issue 3, 2020

Page: [282 - 293] Pages: 12

DOI: 10.2174/1570161117666190408164612

Price: $65

Abstract

Background: In Marfan Syndrome (MFS), aortic dilatation is one of the main cardiovascular manifestations which deteriorate due to the physiological changes during pregnancy. We aimed to assess the up-to-date management and outcomes of aortic root dilation and dissection (AoD) in pregnancy with MFS.

Patients and Methods: A systematic review was conducted and reported according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA). Original studies published between January 1, 2001 and December 31, 2018 and which described the management and/or outcomes of AoD during or after pregnancy in women with MFS were included. Literature searches were conducted. The PubMed search was performed using terms "Marfan Syndrome" [Mesh] and "Pregnancy" [Mesh] whereas the Google Scholar search was for “Marfan” and “Pregnancy”, all words anywhere in the article.

Results: The literature search yielded 177 articles on PubMed and 13,900 articles on Google Scholar. Assessment of full-text articles for eligibility after removal of duplicates from both databases yielded 12 eligible studies to be included in the final review.

Conclusion: Women with MFS are at high risk of aortic dissection during pregnancy and women with aortic root 41-45 mm should consider avoiding pregnancy. Guideline-specific management of aortic aneurysms in pregnancy will reduce the risk of dissection. Diagnosis and Management of MFS need a multidisciplinary approach and team that should start working early in pregnancy. Further studies are needed to optimize medical and surgical approaches in addition to preconception counselling in highrisk subjects.

Keywords: Pregnancy, CVD, Marfan syndrome, aortic root, aneurysm, dissection.

