Cardiac disease in pregnancy and connective tissue disorders
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The most common connective tissue disorder that effects the cardiovascular system in pregnancy is Marfan's syndrome, which will be reviewed in this section.
Connective Tissue Disorder
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.
- 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.
- 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.
- 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.
- Immer FF, Bansi AG, Immer-Bansi AS, McDougall J, Zehr KJ, Schaff HV; et al. (2003). "Aortic dissection in pregnancy: analysis of risk factors and outcome". Ann Thorac Surg. 76 (1): 309–14. PMID 12842575.
- Rossiter JP, Repke JT, Morales AJ, Murphy EA, Pyeritz RE (1995). "A prospective longitudinal evaluation of pregnancy in the Marfan syndrome". Am J Obstet Gynecol. 173 (5): 1599–606. PMID 7503207.
- Meijboom LJ, Vos FE, Timmermans J, Boers GH, Zwinderman AH, Mulder BJ (2005). "Pregnancy and aortic root growth in the Marfan syndrome: a prospective study". Eur Heart J. 26 (9): 914–20. doi:10.1093/eurheartj/ehi103. PMID 15681576.