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Pregnant women with heart disease have an increased risk of both maternal and fetal complications. Given that an estimated 1 million women in the United States have underlying congenital heart disease, the likelihood that a health care provider will care for such a patient is increasing. Certain conditions, such as pulmonary hypertension or prior peripartum cardiomyopathy, carry such high risk that they may contraindicate a pregnancy. In the Western world, the most common maternal heart disease is congenital, accounting for about 80% of patients. In the developing world, rheumatic valvular heart disease predominates.
This chapter provides an overview of prepregnancy counseling for women with heart disease, with an emphasis on its advantages for this patient population. We also discuss the scoring systems and tests used to ascertain the risk of maternal and fetal complications for patients hoping to become pregnant.
Ideally, risk assessment in patients with heart disease takes place before a woman becomes pregnant to present her with as accurate a prognosis as possible for both herself and her future child. If counseling was not provided before conception, it should be provided as early as possible during pregnancy. Counseling should involve the patient, her spouse or partner, and any other family members or significant individuals she chooses. The health care providers discuss the effect of pregnancy on the maternal cardiovascular system, whether this risk will change over time or with treatment, and how pregnancy may affect the long-term health of the woman. They also counsel the patient on risks to the fetus and alternative options, such as adoption or surrogacy. Additionally, health care providers may refer women to centers with expertise in adult congenital heart disease and high-risk pregnancy care. Whenever possible, we believe that counseling should involve both cardiologists and fetal-maternal medicine specialists. In most cases when the mother’s condition may be inherited, genetics counselors should also be involved. Prepregnancy counseling allows early assessment of the patient’s history, exam findings, imaging and electrocardiographic (ECG) studies, and possibly exercise testing before the advent of the physiologic changes inherent with pregnancy. Counseling can help a woman make important decisions regarding surgical repair before pregnancy or to choose the appropriate timing of pregnancy.
Prepregnancy counseling also opens the door to discussions regarding the risks of congenital abnormalities. Genetic counseling should be a major focus in these discussions with specific details regarding the potential inheritance of certain lesions. The background congenital heart disease rate of 0.6% to 2.0% may be altered by an individual’s family history, specific type of lesion, and exposure to teratogens. The risk for congenital heart disease in a fetus carried by a mother with congenital heart disease is estimated to be 4% to 6%, with left-sided heart lesions carrying an even higher risk. Diseases such as Marfan syndrome or 22q11 deletions carry a 50% inheritance risk and warrant discussion for in utero genetic testing. Having the opportunity to discuss these risks and the availability of genetic testing options before the start of pregnancy gives women time to discuss and contemplate these often-difficult ethical and personal considerations.
In sum, the prepregnancy counseling session provides women and couples essential information to help plan and determine the appropriate prepregnancy testing, surgical repair, pregnancy timing, and antepartum management strategies that best fit their lives. We strongly encourage all women with underlying cardiovascular disease to take advantage of this option.
Numerous scoring systems and tests can help assess the risk of both maternal and fetal complications in patients with heart disease. These include the CARPREG risk score, the World Health Organization (WHO) risk classification system, the measurement of maternal serum B-type natriuretic peptides (BNPs), and cardiopulmonary exercise testing.
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