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This chapter should be cited as follows:
Tolcher MC, Ermis P, et al, Glob. libr. women's med.,
ISSN: 1756-2228; DOI 10.3843/GLOWM.409403

The Continuous Textbook of Women’s Medicine SeriesObstetrics Module

Volume 13

Obstetric emergencies

Volume Editor: Dr María Fernanda Escobar Vidarte, Fundación Valle del Lili, Cali, Colombia

Chapter

Cardiac Disease in Pregnancy

First published: August 2024

Study Assessment Option

By completing 4 multiple-choice questions (randomly selected) after studying this chapter readers can qualify for Continuing Professional Development awards from FIGO plus a Study Completion Certificate from GLOWM
See end of chapter for details

INTRODUCTION

Pregnant patients with cardiac disease can be intimidating for obstetricians and gynecologists, particularly patients with preexisting, complex congenital cardiac disease. Cardiac disease complicates <5% of pregnancies but is a major cause of maternal morbidity and mortality.1 Given the relative infrequency of cardiac disease and the complexity of repaired and unrepaired congenital disease, multidisciplinary management involving maternal-fetal medicine experts, especially those with clinical interest or expertise in managing these patients, in collaboration with cardiologists, anesthesiologists, and intensivists with interest in care of the pregnant patient is essential to minimize complications. Complications of cardiac disease are currently a leading cause of the rising maternal mortality rates, and advances in congenital cardiac disease diagnosis and management will undoubtedly lead to an increase in this population of pregnant women. Finally, pregnancy at advanced maternal age with increased co-morbidities including hypertension and diabetes will likely result in an increase in cardiovascular complications of pregnancy.

PHYSIOLOGIC CHANGES

Cardiopulmonary physiologic adaptations to pregnancy have been well-described and are similar in nature to those that occur during exercise/stress. Most notable for cardiac patients are an increase in blood volume, cardiac output, and heart rate, and a decrease in peripheral vascular resistance.2 A disproportionate increase in plasma volume as compared to red cell mass results in the physiologic anemia of pregnancy. These physiologic changes begin at less than 10 weeks of gestation. Additionally, uterine blood flow increases from <1% of cardiac output in non-pregnant women to approximately 25% of cardiac output at term.3 Finally, maternal position can significantly affect cardiac output by decreasing venous return; supine position can decrease cardiac output by up to 30% and should be avoided for extended periods of time.

RISK STRATIFICATION

Fundamental to the management of women with cardiac disease is assessing the risk of the pregnancy to cardiac status and the risks of cardiac status to the pregnancy. Various risk profile scores have been developed to quantify risks of pregnancy based on underlying condition, functional status, and anticipated changes in pregnancy. The CARPREG (Cardiac Disease in Pregnancy) scoring system utilized four predictors: (1) prior cardiac event (heart failure, stroke or arrhythmia), (2) poor maternal functional class (New York Heart Association (NYHA) class >II or cyanosis (SaO2 <90%)), (3) ventricular systolic dysfunction (ejection fraction <40%), or (4) left heart obstruction. These factors were found to be predictive of maternal cardiac complications with a risk of 5% with zero factors, 27% with one factor, and 75% with more than one factor.4 One study compared the predictive ability for adverse perinatal outcomes of the modified World Health Organization (mWHO) classification to the CARPREG.5 Table 1 lists the mWHO classification for cardiac disease in pregnancy. The mWHO classification was found to be more predictive than CARPREG with significant differences in adverse outcomes among the four mWHO groups (17.5% (mWHO group 1), 21% (mWHO group 2), 42% (mWHO group 3), and 85.7% (mWHO group 4), p = 0.0001). The three CARPREG groups (no risk factors, 1 risk factor, and ≥1 risk factor) had more similar rates of complications (22%, 31.6%, and 42.9%, respectively). Since the mWHO classification has been shown to better predict obstetric and neonatal complications as compared to CARPREG,5 we typically recommend the mWHO classification for prognosticating (Table 1). A subsequent risk index, CARPREG II, was developed that includes 10 predictors (prior cardiac events or arrhythmias, baseline NYHA class III or IV or cyanosis, mechanical valve, ventricular dysfunction, high risk left-sided valve disease or left ventricular outflow obstruction, pulmonary hypertension, coronary artery disease, high risk aortopathy, no prior cardiac intervention, and late pregnancy assessment).6 Validation of the CARPREG II index is needed to determine generalizability.

