This document discusses heart disease in pregnancy. It notes that pregnant women with heart disease are at higher risk for complications. However, the changes of pregnancy can make diagnosing heart conditions difficult. Key signs of heart disease include severe dyspnea, syncope with exertion, and chest pain. If heart disease is suspected, confirmatory tests should be done while accounting for normal pregnancy changes. Pre-conception counseling is important to advise patients on risk levels and necessary pre-pregnancy treatment. Certain high-risk conditions like severe valvular disease may require terminating the pregnancy due to prohibitive maternal mortality risks.

1. Chapter 39
Cardiac Diseases
Daniel G. Blanchard, MD, and Ralph Shabetai, MD
pregnancy. The most important of these are increases in blood volume,
cardiac output, and heart rate. These adaptations exacerbate the symp-
Diagnosis of Heart Disease toms and clinical signs of heart disease and may necessitate significant
in Pregnancy escalation in treatment.
Cardiac risk varies among the specific forms of heart disease and
Pregnant women with heart disease are at higher risk for cardiovascu- also with severity. During prepregnancy counseling, the physician
lar complications during pregnancy and also have a higher incidence should describe the nature of the heart disease in terms comprehensi-
of neonatal complications.1 However, the significant hemodynamic ble to the prospective parents. The risk to the woman, which can vary
changes that accompany pregnancy make the diagnosis of certain from negligible to prohibitive, should be spelled out as clearly as pos-
forms of cardiovascular disease difficult. During normal pregnancies, sible.7 On this basis, the patient may be advised either that the con-
women frequently experience dyspnea, orthopnea, easy fatigability, templated pregnancy is safe, will be uncomfortable and will necessitate
dizzy spells, and, occasionally, even syncope. On physical examination, treatment, carries a significantly increased risk, or would be extremely
dependent edema, rales in the lower lung fields, visible neck veins, and dangerous and should not be undertaken.
cardiomegaly are commonly found. Systolic murmurs occur in more In the case of certain cardiac conditions, the patient should be
than 95% of pregnant women, and internal mammary flow murmurs strongly advised to undergo the necessary treatment before pregnancy
and venous hums are common. A third heart sound (S3 gallop) is often and to allow several months to elapse before becoming pregnant.
present.2 Nevertheless, certain findings indicate heart disease in preg- Examples in this category include the following:
nancy and should suggest the presence of a significant cardiovascular
abnormality. These symptoms include severe dyspnea, syncope with Large intracardiac shunt (atrial or ventricular septal defect) with
exertion, hemoptysis, paroxysmal nocturnal dyspnea, and chest pain mild to moderate pulmonary hypertension
related to exertion. Physical signs of organic heart disease include a Patent ductus arteriosus (PDA) with mild to moderate pulmonary
fourth heart sound (S4 gallop), cyanosis, clubbing, diastolic murmurs, hypertension
sustained cardiac arrhythmias, and loud, harsh systolic murmurs.3 Severe coarctation of the aorta
If there is a strong suspicion of heart disease during pregnancy, Severe mitral stenosis or regurgitation
confirmatory diagnostic tests should be initiated. The changes of Severe aortic stenosis or regurgitation
normal pregnancy must be recognized so that the findings are not Tetralogy of Fallot
misinterpreted. For example, nonspecific ST segment and T-wave Various congenital malformations and acquired heart diseases
abnormalities and shifts in the electrical axis can occur.4 Pregnancy
also produces changes in the echocardiogram, including alterations in Again, it is imperative that the palliative procedure be carried out
cardiac dimensions and performance. The internal dimensions of all before pregnancy is undertaken and that a year or so elapse before
the cardiac chambers are increased, and slight regurgitation through pregnancy occurs. Flexibility in clinical judgment is necessary, however.
the four valves is frequently observed. The ejection fraction (EF) and A woman with moderately severe valvular disease may require a pros-
stroke volume are concomitantly larger, and the cardiac output is thetic valve in the future. In such a case, the patient should be advised
increased.5 A small pericardial effusion can be a normal finding in to have her family before valve replacement—with its associated anti-
pregnant women.6 Both radiographic and radionuclide diagnostic pro- coagulant risk—is required8 (see Pregnancy in Patients with Artificial
cedures should be avoided during pregnancy unless the procedure is Heart Valves, later). The valvular heart lesions associated with high and
deemed essential for the health and safety of the mother. low maternal and fetal risk during pregnancy are listed in Tables 39-1
and 39-2.
As previously noted, some cardiac disorders are so serious in nature
Pre-Conception Counseling that the physiologic changes of a superimposed pregnancy pose pro-
hibitive risks to the mother; they carry such a high maternal mortality
If a woman plans to become pregnant but knows that she has heart risk that pregnancy is contraindicated. In such circumstances, patients
disease, she and her physicians must be fully aware of several funda- must be strongly cautioned against becoming pregnant. If such a
mental principles. The cardiovascular system undergoes specific adap- patient is seen for the first time when she is already pregnant, termina-
tations to meet the increased demands of the mother and fetus during tion of the pregnancy is recommended. The most serious of the cardiac

2. 798 CHAPTER 39 Cardiac Diseases
TABLE 39-1 VALVULAR HEART LESIONS TABLE 39-3 HIGH-RISK MATERNAL
ASSOCIATED WITH HIGH CARDIOVASCULAR DISORDERS
MATERNAL AND/OR FETAL RISK
Estimated Maternal
DURING PREGNANCY
Disorder Mortality Rate (%)
Severe AS with or without symptoms Aortic valve stenosis 10-20
AR with NYHA functional class III-IV symptoms Coarctation of the aorta 5
MS with NYHA functional class II-IV symptoms Marfan syndrome 10-20
MR with NYHA functional class III-IV symptoms Peripartum cardiomyopathy 15-60
Aortic and/or mitral valve disease resulting in severe pulmonary Severe pulmonary hypertension 50
hypertension (pulmonary pressure greater than 75% of Tetralogy of Fallot 10
systemic pressures)
Aortic and/or mitral valve disease with significant LV dysfunction
(EF < 40%)
Mechanical prosthetic valve requiring anticoagulation
Marfan syndrome with or without AR If a patient with one of these disorders presents when she is already
pregnant, she should be strongly urged to consider early termina-
AR, aortic regurgitation; AS, aortic stenosis; EF, ejection fraction; LV, tion. A carefully planned suction curettage before 13 weeks’ gestation
left ventricular; MR, mitral regurgitation; MS, mitral stenosis; NYHA,
would place such a patient at minimal risk. Termination of pregnancy
New York Heart Association.
Reproduced with permission from Bonow RO, Carabello B, DeLeon AC, beyond 13 weeks increases the risk to the mother, because many of
et al: ACC/AHA 2006 guidelines for the management of patients with the cardiovascular alterations that occur in pregnancy have taken
valvular heart disease. Circulation 114:84, 2006. place.
Infective endocarditis often causes rapid and serious deterioration
of the cardiac status, posing a major threat to the life and health of the
TABLE 39-2 VALVULAR HEART LESIONS mother and, therefore, of the fetus as well. Scrupulous attention to
ASSOCIATED WITH LOW prophylaxis against endocarditis is critical during pregnancy. Pregnant
MATERNAL AND FETAL RISK women must pay meticulous attention to their dental health; if they
have cardiac lesions susceptible to infective endocarditis, neglect of
DURING PREGNANCY
antibacterial prophylaxis could have dire consequences. In general,
Asymptomatic AS with low mean gradient (<25 mm Hg and aortic women with valvular heart disease should have antibiotic prophylaxis
valve area >1.5 cm2) in the presence of normal LV systolic at the time of delivery.10,11
function (EF > 50%) The prospective parents will want to know not only about the risk
NYHA functional class I or II AR with normal LV systolic function to the health and life of the future mother but also about the fetal risks.
NYHA functional class I or II MR with normal LV systolic function
One of the most important questions is whether the mother’s heart
MVP with no MR or mild to moderate MR with normal LV systolic
disease is hereditary and, if so, what is the risk that the infant will be
function
Mild MS (mitral valve area >1.5 cm2, gradient <5 mm Hg) without born with the same defect. A detailed family cardiac history must be
severe pulmonary hypertension obtained before pregnancy, especially if the prospective mother has
Mild to moderate pulmonary valve stenosis heart disease.
Some of the cardiomyopathies, especially hypertrophic forms, may
AR, aortic regurgitation; AS, aortic stenosis; EF, ejection fraction; LV, be inherited in a mendelian manner.12 Familial dilated cardiomyopathy
left ventricular; MR, mitral regurgitation; MS, mitral stenosis; MVP,
has also been described. Approximately 20% of idiopathic dilated car-
mitral valve prolapse; NYHA, New York Heart Association.
Reproduced with permission from Bonow RO, Carabello B, DeLeon AC, diomyopathy is inherited.13 There is a strong familial tendency in
et al: ACC/AHA 2006 guidelines for the management of patients with certain congenital malformations, such as PDA and arterial septal
valvular heart disease. Circulation 114:84, 2006. defect (ASD). Additionally, mothers with congenital heart disease
may have children with unrelated congenital malformations: this risk
appears to be approximately 5%.3,14 Also, pregnant women with
disorders are those involving pulmonary hypertension, particularly advanced heart disease, especially those with low cardiac output or
those associated with a right-to-left shunt in cardiac blood flow severe hypoxia, experience a greatly increased incidence of spontane-
(Eisenmenger syndrome). Low cardiac output states and entities ous abortion, stillbirths, and small or deformed children.9 For most
in which there is an increased risk of aortic dissection (Marfan pregnant women with heart disease, vaginal delivery (with a low
syndrome) also represent an extraordinarily high risk of maternal threshold for forceps or vacuum assistance) is recommended. Elective
mortality. These high-risk maternal cardiovascular disorders are cesarean section is recommended in cases of Marfan syndrome or
listed in Table 39-3. aortic aneurysm of any cause.3
In some women with specific dangerous cardiovascular diseases, Today’s prospective mother wants to know about the risks to her
pregnancy is contraindicated because of the substantial risk of mater- fetus of drugs, other therapies, and diagnostic tests that are used to
nal death.9 Examples include the following: treat heart disease. Echocardiography poses no threat to the fetus, but
radiation incurred with radionuclide angiography, cardiac catheteriza-
Dilated cardiomyopathy or left ventricular dysfunction tion with contrast angiography, or computed tomography pose a
(EF < 40%) of any cause potential hazard to the fetus. If these studies are required, they should
Severe pulmonary hypertension of any cause be performed before pregnancy occurs; they should be repeated there-
Marfan syndrome, especially with aortic root dilation (diameter after only if mandated for the safety of the mother, and pelvic shielding
> 4 cm) should be used.

