Marfan syndrome Flashcards
1
Q
What is your preconceptual counselling for a woman with Marfan syndrome
A
Maternal risks:
- Aortic dissection
- Pre-exsiting HTN –> preeclampsia
- Maternal death
- Cervical insufficiency
- Risks associated with aortic and mitral regurgitation if present.
Fetal risks:
- Autosomal dominant inheritance; 50% chance.
- PTB
- FGR
- Fetal death
Risk of passing on to offspring + risks to mum:
- Adoption
- Egg donor + surrogacy
- Childlessness
Risk of dissection:
- CT or MR angiography
- ECHO
- Repair of aortic root pre-pregnancy if >=45mm
- Recommend against pregnancy if aortic root >=45mm or hx of dissection.
2
Q
Outline a management plan for a woman with Marfans during pregnancy in addition to routine care:
A
During pregnancy:
- MDT: cardiologist, MFM, anaesthetist
- ECHO every month if aortic root >40mm and every trimester if aortic root <40mm.
- Beta-blocker e.g. labetalol or metoprolol CR: minimises progression of aortic dilatation and risk of dissection.
- Serial growth scans.
Delivery:
- Tertiary unit with cardiothoracic surgery.
- Minimise BP/CO surges: epidural, beta-blockers, shorten 2nd stage (instrument, passive 2nd stage).
- Elective CS if aortic diameter >45 mm or hx of dissection.
Postpartum:
- ECHO 6 months postpartum.