1

Modified World Health Organization (mWHO) risk classification by cardiac condition. Adapted from the European Society of Cardiology guidelines on the management of cardiovascular diseases during pregnancy.7


I

II

II–III

III

IV

Cardiac condition

Uncomplicated, small or mild

  • pulmonary stenosis
  • patent ductus arteriosus
  • mitral valve prolapse
  • Successfully repaired simple lesions
  • atrial or ventricular septal defect
  • patent ductus arteriosus
  • anomalous pulmonary venous drainage

Atrial or ventricular ectopic beats, isolated

Unrepaired atrial or ventricular septal defect

Repaired tetralogy of Fallot

Most arrhythmias (e.g. SVT)

Turner syndrome without aortic dilation

Mild left ventricular impairment (EF >45%)

Hypertrophic cardiomyopathy

Native or tissue valvular heart disease not considered WHO class I or IV (mild mitral stenosis, moderate aortic stenosis)

Marfan syndrome or other aortopathy without aortic dilation

Aorta <45 mm with bicuspid aortic valve pathology

Repaired coarctation

Atrioventricular septal defect

Moderate left ventricular impairment (EF 30–45%)

Previous PPCM without any residual left ventricular impairment

Mechanical valve

Systemic right ventricle with good or mildly decreased ventricular function

Fontan circulation, uncomplicated

Unrepaired cyanotic heart disease

Other complex heart disease

Moderate mitral stenosis

Severe asymptomatic aortic stenosis

Moderate aortic dilation (40–45 mm in

Marfan syndrome or other aortopathy; 45–50 mm in bicuspid aortic valve, Turner syndrome ASI 20–25 mm/m2, tetralogy of Fallot <50 mm)

Ventricular tachycardia

Pulmonary arterial hypertension

Severe systemic ventricular dysfunction (EF <30%, NYHA III–IV)

Previous PPCM with any residual left ventricular impairment

Severe mitral stenosis

Severe symptomatic aortic stenosis

Systemic right ventricle with moderate or severely decreased ventricular function

Severe aortic dilation (>45 mm in Marfan syndrome or other aortopathy, >50 mm in bicuspid aortic valve, Turner syndrome ASI >25 mm/m2, tetralogy of Fallot >50 mm)

Vascular Ehlers-Danlos

Severe (re)coarctation

Fontan with any complication

Risk

No detectable increased risk of maternal mortality and no/mild increase in morbidity

Small increased risk of maternal mortality or moderate increase in morbidity

Significantly increased risk of maternal mortality or severe morbidity.

Intermediate increased risk of maternal mortality or moderate to severe increased risk of maternal morbidity

Extremely high risk of maternal mortality or severe morbidity

Cardiac event rate

2.5–5%

7.5–10.5%

10–19%

19–27%

40–100%

Counseling

Yes

Yes

Yes

Expert counseling required

Expert counseling required. Pregnancy contra-indicated. If pregnancy occurs, termination should be discussed.

Care during pregnancy

Local center

Local center

Referral center

Expert center for cardiac disease in pregnancy

Expert center for cardiac disease in pregnancy

Minimal visit frequency (in addition to routine prenatal care)

1–2 during pregnancy

1 visit each trimester

Every other month

Monthly or every other month

Monthly

Delivery location

Local center

Local center

Referral center

Expert center for cardiac disease in pregnancy

Expert center for cardiac disease in pregnancy

SVT, supraventricular tachycardia; EF, ejection fraction; PPCM, peripartum cardiomyopathy; ASI, aortic size index; NHYA, New York Heart Association

VALVULAR LESIONS

Pulmonary stenosis

Isolated pulmonary stenosis is generally well-tolerated in pregnancy. Severe obstruction places the patient at higher risk for right-sided heart failure.

Management pearls

Pre-pregnancy or antenatal evaluation should include an echocardiogram to determine the severity of the outflow obstruction. Epidural analgesia and vaginal delivery are not contraindicated.

Tricuspid lesions

Tricuspid stenosis or regurgitation lesions are well-tolerated in pregnancy. However, in severe cases, tricuspid regurgitation can be associated with right-sided heart failure, pulmonary hypertension, and/or arrhythmia.

Management pearls

Pre-pregnancy or antenatal evaluation should include an echocardiogram to determine the severity of the valvular disease. Epidural analgesia and vaginal delivery are not contraindicated.