3. CHAPTER 39 Cardiac Diseases 799
Maternal infection with the virus that causes German measles vena cava, accounting for lower cardiac output. This is one reason why
(rubella) is associated with a high risk of congenital malformation of some obstetricians prefer to manage labor with the patient in the left
the fetal heart as well as PDA. If the patient has not had German decubitus position.
measles as a child and has never been inoculated against it and her
antibody titer confirms the absence of immunity, she should be vacci-
nated some months before becoming pregnant. Cardiac Performance
Every pregnant woman who is known or thought to have heart Echocardiographic studies have shown increases in the left ventricular
disease should, at a minimum, be evaluated once by a cardiologist who fiber shortening velocity and in EF. These changes do not necessarily
understands the cardiovascular adaptations to pregnancy. The cardi- indicate increased myocardial contractility but may simply be the
ologist will prescribe necessary diagnostic studies and treatments and, result of decreased peripheral vascular resistance and increased preload.
of equal importance, will not allow unnecessary ones. The effects of In any case, stroke volume is increased, and cardiac output is further
heart disease can often be ameliorated by correcting coexisting medical augmented by the 10% to 15% increase in heart rate that characterizes
problems, such as anemia, chronic infection, anxiety, thyroid dysfunc- normal pregnancy.18
tion, hypertension, and arrhythmia. Demands on the cardiovascular system increase significantly
during labor and delivery. Pain increases sympathetic tone, and uterine
contractions induce wide swings in the systemic venous return. With
placental separation, autotransfusion of at least 500 mL takes place,
Cardiovascular Adaptations placing an acute load on the diseased heart, unless offset by blood loss.
These large shifts in blood volume can precipitate, on the one hand,
to Pregnancy shock, and on the other, pulmonary edema in women with severe heart
disease.
Increased Blood Volume If a chest radiograph is obtained in a pregnant woman, the cardiac
The cardiovascular alterations observed in pregnancy are discussed in silhouette often appears slightly enlarged owing to the combined
detail in Chapter 7 but are reviewed here briefly. It is worthwhile to effects of volume overload and elevation of the diaphragm. Routine
reconsider and emphasize some of the most important cardiovascular echocardiographic studies have demonstrated that a small, silent peri-
changes that occur during pregnancy, because they may significantly cardial effusion is quite common.6
alter the course of cardiac disease or may themselves be influenced by
a specific disorder.
Blood volume and cardiac output increase during pregnancy.15 The Electrocardiographic Changes
uterus hypertrophies, endometrial vascularization is greatly increased, The mean QRS axis may shift to the left19 as a result of the elevated
and the placenta becomes a highly vascular structure that functions to diaphragm. In later pregnancy, the axis may shift to the right when the
some extent as an arteriovenous shunt. In addition, generalized arte- fetus descends into the pelvis. Minor ST segment and T-wave changes
riolar dilation develops, mediated most probably by estrogen. These may be observed, usually in lead III but sometimes aVF as well. Less
mechanisms combine to lower systemic vascular resistance and increase often, T inversions may appear transiently in the left precordial leads.
the pulse pressure. The total blood volume rises steadily during the Occasionally, small Q waves may accompany T-wave inversion in leads
first trimester and is increased by almost 50% by the 30th week, III and aVF. These changes are seldom of sufficient magnitude to raise
remaining more or less constant thereafter.16 Several mechanisms are the question of ischemic heart disease, which in any case is relatively
responsible for increasing blood volume in pregnancy, including uncommon in pregnancy, especially if the mother is young and free
steroid hormones of pregnancy, elevated plasma renin activity, and from symptoms. Extrasystoles and supraventricular tachycardia are
elevated plasma aldosterone levels. Human placental lactogen, atrial more common during pregnancy. Symptoms of palpitations are
natriuretic factor, and other peptides may also play significant roles in common during pregnancy but only rarely signify the presence of
governing changes of blood volume in pregnancy. Hypervolemia also organic heart disease.
occurs with trophoblastic disease, indicating that a fetus is not essential
for its development. Heart rate increases by 10 to 20 beats/min. In a
normal pregnancy, blood pressure does not increase, because the
increased intravascular volume is balanced by decreased peripheral General Guidelines
vascular resistance mediated by the placenta. Plasma volume tends to
increase more than the red blood cell mass, accounting for a “physio-
for Management
logic anemia” that is common in pregnancy. Treatment with iron cor- During treatment of all pregnant patients with heart disease, priority
rects the anemia which, if left untreated, may become significant must be given to maternal health, but all possible therapeutic measures
(hematocrit as low as 33% and hemoglobin 11 g/dL). should also be taken to protect the developing fetus. The aspects of
management are outlined in Table 39-4.
Because pregnancy increases the demands on the heart, physical
Cardiac Output exertion frequently must be restricted, especially if it causes symptoms.
Cardiac output rises during the first few weeks of pregnancy and is Some women with certain forms of cardiac disease, such as significant
30% to 45% above the nonpregnant level by the 20th week, remaining mitral stenosis and cardiomyopathy, tolerate pregnancy poorly and
there until term.15 The increase in cardiac output in the first trimester cannot endure physical exertion. They may require strict bed rest for
begins rapidly and peaks between the 20th and 26th week. Early in the duration of the pregnancy, particularly during the last trimester.
pregnancy, the dominant factor is elevated stroke volume; later, Women with heart disease have a limited ability to increase cardiac
increased heart rate predominates.17 In late pregnancy, the enlarged output to meet increased metabolic demands and should minimize the
uterus partially impedes venous return by compressing the inferior demands placed on the heart from physical activity.

4. 800 CHAPTER 39 Cardiac Diseases
TABLE 39-4 CARDIAC DISEASE IN inhibitors during pregnancy.25,26 These complications suggest a pro-
PREGNANCY: ASPECTS found and deleterious effect on fetal renal function, leading to decreased
renal function and oligohydramnios, as well as neonatal renal failure.
OF MANAGEMENT
ACE inhibitors are absolutely contraindicated during pregnancy.
Activity restriction Another class of antihypertensive drugs, the angiotensin receptor
Diet modification blockers, also may affect fetal renal function and are likewise absolutely
Team approach for medical care contraindicated in pregnancy.
Infection control The indications and possible adverse effects of commonly pre-
Immunizations scribed cardioactive drugs during pregnancy are summarized in
Prophylaxis against bacterial endocarditis
Table 39-5.
Prophylaxis against rheumatic fever
Interruption of pregnancy
Counseling
Contraception or sterilization
Team Approach to Medical Care
Cardiovascular surgery Medical care for pregnant women with heart disease is best provided
Cardiovascular drugs through the cooperative efforts of a cardiologist who is familiar with
the hemodynamic changes of pregnancy and an obstetrician. Frequent
visits to both specialists, along with open consultations, can provide
the patient with consistent advice and reassurance and can circumvent
Cardiovascular Drugs the worry and anxiety created by confusing and conflicting informa-
Some of the drugs commonly used in the management of cardiovas- tion. In addition, the anesthesiologist needs to be consulted during
cular disease have potentially harmful effects on the developing embryo the antepartum period to outline the anticipated approach to intra-
and fetus. For example, there is no question that oral anticoagulants partum management, a time of maximum risk for most of these
are potential teratogens when administered in the first trimester (see women. The role of the anesthesiologist and the approach to women
Chapter 20 and later discussion in this chapter). The “warfarin embry- with pregnancies complicated by cardiac disease are summarized in
opathy syndrome,” consisting of nasal hypoplasia, optic atrophy, digital Chapter 56.
abnormalities, and mental impairment, occurs in a minority of cases.
The actual risk of warfarin embryopathy is difficult to estimate and
has ranged from 4% to 67% in various reports.20,21 A risk of 4% to 10%
seems more reasonable.22 There is some evidence that embryopathy is
Congenital Heart Disease
less likely if the warfarin dose is 5 mg/day or less.23 The fetal risks A number of simple congenital malformations are compatible with a
continue beyond the first trimester, because warfarin increases the normal or nearly normal pregnancy. Congenital malformations previ-
possibility of both fetal and intrauterine bleeding. ously associated with high maternal morbidity and mortality and fetal
Anticoagulation presents a significant practical problem in the wastage now frequently end with a satisfactory outcome because of
management of atrial fibrillation, systemic or pulmonary embolism, palliative or corrective surgery. Despite recent advances, however,
thrombophlebitis, and pulmonary hypertension in pregnancy. The most women with congenital heart disease who become pregnant still have
vexing problem arises in the setting of prosthetic heart valves10,11 (dis- a significant risk of miscarriage, cardiac complications, and premature
cussed later). In the case of mechanical valve prostheses, warfarin delivery.27
appears to be superior to heparin in preventing valvular thrombosis. The care of adults with congenital heart malformation is an impor-
Although heparin is safer for the fetus, there is probably an increased tant and growing branch of cardiology7,28,29 that requires the coopera-
risk for the mother.20,24 This is a complex medical issue, and no random- tive efforts of medical and pediatric cardiologists, cardiac surgeons,
ized trial to determine the optimal anticoagulant therapy for women and, in the case of pregnancy, obstetricians and anesthesiologists.30,31
with a prosthetic valve has been conducted. Therefore, recommenda-
tions are based on smaller studies and on clinical judgment.10,11 No
single regimen is likely to be applicable to all such cases, because the Left-to-Right Shunt
issue is complicated by the type and generation of the prosthetic valve,
the cardiac rhythm, and the size and contractility of the cardiac Atrial Septal Defect
chambers. ASD may be undiscovered before pregnancy, because symptoms are
β-Adrenergic blocking agents, used for the treatment of hyper- often absent and the physical findings are not blatant. Other causes of
tension and tachyarrhythmia, have been associated with neonatal left-to-right shunt, such as PDA and ventricular septal defect (VSD),
respiratory depression, sustained bradycardia, and hypoglycemia when are more likely to be discovered and treated in infancy or childhood.
administered late in pregnancy or just before delivery. However, if they Physicians should be alert to the higher possibility of uncorrected
are used judiciously in selected cases (e.g., in women with cardiomy- defects in women who have immigrated from an undeveloped
opathy and heart failure), β-blockers are usually well tolerated. country.
The thiazide diuretics are another class of drugs that can produce Closure of uncomplicated large ostium secundum ASD is straight-
harmful effects on the fetus—especially if they are used in the third forward and safe and usually is curative. Therefore, the procedure
trimester or for extended periods—and may impair normal expansion should be done before pregnancy. Many ASDs can now be closed per-
of plasma volume. Rarely, severe neonatal electrolyte imbalance, cutaneously, using a “clamshell” or “umbrella” device inserted via a
jaundice, thrombocytopenia, liver damage, and even death have been transvenous catheter.32 If the patient is unwilling to undergo ASD
reported. closure, she can be advised that the lesion is unlikely to complicate
There have been numerous reports of fetal and neonatal renal pregnancy and labor, provided that pulmonary hypertension is not
complications after the use of angiotensin-converting enzyme (ACE) present.3

