A 26-year-old woman with Marfan syndrome is counselled before pregnancy. Echocardiography shows an aortic root diameter of 4.6 cm. What is the most appropriate recommendation?
- A Pregnancy is safe with annual echocardiography
- B Proceed with pregnancy but deliver by elective caesarean at 37 weeks
- C Avoid pregnancy until aortic root replacement is performed ✓
- D Proceed with pregnancy on labetalol alone without further assessment
Explanation
An aortic root of 4.0 cm or greater in Marfan syndrome places the patient at high risk of aortic dissection during pregnancy, when haemodynamic stress peaks. Guidelines recommend counselling against pregnancy until prophylactic aortic root replacement is done. A root below 4.0 cm generally permits pregnancy with beta blockade and serial echocardiography every 4 to 8 weeks. Beta blockade alone does not reduce dissection risk adequately at this root size, and mode of delivery does not address the primary hazard.
Reference: Williams Obstetrics, 26th ed.
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Written and medically reviewed by the StethoPrep medical team.