Mitral stenosis

Rheumatic heart disease remains the most common cause of mitral stenosis worldwide. Due to restricted flow across a stenotic mitral valve, left atrial pressures may be elevated. Additionally, an elevated transmitral gradient may be further elevated by pregnancy-induced tachycardia and increased stroke volume, leading to increased atrial pressures, dyspnea, and pulmonary edema. Patients with moderate (1–1.5 cm2 valve area) or severe stenosis (<1 cm2 valve area) are at highest risk of potential complications including pulmonary edema, pulmonary hypertension, and atrial fibrillation. Severe mitral stenosis is considered a contraindication to pregnancy.

Management pearls

Pre-pregnancy or antenatal evaluation should include an echocardiogram to evaluate degree of stenosis. Electrocardiogram should also be performed to exclude atrial fibrillation. Tachycardia (heart rate >100 beats per minute) should be prevented with the use of beta blockers and avoidance of terbutaline. Diuretics (e.g. furosemide) can be used to treat pulmonary edema. Since inadequate preload can decrease cardiac output, a “slow” epidural with avoidance of abrupt sympathectomy is recommended.

Aortic stenosis

Aortic stenosis (AS) can result in outflow obstruction along with left ventricular hypertrophy. Stenosis is frequently associated with a bicuspid aortic valve in the younger patient population and, therefore, the combination can be seen during pregnancy. Severe disease is characterized by a valve area <1 cm2 or mean gradient >40 mmHg. The presence of a stenotic valve leads to a fixed cardiac output and thus correction, especially in cases of severe disease, is recommended prior to conception. Hypovolemia, hypotension, and bradycardia can result in decreased cardiac output leading to angina secondary to decreased coronary artery perfusion, syncope, hypervolemia and pulmonary edema, and sudden death. Women with severe disease are at significant risk of complications of pregnancy, particularly those with severe, symptomatic AS. A recent study of women with severe AS showed a 35% hospitalization rate during pregnancy with heart failure and arrhythmias being the most common complications.8 Heart failure occurred in 6.7% of women with severe, asymptomatic AS and 26.3% of women with severe, symptomatic AS. Severe AS is a contraindication to pregnancy.

Management pearls

Pre-pregnancy or antenatal evaluation should include an echocardiogram to evaluate degree of stenosis, gradient, and ejection fraction. Electrocardiogram should also be performed. Epidural analgesia should be avoided due to the risk of hypotension.

Mitral and aortic regurgitation

Both mitral and aortic regurgitation are generally well-tolerated in pregnancy. Long-standing and/or severe regurgitation may lead to left ventricular dysfunction or left atrial enlargement. Complications including pulmonary edema or arrhythmias are rare. Caution should be taken to avoid bradycardia, increase systemic vascular resistance, or medications that can suppress myocardial function.

Management pearls

Pre-pregnancy or antenatal evaluation should include an echocardiogram and electrocardiogram. Epidural analgesia and vaginal delivery are not contraindicated.

Prosthetic valves

The primary consideration for women with prosthetic valves in pregnancy is anticoagulation. There are two types of prosthetic valves, bioprosthetic (tissue) and mechanical, and the approach to anticoagulation differs based upon the type of valve used for replacement. Women with mechanical valves require anticoagulation for life, whereas women with bioprosthetic valves are typically managed with aspirin alone in the absence of another indication for anticoagulation. Although warfarin has been associated with a lower risk of thrombotic events in pregnancy,9 there is an increased risk for teratogenesis and its use should be limited to the highest risk patients and when the dose is typically less than 5 mg/day. Either low molecular weight heparin or continuous IV unfractionated heparin may be utilized, assuming anti-Xa or partial thromboplastin time (PTT) are monitored, respectively (Table 2).10 In addition to the potential for thrombosis of the valve, valve failure can also occur during pregnancy. Low-dose aspirin (75–100 mg daily) should be given in addition to anticoagulation for patients with prosthetic valves at increased risk for valve thrombosis.11

2

Options for anticoagulation.