5. CHAPTER 39 Cardiac Diseases 801
TABLE 39-5 INDICATIONS FOR AND POSSIBLE ADVERSE EFFECTS OF COMMONLY PRESCRIBED
CARDIOACTIVE DRUGS ON MOTHER AND FETUS
Drug Use in Pregnancy Potential Side Effects Breastfeeding Risk Category*
Adenosine Maternal and fetal arrhythmias No side effects reported; data on use during Data NA C
first trimester are limited
Amiodarone Maternal arrhythmias IUGR, prematurity, congenital goiter, Not C
hypothyroidism and hyperthyroidism, recommended
transient bradycardia, prolonged QT in the
newborn
ACEIs and Hypertension Oligohydramnios, IUGR, prematurity, neonatal Compatible X
angiotensin hypotension, renal failure, anemia, death,
receptor skull ossification defect, limb contractures,
blockers patent ductus arteriosus
β-Blockers Hypertension, maternal arrhythmias, Fetal bradycardia, low placental weight, possible Compatible; Acebutolol: B
myocardial ischemia, mitral IUGR, hypoglycemia; no information on monitoring Labetalol: C
stenosis, hypertrophic carvedilol of infant’s Metoprolol: C
cardiomyopathy, hyperthyroidism, heart rate Propranolol: C
Marfan syndrome recommended Atenolol: D
Digoxin Maternal and fetal arrhythmias, No evidence for unfavorable side effects on the Compatible C
heart failure fetus
Diltiazem Myocardial ischemia, tocolysis Limited data; increased incidence of major birth Compatible C
defects
Disopyramide Maternal arrhythmias Limited data; may induce uterine contraction Compatible C
and premature delivery
Diuretics Hypertension, congestive heart Hypovolemia leads to reduced uteroplacental Compatible C
failure perfusion, fetal hypoglycemia,
thrombocytopenia, hyponatremia,
hypokalemia; thiazide diuretics can inhibit
labor and suppress lactation
Flecainide Maternal and fetal arrhythmias Limited data; 2 cases of fetal death after Compatible C
successful treatment of fetal SVT reported,
but relation to flecainide uncertain
Heparin Anticoagulation None reported Compatible C
Hydralazine Hypertension None reported Compatible C
Lidocaine Local anesthesia, maternal No evidence for unfavorable fetal effects; high Compatible C
arrhythmias serum levels may cause CNS depression at
birth
Nifedipine Hypertension, tocolysis Fetal distress related to maternal hypotension Compatible C
reported
Nitrates Myocardial infarction and ischemia, Limited data; use is generally safe; few cases Data NA C
hypertension, pulmonary edema, of fetal heart rate deceleration and
tocolysis bradycardia have been reported
Procainamide Maternal and fetal arrhythmias Limited data; no fetal side effects reported Compatible C
Propafenone Fetal arrhythmias Limited data; fetal death reported after direct Data NA C
intrauterine administration in fetuses with
fetal hydrops
Quinidine Maternal and fetal arrhythmias Minimal oxytocic effect, high doses may cause Compatible C
premature labor or abortion; transient
neonatal thrombocytopenia and damage to
eighth nerve reported
Sodium Hypertension, aortic dissection Limited data; potential thiocyanate fetal toxicity, Data NA C
nitroprusside fetal mortality reported in animals
Sotalol Maternal arrhythmias, hypertension, Limited data; 2 cases of fetal death and 2 cases Compatible; B
fetal tachycardia of significant neurologic morbidity in monitoring
newborns reported, as well as bradycardia in of infant’s
newborns heart rate
recommended
Verapamil Maternal and fetal arrhythmias, Limited data; other than 1 case of fetal death of Compatible C
hypertension, tocolysis uncertain cause, no adverse fetal or newborn
effects reported
Warfarin Anticoagulation Crosses placental barrier; fetal hemorrhage in Compatible X
utero, embryopathy, CNS abnormalities
ACE, angiotensin-converting enzyme inhibitor; CNS, central nervous system; IUGR, intrauterine growth retardation; NA, not available; SVT,
supraventricular tachycardia.
*U.S. Food and Drug Administration classification of drug risk. B: Either animal reproduction studies have not demonstrated a fetal risk but there are
no controlled studies in pregnant women, or animal reproduction studies have shown an adverse effect that was not confirmed in controlled studies
in women. C: Either studies in animals have revealed adverse effects on the fetus and there are no controlled studies in women, or studies in women
and animals are not available. Drug should be given only if the potential benefits justify the potential risks to the fetus. D: There is positive evidence
of human fetal risk, but the benefits from use in pregnant women may be acceptable despite the risk. X: Studies in animals or human beings have
demonstrated fetal abnormalities. The risk of the use of the drug in pregnant women clearly outweighs any possible benefit. The drug is
contraindicated in women who are or may become pregnant.
Source: Drug Information for the Health Care Professional (USDPI Vol 1). Micromedex, 23rd ed, January 1, 2003. (Adapted and modified from Elkayam
U. Pregnancy and cardiovascular disease. In Zipes DP, Libby P, Bonow RO, Braunwald E (eds): Braunwald’s Heart Disease: A Textbook of Cardiovascular
Medicine, 7th ed. Philadelphia: Elsevier, 2005, p 1965.)

6. 802 CHAPTER 39 Cardiac Diseases
Metcalfe and colleagues33 reported one maternal death among 219
pregnancies in 113 women with ASD. Peripheral vasodilation, if any-
thing, reduces the left-to-right shunt.17 Because ASD in young women
is not associated with heart failure, diuretics and extreme limitation of
LA
intravenous infusion are not warranted. A small percentage of patients
with ASD have atrial flutter or fibrillation, which usually is paroxysmal.
This arrhythmia can be managed along conventional lines, often
with digoxin if necessary. The prospective mother should be informed
that closure of the defect does not prevent atrial fibrillation once the
arrhythmia has occurred.
ASD can be difficult to diagnose during pregnancy. The murmur RA
associated with ASD may be inconspicuous, being a pulmonary ejec-
RV
tion systolic murmur and therefore not unlike the physiologic murmur
of pregnancy. However, the second heart sound is widely split and may
be fixed throughout the respiratory cycle, a distinctly abnormal finding.
The electrocardiogram (ECG) shows incomplete right bundle branch
block and, in the case of the much more common ostium secundum
defect, right axis deviation. In the less common ostium primum defect,
marked left axis deviation accompanies incomplete right bundle
branch block. The chest radiograph shows right atrial and right ven-
tricular enlargement, prominent pulmonary arteries, and plethoric
lung fields. Echocardiography establishes or confirms the diagnosis LA
(Fig. 39-1), obviating the need for cardiac catheterization in many
cases.
Complicated Atrial Septal Defect
If atrial arrhythmias recur frequently—and especially if the heart rate
is difficult to control—catheter ablation is successful in restoring RA
normal sinus rhythm without the need for antiarrhythmic drugs. In
most cases, this procedure should not be done until after delivery RV
because of the extensive radiation exposure that is needed. In rare
instances, pregnancy and labor may be associated with a paradoxical
systemic embolus resulting from a thrombus migrating from the infe-
rior vena cava across the ASD into the left atrium.
In the uncommon event that the patient is more than 35 years old
and has an uncorrected large ASD, the likelihood of chronic atrial
fibrillation, right ventricular dysfunction, and pulmonary hyperten- FIGURE 39-1 Transesophageal echocardiographic image of atrial
sion rises significantly. Pregnancy is not advised if any of these sequelae septal defect (ostium secundum). In the upper panel, a large defect
is present. If the patient insists on going through with the pregnancy, in the interatrial septum is present. In the lower panel, the blue/
prolonged bed rest will be required, and vigorous treatment of heart yellow color represents blood flow from the left atrium (LA) into the
failure may be needed. The maternal risk is increased, and there is sig- right atrium (RA). RV, right ventricle.
nificant risk of fetal loss. Although warfarin is generally recommended
for chronic atrial fibrillation, its use is best avoided (especially in the
first trimester); aspirin would be a reasonable compromise. Severe These findings constitute the maladie de Roger. Prophylaxis against
pulmonary hypertension is an uncommon feature of an ostium secun- infective endocarditis is indicated, but otherwise this lesion has no
dum ASD but is a contraindication to pregnancy. The ostium primum effect on pregnancy or labor.
ASD, which is associated with Down syndrome and poses a risk of When the defect is in the membranous septum, spontaneous
endocarditis, is more often associated with severe pulmonary hyper- closure is rare. In the absence of significant pulmonary vascular disease,
tension. Infective endocarditis rarely, if ever, complicates a simple the same pansystolic murmur and thrill are found. If the shunt is large,
ostium secundum ASD; therefore, prophylaxis during labor is not however, the lung fields are plethoric on chest radiography, and the
warranted. heart and pulmonary arteries are enlarged. The classic ECG shows a
pattern of biventricular hypertrophy. In such cases, flow through the
Ventricular Septal Defect pulmonary vascular bed is usually at least twice the systemic cardiac
The clinical spectrum and risk of VSD may range from so mild that it output. Patients with a relatively large, uncomplicated left-to-right
has little or no effect on pregnancy to so high that maternal or fetal shunt through a VSD tolerate pregnancy well and, in this respect, are
death can occur. Small defects in the muscular ventricular septum comparable to patients with ASD. However, prophylaxis against endo-
frequently close spontaneously during childhood. However, these carditis is essential in cases of VSD.
defects occasionally persist, allowing a small left-to-right shunt, mani- Here it is appropriate to detour from clinical description to
fested by a loud pansystolic murmur along the left sternal border pathophysiology. Pulmonary vascular resistance is calculated as the
accompanied by a coarse thrill. The chest radiograph is often normal, pressure drop across the pulmonary vascular bed divided by the flow
as is the ECG. The echocardiogram is usually diagnostic (Fig. 39-2). through it:

7. CHAPTER 39 Cardiac Diseases 803
where P is the pressure drop and Q is the flow across the pulmonary
vascular bed.
A patient at one extreme may have a large shunt with pulmonary
flow of 20 L/min and an R of 3 units, yielding an MPAP of 55 mm Hg
(assuming a normal MPCWP of 5 mm Hg). At the other extreme, a
patient with pulmonary vascular disease may have a pulmonary blood
flow of 7 L/min and an R of 7 units, yielding an MPAP of 44 mm Hg.
The higher the pulmonary vascular resistance (R), the greater the
maternal risk. The risk is prohibitive when R reaches the systemic level
(approximately 15 Wood units). In borderline cases (e.g., patients with
R between 5 and 8 units), a pulmonary arteriolar vasodilating agent is
rv lv sometimes administered to determine whether the increased resistance
is partially reversible or completely irreversible. An increase in pulmo-
nary vascular resistance of 3 to 4 units is considered mild, 5 to 7 units
is moderate, and more than 8 units is severe.
VSD may be associated with considerable increase in pulmonary
vascular resistance, reflecting occlusive disease of the small pulmonary
arteries and arterioles. This development, if it is to occur, usually does
so in childhood; unless corrected, it leads to the Eisenmenger syn-
drome (discussed later). However, a small number of adults may
survive with VSD and pulmonary vascular resistance that is signifi-
la
cantly elevated but falls short of the Eisenmenger syndrome. Such
patients are at high risk for death during pregnancy or labor, and there
is a high risk of fetal impairment or loss. The patient should be told
ra that early therapeutic abortion would be the safest option, and that
later pregnancy would be hazardous and would require intensive care,
with physical exercise strictly curtailed and prolonged bed rest enforced.
The combination of decreased physical activity, pulmonary hyperten-
lv sion, and pulmonary vascular disease would constitute a sound ratio-
rv nale for instituting anticoagulation, which is another reason why
pregnancy is better avoided or terminated.
Some authorities strongly advise that delivery be effected prema-
turely by means of cesarean section and urge sterilization at the same
operation. The dangers must be thoroughly understood by women in
this category who insist on continuing pregnancy.
FIGURE 39-2 Transesophageal echocardiographic image of a
Patent Ductus Arteriosus
small muscular ventricular septal defect. In the upper panel, a The loud, continuous or machinery murmur of typical PDA with
small communication is seen (arrow) between the right ventricle (rv) a large left-to-right shunt and no pulmonary vascular disease is so
and left ventricle (lv). In the lower panel, color imaging confirms blood striking that the lesion is almost invariably detected and corrected in
flow between the two chambers. la, left atrium; ra, right atrium. infancy or childhood. Occasionally, however, women of childbearing
age or pregnant women from underdeveloped countries may present
R = (MPAP − MPCWP)/Qpulm with a PDA. If the left-to-right shunt is large, the circulation is hyper-
dynamic, with a wide arterial pulse pressure, low arterial diastolic
where R is pulmonary vascular resistance in Wood units; MPAP and pressure, and hyperactive precordium. The heart may be somewhat
MPCWP (in mm Hg) are the mean pulmonary arterial and capillary enlarged to clinical and radiologic examination, and the ECG may
wedge pressures, respectively; and Qpulm is total flow through the right show left ventricular hypertrophy. The echocardiogram is useful for
heart and pulmonary circulation (i.e., cardiac output plus left-to-right demonstrating a shunt between the two great vessels (Fig. 39-3). The
shunt) in liters per minute. signs of hyperdynamic circulation resulting from the PDA are
Resistance can be described in Wood units or in dyne·s/cm5. The exaggerated by pregnancy.
Wood unit has the merit of simplicity and is derived from clinical units Because the murmur of PDA is systolic-diastolic, it is commonly
of pressure and flow. The more fundamental but less friendly dyne·s/ referred to as a “continuous” murmur, although it usually peaks late in
cm5 can be obtained by multiplying Wood units by 80. Normal pul- systole. Because of its characteristics, the murmur is also referred to as
monary vascular resistance is 0.5 to 1.5 units. When a clinician is faced a “machinery” murmur. It is maximal in the left infraclavicular region.
with a pregnant woman with pulmonary hypertension, the key to her It must be distinguished from a venous hum, which is loudest in the
risk during pregnancy lies in the pulmonary vascular resistance. High neck rather than the infraclavicular area. Venous hum is common in
flow, by itself, can be the mechanism for pulmonary hypertension pregnant women, and it changes dramatically with changes in the
without dangerous elevation in the resistance. This mechanism can be position of the head.
appreciated by rewriting the resistance equation to read Division or occlusion of the PDA should be accomplished before
pregnancy is undertaken. Currently, most PDAs can be closed by the
P=Q·R insertion of an occluder device delivered via a percutaneous intravas-

8. 804 CHAPTER 39 Cardiac Diseases
Head and
upper extremities
AO SVC
Aorta
PA
PA
Right
PA lung PV Left
lung
LA PV
PA
RA
LV
FIGURE 39-3 Transesophageal echocardiographic image of a Descending
patent ductus arteriosus. A communication is present between the aorta
RV
proximal portion of the descending aorta (AO) and the pulmonary
artery (PA). Color imaging (arrow) confirms blood flow from the aorta
into the PA. IVC
cular catheter.34 If a patient does become pregnant before PDA occlu-
sion, an uncomplicated left-to-right shunt can be managed safely.
Endocarditis is a risk in patients with PDA, and antibiotic prophylaxis
is required. Embolic complications of infective endocarditis and end-
arteritis secondary to PDA may take the form of infected pulmonary
emboli. The patient becomes febrile with respiratory symptoms, and
the chest radiograph shows multiple opacities and infiltrates. Trunk and
lower extremities
The leading cause of Eisenmenger syndrome is a large VSD, fol-
lowed in prevalence by a large PDA. As with VSD, individuals with PDA Arterial blood Venous and arterial
may sustain severe increases in pulmonary vascular resistance with the (fully saturated) blood
corresponding pulmonary hypertension and right ventricular hyper- Cyanosis visible
trophy, yet fall short of Eisenmenger physiology. The maternal risk
Small admixture of Venous blood
during pregnancy is high in this situation, similar to that encountered venous blood
in VSD with equivalent pathology. Treatment is the same as in VSD No visible cyanosis
with Eisenmenger syndrome. When the pulmonary pressure rises, the
aortopulmonary shunt decreases, and the murmur becomes progres- FIGURE 39-4 Eisenmenger complex. Here the cause of right-to-left
sively quieter and shorter, until it finally disappears. shunt across the ventricular septal defect is increased pulmonary
In general, the woman with uncomplicated PDA tolerates preg- vascular resistance arising in the small pulmonary arteries and
nancy well. If pulmonary hypertension supervenes, the risk to the arterioles. IVC, inferior vena cava; LA, left atrium; LV, left ventricle;
PA, pulmonary artery; PV, pulmonary vein; RA, right atrium; RV, right
mother becomes significant. Therefore, if pulmonary hypertension is
ventricle; SVC, superior vena cava. (Reprinted by permission of the
suspected and documented, termination of pregnancy is strongly publisher. From Taussig HB: Congenital Malformations of the Heart.
recommended. Cambridge, Mass, Harvard University Press, 1960. Copyright © 1960
by the Commonwealth Fund by the President and Fellows of Harvard
College.)
Eisenmenger Syndrome
Eisenmenger syndrome is characterized by a congenital communica-
tion between the systemic and pulmonary circulations and increased ventricles. It is more common in girls and develops at a young age.
pulmonary vascular resistance, either to systemic level (so that there is Therefore, when increased pulmonary vascular resistance is detected
no shunt across the defect) or exceeding systemic (allowing right-to- in a child with a large VSD, operative closure must be done as soon as
left shunting). As mentioned, the most common underlying defect is possible to prevent the development of Eisenmenger pathophysiology.
a large VSD, followed in prevalence by a large PDA. Eisenmenger Once this has appeared, pulmonary hypertension is irreversible, and
pathophysiology is less common in ASD. Occasionally, this type of the VSD is consequently inoperable (unless lung transplantation is
pathophysiology develops in other, less common defects. By the time performed as well).
the syndrome is fully developed, it is often difficult clinically to diag- The major clues that pulmonary vascular resistance is increasing
nose the underlying defect. For this discussion, the VSD serves as a are (1) diminution of evidence of a left-to-right shunt and (2) the
good model (Fig. 39-4). appearance of progressively severe pulmonary hypertension. The pan-
Eisenmenger pathophysiology develops only if the defect is large systolic murmur of VSD or the continuous murmur of PDA is replaced
and is not restrictive, resulting in equal systolic pressure in the two by a short ejection systolic murmur. The lungs are no longer plethoric

