Cardiovascular conditions, including cardiomyopathy, are responsible for approximately 26% of all pregnancy-related deaths. In the United States, because incidence of rheumatic heart disease has markedly declined, most heart problems during pregnancy result from congenital heart disease. However, in Southeast Asia, Africa, India, the Middle East, and parts of Australia and New Zealand, rheumatic heart disease is still common.
Despite dramatic improvements in survival and quality of life for patients with severe congenital heart defects and other heart disorders, pregnancy remains inadvisable for women with certain high-risk conditions such as the following (1 General reference Cardiovascular conditions, including cardiomyopathy, are responsible for approximately 26% of all pregnancy-related deaths. In the United States, because incidence of rheumatic heart disease... read more ):
Pulmonary hypertension Pulmonary Hypertension Pulmonary hypertension is increased pressure in the pulmonary circulation. It has many secondary causes; some cases are idiopathic. In pulmonary hypertension, pulmonary vessels may become constricted... read more (pulmonary artery systolic pressure > 25 mm Hg) caused by any condition, including Eisenmenger syndrome
Coarctation of the aorta Coarctation of the Aorta Coarctation of the aorta is a localized narrowing of the aortic lumen that results in upper-extremity hypertension, left ventricular hypertrophy, and, if severe, malperfusion of the abdominal... read more if uncorrected or if accompanied by an aneurysm
Severe symptomatic aortic stenosis Aortic Stenosis Aortic stenosis (AS) is narrowing of the aortic valve, obstructing blood flow from the left ventricle to the ascending aorta during systole. Causes include a congenital bicuspid valve, idiopathic... read more
or severe mitral stenosis Mitral Stenosis Mitral stenosis is narrowing of the mitral orifice that impedes blood flow from the left atrium to the left ventricle. The usual cause is rheumatic fever. Common complications are pulmonary... read more Bicuspid aortic valve with ascending aorta diameter > 50 mm
A single ventricle and impaired systolic function (whether treated with the Fontan procedure or not)
Cardiomyopathy Overview of Cardiomyopathies A cardiomyopathy is a primary disorder of the heart muscle. It is distinct from structural cardiac disorders such as coronary artery disease, valvular disorders, and congenital heart disorders... read more with ejection fraction < 30% or New York Heart Association (NYHA) class III or IV heart failure (see table )
General reference
1. European Society of Gynecology (ESG); Association for European Paediatric Cardiology (AEPC); German Society for Gender Medicine (DGesGM): ESC Guidelines on the management of cardiovascular diseases during pregnancy: the Task Force on the Management of Cardiovascular Diseases During Pregnancy of the European Society of Cardiology (ESC). Eur Heart J 32 (24):3147–3197, 2011. doi: 10.1093/eurheartj/ehr218
Pathophysiology of Heart Disease in Pregnancy
Pregnancy stresses the cardiovascular system, often worsening known heart disease; mild heart disease may first become evident during pregnancy.
Stressors include decreased hemoglobin 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 weeks gestation.
During labor, cardiac output increases about 20% with each uterine contraction; other stressors include straining during the 2nd stage of labor and the increase in venous blood returning to the heart from the contracting uterus. Cardiovascular stressors do not return to prepregnancy levels until several weeks after delivery.
Symptoms and Signs of Heart Disease in Pregnancy
Findings resembling heart failure Heart Failure (HF) Heart failure (HF) is a syndrome of ventricular dysfunction. Left ventricular (LV) failure causes shortness of breath and fatigue, and right ventricular (RV) failure causes peripheral and abdominal... read more 
(eg, mild dyspnea, systolic murmurs, jugular venous distention, tachycardia, dependent edema, mild cardiomegaly seen on chest x-ray) typically occur during normal pregnancy or may result from heart disease. Diastolic or presystolic murmurs are more specific for heart disease.
Heart failure can cause premature labor or arrhythmias. Risk of maternal or fetal death correlates with New York Heart Association (NHYA) functional classification, which is based on the amount of physical activity that causes symptoms of heart failure.
Risk is increased only if symptoms
Occur during mild exertion (NYHA class III)
Occur during minimal or no exertion (NYHA class IV)
Diagnosis of Heart Disease in Pregnancy
History and physical examination
Usually echocardiography
Diagnosis of heart disease during pregnancy is usually based on clinical evaluation and echocardiography.
Because genetics can contribute to the risk of heart disease, genetic counseling and fetal echocardiography should be offered to women with congenital heart disease.