Option

Medication

Monitoring

Comments

1

LMWH

enoxaparin 1 mg/kg subcutaneous every 12 hours

Anti-Xa level 4–6 hours post injection

Goal level 1 (0.8–1.2) U/mL


2

Dose-adjusted continuous UFH

aPTT ≥2 times normal

Heparin-induced thrombocytopenia more common than with LMWH

3

Option 1 or 2 through 12 weeks of gestation, then warfarin until 36 weeks or close to delivery, then resume option 1 or 2

INR 3 (2.5–3.5)


4

Warfarin throughout pregnancy until close to delivery

INR 3 (2.5–3.5)

For patients at highest risk given potential for warfarin embryopathy.

For patients with daily dose <5 mg.

LMWH, low molecular weight heparin; UFH, unfractionated heparin, aPTT, activated partial thromboplastin time; INR, international normalized ratio

Management pearls

Pre-pregnancy or antenatal evaluation should include an echocardiogram and electrocardiogram. Epidural analgesia and vaginal delivery are not contraindicated. However, anticoagulation must be held around the time of delivery with durations dependent upon which anticoagulant and the dose.

OTHER CONGENITAL HEART DISEASE

Atrial septal defect, ventricular septal defect, and patent ductus arteriosus

These lesions are overall low risk conditions for pregnancy. Paradoxical emboli can occur in the setting of septal defects; thus, air filters for intravenous lines should be considered. Arrhythmias may also occur but are rare. Perhaps the biggest concern with these lesions is the potential of the development of elevated pulmonary arterial pressures eventually leading to Eisenmenger syndrome.

Management pearls

Pre-pregnancy or antenatal evaluation should include an echocardiogram to evaluate the size of the lesion, shunt flow, and pulmonary artery pressures. Baseline electrocardiogram should also be performed. Epidural analgesia and vaginal delivery are not contraindicated.

Eisenmenger syndrome

This occurs when pulmonary arterial pressures exceed systemic pressures and right-to-left shunt across an intracardiac defect results in hypoxemia. Eisenmenger syndrome is one of the most dangerous conditions encountered in pregnancy, and the mortality rate is high at 30%;12 therefore, termination of pregnancy should be discussed with the patient. Maternal hypoxia results in increased fetal risks of intrauterine fetal growth restriction, preterm delivery, low birth weight, and perinatal death.13,14,15

Management Pearls

Pre-pregnancy or antenatal evaluation should include pulse oximetry, chest radiography, and electrocardiogram. Serial transthoracic and transesophageal echocardiography should be performed to evaluate the degree of shunting, right ventricular function, and pulmonary arterial pressures. Treatment with pulmonary artery vasodilators may be needed and prophylactic anticoagulation is recommended. Epidural analgesia is contraindicated (Table 3).

3

Approach to the most common high-risk cardiac lesions in pregnancy.

Condition

Main concern

Goals of therapy

What to look for

Anticoagulation

Assisted second stage (i.e., avoid Valsalva)

Pulmonary hypertension or Eisenmenger syndrome

Hypoxia (right-to-left shunt for Eisenmenger syndrome)

Prevent hypotension and increase in pulmonary vascular resistance (avoid hypoxia, acidemia)

Hypoxia (cyanosis, clubbing)

Yes, prophylactic

Yes

Systemic ventricular dysfunction

Heart failure

Standard heart failure therapy

Signs of heart failure, edema, dyspnea

Yes, depending on severity

Yes

Severe mitral stenosis

Pulmonary edema and arrhythmia

Prevent tachycardia, fluid overload, and hypotension

Pulmonary edema, atrial fibrillation, SVT

If atrial fibrillation

Consider

Severe aortic stenosis

Fixed cardiac output

Beta blockade to increase LV filling time; avoid bradycardia, hypovolemia or hypervolemia

Angina, syncope, hypervolemia

No

Yes

Dilated aortic root

Aortic dissection

Beta blockade to decrease aortic dilation

Tachycardia, hypertension, aortic dissection

No

Yes

Severe coarctation

Hypertension, aortic dissection, heart failure

Normotensive

Monitor lower and upper extremity blood pressures, hypertension

No

Yes

LV, left ventricle; SVT, supraventricular tachycardia

Hypertrophic cardiomyopathy

Women with hypertrophic cardiomyopathy (HCM) generally tolerate pregnancy well. Heart failure (15%) and arrhythmia (12%) are the most common complications.16 In one study, there were no differences observed in pregnancy outcomes among women with obstructive versus non-obstructive HCM.16

Management pearls

Pre-pregnancy or antenatal evaluation should include an echocardiogram and electrocardiogram. Beta blockade should be continued in patients already taking them and considered in cases where new symptoms occur, for rate control with atrial fibrillation, and to suppress ventricular arrhythmias.7 Epidural anesthesia should be used with caution in patients with severe obstruction. In the presence of diastolic dysfunction, volume overload should be avoided.

