NYHA class
Symptoms
I
Cardiac disease present, but no symptoms and no limitation in ordinary physical activity, e.g., no shortness of breath with walking (>3–4 city blocks), climbing stairs (two flights), etc.
II
Mild symptoms (mild shortness of breath and/or angina) and slight limitation during ordinary activity
III
Marked limitation in activity due to symptoms, even during less-than-ordinary activity, e.g., walking short distances (<100 m). Comfortable only at rest
IV
Severe limitations. Experiences symptoms even while at rest. Mostly bedbound patients
Table 2
Predictors of maternal cardiovascular events and risk score based on the CARPREG study
Cardiovascular event prior to pregnancy (heart failure, transient ischemic attack, stroke, or arrhythmia) |
Baseline NYHA functional class > II or cyanosis |
Obstructive left ventricular lesions (by echocardiography: mitral valve area <2 cm2, aortic valve area <1.5 cm2, peak LV outflow tract gradient > 30 mmHg) |
Left ventricular systolic dysfunction (ejection fraction <40 %) |
Table 3
Modified World Health Organization (WHO) classification of maternal cardiovascular risk principles
Risk class | Risk of pregnancy by medical condition |
---|---|
I | No or mild increase in maternal morbidity, and no discernable increased risk of maternal mortality |
II | Moderate increase in maternal morbidity and small increased risk of mortality |
III | Potential for severe maternal morbidity and significantly increased risk of mortality. Expert counseling regarding risk in pregnancy required. If pregnancy is decided upon, multidisciplinary cardiac and obstetric monitoring needed throughout pregnancy, childbirth, and puerperium |
IV | Extremely high risk of severe maternal morbidity or mortality; pregnancy contraindicated. If pregnancy occurs, termination should be discussed. If pregnancy continues, care as class III with multidisciplinary team |
Table 4
Modified WHO classification of maternal risk by cardiovascular disease
Conditions in which pregnancy risk is WHO I |
Mild pulmonic stenosis, patent ductus arteriosus, or mitral valve prolapse, if uncomplicated |
Repaired atrial or ventricular septal defect, patent ductus arteriosus, anomalous pulmonary venous drainage |
Isolated atrial or ventricular premature contractions |
Conditions in which pregnancy risk is WHO II to III |
WHO II (if otherwise asymptomatic and uncomplicated) |
Uncorrected atrial or ventricular septal defect |
Tetralogy of Fallot, repaired |
Majority of arrhythmias |
WHO II–III (depending on individual) |
Mild left ventricular systolic dysfunction |
Hypertrophic cardiomyopathy |
Valvular regurgitation or stenosis (native or prosthetic) not considered WHO I or IV |
Marfan syndrome in the absence of aortic dilation |
Aortic dilation (<45 mm) associated with bicuspid aortic valve |
Repaired aortic coarctation |
WHO III |
Prosthetic mechanical valve |
Systemic right ventricle |
Fontan circulation |
Cyanotic congenital heart disease (unrepaired) |
Complex congenital heart disease |
Marfan syndrome with aortic dilation of 40–45 mm |
Bicuspid aortic valve with aortic dilation of 45–50 mm |
Conditions in which pregnancy risk is WHO IV (pregnancy contraindicated) |
Pulmonary arterial hypertension (of any cause) |
Severe left ventricular systolic dysfunction (LVEF <30 %, NYHA III–IV) |
Prior peripartum cardiomyopathy, with any residual left ventricular systolic dysfunction |
Severe mitral stenosis, severe symptomatic aortic stenosis |
Marfan syndrome with aortic dilation of >45 mm |
Bicuspid aortic valve with aortic dilation of >50 mm |
Severe aortic coarctation |
The evaluation of regurgitant valvular lesions should include a complete evaluation of symptoms, echocardiographic evaluation of regurgitation severity, left ventricular dimensions, and systolic function [6]. Ideally, valvular dysfunction should be assessed prior to pregnancy to establish the stability of the regurgitation over time, to counsel on maternal and fetal risk, and to initiate therapy appropriate for pregnancy. Patients with severely regurgitant valves are recommended against pregnancy prior to valve repair or replacement. In moderate-to-severe regurgitant valves, exercise testing is recommended to fully characterize the patient’s functional capacity, the hemodynamic consequences of the lesion, and evaluate for inducible pulmonary hypertension. Symptoms during pregnancy can typically be managed conservatively with judicious use of diuretics for congestive symptoms and afterload-reducing agents, such as hydralazine. Angiotensin Converting Enzyme (ACE) inhibitors and angiotensin receptor blockers, which are also afterload-reducing agents, are strictly contraindicated in pregnancy due to their teratogenicity.