Treatment of Heart Disease in Pregnancy
Avoidance of warfarin, angiotensin-converting enzyme (ACE) inhibitors, angiotensin II receptor blockers (ARBs), aldosterone antagonists, thiazide diuretics, and certain antiarrhythmics (eg, amiodarone)
For New York Heart Association (NYHA) class III or IV, activity restriction and possibly bed rest after 20 weeks
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 disease in pregnancy should attend the labor and ideally should be consulted prenatally. 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 heart disease should be optimally treated medically and, if indicated (eg, if due to a valvular heart disease), treated surgically. Women with class III or IV heart failure or another high-risk disorder (listed above) may be advised to obtain an early therapeutic abortion.
Some women with heart disease and poor cardiac function require digoxin 0.25 mg orally once a day plus bed rest or limited activity, beginning at 20 weeks. Cardiac glycosides (eg, digoxin, digitoxin) cross the placenta, but neonates (and children) are relatively resistant to their toxicity. ACE inhibitors and ARBs are contraindicated because they may cause fetal renal damage. Aldosterone antagonists (spironolactone, eplerenone) should be avoided because they may cause feminization of a male fetus. Other treatments for heart failure (eg, nonthiazide 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 Atrial Fibrillation Atrial fibrillation is a rapid, irregularly irregular atrial rhythm. Symptoms include palpitations and sometimes weakness, effort intolerance, dyspnea, and presyncope. Atrial thrombi may form... read more may accompany cardiomyopathy Overview of Cardiomyopathies A cardiomyopathy is a primary disorder of the heart muscle. It is distinct from structural cardiac disorders such as coronary artery disease, valvular disorders, and congenital heart disorders... read more or valvular disorders Overview of Cardiac Valvular Disorders Any heart valve can become stenotic or insufficient (also termed regurgitant or incompetent), causing hemodynamic changes long before symptoms occur. Depending on which valve is involved, the... read more . Rate control is usually similar to that in nonpregnant patients, with beta-blockers, calcium channel blockers, or digoxin (see Medications for Arrhythmias Medications for Arrhythmias The need for treatment of arrhythmias depends on the symptoms and the seriousness of the arrhythmia. Treatment is directed at causes. If necessary, direct antiarrhythmic therapy, including antiarrhythmic... read more ). Certain antiarrhythmics (eg, amiodarone) should be avoided. If pregnant patients have new-onset atrial fibrillation or hemodynamic instability or if medications do not control ventricular rate, 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. Standard or low molecular weight heparin is used. Neither standard heparin nor low molecular weight heparins cross the placenta, but low molecular weight heparins may have less risk of thrombocytopenia. Warfarin crosses the placenta and may cause fetal abnormalities (see table ), particularly during the 1st trimester. However, risk is dose-dependent, and incidence is very low if the dose is ≤ 5 mg per day. Warfarin use during the last month of pregnancy has risks. Rapid reversal of warfarin’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 tachycardia Treatment Reentrant supraventricular tachycardias (SVT) involve reentrant pathways with a component above the bifurcation of the His bundle. Patients have sudden episodes of palpitations that begin and... read more or ventricular tachycardia Treatment Ventricular tachycardia is ≥ 3 consecutive ventricular beats at a rate ≥ 120 beats/minute. Symptoms depend on duration and vary from none to palpitations to hemodynamic collapse and death. Diagnosis... read more is the same as for nonpregnant patients.
Endocarditis prophylaxis
For pregnant patients with a structural heart disorder, indications and use of endocarditis prophylaxis Prevention Infective endocarditis is infection of the endocardium, usually with bacteria (commonly, streptococci or staphylococci) or fungi. It may cause fever, heart murmurs, petechiae, anemia, embolic... read more 
for nonobstetric events are the same as those for nonpregnant patients. The American Heart Association guidelines do not recommend endocarditis prophylaxis for vaginal and cesarean deliveries because the rate of bacteremia is low. However, in the highest-risk patients (eg, those with prosthetic heart materials, a history of endocarditis, an unrepaired congenital cyanotic heart lesion, or a heart transplant with a valvulopathy), prophylaxis is often considered when the membranes rupture, even though no evidence indicates any benefit.
If patients with a structural heart disorder develop chorioamnionitis Intraamniotic Infection Intraamniotic infection is infection of the chorion, amnion, amniotic fluid, placenta, or a combination. Infection increases risk of obstetric complications and problems in the fetus and neonate... read more or another infection (eg, pyelonephritis) requiring hospital admission, the antibiotics used to treat the infection should cover the pathogens most likely to cause endocarditis.
Key Points
Pregnancy may not be advisable for women with certain high-risk heart disease (eg, pulmonary hypertension, coarctation of the aorta if uncorrected or accompanied by an aneurysm, Marfan syndrome with aortic root diameter of > 4.5 cm, severe symptomatic aortic stenosis, severe mitral stenosis, bicuspid aortic valve with ascending aorta > 50 mm, a single ventricle with impaired systolic function, cardiomyopathy, NYHA class III or IV heart failure).