Coarctation of the aorta

Although uncorrected or native coarctation of the aorta is a high risk condition in pregnancy, it is rarely encountered in pregnancy as most patients undergo a corrective procedure in childhood. However, patients who have undergone repair remain at risk for complications including hypertension, aortic aneurysm, and re-coarctation. For monitoring of coarctation, a so called “peak-to-peak” is measured referring to the peak pressures proximal and distal to the narrowing in the aorta. A gradient exceeding 20 mmHg is considered an indication for surgical repair. Since the peak-to-peak gradient is a cardiac catheterization-based measurement, the mean gradient is utilized as an approximation on echocardiography. Clinically, peripheral blood pressures can be monitored in the upper and lower extremities; normally, the blood pressure in the upper extremities is lower than blood pressure in the lower extremities. Increasing blood pressure in the upper extremities over the lower extremities warrants workup for potentially worsening coarctation. Important associations with coarctation of the aorta include a bicuspid aortic valve (up to 40%) and intracranial aneurysms (10%). Therefore, magnetic resonance angiography of the thoracic aorta and the intracranial vessels is recommended for all patients.17

Management pearls

Pre-pregnancy or antenatal evaluation should include an echocardiogram to determine the severity of narrowing or re-coarctation. With corrected disease, epidural analgesia and vaginal delivery are not contraindicated. With uncorrected disease, epidural should be used with caution to avoid hypotension.

Tetralogy of Fallot

Tetralogy of Fallot is a complex cardiac disease consisting of four components: (1) ventricular septal defect, (2) overriding aorta, (3) right ventricular outflow obstruction, and (4) right ventricular hypertrophy. Most patients will have undergone surgical correction but may have right-sided heart failure, pulmonary regurgitation, dysrhythmias, shunting, pulmonary hypertension, aortic root dilation, or aortic insufficiency.

Management pearls

Pre-pregnancy or antenatal evaluation should include an echocardiogram to determine anatomy and valvular and ventricular function. Epidural analgesia and vaginal delivery are not contraindicated.

Aortopathy

Aortic aneurysm/dilation and aortic dissection are the major concerns in the setting of aortopathies including bicuspid aortic valve, Marfan syndrome, vascular Ehlers-Danlos syndrome, and Loeys-Dietz syndrome. Most data for aortic dissection is from women with Marfan syndrome in pregnancy where aortic dissection has been reported to occur in 5–10% of pregnancies.18,19,20 Due to this risk, pregnancy is contraindicated in Marfan syndrome when the aortic root diameter is ≥45 mm and in those with diameter ≥50 mm without Marfan syndrome and preconception repair is recommended.7,21 Beta blockers are typically prescribed to prevent tachycardia and hypertension, though the evidence for the efficacy of beta blockade for the prevention of aortic dissection is limited.22 Women with clinical features suggestive of Marfan syndrome should undergo further work-up (e.g. joint hypermobility, spontaneous pneumothorax, chest deformities, wide arm span, scoliosis, or arachnodactyly).23 Patients with aortopathies should also be counseled about the inheritance pattern of the disease (e.g. Marfan syndrome is autosomal dominant). Pregnancy is best avoided in those with vascular Ehlers-Danlos syndrome due to the high risk of mortality.7

Management pearls

Women with known aortic disease or aortic syndrome should have imaging of the entire aorta (computed tomography (CT) or magnetic resonance imaging (MRI)) prior to pregnancy. Pre-pregnancy or antenatal evaluation should include serial echocardiography with measurement of the aortic root every trimester or more frequently if concern for rapid enlargement. Beta blockade should be utilized to maintain a heart rate <100 beats per minutes. Epidural analgesia is not contraindicated. If the ascending aortic root diameter is 40–45 mm, vaginal delivery with expedited second stage (instrumental delivery) and regional anesthesia should be considered. Expedited second stage is recommended to avoid blood pressure peaks as this can increase the risk of aortic dissection.7 Cesarean delivery may also be considered if the aortic diameter is 40–45 mm based on the individual situation, especially in patients with Marfan or Loeys-Dietz syndrome. Cesarean delivery should be considered when the aortic diameter exceeds 45 mm in all aortopathies, and cesarean is recommended in all cases of vascular Ehlers-Danlos syndrome type IV or acute or chronic aortic dissection.

