Rupture of a Type B Aortic Dissection in a Postpartum Patient with Marfan Syndrome

Abstract

Background Pregnant women with Marfan syndrome (MFS) are known to be at increased risk of aortic dissection; however, cases of aortic rupture are extremely rare. There is lack of consensus on the exact site and size of aortic diameter measurement that increases this risk, and whether this applies to both Type A and Type B dissections.

Case A 23-year-old G2P1001 with known Marfan syndrome who underwent an uncomplicated antepartum and intrapartum course. She experienced persistent backache 10 days postpartum that led to the diagnosis of Stanford Type B dissection. The patient was hospitalized for close observation. Dissection progressed to aortic rupture within 24 hours that required emergent thoracic endovascular aortic repair. She had an uncomplicated postoperative course.

Conclusion Our report demonstrates rupture of a known aortic dissection within a very short time in the postpartum period. The case highlights the importance of patient education and close surveillance especially in the postpartum period. It also brings home the value of imaging of the whole aorta rather than focusing on the ascending alone. Multidisciplinary care and timely diagnosis and intervention likely led to the favorable outcome in our case.

Keywords

Marfan syndrome - pregnancy - aortic rupture - Type B aortic dissection

• References

• 1 Judge DP, Dietz HC. Marfan's syndrome. Lancet 2005; 366 (9501): 1965-1976
• 2 Collins MJ, Dev V, Strauss BH, Fedak PW, Butany J. Variation in the histopathological features of patients with ascending aortic aneurysms: a study of 111 surgically excised cases. J Clin Pathol 2008; 61 (04) 519-523
• 3 Trotter SE, Olsen EG. Marfan's disease and Erdheim's cystic medionecrosis. A study of their pathology. Eur Heart J 1991; 12 (01) 83-87
• 4 Manalo-Estrella P, Barker AE. Histopathologic findings in human aortic media associated with pregnancy. Arch Pathol 1967; 83 (04) 336-341
• 5 Goland S, Elkayam U. Cardiovascular problems in pregnant women with Marfan syndrome. Circulation 2009; 119 (04) 619-623
• 6 Goland S, Elkayam U. Pregnancy and Marfan syndrome. Ann Cardiothorac Surg 2017; 6 (06) 642-653
• 7 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
• 8 Pyeritz RE. Maternal and fetal complications of pregnancy in the Marfan syndrome. Am J Med 1981; 71 (05) 784-790
• 9 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
• 10 Kim SY, Wolfe DS, Taub CC. Cardiovascular outcomes of pregnancy in Marfan's syndrome patients: a literature review. Congenit Heart Dis 2018; 13 (02) 203-209
• 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 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. Circulation 2010; 121 (13) e266-e369
• 13 Meijboom LJ, Vos FE, Timmermans J, Boers GH, Zwinderman AH, Mulder BJ. Pregnancy and aortic root growth in the Marfan syndrome: a prospective study. Eur Heart J 2005; 26 (09) 914-920
• 14 Omnes S, Jondeau G, Detaint D. , et al. Pregnancy outcomes among women with Marfan syndrome. Int J Gynaecol Obstet 2013; 122 (03) 219-223
• 15 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 (01) 132-137
• 16 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 ≤ 45 mm. Eur J Obstet Gynecol Reprod Biol 2018; 230: 68-72
• 17 Erbel R, Aboyans V, Boileau C. , et al; ESC Committee for Practice Guidelines; The Task Force for the Diagnosis and Treatment of Aortic Diseases of the European Society of Cardiology (ESC). 2014 ESC Guidelines on the diagnosis and treatment of aortic diseases: document covering acute and chronic aortic diseases of the thoracic and abdominal aorta of the adult. Eur Heart J 2014; 35 (41) 2873-2926
• 18 Boodhwani M, Andelfinger G, Leipsic J. , et al; Canadian Cardiovascular Society. Canadian Cardiovascular Society position statement on the management of thoracic aortic disease. Can J Cardiol 2014; 30 (06) 577-589
• 19 Regitz-Zagrosek V, Roos-Hesselink JW, Bauersachs J. , et al; ESC Scientific Document Group. 2018 ESC Guidelines for the management of cardiovascular diseases during pregnancy. Eur Heart J 2018; 39 (34) 3165-3241
• 20 Loeys BL, Dietz HC, Braverman AC. , et al. The revised Ghent nosology for the Marfan syndrome. J Med Genet 2010; 47 (07) 476-485
• 21 Wright M, Connolly H. Management of Marfan syndrome and related disorders. In: Post T. , ed. UpToDate Website. https://www.uptodate.com/contents/management-of-marfan-syndrome-and-related-disorders . Accessed April 13, 2019
• 22 Roman MJ, Rosen SE, Kramer-Fox R, Devereux RB. Prognostic significance of the pattern of aortic root dilation in the Marfan syndrome. J Am Coll Cardiol 1993; 22 (05) 1470-1476
• 23 Goldstein SA, Evangelista A, Abbara S. , et al. Multimodality imaging of diseases of the thoracic aorta in adults: from the American Society of Echocardiography and the European Association of Cardiovascular Imaging: endorsed by the Society of Cardiovascular Computed Tomography and Society for Cardiovascular Magnetic Resonance. J Am Soc Echocardiogr 2015; 28 (02) 119-182
• 24 Davies RR, Gallo A, Coady MA. , et al. Novel measurement of relative aortic size predicts rupture of thoracic aortic aneurysms. Ann Thorac Surg 2006; 81 (01) 169-177
• 25 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) 2545-2551
• 26 Smith K, Gros B. Pregnancy-related acute aortic dissection in Marfan syndrome: a review of the literature. Congenit Heart Dis 2017; 12 (03) 251-260
• 27 Elkayam U, Ostrzega E, Shotan A, Mehra A. Cardiovascular problems in pregnant women with the Marfan syndrome. Ann Intern Med 1995; 123 (02) 117-122
• 28 Roman MJ, Pugh NL, Hendershot TP. , et al; GenTAC Investigators Dietz Harry C, Habashi Jennifer, Prakash Siddharth K, Maslen Cheryl L, Song Howard K, Bavaria Joseph E, Milewski Karianna, Weinsaft Jonathan W, McDonnell Nazli, Asch Federico M, Tolunay H Eser, Desvigne-Nickens Patrice, Tseng Hung, Kroner Barbara 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
• 29 de Beaufort HWL, Trimarchi S, Korach A. , et al. Aortic dissection in patients with Marfan syndrome based on the IRAD data. Ann Cardiothorac Surg 2017; 6 (06) 633-641
• 30 Umaña JP, Miller DC, Mitchell RS. What is the best treatment for patients with acute type B aortic dissections--medical, surgical, or endovascular stent-grafting?. Ann Thorac Surg 2002; 74 (05) S1840-S1843 , discussion S1857–S1863