9. CHAPTER 39 Cardiac Diseases 805
but show large central pulmonary arteries and small peripheral arteries until term, treated with oxygen and heparin, and delivered by cesarean
characteristic of severe pulmonary hypertension. Because the shunt section. One patient died a month after delivery. Most of the infants
has disappeared, the radiographic cardiothoracic ratio returns to were small, and one died. Despite this better-than-average outcome,
normal but the main pulmonary segment is prominent. There is pregnancy should not be encouraged in women with Eisenmenger
usually a striking right ventricular heave, a loud and palpable pulmo- syndrome or in those with a systemic level of pulmonary hypertension
nary valve closure sound, and an ejection sound in early systole. When of any cause.
concentric ventricular hypertrophy gives way to dilatation and right-
sided heart failure, evidence of tricuspid regurgitation appears. Until
then, the mean venous pressure is normal but the amplitude of the a Primary Pulmonary Hypertension
wave may be increased, reflecting decreased right ventricular diastolic Severe idiopathic (“primary”) pulmonary hypertension, like the
compliance. Eisenmenger syndrome, carries a high risk in pregnancy, and the
If pulmonary vascular resistance is significantly higher than sys- same principles apply to its management. Pregnancy is not advised in
temic levels, right-to-left shunting of blood occurs and causes cyanosis women with this condition, because the mortality rate approaches
and clubbing of the fingers and toes. This shunting of deoxygenated 50%.40
blood into the systemic circulation leads to hypoxemia and triggers a Severe pulmonary hypertension can result from taking appetite-
reactive erythrocytosis as the system attempts to increase peripheral suppressing drugs. The fenfluramine-phentermine regimen (“fen-
oxygen delivery. This increases blood viscosity and can cause sludging phen”) was a notorious culprit41 and was withdrawn from the market.
and decreased flow of blood, especially in small vessels. A high hemat- Treatment strategies include vasodilators, sometimes by chronic intra-
ocrit value, however, is not an automatic indication for serial phle- venous infusion, and nitric oxide inhalation. In some 25% of cases,
botomy, because this approach can lead to iron deficiency and pulmonary arterial pressure is lowered by prostacyclin infusion.42 This
microcytosis. Tissue hypoxia may then actually worsen, a particularly response predicts a favorable response to chronic oral nifedipine
undesirable result in pregnancy. Phlebotomy is reserved for patients administration and a good prognosis. Balloon atrial septostomy,43,44
without evidence of iron deficiency on laboratory testing who have through the foramen ovale or via transseptal puncture, can be used, in
symptoms of hyperviscosity, including headache, dizziness, visual dis- extreme cases of pulmonary hypertension, to relieve right heart pres-
turbance, myalgia, and bleeding diathesis. Quantitative volume replace- sure, usually as a bridge to transplantation.
ment is necessary during phlebotomy.
Attempted surgical correction of a congenital cardiac shunt after
Eisenmenger syndrome is present usually results in the death of the
patient.35 Many patients ultimately die of right-sided heart failure, Congenital Obstructive
pulmonary hypertension, or pulmonary hemorrhage.36 Lesions
The woman with Eisenmenger syndrome must be informed that
pregnancy carries a mortality risk of about 50%.37 Even if the mother Some congenital cardiac malformations are characterized by obstruc-
survives, the outcome for the fetus is likely to be poor, because the fetal tion to left or right ventricular outflow. The more common examples
mortality rate exceeds 50% in cyanotic women with Eisenmenger syn- include pulmonary stenosis, aortic stenosis, and coarctation of the
drome.36 Sudden death may occur at any time, but labor, delivery, and aorta. The hypoplastic left heart syndrome seldom allows survival to
particularly the early puerperium seem to be the most dangerous childbearing age, and those who do survive usually have undergone a
periods.38 Any significant fall in venous return, regardless of cause, major palliative procedure, such as construction of a ventriculoaortic
impairs the ability of the right heart to pump blood through the high, conduit with a prosthetic valve, that would constitute a strong contra-
fixed pulmonary vascular resistance. Hypotension and shock can occur indication to pregnancy.
quickly and are often unresponsive to medical therapy.
The major physiologic difficulty in pulmonary hypertension is
maintenance of adequate pulmonary blood flow. Any event or condi- Mitral Stenosis
tion that decreases venous return, such as vasodilation on the systemic Congenital mitral stenosis is a rare malformation. When it is associated
side of the circulation from epidural anesthesia or pooling of blood in with an ASD, it constitutes the Lutembacher syndrome. Survival to
the lower extremities from vena caval compression, decreases preload childbearing age is usual. Both lesions tend to promote atrial fibrilla-
to the right ventricle and pulmonary blood flow. Therefore, manage- tion. Ideally, the mitral valve and the atrial defect should be repaired
ment during pregnancy centers on the maintenance of pulmonary before pregnancy.
blood flow. If the patient insists on continuing her pregnancy, limita-
tion of physical activity is essential, as is the use of pressure-graded
elastic support hose, low-flow home oxygen therapy, and monthly Aortic Stenosis
monitoring of blood and platelet counts. Because of the precarious
(see also the later section on aortic stenosis under
physiologic balance, a planned delivery should be performed with
Aortic Valve Disease)
intensive care monitoring, including a Swan-Ganz catheter and provi-
sions for skilled obstetric anesthesia care. Anesthetic considerations for Bicuspid aortic valve is one of the more common congenital malfor-
this entity are discussed in Chapter 56. mations that may lead to aortic stenosis, regurgitation, or both (Fig.
On a more optimistic note, a report published in 1995 described 39-5). Often, aortic stenosis is not present during early life but pro-
13 pregnancies in 12 women with Eisenmenger syndrome who elected gresses over time because of valve calcification and gradual restriction
not to accept advice to terminate pregnancy.39 Mean systolic pulmo- in leaflet motion. The bicuspid valve may occur as an isolated defect
nary arterial pressure was 113 mm Hg. Three spontaneous abortions, or in combination with other congenital anomalies, most commonly
one premature labor, and two maternal deaths occurred. The seven aortic coarctation. Congenital aortic stenosis, on the other hand, can
patients who reached the end of the second trimester were hospitalized be severe at birth and may cause severe left ventricular hypertrophy

10. 806 CHAPTER 39 Cardiac Diseases
LA
AV
PA
RVOT
RV
FIGURE 39-5 Transesophageal echocardiographic image of a
bicuspid aortic valve. During systole, only two aortic valve (AV)
leaflets are seen. LA, left atrium; RVOT, right ventricular outflow tract.
FIGURE 39-6 Transesophageal echocardiographic image of
pulmonic stenosis. The pulmonic valve leaflets exhibit characteristic
that limits the ability of the heart to respond to demands for increased doming (arrow). PA, pulmonary artery; RV, right ventricle.
cardiac output.
In the syndrome of severe congenital aortic stenosis, the pulses are
of slow upstroke and diminished amplitude. Unlike adults with
acquired aortic stenosis, children and young adults with congenital Vasodilators, helpful in patients with heart failure of other etiology,
aortic stenosis have an abnormally loud aortic valve closure sound. Left are dangerous in patients with aortic stenosis, because the impeded left
ventricular ejection is prolonged, so that the aortic valve closure sound ventricle may not be able to fill the dilated peripheral vascular bed. It
may occur after the pulmonary valve closure sound. Therefore, split- should be remembered that the lowered systemic vascular resistance
ting of the second heart sound is paradoxical and is heard in expiration of pregnancy adversely affects aortic stenosis. The obstructed left ven-
instead of inspiration. Often a loud ejection sound is heard in early tricle is limited in its ability to fill the dilated peripheral bed, a situation
systole. The duration of the ejection murmur and the time to its peak that can lead to syncope or more serious manifestations of limited,
intensity increase with worsening severity of aortic stenosis. relatively fixed cardiac output.
The ECG shows severe left ventricular hypertrophy. The chest
radiograph is characterized by poststenotic dilatation of the aorta.
Although some patients complain of dyspnea, chest pain, and syncope, Pulmonic Stenosis
others remain asymptomatic. The lesion can be recognized and its The murmur of pulmonic stenosis is loud and is often accompanied
severity assessed by Doppler echocardiography. by a thrill. The lesion is usually detected in early childhood and is likely
Critical calcific aortic stenosis is usually treated by aortic valve to have been corrected before the childbearing age. Expectant mothers
replacement in older patients, but aortic valve repair is often possible who have not had adequate health supervision in childhood may have
in younger women of childbearing age with congenital aortic stenosis. unrecognized pulmonary stenosis.
If aortic stenosis is severe—and especially if it is symptomatic—the The diagnosis is suggested by a long, harsh systolic murmur over
woman should be advised against becoming pregnant. She should be the upper left sternal border that is usually preceded by an ejection
advised that, if the aortic valve must be replaced, pregnancy and labor sound. The venous pressure is normal, but there are striking a waves
would be difficult and dangerous because of the need for anticoagulant in the jugular venous pulse. The pulmonary valve closure sound is
treatment after a mechanical prosthesis is implanted. Maternal mortal- usually too soft to hear when pulmonary stenosis is severe. Severe
ity rates as high as 17% have been reported,45 although more recent pulmonary stenosis causes massive concentric right ventricular
data have suggested a somewhat lower risk. However, these studies also hypertrophy; this is manifested by a left parasternal heave and by tall
emphasize the adverse effects of severe maternal aortic stenosis on fetal R waves and deeply inverted T waves in the right precordial leads of
outcomes, including increased rates of preterm delivery and intrauter- the ECG. Tall, pointed P waves are also present, denoting right atrial
ine growth restriction.46 If aortic stenosis is moderate in severity, the enlargement.
patient should be advised to complete her pregnancies before the aortic Right ventricular enlargement and poststenotic dilatation of the
valve is replaced. Labor can be managed in such cases without a high main and left pulmonary arteries are seen on the chest radiograph,
maternal or fetal risk, but assisted shortening of the second stage of which also may show slightly diminished peripheral pulmonary vas-
labor is recommended.47 culature. Echocardiography demonstrates limited opening of the pul-
Strict limits on physical exertion and prolonged periods of bed rest monic valve leaflets (Fig. 39-6), right ventricular hypertrophy, and
may be required. Left ventricular failure may appear and may necessi- abnormally high velocity of blood flow in the pulmonary artery.
tate the use of diuretic agents and digitalis. Rarely, even in the presence Doppler echocardiography also allows calculation of the right ven-
of severe aortic stenosis, heart failure may be due to another cause (e.g., tricular pressure and the systolic pressure gradient across the valve.
peripartum cardiomyopathy).48 Prophylaxis against bacterial endocar- These pressures can also be measured directly in the hemodynamics
ditis at delivery is recommended. laboratory (Fig. 39-7).