Treat heart failure and arrhythmias during pregnancy as for nonpregnant patients, except avoid certain medications (eg, warfarin, ACE inhibitors, ARBs, aldosterone antagonists, thiazide diuretics, certain antiarrhythmics such as amiodarone).
Treat most pregnant patients who have atrial fibrillation with standard or low molecular weight heparin.
Indications for endocarditis prophylaxis for pregnant patients with a structural heart disorder are the same as those for other patients.
Valvular Stenosis and Insufficiency in Pregnancy
During pregnancy, valvular stenosis and regurgitation (insufficiency) most often affect the mitral and aortic valves. Mitral stenosis Mitral Stenosis Mitral stenosis is narrowing of the mitral orifice that impedes blood flow from the left atrium to the left ventricle. The usual cause is rheumatic fever. Common complications are pulmonary... read more is the most common valvular disorder during pregnancy.
Pregnancy amplifies the murmurs of mitral stenosis and aortic stenosis Aortic Stenosis Aortic stenosis (AS) is narrowing of the aortic valve, obstructing blood flow from the left ventricle to the ascending aorta during systole. Causes include a congenital bicuspid valve, idiopathic... read more but diminishes those of mitral and aortic regurgitation Aortic Regurgitation Aortic regurgitation (AR) is incompetency of the aortic valve causing backflow from the aorta into the left ventricle during diastole. Causes include valvular degeneration and aortic root dilation... read more 
. 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 Atrial Fibrillation Atrial fibrillation is a rapid, irregularly irregular atrial rhythm. Symptoms include palpitations and sometimes weakness, effort intolerance, dyspnea, and presyncope. Atrial thrombi may form... read more is also common.
Treatment
For mitral stenosis, prevention of tachycardia, treatment of pulmonary edema and atrial fibrillation, and sometimes valvotomy
For aortic stenosis, surgical correction before pregnancy if possible
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 (eg, for endocarditis prophylaxis Prevention Infective endocarditis is infection of the endocardium, usually with bacteria (commonly, streptococci or staphylococci) or fungi. It may cause fever, heart murmurs, petechiae, anemia, embolic... read more ).
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. Tachycardia should be prevented so that diastolic flow through the stenotic mitral valve can be maximized.
If pulmonary edema occurs, loop diuretics can be used.
If atrial fibrillation occurs, anticoagulation and control of heart rate are necessary. Control of heart rate is usually similar to that in nonpregnant patients and involves beta-blockers, calcium channel blockers, or digoxin (see Medications for Arrhythmias Medications for Arrhythmias The need for treatment of arrhythmias depends on the symptoms and the seriousness of the arrhythmia. Treatment is directed at causes. If necessary, direct antiarrhythmic therapy, including antiarrhythmic... read more ).
During labor, conduction anesthesia (eg, slow epidural infusion) is usually preferred.
Aortic stenosis
Aortic stenosis Treatment Aortic stenosis (AS) is narrowing of the aortic valve, obstructing blood flow from the left ventricle to the ascending aorta during systole. Causes include a congenital bicuspid valve, idiopathic... read more should be corrected before pregnancy if possible because surgical repair during pregnancy has more risks and catheter valvuloplasty is not very effective.
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 is preferred. Cesarean delivery Cesarean Delivery Cesarean delivery is surgical delivery by incision into the uterus. Up to 30% of deliveries in the US are cesarean. The rate of cesarean delivery fluctuates. It has recently increased, partly... read more is done if indicated for obstetric reasons.
Other Heart Disorders in Pregnancy
Mitral valve prolapse
Mitral valve prolapse Mitral Valve Prolapse (MVP) Mitral valve prolapse (MVP) is a billowing of mitral valve leaflets into the left atrium during systole. The most common cause is idiopathic myxomatous degeneration. MVP is usually benign, but... read more 
occurs more frequently in younger women and tends to be familial. Mitral valve prolapse is usually an isolated abnormality that has no clinical consequences; however, patients may also have some degree of mitral regurgitation. Rarely, mitral valve prolapse occurs with Marfan syndrome Marfan Syndrome Marfan syndrome consists of connective tissue anomalies resulting in ocular, skeletal, and cardiovascular abnormalities (eg, dilation of ascending aorta, which can lead to aortic dissection)... read more 
or an atrial septal defect Atrial Septal Defect (ASD) An atrial septal defect (ASD) is an opening in the interatrial septum, causing a left-to-right shunt and volume overload of the right atrium and right ventricle. Children are rarely symptomatic... read more .
Women with mitral valve prolapse and resulting mitral regurgitation Mitral Regurgitation Mitral regurgitation (MR) is incompetency of the mitral valve causing flow from the left ventricle (LV) into the left atrium during ventricular systole. MR can be primary (common causes are... read more 
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.
