Cardiac disease in pregnancy and connective tissue disorders

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Cardiac disease in pregnancy Microchapters




Epidemiology and Demographics

Risk Factors


History and Symptoms

Physical Examination


Exercise Testing

Radiation Exposure

Chest X Ray





Pulmonary artery catheterization
Cardiac catheterization
Cardiac Ablation


Cardiovascular Drugs in Pregnancy

Labor and delivery

Resuscitation in Late Pregnancy

Contraindications to pregnancy

Special Scenarios:

I. Pre-existing Cardiac Disease:
Congenital Heart Disease
Repaired Congenital Heart Disease
Pulmonary Hypertension
Rheumatic Heart Disease
Connective Tissue Disorders
II. Valvular Heart Disease:
Mitral Stenosis
Mitral Regurgitation
Aortic Insufficiency
Aortic Stenosis
Mechanical Prosthetic Valves
Tissue Prosthetic Valves
III. Cardiomyopathy:
Dilated Cardiomyopathy
Hypertrophic Cardiomyopathy
Peripartum Cardiomyopathy
IV. Cardiac diseases that may develop During Pregnancy:
Acute Myocardial Infarction

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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.

Connective Tissue Disorder

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

Post-Partum Management

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.

Related Chapter

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


  1. 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.
  2. 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.
  3. 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.

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