2026 SOAP Annual Meeting

New Post-conceptional Diagnosis of Marfan, Bicuspid Aortic Valve, and Ascending Aorta Dilation: Case Considerations and Multidisciplinary Management Through Pregnancy and Delivery
Liliana Goelkel-Garcia, MD; Roberto C Blanco, MD; Sadaf Mirkarimi, MD; Iryna Chugaieva, MD
Department of Anesthesiology, University of Minnesota

MARFAN SYNDROME - BACKGROUND

•Autosomal dominant disorder of connective tissue.

•Involvement of cardiovascular, skeletal, ocular, and other systems.

•US prevalence: 1 in 5,000, both genders equally affected.

•80% of patients have cardiovascular involvement, including aortic dilatation, aortic regurgitation, and mitral and tricuspid valve prolapse with or without regurgitation.

•Leading cause of morbidity and mortality is aortic dissection

•Risk of aortic dissection, even without prior aortic dilation

•Higher risk with aortic diameter >4.5 cm

•Often remains undiagnosed prior to pregnancy

•Hemodynamic changes of pregnancy increase aortic wall stress

CASE PRESENTATION

•20-year-old primigravida with marfanoid habitus

•1st trimester: confirmed Marfan syndrome

•Bicuspid aortic valve, mild AI

•Aortic root dilation: 4.5 cm (Z +5.1)

•Ascending aorta: 3.3 cm (Z +2.0)

•PFO (L→R), mildly dilated LV

•Multidisciplinary Management, Metoprolol (HR <100, BP <130/90), Serial echocardiography (q4–8 weeks)

•Planned postpartum aortic root surgery

•Pregnancy complication: Fetal growth restriction (EFW 11%, AC 3%)

•Delivery: Scheduled C-section at 37w3d with CV surgery team on standby. Combined spinal-epidural anesthesia

•Tight hemodynamic control (esmolol, nicardipine, phenylephrine)

•Outcome: Uneventful delivery, EBL 725 mL. No transfusion required

•Post-op CVICU monitoring → discharged home

LEARNING POINTS

•Dissection risk is higher when aortic diameter >4.5 cm

•Preconception counseling:

•Avoidance of pregnancy

•Prophylactic aortic repair

•Beta-blockers may reduce aortic dilation but require fetal growth monitoring

•Vaginal delivery in low-risk cases

•Cesarean recommended with larger aortic diameters or added risk factors

•Intrapartum goal: minimize hemodynamic stress

•Multidisciplinary management is critical for optimal maternal and fetal outcomes

REFERENCES

•Lipscomb K.J., Smith J.C., et al. Outcome of pregnancy in women with Marfan's syndrome. Br J Obstet Gynaecol. 1997; 104: 201-206

•Lind J, et al. The Marfan syndrome and pregnancy: a retrospective study in a Dutch population. Eur J Obstet Gynecol Reprod Biol. 2001 Sep;98(1):28-35. doi: 10.1016/s0301-2115(01)00314-1. PMID: 11516796.

•Rahman J, et al. Obstetric and gynecologic complications in women with Marfan syndrome. J Reprod Med. 2003 Sep;48(9):723-8. PMID: 14562639.

•Elkayam U, et al. Cardiovascular problems in pregnant women with Marfan syndrome. Ann Intern Med. 1995 Jul 15;123(2):117-22. doi: 10.7326/0003-4819-123-2-199507150-00007. PMID: 7778824.

•Cox DA, et al. Management of the pregnant woman with Marfan syndrome complicated by ascending aorta dilation. Arch Gynecol Obstet. 2014 Oct;290(4):797-802. doi: 10.1007/s00404-014-3307-4. Epub 2014 Jun 26. PMID: 24966118.

•Goland S, et al. Pregnancy and Marfan syndrome. Ann Cardiothorac Surg. 2017 Nov;6(6):642-653. doi: 10.21037/acs.2017.10.07. PMID: 29270376; PMCID: PMC5721114.]

Image: Baban A, et al. Unique Features of Cardiovascular Involvement and Progression in Children with Marfan Syndrome Justify Dedicated Multidisciplinary Care. Journal of Cardiovascular Development and Disease. 2024; 11(4):114. https://doi.org/10.3390/jcdd11040114].

2026 SOAP Annual Meeting

New Post-conceptional Diagnosis of Marfan, Bicuspid Aortic Valve, and Ascending Aorta Dilation: Case Considerations and Multidisciplinary Management Through Pregnancy and Delivery.

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