A 25-year-old woman with Marfan syndrome wishes to conceive. Echocardiography shows an aortic root diameter of 4.8 cm. What is the MOST appropriate advice?
- A Conceive after elective aortic root replacement, as root diameter above 4.5 cm carries high risk of dissection in pregnancy ✓
- B Pregnancy is safe with six-monthly echocardiographic surveillance
- C Proceed with pregnancy but deliver by elective caesarean at 34 weeks
- D Proceed with pregnancy on labetalol and plan vaginal delivery with epidural analgesia
Correct answer: A. Conceive after elective aortic root replacement, as root diameter above 4.5 cm carries high risk of dissection in pregnancy
Explanation
In Marfan syndrome, an aortic root diameter greater than 4.5 cm places the patient in modified WHO class IV, with maternal mortality risk of 50 percent or more from aortic dissection, and pregnancy is contraindicated until surgical repair. Beta blockade and surveillance are appropriate only for roots below 4.5 cm. Early delivery does not reduce dissection risk, which accumulates throughout pregnancy and peaks in the third trimester and puerperium, so option C fails to address the core hazard.
Reference: Williams Obstetrics, 26th ed.
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Written and medically reviewed by the StethoPrep medical team.