11. CHAPTER 39 Cardiac Diseases 807
Aorta LV
LV
Septal
RV defect RV
Pulmonary
artery
Tetralogy of Fallot Normal
FIGURE 39-8 Tetralogy of Fallot. The anatomic pathology (left)
compared with normal (right). Note the ventricular septal defect, the
aorta (which overrides the defect), the pulmonary stenosis, and the
right ventricular hypertrophy. LV, left ventricle; RV, right ventricle.
(Reprinted by permission of the publisher. From Taussig HB:
Congenital Malformations of the Heart. Cambridge, Mass, Harvard
University Press, 1960. Copyright © 1960 by the Commonwealth
Fund of the President and Fellows of Harvard College.)
FIGURE 39-7 Pressure tracings in severe pulmonary stenosis.
Pulmonary pressure is extremely low and appears damped. Right
ventricular pressure is suprasystemic. (From Shabetai R, Adolph RJ:
Principles of cardiac catheterization. In Fowler NO [ed]: Cardiac
Diagnosis and Treatment. Hagerstown, MD: Harper & Row, 1980,
p 106.)
RV
Pulmonic stenosis is generally well tolerated so that neither preg-
nancy nor labor poses a significant threat.49 Prophylaxis against infec-
tive endocarditis is necessary. More severe pulmonary stenosis requires LV
AO
treatment. Unlike aortic stenosis, however, critical pulmonary stenosis
does not require valve replacement or open repair. Most cases are
treated successfully with transvenous balloon valvuloplasty.50 Ideally, MV
this should be carried out before pregnancy is undertaken; if a woman
does become pregnant and develops intractable right-sided heart
failure, the procedure can still be safely performed (but at some risk
to the fetus). Extreme pulmonary stenosis (right ventricular systolic
pressure > systemic systolic pressure) is a contraindication to preg- FIGURE 39-9 Transthoracic echocardiographic image of tetralogy
nancy until the lesion is adequately treated. of Fallot. A large ventricular septal defect is present, and the aorta
(AO) overrides the interventricular septum. LV, left ventricle; MV,
mitral valve; RV, right ventricle.
Right-to-Left Shunt without Pulmonary
Hypertension (Tetralogy of Fallot) outflow obstruction that diverts blood flow through the VSD. In the
The congenital cyanotic heart diseases discussed so far have been asso- typical case, right and left ventricular systolic pressures are equal but
ciated with a communication between the pulmonary and systemic the pulmonary artery pressure is exceedingly low. A loud, long systolic
circulations and pulmonary vascular resistance sufficiently high to murmur is audible along the left sternal border. The murmur is caused
cause a right-to-left shunt. However, cyanosis occurs in other congeni- by an abnormal flow pattern through the obstructed right ventricular
tal malformations, in which there is a defect between the right and left outflow tract. The pulmonary valve closure sound is usually inaudible.
sides of the heart but also right ventricular outflow obstruction (Figs. Patients are usually cyanotic and often have significant clubbing of the
39-8 and 39-9). Examples include the tetralogy of Fallot and tricuspid fingers and toes. The hematocrit value is greatly elevated because of
atresia. the severe erythrocytosis. Phlebotomy is not indicated to treat the
Tetralogy of Fallot is used to illustrate this class of congenital mal- hematocrit level per se but is indicated if symptoms of hyperviscosity
formation of the heart, because it is by far the most common form of occur. Ignoring this important therapeutic principle leads to a micro-
cyanotic congenital heart disease encountered in pregnancy. Moreover, cytic anemia that further complicates pregnancy. The ECG shows
the offspring of a mother with tetralogy of Fallot has a 2% to 13% severe right ventricular hypertrophy. The chest radiograph is charac-
chance of inheriting the condition.51 The syndrome includes (1) a large terized by a normal-sized heart and a concavity in the region where
defect high in the ventricular septum; (2) pulmonary stenosis, which the pulmonary artery should be (Fig. 39-11). As in all malformations
may be at the valve itself but more commonly is in the infundibulum of this general type, the lung fields are oligemic, showing small vessels
of the right ventricle; (3) dextroposition of the aorta so that the aortic throughout.
orifice sits astride the VSD and overrides, at least in part, the right Most adults born with the tetralogy of Fallot and lesions with
ventricle; and (4) right ventricular hypertrophy (Fig. 39-10). similar pathophysiology have undergone surgical treatment before
A wide spectrum of clinical presentations may be present, depend- reaching young adulthood. Children raised in undeveloped countries
ing on the relative size of the VSD and the degree of right ventricular are an important exception. Many patients have had surgery to close

12. 808 CHAPTER 39 Cardiac Diseases
The cyanotic patient with tetralogy of Fallot has special problems
Head and
upper extremities during pregnancy. The reduced systemic vascular resistance of preg-
nancy causes more blood to shunt from right to left, leaving less to
flow to the pulmonary circulation. This intensifies hypoxemia and can
lead to syncope or death. Maintenance of venous return is crucial. The
SVC most dangerous times for these women are late pregnancy and the
Aorta early puerperium, because venous return is impeded by the large
PA
PA gravid uterus near term and by peripheral venous pooling after deliv-
ery. Pressure-graded elastic support hose are recommended. Blood loss
Right
lung PV Left during labor may compromise venous return, and blood volume must
PA lung be promptly and adequately restored. Anesthetic considerations during
delivery are discussed in detail in Chapter 56. Antibiotic prophylaxis
LA PV should be used in these susceptible patients at delivery.
Because of the combined high maternal risk and high incidence of
fetal loss, pregnancy is discouraged in women with uncorrected tetral-
RA ogy of Fallot. The prognosis is particularly bleak in those women with
LV a history of repeated syncopal episodes, a hematocrit level greater than
Descending 60%, or a right ventricular systolic pressure greater than 120 mm Hg.
RV aorta If a young woman with untreated tetralogy of Fallot requests prepreg-
nancy counseling, she should be advised to undergo surgical correction
before pregnancy. Pregnancy does not represent a significantly
IVC
increased risk for patients in whom the VSD has been patched and the
pulmonary stenosis corrected.
Coarctation of the Aorta
Coarctation of the aorta is a congenital defect in the area of the aorta
where the ligamentum arteriosum and the left subclavian artery insert
(the distal portion of the aortic arch). The malformation may be
Trunk and simple or complex, and it is either isolated or associated with PDA and
lower extremities
other malformations, notably aortic stenosis and aortic regurgitation
Arterial blood Venous and arterial secondary to a bicuspid aortic valve. It may also occur in women with
(fully saturated) blood Turner syndrome. The lesion should be detected and treated surgically
Cyanosis visible or by balloon dilation in infancy or childhood, but it may be present
in women who are, or want to become, pregnant.
Small admixture of Venous blood
venous blood Typical features include the following:
No visible cyanosis
Upper extremity hypertension but lower extremity hypotension
FIGURE 39-10 Tetralogy of Fallot. Blood shunts from left to right Visible and palpable collateral arteries in the scapular area
through the ventricular septal defect because its flow to the lungs is A late systolic murmur, usually loudest over the interscapular
impeded by pulmonary stenosis; this results in cyanosis. IVC, inferior region
vena cava; LA, left atrium; LV, left ventricle; PA, pulmonary artery; PV, Femoral pulses that lag behind the carotid pulses and are of
pulmonary vein; RA, right atrium; RV, right ventricle; SVC, superior
diminished amplitude
vena cava. (Reprinted by permission of the publisher. From Taussig
Notching of the inferior rib borders seen on the chest radiograph
HB: Congenital Malformations of the Heart. Cambridge, Mass,
Harvard University Press, 1960. Copyright © 1960 by the and resulting from erosion by arterial collaterals that bridge the
Commonwealth Fund by the President and Fellows of Harvard coarctation
College.)
Electrocardiographic evidence of severe left ventricular hypertro-
phy strongly suggests associated aortic stenosis. Surgical grafting or
the VSD and relieve the pulmonary stenosis, constituting virtual “total percutaneous intravascular balloon dilation reduces the upper extrem-
repair” and rendering them potentially safe candidates for pregnancy ity hypertension, but blood pressure does not always return to normal,
and delivery. However, the operation is not curative. Significant and hypertension may recur in later life.
arrhythmia and conduction defects that may eventually lead to the Whenever possible, the operation should be performed before
need for electronic cardiac pacing or an implantable defibrillator may pregnancy; otherwise, the maternal mortality rate is approximately
occur years after an apparently successful operation. Other sequelae 3%. Coarctation is associated with congenital berry aneurysm of the
and residua include only partial relief of the right ventricular outflow circle of Willis and hemorrhagic stroke. The risk of stroke may increase
obstruction and pulmonic regurgitation. This latter problem is usually during labor because of transient elevations in blood pressure. Patients
well tolerated early but may lead to right-sided heart failure, necessitat- are at risk for aortic dissection and infective endocarditis involving
ing reoperation. In addition, women with repaired tetralogy of Fallot an abnormal aortic valve; these risks increase during pregnancy.53
and significant pulmonic regurgitation have a higher risk of decom- Hypertension often worsens as well.54 Coarctation is also associated
pensation during pregnancy.52 with an increased frequency of preeclampsia.31 The operation does not

13. CHAPTER 39 Cardiac Diseases 809
A B
C D
FIGURE 39-11 Tetralogy of Fallot. A, Chest radiograph. Note concavity in the area of the pulmonary artery, oligemic lungs, and right aortic
arch. B, Right ventriculogram. Note the narrow right ventricular outflow tract. C, Further clarification of the pulmonary arteries. The left ventricle
is slightly opacified via the ventricular septal defect. D, The associated right-sided aortic arch is now visible. (From Shabetai R, Adolph RJ:
Principles of cardiac catheterization. In Fowler NO [ed]: Cardiac Diagnosis and Treatment. Hagerstown, MD: Harper & Row, 1980, p 106.)
require cardiopulmonary bypass and can be carried out with safety for dissection and rupture. A number of centers are now performing
the mother and with less fetal risk than accompanies open heart balloon dilation with stent implantation for adults with unoperated
surgery with cardiopulmonary bypass. Although transvascular balloon aortic coarctation, but large, multicenter studies are currently not
dilation of aortic coarctation is a viable option for children and infants available.56
with coarctation, its use in adults is controversial.55 The procedure If delivery must be undertaken in cases of unoperated coarctation,
is well accepted for treatment of postsurgical renarrowing of the blood pressure can be titrated with β-adrenergic–blocking agents
coarctation, but de novo balloon angioplasty carries a risk of aortic delivered by intravenous drip.

