Cardiac disease in pregnancy and connective tissue disorders

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

Of note, there have been case reports of congenital heart block in infants born to parents with congenital heart disease.

Marfan's Syndrome
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.

Considerations for a pregnant patient with Marfan's Syndrome

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


 * Most common in 3rd trimester or 1st stage of labor


 * 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.


 * 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.


 * 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.


 * Prophylactic beta-blockers appear to be helpful.
 * During labor, pushing should be kept to a minimm, with an assisted second stage if necessary.
 * Surgery recommended pre-conception if root diameter >40 mm and during gestation if > 55 mm.

Related Chapter
For a more detailed review of Marfan's syndrome, click here.