Acute Aortic Dissection in the Third Trimester of Pregnancy as an Initial Presentation of Marfan Syndrome

Authors

  • Marta Reis Santos Department of Obstetrics, Gynecology and Reproductive Medicine. Hospital de Santa Maria. Unidade Local de Saúde de Santa Maria. Lisboa. Portugal. https://orcid.org/0009-0005-5923-3540
  • Alexandre Valentim Lourenço Department of Obstetrics, Gynecology and Reproductive Medicine. Hospital de Santa Maria. Unidade Local de Saúde de Santa Maria. Lisboa. Portugal.
  • Maria Pulido Valente Department of Obstetrics, Gynecology and Reproductive Medicine. Hospital de Santa Maria. Unidade Local de Saúde de Santa Maria. Lisboa. Portugal. https://orcid.org/0000-0003-1173-6171

DOI:

https://doi.org/10.20344/amp.22287

Keywords:

Aortic Aneurysm, Aortic Dissection, Marfan Syndrome, Pregnancy Complications, Cardiovascular, Pregnancy Trimester, Third

Abstract

Aortic dissection is rare and more common in men. In women, it is more frequent during pregnancy, especially in the third trimester. We present the case of a 30-year-old pregnant woman diagnosed with type B aortic dissection at 29 weeks of gestation. Following the diagnosis, fetal maturation was initiated, and the patient was transferred to a tertiary care center. Due to uncontrolled hypertension and persistent pain, and after evaluating the maternal and fetal risk-benefit, a cesarean section was performed at 29 weeks and six days. During the postoperative period, a multi-drug regimen was required for blood pressure control, but the patient recovered and was discharged. Genetic testing, prompted by a family history of sudden death (brother) and aortic dissection (father), identified a mutation in the FBN1 gene, confirming Marfan syndrome. This case highlights the importance of comprehensive patient history, the challenges of aortic dissection during pregnancy, and the need for a multidisciplinary approach in these cases.

Downloads

References

Bossone E, Carbone A, Eagle KA. Gender differences in acute aortic dissection. J Pers Med. 2022;12:1148.

Yuan S. Aortic dissection during pregnancy: a difficult clinical scenario. Clin Cardiol. 2013;36:576-584.

Meng X, Han J, Wang L, Wu Q. Aortic dissection during pregnancy and postpartum. J Card Surg. 2021;36:2510-7.

Russo M, Boehler-Tatman M, Albright C, David C, Kennedy L, Roberts AW, et al. Aortic dissection in pregnancy and the postpartum period. Semin Vasc Surg. 2022;35:60-8.

Rimmer L, Heyward-Chaplin J, South M, Gouda M, Bashir M. Acute aortic dissection during pregnancy: trials and tribulations. J Card Surg. 2021;36:1799-805.

Mazzolai L, Teixido-Tura G, Lanzi S, Boc V, Bossone E, Brodmann M, et al. 2024 ESC guidelines for the management of peripheral arterial and aortic diseases. Eur Heart J. 2024;45:3538-700.

McNally E. Aortic dissection with pregnancy—anticipating prepartum and postpartum risk. JAMA Cardiol. 2021;6:66-7.

Regitz-Zagrosek V, Roos-Hesselink JW, Bauersachs J, Blomström-Lundqvist C, Cífková R, De Bonis M, et al. 2018 ESC Guidelines for the management of cardiovascular diseases during pregnancy. Eur Heart J. 2018;39:3165-241.

Smith K, Gros B. Pregnancy-related acute aortic dissection in Marfan syndrome: a review of the literature. Congenit Heart Dis. 2017;12:251-60.

Kano HD, Sun W. Risk factors for aortic dissection. US Cardiol. 2023;17:e17.

Rogers AM, Hermann LK, Booher AM, Nienaber CA, Williams DM, Kazerooni EA, et al. Sensitivity of the aortic dissection detection risk score, a novel guideline-based tool for identification of acute aortic dissection at initial presentation. Circulation. 2011;123:2213-8.

Czerny M, Grabenwöger M, Berger T, Aboyans V, Della Corte A, Chen EP, et al. EACTS/STS guidelines for diagnosing and treating acute and chronic syndromes of the aortic organ. Ann Thorac Surg. 2024;118:5-115.

Committee Opinion No. 723: guidelines for diagnostic imaging during pregnancy and lactation. Obstet Gynecol. 2017;130:e210-6.

Gersony DR, McClaughlin MA, Jin Z, Gersony WM. The effect of beta-blocker therapy on clinical outcome in patients with Marfan’s syndrome: a metaanalysis. Int J Cardiol. 2007;114:303-8.

Roberts EA, Pistner A, Osobamiro O, Banning S, Shalhub S, Albright C, et al. Beta-blocker use during pregnancy correlates with less aortic root dilatation in patients with Marfan’s syndrome. Aorta. 2023;11:63-70.

Guimarães T, Magalhães A, Veiga A, Fiuza M, Ávila W, Pinto FJ. Cardiopatia e gravidez – o estado da arte. Rev Port Cardiol. 2019;38:373-83.

Sibai BM. Etiology and management of postpartum hypertension-preeclampsia. Am J Obstet Gynecol. 2012;206:470-5.

Downloads

Published

2025-03-26

How to Cite

1.
Reis Santos M, Valentim Lourenço A, Pulido Valente M. Acute Aortic Dissection in the Third Trimester of Pregnancy as an Initial Presentation of Marfan Syndrome. Acta Med Port [Internet]. 2025 Mar. 26 [cited 2025 Apr. 1];. Available from: https://actamedicaportuguesa.com/revista/index.php/amp/article/view/22287

Issue

Section

Case Report