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    Cardiovascular morbidity and mortality in pregnancy

     

    Aortopathy and aortic dissection

    Aortic dissection is rare in pregnancy and can occur in patients without an underlying aortopathy. There are a number of conditions, including Marfan syndrome, Loeys-Dietz syndrome, other familial aortopathies, bicuspid aortic valves (which may be associated with an aortopathy), and Turner syndrome which carry a significantly higher risk of aortic dissection during pregnancy. Specific aortic diameters at which risk is considered prohibitive or prophylactic surgery is recommended vary between the various diseases.28 Whenever possible, preconceptual counseling and assessment of aortic size should be done to determine the risk of pregnancy, and prophylactic aortic surgery may be recommended prior to pregnancy to decrease the risk of dissection during pregnancy. Close follow-up of aortic size during pregnancy and carefully planned delivery are necessary to minimize risk for women with aortic disease. Cesarean delivery is usually recommended for patients at high risk of dissection.5,6

     

    Pulmonary hypertension 

    Pulmonary hypertension may be seen as a primary disease (pulmonary arterial hypertension) or secondary to cardiac disease such as CHD and valvular disease, lung disease, thromboembolic disease or other disorders. Defined as a mean arterial pressure of 25 mmHg or greater as assessed at catheterization, the degree of pulmonary hypertension ranges from mild to severe. Eisenmenger syndrome is a particularly severe form of pulmonary hypertension due to an unrepaired shunt, and it is associated with a very high rate of maternal morbidity and mortality. The hemodynamic changes of pregnancy are not well-tolerated in women with pulmonary hypertension and an increase in pulmonary pressure, decline in RV function, clinical heart failure, and syncope may occur, resulting in death rates as high as 50%.29 For this reason, pregnancy is usually contraindicated in mothers with pulmonary hypertension and termination of pregnancy — preferably in a pulmonary hypertension facility — is often recommended.5,6,7,29 Newer therapies for treatment of pulmonary hypertension are available and can improve symptom status and mortality, but many of these drugs have significant fetal toxicity (Table 1). For women who choose to continue pregnancy, careful multidisciplinary care (including a pulmonary hypertension specialist, cardiologist, and maternal-fetal medicine specialist)  in a pulmonary hypertension center is required with very frequent monitoring, consideration of early planned delivery, and prolonged postpartum care.28

    Planning for pregnancy

    Preconceptual counseling is necessary for women with preexisting CVD to clearly understand the risk to both mother and fetus. Education about CVD and pregnancy risk should begin at a young age, ideally involving a discussion of both maternal and fetal risk and appropriate contraceptive options. For women with CHD and women with some types of cardiomyopathy and familial aortopathies (such as Marfan syndrome), genetic counseling may be appropriate. Obstetricians should not rely solely on a patient’s self-reported history about her diagnosis or her symptom status. 

    Women with preexisting CVD should be referred to a maternal-fetal medicine specialist and a cardiologist who can work in collaboration to fully address maternal and fetal risks. For women with CHD in particular, consultation with an adult congenital heart disease subspecialist should be obtained to assess the specific nature of the original defect, the specific type of surgical procedure(s) or device interventions performed, presence of absence of any residual lesions, shunts, valvular dysfunction, ventricular dysfunction, arrhythmias, prior heart failure or stroke, pulmonary hypertension, residual cyanosis, or other comorbidities that may impact pregnancy risk.5-8 Cardiac imaging is imperative.  Most commonly, echocardiography is performed, but cardiac magnetic resonance imaging, computed tomography angiography, and invasive cardiac catheterization may be required.  Assessment of functional capacity with formal stress testing may be a valuable tool. Preconceptual counseling also should include assessment of all medications for their benefits and risks, including assessment for potentially teratogenic drugs. In some cases, alternate therapy may be able to be substituted prior to conception. 

    NEXT: Prediction of risk

    Beth Brickner, MD
    Dr. Brickner is co-director of the Adult Congenital Heart Disease Program at UT Southwestern Medical Center, Dallas, Texas.

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