In acute severe regurgitation refractory to therapy, surgery may be unavoidable. If the fetus is mature enough, delivery should occur prior to surgery. In patients with any valvular regurgitation, vaginal delivery is preferable, and symptomatic patients should receive regional anesthesia with epidural or spinal to minimize the cardiovascular consequences of catecholamine surges. In some cases, an assisted second stage of labor may be appropriate [1].
Congenital bicuspid aortic valves are often associated with an underlying aortopathy, and it is important to screen for concomitant aortic dilation and aneurysm. Likewise, there is an important link between bicuspid aortic valves and coarctation of the aorta, and individuals with bicuspid aortic valves should be screened for this condition as well. Approximately 50 % of patients with bicuspid aortic valve and aortic valve pathology (aortic stenosis or aortic regurgitation) have dilation of the ascending aorta [7]. Also, dilation is often maximal at the distal end of the ascending aorta, which cannot be adequately visualized with transthoracic echocardiography, and Magnetic Resonance Imaging (MRI) or Compted Tomography (CT) of the chest should be performed prior to conception. Pre-pregnancy surgery should be considered when the thoracic aortic diameter reaches 50 mm [8]. Immer et al. [9] found that increased aortic root dilation during pregnancy in patients with bicuspid aortic valve carries a significant risk of type A aortic dissection.
Our patient underwent a transthoracic echocardiogram, with visualization of the aortic root, which was mildly dilated. The entire ascending aorta, aortic arch, and descending aorta were not fully visualized. Dedicated MRI of the aorta without contrast administration (an optimal choice to decrease radiation exposure) showed stable dilation of the aortic root and no evidence of coarctation, thoracic aneurysm, or dissection. She underwent a normal pregnancy, with regularly scheduled follow-up with her cardiologist. In the setting of congenital bicuspid aortic valve disease, screening of first-degree relatives, including children, siblings, and parents, is recommended [8].
Case 2: Arrhythmia in Pregnancy
A 31-year-old woman presents to the emergency department at 35-weeks gestation with acute shortness of breath, chest pressure, and sudden onset of palpitations for the preceding 30 minutes. Her medical history is notable for a history of hyperthyroidism for which she received radioactive iodine ablation. She takes levothyroxine 75 mcg by mouth daily, which was recently increased in the first trimester. Six years ago, she had presented to the emergency department with thyrotoxicosis and a supraventricular tachycardia. Her initial vital signs demonstrate a heart rate of 164 beats per minute, blood pressure of 109/56 mmHg, and respiratory rate of 26 respirations per minute. An electrocardiogram is shown in Fig. 1. Laboratory values are not yet obtained. Carotid sinus pressure is applied at the bedside, which decreases the rate to 146 beats per minute (bpm) briefly. Adenosine 6 mg IV once is administered, with a two-second pause and termination of the arrhythmia with conversion to sinus rhythm at 83 bpm. Her shortness of breath and chest discomfort resolve. She is admitted for further monitoring on telemetry, but she remains in sinus rhythm for 24 hours (h) and is discharged home without medication changes. Twelve days later, she re-presents with recurrent symptoms and is found to have a supraventricular tachycardia at 157 bpm. She is given adenosine 6 mg IV once, with resolution of the tachycardia and reestablishment of sinus rhythm. During the preceding 10 days, she noted intermittent palpitations for 1–2 min before resolving either on their own or with vagal maneuvers. She is admitted for monitoring and started on digoxin 0.125 mg daily. She undergoes induction of labor on hospital day three, with placement of an epidural, and delivery of a healthy baby boy at 37 0/7 weeks gestation. On hospital day six, she undergoes an electrophysiology study and atrioventricular nodal reentrant tachycardia (AVNRT) ablation. She is discharged on hospital day seven in stable condition.
Fig. 1
ECG of supraventricular tachycardia
Premature extra beats and atrial tachyarrhythmias are more frequent and may even manifest for the first time, during pregnancy. The sensation of palpitations is common in pregnancy, with sinus tachycardia, premature atrial contractions, and premature ventricular complexes representing the most common findings [10, 11]. Premature beats manifest most frequently during the second trimester. They are not associated with adverse maternal or fetal outcomes and require treatment only if symptoms are intolerable to the mother. The occurrence of atrial fibrillation or atrial flutter is relatively rare during pregnancy and is usually associated with hyperthyroidism or structural cardiac disease [1], such as underlying valvular disease, congestive heart failure, or congenital heart disease.