Graphical Abstract
[1]
Smith K, Gros B. Pregnancy-related acute aortic dissection in Marfan syndrome: A review of the literature. Congenit Heart Dis 2017; 12(3): 251-60.
[http://dx.doi.org/10.1111/chd.12465] [PMID: 28371362]
[2]
Kim SY, Wolfe DS, Taub CC. Cardiovascular outcomes of pregnancy in Marfan’s syndrome patients: A literature review. Congenit Heart Dis 2018; 13(2): 203-9.
[http://dx.doi.org/10.1111/chd.12546] [PMID: 29063738]
[3]
Judge DP, Dietz HC. Marfan’s syndrome. Lancet 2005; 366(9501): 1965-76.
[http://dx.doi.org/10.1016/S0140-6736(05)67789-6] [PMID: 16325700]
[4]
Rosenberger LH, Adams JD, Kern JA, Tracci MC, Angle JF, Cherry KJ. Complicated postpartum type B aortic dissection and endovascular repair. Obstet Gynecol 2012; 119(2 Pt 2): 480-3.
[http://dx.doi.org/10.1097/AOG.0b013e3182390622] [PMID: 22270446]
[5]
Pyeritz RE. Maternal and fetal complications of pregnancy in the Marfan syndrome. Am J Med 1981; 71(5): 784-90.
[http://dx.doi.org/10.1016/0002-9343(81)90365-X] [PMID: 7304650]
[6]
Ammash NM, Sundt TM, Connolly HM. Marfan syndrome-diagnosis and management. Curr Probl Cardiol 2008; 33(1): 7-39.
[http://dx.doi.org/10.1016/j.cpcardiol.2007.10.001] [PMID: 18155514]
[7]
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(6): 253-62.
[http://dx.doi.org/10.1097/CRD.0b013e3181bb83d3] [PMID: 19829173]
[8]
Zhang M, Zhou Y, Peng Y, Jin L. Two rare missense mutations in the fibrillin‑1 gene associated with atypical cardiovascular manifestations in a Chinese patient affected by Marfan syndrome. Mol Med Rep 2018; 18(1): 877-81.
[http://dx.doi.org/10.3892/mmr.2018.9041] [PMID: 29845260]
[9]
Isekame Y, Gati S, Aragon-Martin JA, Bastiaenen R, Kondapally Seshasai SR, Child A. Cardiovascular management of adults with Marfan Syndrome. Eur Cardiol 2016; 11(2): 102-10.
[http://dx.doi.org/10.15420/ecr/2016:19:2] [PMID: 30310455]
[10]
Zamłyński J, Olejek A, Gajewska-Kucharek A, et al. Three pregnancies in a Marfan syndrome patient after a mitral and tricuspid valve surgery. Ginekol Pol 2013; 84(4): 314-7.
[http://dx.doi.org/10.17772/gp/1583] [PMID: 23700867]
[11]
Baumgartner H, Bonhoeffer P, De Groot NM, et al. Task force on the management of grown-up congenital heart disease of the European society of cardiology (ESC); association for European paediatric cardiology (AEPC); ESC committee for practice guidelines (CPG).. ESC guidelines for the management of grown-up congenital heart disease (new version 2010). Eur Heart J 2010; 31(23): 2915-57.
[http://dx.doi.org/10.1093/eurheartj/ehq249] [PMID: 20801927]
[12]
Burman ED, Keegan J, Kilner PJ. Aortic root measurement by cardiovascular magnetic resonance: specification of planes and lines of measurement and corresponding normal values. Circ Cardiovasc Imaging 2008; 1(2): 104-13.
[http://dx.doi.org/10.1161/CIRCIMAGING.108.768911] [PMID: 19808527]
[13]
Silversides CK, 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(3): e80-97.
[http://dx.doi.org/10.1016/S0828-282X(10)70355-X] [PMID: 20352138]
[14]
Hiratzka LF, Bakris GL, Beckman JA, 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. 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): 27-129.
[http://dx.doi.org/10.1016/j.jacc.2010.02.015] [PMID: 20359588]
[15]
Gott VL, Greene PS, Alejo DE, et al. Replacement of the aortic root in patients with Marfan’s syndrome. N Engl J Med 1999; 340(17): 1307-13.
[http://dx.doi.org/10.1056/NEJM199904293401702] [PMID: 10219065]
[16]
Tran TP, Khoynezhad A. Current management of type B aortic dissection. Vasc Health Risk Manag 2009; 5(1): 53-63.
[PMID: 19436678]
[17]
Loeys BL, Dietz HC, Braverman AC, et al. The revised Ghent nosology for the Marfan syndrome. J Med Genet 2010; 47(7): 476-85.
[http://dx.doi.org/10.1136/jmg.2009.072785] [PMID: 20591885]
[18]
Study Quality Assessment Tools | National Heart, Lung, And Blood Institute (NHLBI) [online] 2019. [cited 2019] Available at:. https://www.nhlbi.nih.gov/health-topics/study-quality-assessment-tools
[19]
Minsart AF, Mongeon FP, Laberge AM, Morin F, Dore A, Leduc L. Obstetric and cardiac outcomes in women with Marfan syndrome and an aortic root diameter ≤ 45mm. Eur J Obstet Gynecol Reprod Biol 2018; 230: 68-72.
[http://dx.doi.org/10.1016/j.ejogrb.2018.09.012] [PMID: 30243228]
[20]
Lim JCE, Cauldwell M, Patel RR, et al. Management of Marfan Syndrome during pregnancy: A real world experience from a Joint Cardiac Obstetric Service. Int J Cardiol 2017; 243: 180-4.
[http://dx.doi.org/10.1016/j.ijcard.2017.05.077] [PMID: 28606654]
[21]
Liu YY, Li HY, Jiang WJ, et al. Treatment of patients with aortic disease during pregnancy and after delivery. J Int Med Res 2017; 45(4): 1359-68.
[http://dx.doi.org/10.1177/0300060517711088] [PMID: 28587539]
[22]
Kuperstein R, Cahan T, Yoeli-Ullman R, Ben Zekry S, Shinfeld A, Simchen MJ. Risk of aortic dissection in pregnant patients with the marfan syndrome. Am J Cardiol 2017; 119(1): 132-7.
[http://dx.doi.org/10.1016/j.amjcard.2016.09.024] [PMID: 27788933]
[23]
Hassan N, Patenaude V, Oddy L, Abenhaim HA. Pregnancy outcomes in Marfan syndrome: a retrospective cohort study. Am J Perinatol 2015; 32(2): 123-30.