Beta-blockers are indicated for recurrent arrhythmias. Rarely, thrombi and systemic emboli (due to concomitant atrial fibrillation) develop and require anticoagulation.
Congenital heart disease
For most asymptomatic patients, risk is not increased during pregnancy. However, patients with Eisenmenger syndrome Eisenmenger Syndrome Eisenmenger syndrome is a complication of uncorrected large intracardiac or aortic to pulmonary artery left-to-right shunts. Increased pulmonary resistance may develop over time, eventually... read more (now rare), primary pulmonary hypertension Pulmonary Hypertension Pulmonary hypertension is increased pressure in the pulmonary circulation. It has many secondary causes; some cases are idiopathic. In pulmonary hypertension, pulmonary vessels may become constricted... read more , or perhaps isolated pulmonary stenosis Pulmonic Stenosis Pulmonic stenosis (PS) is narrowing of the pulmonary outflow tract causing obstruction of blood flow from the right ventricle to the pulmonary artery during systole. Most cases are congenital... read more are predisposed, for unknown reasons, to sudden death during labor, during the postpartum period (the 6 weeks after delivery), or after abortion at > 20 weeks gestation. Thus, pregnancy is inadvisable. If these patients become pregnant, they should be closely monitored with a pulmonary artery catheter and/or 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 Marfan Syndrome Marfan syndrome consists of connective tissue anomalies resulting in ocular, skeletal, and cardiovascular abnormalities (eg, dilation of ascending aorta, which can lead to aortic dissection)... read more are at increased risk of aortic dissection Aortic Dissection Aortic dissection is the surging of blood through a tear in the aortic intima with separation of the intima and media and creation of a false lumen (channel). The intimal tear may be a primary... read more 
and rupture of aortic aneurysms Overview of Aortic Aneurysms Aneurysms are abnormal dilations of arteries caused by weakening of the arterial wall. Common causes include hypertension, atherosclerosis, infection, trauma, systemic rheumatic diseases, and... read more 
during pregnancy. Bed rest, beta-blockers, avoidance of Valsalva maneuvers, and measurement of aortic diameter with echocardiography are required.
Peripartum cardiomyopathy
Heart failure with no identifiable cause (eg, myocardial infarction, valvular disorder) can develop between the last month of pregnancy and 6 months postpartum in patients without a previous heart disorder (1 General reference Cardiovascular conditions, including cardiomyopathy, are responsible for approximately 26% of all pregnancy-related deaths. In the United States, because incidence of rheumatic heart disease... read more ). Risk factors include
Multiparity
Age ≥ 30
Multifetal pregnancy
Preeclampsia
Recurrence is likely in subsequent pregnancies, particularly in patients with residual cardiac dysfunction. Risk of death in a subsequent pregnancy may be as high as 50%; therefore, future pregnancies are not recommended (2 General reference Cardiovascular conditions, including cardiomyopathy, are responsible for approximately 26% of all pregnancy-related deaths. In the United States, because incidence of rheumatic heart disease... read more ).
Treatment is as for heart failure Treatment Heart failure (HF) is a syndrome of ventricular dysfunction. Left ventricular (LV) failure causes shortness of breath and fatigue, and right ventricular (RV) failure causes peripheral and abdominal... read more . Angiotensin-converting enzyme (ACE) inhibitors and aldosterone are relatively contraindicated but may be used when the expected benefit clearly exceeds the potential risks.
General reference
1. Sliwa K, Hilfiker-Kleiner D, Petrie MC, et al: Current state of knowledge on aetiology, diagnosis, management, and therapy of peripartum cardiomyopathy: A position statement from the Heart Failure Association of the European Society of Cardiology Working Group on peripartum cardiomyopathy. Eur J Heart Fail 12 (8):767–778, 2010. doi: 10.1093/eurjhf/hfq120
2. Elkayam U: Risk of subsequent pregnancy in women with a history of peripartum cardiomyopathy. J Am Coll Cardiol 64(15):1629-1636, 2014. doi:10.1016/j.jacc.2014.07.961
Drugs Mentioned In This Article
| Drug Name | Select Trade |
|---|---|
warfarin |
Coumadin, Jantoven |
angiotensin ii |
GIAPREZA |
amiodarone |
Cordarone, Nexterone, Pacerone |
digoxin |
Digitek , Lanoxicaps, Lanoxin, Lanoxin Pediatric |
spironolactone |
Aldactone, CAROSPIR |
eplerenone |
Inspra |
heparin |
Hepflush-10 , Hep-Lock, Hep-Lock U/P, Monoject Prefill Advanced Heparin Lock Flush, SASH Normal Saline and Heparin |