GENERAL ANTEPARTUM CARE

Monitoring patients for signs and symptoms of cardiac decompensation and failure, including dyspnea, fatigue, palpitations, orthopnea, and edema, is of utmost importance. Since fatigue and shortness of breath are not uncommon complaints of pregnancy, physical examination, vital signs, laboratory studies, and echocardiography or chest radiography may be utilized to distinguish “normal” from “abnormal” complaints. The most common cardiac complications among women with cardiac disease include pulmonary edema and arrhythmia.24 Fetal echocardiogram is recommended for all women at 20–24 weeks' gestation with a history of congenital heart disease due to the increased risk for fetal cardiac anomaly.

INTRAPARTUM CARE

Historically, expedited and assisted second stage with instrumental delivery including forceps or vacuum delivery has been recommended to avoid Valsalva in patients with cardiac disease. While this blanket recommendation simplifies decision making, assisted second stage is unlikely to be physiologically beneficial to all patients with the spectrum of cardiac disease. With Valsalva, forced expiration against a closed glottis causes increased intrathoracic and intraabdominal pressure, decreased venous return, and an increase in blood pressure and pulse. Reduced venous return/preload leads to decreased cardiac output. Therefore, cardiac lesions that are sensitive to a decrease in preload or an increase in afterload may be negatively impacted. Labor and contractions are associated with a further increase in cardiac output, particularly the second stage of labor. Additionally, the sympathetic response to pain and anxiety during labor results in an increase in heart rate and blood pressure. Epidural anesthesia decreases pain, and thus is expected to decrease tachycardia. However, there are conditions for which the hypotension associated with epidural analgesia may be detrimental (e.g. aortic stenosis). Cesarean delivery is typically only recommended in the setting of heart failure (ejection fraction <35%), aortic root dilation >40–45 mm (depending on underlying genetics diagnosis), and aortic dissection. Spontaneous bacterial endocarditis treatment is not recommended, even in the highest risk patients.

POSTPARTUM CARE

The postpartum period is an important time for vigilance in the care of the cardiac patients. After delivery, venous return and cardiac preload increase secondary to return of blood from the uterus and vena cava decompression. Additionally, systemic vascular resistance increases after delivery resulting in increased afterload. The increase in preload results in an increase in cardiac output of 60–80%.2 Hemodynamic changes resolve over the next 6–12 weeks postpartum but may persist for up to 6 months.25 Close monitoring with telemetry in an intensive care unit setting is recommended for 24–48 hours postpartum in patients considered high risk for arrhythmia or decompensation. Complications may occur up to several weeks postpartum; therefore, close follow-up and extensive patient education is warranted.

ACUTE CONDITIONS

Peripartum cardiomyopathy

Peripartum cardiomyopathy (PPCM) is an idiopathic cardiomyopathy manifested by systolic dysfunction and heart failure that occurs in the final months of pregnancy or up to 5 months postpartum with the majority of cases presenting in the first few weeks postpartum. PPCM is a diagnosis of exclusion with findings of decreased left ventricular ejection fraction and left ventricular dilation. Patients with PPCM present with typical signs and symptoms of heart failure including dyspnea, orthopnea, chest pain, and edema. Death can occur via progressive heart failure or sudden cardiac death related to arrhythmia or thromboembolic events. In severe cases, left ventricular assist devices or heart transplantation have been utilized. B-type natriuretic peptide levels, chest radiography, and echocardiography are useful in pregnancy for assisting with the diagnosis of heart failure. The addition of bromocriptine (accompanied by anticoagulation) to standard heart failure therapy for PPCM may improve outcomes in women with severe PPCM.7

Acute myocardial infarction

The increase in delayed childbearing and prevalence of obesity, diabetes, and hypertension is expected to result in an increase in the rare but serious complication of acute myocardial infarction (MI). Pregnancy increases the risk of MI compared to non-pregnant state, and the reported incidence in pregnancy is 6/100,000. The odds of MI have been reported to be 30-fold higher for women aged 40 or more.26 Perhaps the biggest issue with acute MI in pregnancy is a lack of suspicion on the part of the provider. As in the non-pregnant population, presenting signs can include chest pain, shoulder/jaw pain, and electrocardiogram changes. Serial troponin I serum levels are an important component of the work-up for acute MI. Treatment includes the same treatment as the non-pregnant individual with morphine, oxygen, nitroglycerin, and aspirin along with percutaneous coronary intervention as needed.