14. 810 CHAPTER 39 Cardiac Diseases
more rapid than in acquired complete atrioventricular block. Although
these patients appear to do well during childhood and young adult-
Other Congenital Cardiac hood, the lesion is associated with an unexpectedly high mortality
rate. Therefore, treatment with a pacemaker is indicated in many
Malformations of the cases.58 The pacemaker used should be dual-chamber and rate-
responsive, so that normal cardiovascular dynamics at rest and exercise
Ebstein Anomaly will be preserved. Patients who are untreated or who have received a
Ebstein anomaly is a malformation of the tricuspid valve in which the pacemaker are at slight to no increased risk during pregnancy.
septal leaflet is displaced apically and the anterior leaflet is abnormally
large in size. The deformed tricuspid valve apparatus may be signifi-
cantly incompetent or stenotic, depending on the location of the Additional Malformations
anomalously placed cusps of the valve. In some cases, the malforma- A number of other malformations may be present in women of child-
tion causes impediment to right ventricular outflow. bearing age, including
The clinical features are easily recognized by a cardiologist, and the
echocardiogram is characteristic and reliable (Fig. 39-12). This syn- Other left-to-right or right-to-left shunts
drome is frequently associated with anomalous atrioventricular con- Transposition of the great vessels
duction pathways and with the Wolff-Parkinson-White syndrome. Truncus arteriosus
Patients may also have an ASD with right-to-left shunting and cyano- Single-ventricle double-outlet right ventricle
sis. Supraventricular arrhythmias are also common. Various obstructive lesions
The most favored treatment is reconstruction of the tricuspid valve,
for which satisfactory techniques have now been developed. The opera- The malformations may be multiple and complex. Survival to
tion should be performed before pregnancy is undertaken. Interrup- adulthood depends on at least partial correction, which may have been
tion of anomalous conduction pathways also can be performed during furnished by surgical operation or may be part of the malformation.
surgery. For example, in D-type transposition of the great vessels, the aorta
The Mayo Clinic group57 reported on 111 pregnancies in 44 women arises from the right ventricle and the pulmonary artery from the left.
with Ebstein anomaly resulting in 95 live births, although most of the Survival requires a shunt at some level (ASD, VSD, or PDA) so that
infants had low birth weight. Vaginal delivery was performed in 89% oxygenated blood can enter the systemic circulation.
and cesarean section in 9%; 23 deliveries were premature. Nineteen Some of these women with untreated and delicately balanced
pregnancies ended with spontaneous abortion, and seven ended with lesions bear children, but usually this is not wise to attempt. Transposi-
therapeutic abortion. Congenital heart disease occurred in 6% of the tion of the great vessels is now treated by anastomosis of the aorta to
children of mothers with Ebstein anomaly. the morphologic left ventricle and of the pulmonary artery to the
morphologic right ventricle. Lesions such as single ventricle may be
palliated by the Fontan procedure, in which venous return is connected
Congenital Atrioventricular Block directly to the pulmonary circulation, bypassing the right side of the
Congenital atrioventricular block differs somewhat from heart block heart. Neither procedure constitutes a cure, but successful pregnancy
in adults. The pacemaker is usually junctional, and therefore the QRS can occur.
complex is normal or only slightly widened and the ventricular rate is In summary, patients should be evaluated and tracked by a cardi-
ologist who is experienced in congenital heart disease and by a mater-
nal-fetal medicine specialist with knowledge and experience in
managing pregnancy in women with congenital cardiac lesions.7,31
RV
Rheumatic Heart Disease
Rheumatic Fever
Rheumatic fever is now distinctly uncommon in the United States,
Canada, Western Europe, and Great Britain, but it is still prevalent in
less economically developed countries. Young female immigrants to
the Western world constitute a large proportion of the patients with a
RA history of rheumatic fever. These women are at risk of developing
rheumatic valvular heart disease 10 to 20 years after the initial episode
of rheumatic fever.
Chronic Rheumatic Heart Disease
In the United States, acute rheumatic fever with carditis has been
uncommon for many years, and chronic rheumatic heart disease,
FIGURE 39-12 Ebstein anomaly. The right atrium (RA) and right which manifests years to decades after the episode of acute rheumatic
ventricle (RV) are markedly dilated, and the tricuspid valve is fever, is becoming uncommon among the native childbearing popula-
displaced toward the cardiac apex. tion. Control of rheumatic fever has largely shifted the burden of

15. CHAPTER 39 Cardiac Diseases 811
ECG
mm Hg LA
100
LV
75
50
LA AO
25
LV
0
A 1 sec.
FIGURE 39-14 Transesophageal echocardiographic image of
ECG mitral stenosis. During diastole, opening of the mitral valve is
restricted by scarring and fusion of the leaflet tips. Characteristic
doming of the leaflet is also present. AO, aorta; LA, left atrium; LV,
mm Hg left ventricle.
40
LV
eventually supervenes, causing a fall in cardiac output and escalation
of left atrial hypertension, especially if the ventricular rate is not con-
trolled. Atrial fibrillation substantially increases the probability of
20 thrombus in the left atrial appendage and the threat of a subsequent
embolic stroke.
Wedge
Effect of Pregnancy
Pregnancy drastically stresses the circulation in women with severe
B 0
mitral stenosis. The increased blood volume, heart rate, and cardiac
FIGURE 39-13 Hemodynamics of mitral valve disease. A, Mitral output raise left atrial pressure to a level that causes severe pulmonary
stenosis. The diastolic pressure gradient (shaded area) between the congestion, leading to progressive exertional dyspnea, orthopnea, par-
left atrium (LA) and left ventricle (LV) persists to end-diastole. oxysmal nocturnal dyspnea, and pulmonary edema. Women who have
B, Mitral regurgitation. Note the large systolic pressure wave of the not been receiving antenatal care often present initially with severe
pulmonary wedge pressure tracing. The diastolic pressure gradient is pulmonary edema during pregnancy. In long-standing cases, severe
limited to early diastole. ECG, electrocardiogram. right-sided heart failure develops. Infective endocarditis, pulmonary
embolism, and massive hemoptysis may also occur. The maternal risk
for death is highest in the third trimester and in the puerperium.10,11
rheumatic heart disease from teenagers to women in the third and
fourth decades of life. Significant Mitral Stenosis without
The characteristic lesion of rheumatic heart disease is mitral steno- Heart Failure
sis, and the next most common manifestation is the combination of Patients who have mitral stenosis without heart failure should be
mitral stenosis with aortic regurgitation. The mitral valve may become advised to undergo percutaneous balloon mitral valvuloplasty and to
both stenotic and incompetent, and the valve may calcify. Pure mitral postpone pregnancy until after full recovery from the procedure. If
regurgitation is almost always nonrheumatic, except in young people they do not follow this advice and do become pregnant, one reasonable
with acute carditis. Similarly, aortic valve disease without mitral course in the first trimester may be pregnancy termination, followed
involvement is seldom rheumatic. Tricuspid regurgitation is a late by mitral valve operation and subsequent pregnancy planning. If this
secondary manifestation that occurs secondary to pulmonary hyper- is not acceptable, the patient can be advised to remain under frequent
tension and right ventricular enlargement. close supervision by the cardiologist and obstetrician and to accept
long periods of rest, prohibition of strenuous activity, salt restriction,
and diuretic treatment. If this type of regimen is followed closely and
Mitral Stenosis is expertly supervised, maternal mortality is low.10,11 Atrial fibrillation
The principal features are enlargement of the left atrium and right signals the need for digitalis, a β-adrenergic blocking agent, or a
ventricle, a diastolic murmur at the cardiac apex, and pulmonary calcium channel blocking agent to maintain a normal heart rate. More
hypertension. Inflow to the left ventricle is impeded by the narrowed than one of these drugs may be needed to achieve the desired result
valve and can be accomplished only by an increased level of pressure without side effects. For patients with atrial fibrillation and significant
in the left atrium (Figs. 39-13 and 39-14). The faster the heart rate, the mitral stenosis, anticoagulant treatment is recommended.
less time in diastole, and the less time for ventricular filling. Left atrial Depending on her course, the woman may have to spend many
pressure therefore is further elevated by tachycardia. Atrial fibrillation weeks in bed and should be admitted to the hospital well in advance

16. 812 CHAPTER 39 Cardiac Diseases
of labor. The supine posture should be avoided as much as possible,
and delivery in the left lateral decubitus position is desirable. The
lithotomy position, with the patient on her back and her feet elevated
in stirrups, is an invitation to pulmonary edema. The crisis of pulmo-
nary edema may appear despite good management. Sedation (to drop
the heart rate and promote cardiac filling and output) and diuretic
treatment must then be followed by prompt delivery if the fetus is
viable.
Percutaneous balloon valvuloplasty is a nonsurgical means to dilate
LV
mitral stenosis and is the current treatment of choice for most patients RV
with symptomatic mitral stenosis.59,60 The procedure can be done
during pregnancy if heart failure is severe, and it appears to be safer
for the fetus than open mitral commissurotomy.61 Lead shielding
RA
should be used, because fluoroscopy is required to guide the balloon
into the mitral orifice. Balloon valvuloplasty should be used with LA
caution during pregnancy, and it should be reserved for women who
are unresponsive to aggressive medical therapy.10,11 If possible, the
procedure should be put off at least until after the first trimester.
Patients with confirmed mitral stenosis and right-sided heart failure
with severe pulmonary congestion should avoid pregnancy until after
the valvular disease is corrected, because the risk of maternal mortality FIGURE 39-15 Transthoracic echocardiographic image of severe
is high. mitral regurgitation. During systole, the mitral valve does not coapt
properly, and an eccentric jet of mitral regurgitation is present
(arrows). LA, left atrium; LV, left ventricle; RA, right atrium; RV, right
Mitral Valve Prolapse ventricle.
In the past, a degree of prolapse of the mitral valve was considered so
prevalent in the general population,62 particularly among young in a small proportion, and the treadmill exercise test may induce ST-
women, that authorities differed as to whether mitral valve prolapse segment depression, indistinguishable from ischemia.68
(MVP) should be considered a normal variant or abnormal. More In most cases, the diagnosis of MVP is made by the physician pro-
exacting echocardiographic criteria have yielded more realistic and viding pre-conception counseling and antenatal care. The examination
much lower estimates of the prevalence of MVP (perhaps 1% of the reveals a systolic click occurring between the first and second heart
female population).63 sounds. The click may or may not be followed by a midsystolic or late
True MVP occurs because portions of the mitral valve apparatus systolic murmur. The click and murmur vary with the patient’s posture
are redundant and, therefore, the leaflets balloon into the left atrium and hydration status. In most patients, no other abnormality is found
during systole. The leaflets may remain coapted during systole, or they on clinical examination. Unless significant mitral regurgitation is
may separate, causing a variable degree of mitral regurgitation. More present, the patient should be told that pregnancy, labor, and delivery
severe prolapse may be caused by myxomatous degeneration of the will be safe and unaffected by the prolapse.
mitral leaflets. These abnormalities of connective tissue may be isolated Patients with MVP and significant mitral regurgitation are far fewer
to the mitral valve, or they may be a part of Marfan syndrome (see in number than those with simple prolapse. The murmur is louder,
later discussion). MVP (and sometimes tricuspid valve prolapse) may longer, and may become pansystolic. Clinical and laboratory evidence
be associated with congenital malformations, notably ASD. of enlargement of the left atrium and ventricle increases with increas-
Mitral regurgitation may be absent, intermittent, or permanent and ing severity and duration of regurgitation. Even modest impairment
may be of any degree of severity. Severe mitral regurgitation greatly of left ventricular function, especially if it is progressive, indicates that
enlarges the left atrium (Fig. 39-15) and ventricle and eventually leads pregnancy may well precipitate heart failure and cannot be lightly
to left ventricular failure and pulmonary hypertension, the latter less undertaken. More obvious left ventricular dysfunction (e.g., EF < 40%)
severe than with mitral stenosis. indicates that the woman should be strongly advised to avoid preg-
Past reports have associated MVP with a number of disorders, nancy. She should then be referred for complete cardiologic evalua-
including stroke, dysautonomia, panic attacks, anxiety, and transient tion2 and surgery to address the mitral regurgitation. In most cases of
ischemic attacks,64,65 but more recent studies66,67 have discounted MVP, the valve can be repaired rather than replaced. It is important to
almost all of these associations. The syndrome of myxomatous mitral appreciate that left ventricular function deteriorates after mitral valve
valve degeneration with prolapse and mitral regurgitation is quite replacement but may improve after mitral valve repair.69 Thereafter, if
uncommon, and many women who were diagnosed with MVP more the result is good and ventricular function is significantly improved,
than 10 years ago do not have any actual pathology. For this reason, it pregnancy may be undertaken successfully.
may be prudent to order an echocardiogram for any woman who was Chest pain and arrhythmias are best managed with β-adrenergic
diagnosed with MVP a number of years ago if she has no symptoms blockers such as atenolol or metoprolol. If symptoms are unusually
or signs of mitral regurgitation. This may prevent needless and repeated pronounced, thyroid function tests should be checked as well. Because
exposure to antibiotics (e.g., before dental procedures). the gravid uterus and vasodilation may add to postural hypoten-
Some women with MVP complain of chest pain, which can be sug- sion, the patient should be informed that she may experience light-
gestive of angina pectoris. Although the coronary arteriogram is headedness, dizziness, or fainting with prolonged standing during
normal, T-wave inversions, especially in leads II, III, and aVF, are found pregnancy.

