Cardiac disease in pregnancy and connective tissue disorders

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Anjan K. Chakrabarti, M.D. [2], Assistant Editor-In-Chief: Cassandra Abueg, M.P.H. [3]

The most common connective tissue disorder that effects the cardiovascular system in pregnancy is Marfan’s syndrome, which will be reviewed in this section.

Marfan syndrome (or Marfan’s syndrome) is a connective tissue disorder most often caused by defects in the Fibrillin-1 gene (FBN1). Patients with Marfan’s syndrome are at significant risk of skeletal, cardiovascular and ocular complications. People with Marfan’s are typically tall, with long limbs and long thin fingers.

• The syndrome has autosomal dominant inheritance, therefore pre pregnancy counseling is important to advise prospective parents about the risks of transmission.

• The clinical outcomes of patients with Marfan’s syndrome during pregnancy are unpredictable.

• Assessment for the mother should include clinical and echocardiographic cardiovascular evaluation should be performed, as well as magnetic resonance imaging or computed tomography assessment of the entire aorta.^[1][2]

• If ascending aorta is larger than 40mm, pregnancy is generally contraindicated. One study has described that pregnancy is safe up to a diameter of 45mm.^[3]

• The mother should have periodic echocardiographic surveillance every 6 to 8 weeks to follow aortic root size.

• There should be a low threshold throughout pregnancy for aortic dissection.

• Complications are most common in 3rd trimester or 1st stage of labor

• Prophylactic beta-blockers like lopressor or metoprolol appear to be helpful.
• During labor, pushing should be kept to a minimm, with an assisted second stage if necessary.
• A C section is recommended if the aorta is >40mm or is increasing in size during pregnancy.
• Infective endocarditis prophylaxis should be considered.
• Surgery recommended pre-conception if aortic root diameter is >40 mm and during gestation if > 55 mm.

Elevated oxytocin levels have been implicated as a persistent risk factor for aortic dissection. If the mother does lactate, then oxytocin levels may stay elevated and may place the mother at risk of aortic dissection.

For a more detailed review of Marfan’s syndrome, click here.

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