Arrhythmias

Pregnancy itself increases the risk of arrhythmia. Premature atrial contractions (PACs) and premature ventricular contractions (PVCs) are the most common arrhythmias and are generally clinically insignificant and do not require treatment. Supraventricular tachycardia (SVT) is a more common arrhythmia that can be resolved with vagal maneuvers or medical therapy (e.g. adenosine) if vagal maneuvers are unsuccessful. Atrial fibrillation or atrial flutter may be treated with a beta blocker or digoxin. Ventricular tachycardia is rare, but is typically associated with congenital heart disease and can be life-threatening. Medications typically used to treat ventricular tachycardia include beta blocker, sotalol, or electrical cardioversion.

4

Healthy (and unhealthy) habits when caring for cardiac pregnant patients.

Encouraged

Discouraged

Euvolemia (strict ins and outs intrapartum and postpartum)

Normal vital signs (blood pressure, pulse, pulse oximetry)

Monitoring for cardiac symptoms (dyspnea, fatigue, orthopnea, chest pain, peripheral edema, palpitations, dizziness/syncope)

Daily low-dose aspirin

Baseline cardiac function testing (history, review of systems, electrocardiogram, echocardiogram)

Fetal echocardiogram

Serial fetal growth ultrasound

Multidisciplinary care (maternal-fetal medicine, cardiology, anesthesiology, genetics, neonatology)

High index of suspicion for heart failure or myocardial infarction

Hypervolemia/hypovolemia

Hypertension/hypotension

Tachycardia/bradycardia

Hypoxia

Routine cesarean unless heart failure, aortic root dilation >45 mm, and aortic dissection

CONTRACEPTION

Appropriate and effective contraception for women with cardiac disease is paramount for avoiding pregnancy in certain conditions or for planning pregnancy in order to optimize cardiac status prior to conception. While there is no evidence comparing contraceptive options in women with cardiac disease, the WHO Medical Eligibility Criteria for Contraceptive Use (WHOMEC) serves as a guide for the safety of various contraceptives in specific medical conditions.27 Generally, combined estrogen and progesterone forms of contraception (e.g. combined oral contraceptive pills) are contraindicated in cardiovascular disease states with an increased thromboembolic risk and increase the risk of venous thromboembolism. Long-acting reversible contraception (LARC) methods, including progestin implants and progestin or copper intrauterine devices, are the most effective forms of contraception with the least systemic adverse effects. Progestin only oral contraceptive or progestin injections are acceptable alternatives, particularly if LARC is not available.

PRACTICE RECOMMENDATIONS

Ideally, patients with known cardiac disease are evaluated prior to conception with an emphasis on baseline physical activity tolerance, baseline cardiac function testing, and counseling on the risks of pregnancy (Tables 3 and 4). Consultation with a cardiologist and maternal-fetal medicine specialist is indicated, if possible. Recommended baseline studies include an electrocardiogram and echocardiography in addition to routine preconception care and detailed medication review. If already pregnant, these tests are recommended as soon as possible for baseline reference and for prognosis discussions. Patients should be frequently assessed for signs and symptoms of cardiac dysfunction. The frequency of follow-up echocardiogram assessment depends on the patient’s condition and should be individualized. Fetal echocardiography, ideally performed at 19–22 weeks, is recommended for pregnancies with maternal congenital heart disease. Periodic assessment of fetal growth and antepartum fetal surveillance may also be undertaken.


CONFLICTS OF INTEREST

Author(s) statement awaited.

REFERENCES

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6

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7

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14

Avila WS, Grinberg M, Snitcowsky R, et al. Maternal and fetal outcome in pregnant women with Eisenmenger's syndrome. Eur Heart J 1995;16(4):460–4.

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Warnes CA, Williams RG, Bashore TM, et al. ACC/AHA 2008 Guidelines for the Management of Adults with Congenital Heart Disease: Executive Summary: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to develop guidelines for the management of adults with congenital heart disease). Circulation 2008;118(23):2395–451.

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Bons LR, Roos-Hesselink JW. Aortic disease and pregnancy. Curr Opin Cardiol 2016;31(6):611–7.

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