Heart disorders account for about 10% of maternal obstetric deaths. In the US, because incidence of rheumatic heart disease has markedly declined, most heart problems during pregnancy result from congenital heart disease. Pregnancy is inadvisable for women with certain high-risk disorders (eg, pulmonary hypertension, severe valvular disorders, prior postpartum cardiomyopathy).

Pathophysiology

Pregnancy stresses the cardiovascular system, often worsening known heart disorders; mild heart disorders may first become evident during pregnancy. Stresses include decreased Hb and increased blood volume, stroke volume, and eventually heart rate. Cardiac output increases by 30 to 50%. These changes become maximal between 28 and 34 wk gestation. During labor, cardiac output increases about 20% with each uterine contraction; other stresses include straining during the 2nd stage of labor and the increase in venous blood returning to the heart from the contracting uterus. Cardiovascular stresses do not return to prepregnancy levels until several weeks after delivery.

Symptoms and Signs

Findings resembling heart failure (eg, mild dyspnea, systolic murmurs, jugular venous distention, tachycardia, dependent edema, mild cardiomegaly seen on chest x-ray—see Heart Failure) typically occur during normal pregnancy or may result from a heart disorder. Diastolic or presystolic murmurs are more specific for heart disorders.

Heart failure can cause premature labor or arrhythmias. Risk of maternal or fetal death correlates with New York Heart Association functional classification, which is based on the amount of physical activity that causes symptoms of heart failure. Risk is not increased if symptoms

• Do not occur during exertion (class I)
• Occur only during significant exertion (class II)

Risk is increased if symptoms

• Occur during mild exertion (class III)
• Occur during minimal or no exertion (class IV)

Diagnosis

Diagnosis is usually based on clinical evaluation and echocardiography.

Treatment

• Avoidance of warfarinSome Trade Names
  COUMADIN
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  , ACE inhibitors, aldosterone antagonists, and certain antiarrhythmics (eg, amiodaroneSome Trade Names
  CORDARONE
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  )
• For NYHA class III or IV, activity restriction and possible bed rest after 20 wk
• Most other usual treatments for heart failure and arrhythmias

Frequent prenatal visits, ample rest, avoidance of excessive weight gain and stress, and treatment of anemia are required. An anesthesiologist familiar with heart disorders in pregnancy should attend the labor. During labor, pain and anxiety are treated aggressively to minimize tachycardia. Women are closely monitored immediately postpartum and are followed for several weeks postpartum by a cardiologist.

Before women with NYHA class III or IV status conceive, the disorder should be optimally treated medically and, if indicated (eg, if due to a valvular heart disorder), treated surgically.

Some women with a heart disorder and poor cardiac function require digoxinSome Trade Names
DIGITEK
LANOXIN
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0.25 mg po once/day plus bed rest, beginning at 20 wk. Cardiac glycosides (eg, digoxinSome Trade Names
DIGITEK
LANOXIN
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, digitoxinSome Trade Names
No US trade name

) cross the placenta, but neonates (and children) are relatively resistant to their toxicity. Women with class IV heart failure may be advised to obtain early therapeutic abortion. ACE inhibitors are contraindicated because they may cause fetal renal damage. Aldosterone antagonists (spironolactoneSome Trade Names
ALDACTONE
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, eplerenoneSome Trade Names
INSPRA
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) should be avoided because they may cause feminization of a male fetus. Other treatments for heart failure (eg, diuretics, nitrates, inotropes) may be continued during pregnancy depending on disease severity and fetal risk, as determined by a cardiologist and a perinatologist.

Arrhythmias: Atrial fibrillation may accompany cardiomyopathy or valvular lesions. Rate control is usually similar to that in nonpregnant patients, with β-blockers, Ca channel blockers, or digoxinSome Trade Names
DIGITEK
LANOXIN
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(see Arrhythmias and Conduction Disorders: Drugs for Arrhythmias). Certain antiarrhythmics (eg, amiodaroneSome Trade Names
CORDARONE
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) should be avoided. In pregnant patients with new-onset atrial fibrillation or hemodynamic instability, cardioversion may be used to restore sinus rhythm. Anticoagulation may be required because the relative hypercoagulability during pregnancy makes atrial thrombi (and subsequent systemic or pulmonary embolization) more likely. Neither standard heparinSome Trade Names
HEPFLUSH-10
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nor low molecular weight heparins cross the placenta, but low molecular weight heparins may have less risk of thrombocytopenia. WarfarinSome Trade Names
COUMADIN
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crosses the placenta and may cause fetal abnormalities (see Table 2: Drugs in Pregnancy: Some Drugs With Adverse Effects During Pregnancy), particularly during the 1st trimester. WarfarinSome Trade Names
COUMADIN
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use during the last month of pregnancy has risks. Rapid reversal of warfarinSome Trade Names
COUMADIN
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's anticoagulant effects may be difficult and may be required because of fetal or neonatal intracranial hemorrhage resulting from birth trauma or because of maternal bleeding (eg, resulting from trauma or emergency cesarean delivery).