[http://dx.doi.org/10.1055/s-0034-1376179] [PMID: 24896139]
[24]
Curry RA, Gelson E, Swan L, et al. Marfan syndrome and pregnancy: maternal and neonatal outcomes. BJOG 2014; 121(5): 610-7.
[http://dx.doi.org/10.1111/1471-0528.12515] [PMID: 24418012]
[25]
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): 392-8.
[http://dx.doi.org/10.1213/ANE.0b013e3182768f78] [PMID: 23302979]
[26]
Omnes S, Jondeau G, Detaint D, et al. Pregnancy outcomes among women with Marfan syndrome. Int J Gynaecol Obstet 2013; 122(3): 219-23.
[http://dx.doi.org/10.1016/j.ijgo.2013.04.013] [PMID: 23810486]
[27]
Katsuragi S, Ueda K, Yamanaka K, et al. Pregnancy-associated aortic dilatation or dissection in Japanese women with Marfan syndrome. Circ J 2011; 75(11): 2545-51.
[http://dx.doi.org/10.1253/circj.CJ-11-0465] [PMID: 21817813]
[28]
Pacini L, Digne F, Boumendil A, et al. Maternal complication of pregnancy in Marfan syndrome. Int J Cardiol 2009; 136(2): 156-61.
[http://dx.doi.org/10.1016/j.ijcard.2008.04.035] [PMID: 18632169]
[29]
Meijboom LJ, Drenthen W, Pieper PG, et al. ZAHARA investigators. Obstetric complications in Marfan syndrome. Int J Cardiol 2006; 110(1): 53-9.
[http://dx.doi.org/10.1016/j.ijcard.2005.07.017] [PMID: 16236373]
[30]
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.
[http://dx.doi.org/10.1016/S0301-2115(01)00314-1] [PMID: 11516796]
[31]
Murdoch JL, Walker BA, Halpern BL, Kuzma JW, McKusick VA. Life expectancy and causes of death in the Marfan syndrome. N Engl J Med 1972; 286(15): 804-8.
[http://dx.doi.org/10.1056/NEJM197204132861502] [PMID: 5011789]
[32]
Finkbohner R, Johnston D, Crawford ES, Coselli J, Milewicz DM. Marfan syndrome. Long-term survival and complications after aortic aneurysm repair. Circulation 1995; 91(3): 728-33.
[http://dx.doi.org/10.1161/01.CIR.91.3.728] [PMID: 7828300]
[33]
Silverman DI, Burton KJ, Gray J, et al. Life expectancy in the Marfan syndrome. Am J Cardiol 1995; 75(2): 157-60.
[http://dx.doi.org/10.1016/S0002-9149(00)80066-1] [PMID: 7810492]
[34]
Cameron DE, Alejo DE, Patel ND, et al. Aortic root replacement in 372 Marfan patients: evolution of operative repair over 30 years. Ann Thorac Surg 2009; 87(5): 1344-9.
[http://dx.doi.org/10.1016/j.athoracsur.2009.01.073] [PMID: 19379862]
[35]
Pearson GD, Devereux R, Loeys B, et al. National heart, lung, and blood institute and national marfan foundation working group. report of the national heart, lung, and blood institute and national marfan foundation working group on research in marfan syndrome and related disorders. Circulation 2008; 118(7): 785-91.
[http://dx.doi.org/10.1161/CIRCULATIONAHA.108.783753] [PMID: 18695204]
[36]
Vahanian A, Baumgartner H, Bax J, et al. Task force on the management of valvular hearth disease of the European society of cardiology; esc committee for practice guidelines. guidelines on the management of valvular heart disease: the task force on the management of valvular heart disease of the European society of cardiology. Eur Heart J 2007; 28(2): 230-68.
[PMID: 17259184]
[37]
Milewicz DM, Dietz HC, Miller DC. Treatment of aortic disease in patients with Marfan syndrome. Circulation 2005; 111(11): e150-7.
[http://dx.doi.org/10.1161/01.CIR.0000155243.70456.F4] [PMID: 15781745]
[38]
Cañadas V, Vilacosta I, Bruna I, Fuster V. Marfan syndrome. Part 2: treatment and management of patients. Nat Rev Cardiol 2010; 7(5): 266-76.
[http://dx.doi.org/10.1038/nrcardio.2010.31] [PMID: 20351702]
[39]
Hosn MA, Goffredo P, Zavala J, et al. Analysis of aortic growth rates in uncomplicated type B dissection. Ann Vasc Surg 2018; 48: 133-40.
[http://dx.doi.org/10.1016/j.avsg.2017.09.023] [PMID: 29217443]
[40]
Evangelista A, Flachskampf FA, Erbel R, et al. European Association of Echocardiography Document Reviewers. Echocardiography in aortic diseases: EAE recommendations for clinical practice. Eur J Echocardiogr 2010; 11(8): 645-58.
[http://dx.doi.org/10.1093/ejechocard/jeq056] [PMID: 20823280]
[41]
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): 1599-606.
[http://dx.doi.org/10.1016/0002-9378(95)90655-X] [PMID: 7503207]
[42]
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): 201-6.
[http://dx.doi.org/10.1111/j.1471-0528.1997.tb11045.x] [PMID: 9070139]
[43]
Yankah CA, Weng Y, Hetzer R, Eds. Aortic root surgery: The biological solution. Springer-Verlag Berlin Heidelberg 2010; pp. 116-46.
[http://dx.doi.org/10.1007/978-3-7985-1869-8]
[44]
Price J, Magruder JT, Young A, et al. Long-term outcomes of aortic root operations for Marfan syndrome: A comparison of Bentall versus aortic valve-sparing procedures. J Thorac Cardiovasc Surg 2016; 151(2): 330-6.
[http://dx.doi.org/10.1016/j.jtcvs.2015.10.068] [PMID: 26704057]
[45]
Nardi P, Pellegrino A, Versaci F, et al. Aortic root surgery in Marfan syndrome: Bentall procedure with the composite mechanical valved conduit versus aortic valve reimplantation with Valsalva graft. J Cardiovasc Med (Hagerstown) 2010; 11(9): 648-54.
[http://dx.doi.org/10.2459/JCM.0b013e3283379998] [PMID: 20150820]

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