AVNRT is the most common supraventricular arrhythmia in pregnant and nonpregnant women. In patients with a preexisting history of supraventricular tachycardia, the incidence of recurrence during pregnancy is as high as 20–44 % in case studies [12, 13]. Previously highly symptomatic tachyarrhythmias should be treated with catheter ablation prior to pregnancy, when possible. During pregnancy, as in our patient, most supraventricular arrhythmias represent a WHO class II risk category, with a small increase in maternal mortality and a moderate increase in morbidity.
Typically, supraventricular tachyarrhythmias are not well tolerated in pregnancy, if rapid and sustained. Thus, the general preference is to restore sinus rhythm. AVNRT or atrioventricular reentrant tachycardia (AVRT) involving an accessory pathway can often be terminated by successful vagal maneuvers, such as having the patient perform the Valsalva maneuver and cough vigorously or by applying carotid sinus pressure. If vagal maneuvers fail, adenosine is the first drug of choice and can be administered safely and intravenously in pregnancy for diagnostic and therapeutic purposes [13, 14]. Adenosine interrupts conduction down the accessory atrioventricular nodal pathway by prolonging the refractory period of the atrioventricular (AV) node and revealing the underlying atrial arrhythmia. Intravenous metoprolol can be used if adenosine fails and serves to slow the ventricular rate to control symptoms but will not typically convert the rhythm to sinus. Direct current synchronized cardioversion is also safe in all stages of pregnancy [13] and is used typically in situations of hemodynamic instability or arrhythmias refractory to medical therapy.
Digoxin is considered safe in pregnancy and can be used to control the ventricular rate but is not an effective medication to use in the acute setting [13]. Other agents, such as specific medications acting on the AV node (beta-blockers, non-dihydropyridine calcium channel blockers; most class C agents) (Table 5), may also be safe and tolerated during pregnancy to treat the symptoms of the arrhythmia. Antiarrhythmic drug therapies can be toxic to the developing fetus and should be discontinued prior to conception [13]. Major controlled studies of antiarrhythmic drugs during pregnancy are lacking. Typically, antiarrhythmic therapy is reserved for hemodynamic compromise or refractory or recurrent arrhythmia. Specific antiarrhythmic medications are considered safer to use in pregnancy, such as sotalol (class B), flecainide (class C), and ibutilide (class C) [13]. They have not demonstrated any adverse fetal effects, but experience with these medications in pregnancy is limited. Amiodarone is usually recommended against in pregnancy. It is a pregnancy class D medication and its use in pregnancy should be restricted to arrhythmias that are resistant to other drugs or are life threatening [15]. Bypass tract or atrioventricular nodal ablation is possible during pregnancy if necessary and is best performed during the second trimester when the fetus has undergone initial development and the mother and fetus can still be adequately shielded from radiation exposure.
Table 5
Federal drug administration pregnancy categories
Risk class | Risk of medication in pregnancy |
---|---|
A | Adequate and well-controlled studies have failed to demonstrate a risk to the fetus in the first trimester of pregnancy (and there is no evidence of risk in later trimesters) |
B | Animal reproduction studies have failed to demonstrate a risk to the fetus, and there are no adequate and well-controlled studies in pregnant women |
C | Animal reproduction studies have shown an adverse effect on the fetus, and there are no adequate and well-controlled studies in humans, but potential benefits may warrant use of the drug in pregnant women despite potential risks |
D | There is positive evidence of human fetal risk based on adverse reaction data from investigational or marketing experience or studies in humans, but potential benefits may warrant use of the drug in pregnant women despite potential risks |
X | Studies in animals or humans have demonstrated fetal abnormalities, and/or there is positive evidence of human fetal risk based on adverse reaction data from investigational or marketing experience, and the risks involved in use of the drug in pregnant women clearly outweigh potential benefits |
Case 3: Valvular Stenosis, Mitral Stenosis
A 28-year-old Indian American woman presents for evaluation at 8-weeks gestation with a recent diagnosis of rheumatic mitral stenosis (MS). This is her second pregnancy, and the first was an uncomplicated pregnancy with vaginal delivery and home birth three years ago with a midwife while living in India. She was noted to have a murmur on exam by her current midwife and referred for further evaluation. She underwent a transthoracic echocardiogram one week ago which demonstrated mild dilation of the left atrium and a characteristic rheumatic deformity of the mitral valve with severe mitral stenosis and a calculated mitral valve area of 1.3 cm2. Prior to her pregnancy, she did not experience dyspnea on exertion, orthopnea, paroxysmal nocturnal dyspnea, or peripheral edema. She does note occasional palpitations that she describes as “skipped beats” for the last four weeks, occurring approximately every other day. She does not exercise regularly and is able to care for her three-year-old son, cook, and clean the home without limitations.