Management of acute supraventricular or ventricular tachycardia is the same as for nonpregnant patients.

Endocarditis prophylaxis: For pregnant patients with a structural heart disorder, indications and use of endocarditis prophylaxis for nonobstetric events are the same as those for nonpregnant patients (see Endocarditis: Prevention). Although rate of bacteremia with uncomplicated vaginal or caesarean delivery is low and routine prophylaxis is not recommended by the American College of Cardiology, many obstetricians give patients with valvular disease prophylactic antibiotics shortly before and after delivery.

Valvular Stenosis and Insufficiency

During pregnancy, stenosis and regurgitation (insufficiency) most often affect the mitral and aortic valves. Pregnancy amplifies the murmurs of mitral and aortic stenosis but diminishes those of mitral and aortic regurgitation. During pregnancy, mild mitral or aortic regurgitation is usually easy to tolerate; stenosis is more difficult to tolerate and predisposes to maternal and fetal complications. Mitral stenosis is especially dangerous; the tachycardia, increased blood volume, and increased cardiac output during pregnancy interact with this disorder to rapidly increase pulmonary capillary pressure, causing pulmonary edema. Atrial fibrillation is also common.

Treatment

Ideally, valvular disorders are diagnosed and treated medically before conception; surgical correction is often recommended for severe disorders. Prophylactic antibiotics are required in certain situations (see Pregnancy Complicated by Disease: Endocarditis prophylaxis).

Mitral stenosis: Patients must be closely observed throughout pregnancy because mitral stenosis may rapidly become more severe. If required, valvotomy is relatively safe during pregnancy; however, open heart surgery increases fetal risk. During labor, conduction anesthesia (eg, epidural or spinal nerve block by a local anesthetic) is usually preferred.

Aortic stenosis: During labor, local anesthesia is preferred, but if necessary, general anesthesia is used. Conduction anesthesia should be avoided because it decreases filling pressures (preload), which may already be decreased by aortic stenosis. Straining, which can suddenly reduce filling pressures and impair cardiac output, is discouraged during the 2nd stage of labor; operative vaginal delivery may be preferred, if feasible. Cesarean delivery is done if indicated (see Abnormalities and Complications of Labor and Delivery: Cesarean Delivery).

Other Heart Disorders in Pregnancy

Mitral valve prolapse: This disorder occurs more frequently in younger women and tends to be familial. Mitral valve prolapse is usually an isolated abnormality but may cause some degree of mitral regurgitation or be accompanied by Marfan syndrome or an atrial septal defect.

Women with mitral valve prolapse generally tolerate pregnancy well. The relative increase in ventricular size during normal pregnancy reduces the discrepancy between the disproportionately large mitral valve and the ventricle.

Patients with mitral regurgitation may require prophylactic antibiotics during delivery. β-Blockers are indicated for recurrent arrhythmias. Rarely, thrombi and systemic emboli develop and require anticoagulation.

Congenital heart disease: For most asymptomatic patients, risk is not increased during pregnancy. However, patients with Eisenmenger's syndrome (now rare), primary pulmonary hypertension, or perhaps isolated pulmonary stenosis are predisposed, for unknown reasons, to sudden death during labor, during the postpartum period (the 6 wk after delivery), or after abortion at > 20 wk gestation. Thus, pregnancy is inadvisable. If these patients become pregnant, they should be closely monitored with a pulmonary artery catheter and an arterial line during delivery. For patients with intracardiac shunts, the goal is to prevent right-to-left shunting by maintaining peripheral vascular resistance and by minimizing pulmonary vascular resistance.

Patients with Marfan syndrome are at increased risk of aortic dissection and rupture of aortic aneurysms during pregnancy. Bed rest, avoidance of Valsalva maneuvers, and measurement of aortic diameter with echocardiography are required.

Peripartum cardiomyopathy: Heart failure with no identifiable cause (eg, MI, valvular disorder) can develop between the last month of pregnancy and 5 mo postpartum in patients without a previous heart disorder. Risk factors include multiparity, age ≥ 30, multifetal pregnancy, and preeclampsia. The 5-yr mortality rate is 50%. Recurrence is likely in subsequent pregnancies, particularly in patients with residual cardiac dysfunction; future pregnancies are therefore not recommended. Treatment is as for heart failure (see Pregnancy Complicated by Disease: Endocarditis prophylaxis).

Last full review/revision December 2008 by Sean C. Blackwell, MD

Content last modified February 2012