A 26-year-old woman with Marfan syndrome is seen in the preconception clinic. Her echocardiogram shows an aortic root diameter of 4.6 cm with mild aortic regurgitation. What is the most appropriate advice?
- A Pregnancy is strongly discouraged until elective aortic root replacement ✓
- B Pregnancy is permitted provided she takes aspirin throughout
- C Pregnancy is permitted with six-monthly echo surveillance
- D Pregnancy is permitted only after the fetus reaches viability
Correct answer: A. Pregnancy is strongly discouraged until elective aortic root replacement
Explanation
In Marfan syndrome, an aortic root diameter above 4.5 cm places the patient in the modified WHO class IV category, where pregnancy is contraindicated because of the high risk of aortic dissection, which peaks in the third trimester and early puerperium. Elective root replacement before conception is advised. Surveillance alone, aspirin, or waiting for viability do not address the mechanical risk of dissection in a dilated root.
Reference: Fernando Arias' Practical Guide to High-Risk Pregnancy and Delivery, 5th ed.
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