17. CHAPTER 39 Cardiac Diseases 813
Mitral Regurgitation Not Caused
by Prolapse
In younger women, mitral regurgitation may be a result of rheumatic
or congenital disease. In older women, mitral regurgitation is more
often a manifestation of hypertension, ischemia, idiopathic myocardial
disease, or infective endocarditis.
Most of the information regarding mitral regurgitation in prolapse
also applies here. In older women, the valve is more likely to be calci-
fied; fewer of the valves are amenable to repair and must be replaced.
The problems posed by prosthetic valves in pregnant women are dis-
cussed later in this chapter; the hemodynamics are illustrated in Figure
39-13, and echocardiography is illustrated in Figure 39-15.
In patients with far-advanced left ventricular dysfunction or failure
who have severe mitral regurgitation, it can be difficult to determine
which is the cause and which the result. In either case, the patient with
a greatly enlarged and hypokinetic ventricle must be advised against
becoming pregnant. Most of the pregnancy would be spent in bed, the
course would be punctuated by episodes of uncompensated congestive
heart failure (any of which could prove fatal or require therapeutic
abortion), and the risk to the fetus would exceed 50%. FIGURE 39-16 Hemodynamic data in aortic stenosis. Left
Pregnancy in patients with mild or moderate mitral regurgitation ventricular pressure is 250/40 mm Hg (normal, 120/10 mm Hg). Aortic
systolic pressure is 130 mm Hg lower than the left ventricular
can be managed safely with a conservative regimen of reduced physical
pressure and shows a slow upstroke and vibrations representing the
activity, salt restriction, and low doses of a diuretic agent. Low-dose
systolic thrill. The record above the aortic pressure tracing is a
digoxin may be helpful if atrial fibrillation supervenes. As mentioned phonocardiogram showing the systolic murmur. Also shown is the
previously, severe mitral regurgitation indicates a need for repair or pulmonary wedge pressure (lowest pressure tracing), which is
replacement of the valve when symptoms and/or early evidence of elevated to equal the left ventricular diastolic pressure. The bottom
declining ventricular function appear.2,70 Clearly, surgical treatment is tracing is the electrocardiogram. (From Shabetai R, Adolph RJ:
best undertaken before pregnancy. If the woman is already pregnant, Principles of cardiac catheterization. In Fowler NO [ed]: Cardiac
the physician should make every effort to help her to carry the preg- Diagnosis and Treatment. Hagerstown, MD: Harper & Row, 1980,
nancy to term using strict medical measures. This course is particularly p 106.)
important if clinical, radiologic, and echocardiographic criteria suggest
that the valve is irreparable and would require replacement. phy is more pronounced, the cavity is smaller, and systolic function is
supranormal.
The left ventricle does not dilate until the ventricle fails, and so a
dilated ventricle in aortic stenosis is an ominous sign that calls for
Aortic Valve Disease rapid intervention. In general, aortic valve replacement is preferred to
percutaneous balloon aortic valvuloplasty, but open heart surgery
Aortic Stenosis presents a high risk to the fetus. For this reason, some have advised
balloon aortic valvuloplasty for treatment of aortic stenosis during
(see also the earlier section on aortic stenosis under Congenital
pregnancy,71 but valve replacement will almost certainly have to be
Obstructive Lesions)
done soon after delivery.
The etiologic mechanism of aortic stenosis commonly is degeneration, Hemodynamic monitoring is recommended during labor in
often of a congenitally bicuspid valve. The problem may be encoun- patients with moderate to severe aortic stenosis. Vaginal delivery is
tered in women a decade or more older than those with rheumatic or preferred, with assisted second stage of labor. If cesarean section is
congenital aortic valve disease. The combination of aortic and mitral performed, some have suggested that general anesthesia is preferred.72
stenosis is usually caused by rheumatic heart disease. Critical aortic See Chapter 56 for more details regarding anesthesia management.
stenosis leads to severe left ventricular hypertrophy and, eventually, to Pregnancy in women with a mechanical aortic valve replacement
left ventricular failure. Before overt heart failure develops, syncope or must be undertaken with great caution and meticulous management,
even sudden death may occur. because continuous anticoagulation is necessary (see Pregnancy in
The characteristic findings include an ejection systolic murmur that Patients with Artificial Heart Valves, later).
is harsher and longer and peaks later than the normal ejection murmur
of pregnancy. It is usually loudest at the second right intercostal space.
If aortic stenosis is severe, the pulse upstroke is slow, and left ventricu- Aortic Regurgitation
lar hypertrophy is evident on the ECG. The echocardiogram is a more The etiologic mechanism of aortic regurgitation is commonly rheu-
sensitive and more specific marker of left ventricular hypertrophy. matic heart disease, in which case mitral stenosis often coexists. Other
Doppler echocardiographic measurement of blood flow velocity diseases, such as Marfan syndrome, bicuspid aortic valve, infective
through the aortic valve permits reliable estimation of the systolic endocarditis, and systemic lupus erythematosus, also may cause severe
pressure drop across the valve, as well as calculation of the valve area. aortic regurgitation. This valvular lesion imposes a volume rather than
The hemodynamics are illustrated in Figure 39-16. The left ventricle a pressure overload on the heart and, as such, is usually well tolerated
in women remodels differently than in men. The concentric hypertro- in pregnancy and labor.15

18. 814 CHAPTER 39 Cardiac Diseases
The diagnosis is usually based on the finding of a typical, high- ditis that did not progress to cardiomyopathy. Dilated cardiomyopathy
pitched, blowing diastolic murmur and can be quantified by Doppler may be the outcome of an autoimmune response to a myocardial
echocardiography. Both pregnancy and aortic regurgitation contribute injury, most commonly viral myocarditis. The exact role of alcohol is
to hypervolemia and peripheral vasodilatation. A prolonged course unclear, but it is at least a major aggravating factor in some cases.
without decompensation is characteristic of chronic aortic regurgita- Patients may have symptoms and signs of heart failure for which
tion; once heart failure appears, however, the course may progress no cause can be found on clinical and laboratory examination. Weight
rapidly downhill. is increased, the jugular venous pressure is elevated, and the heart is
Traditionally, aortic valve replacement is not recommended until enlarged. An S3 gallop is often present, frequently accompanied by the
symptoms of heart failure (most notably exertional dyspnea) occur or murmurs of mitral and tricuspid regurgitation, which develop as a
left ventricular dysfunction/enlargement is seen on echocardiography. consequence of cardiac dilatation. The ECG is usually abnormal, often
Repair of aortic regurgitation is much less successful than repair of showing left ventricular hypertrophy or left bundle branch block.
mitral regurgitation. For a woman who is contemplating pregnancy, Echocardiography shows enlargement and hypocontractility of the
the need for aortic valve replacement constitutes the grounds on which ventricles. The patients are subject to mural thrombus in the cardiac
the medical advisor should caution against pregnancy and make the chambers with a consequent risk of stroke or pulmonary embolism.
patient fully understand the consequences of choosing otherwise. If Established dilated cardiomyopathy, even when heart failure is com-
left ventricular dysfunction and heart failure are absent, carefully pensated, is a contraindication to pregnancy.
supervised pregnancy is in order, and the woman should be encour- It was formerly thought that dilated cardiomyopathy was sporadic
aged to complete her family before cardiac dysfunction and the need and not familial, but inherited cases have now been observed. It is
for valve replacement arise. estimated that 20% of cases are genetic in origin.13 Therefore, if an
In many cases, the cause of aortic regurgitation is unclear. Special extensive family history of heart failure is present, the prospective
care must be taken to rule out aortic aneurysm or dissection, especially mother and father should be informed of the potential risk of genetic
if aortic regurgitation is associated with Marfan syndrome or coarcta- transmission.
tion of the aorta, because these conditions can result in aortic rupture In a young woman with severe dilated cardiomyopathy, manifested
and constitute strong reasons to advise against pregnancy. by greatly impaired ventricular function and drastically reduced exer-
cise capacity, cardiac transplantation should be considered. Successful
pregnancy has been reported in women who have undergone heart or
heart-lung tranplantation.76-78
Drug-Induced Valvular
Heart Disease Peripartum Cardiomyopathy
A recently recognized cause of deformity and regurgitation of the Peripartum cardiomyopathy is a form of dilated cardiomyopathy that
cardiac valves is ingestion of the drug combination, fenfluramine- occurs in the last month of pregnancy or during the first 5 months
phentermine. The revelation of this side effect led to withdrawal of this after delivery, in the absence of previous heart disease.79 The incidence
drug combination from the market in the late 1990s. The mechanism in the United States is 1 case per 3000 to 4000 live births. Additional
of the effect of these drugs is unclear, and there is evidence that valvular diagnostic criteria include a left ventricular EF of less than 45% and,
lesions may sometimes gradually improve after discontinuation of the most importantly, the absence of other identifiable causes of heart
drugs.73 In some cases, however, valve surgery has been necessary.74 failure.80 Whether the peripartum or postpartum state somehow con-
stitutes the original myocardial insult or is an aggravating factor in
individuals susceptible to cardiomyopathy for other reasons is not
Cardiomyopathy known.81 It has been suggested that some cases are caused by active
myocarditis,82 but other investigators have reported that the incidence
Cardiomyopathy is a disorder of myocardial structure or function. A of myocarditis is the same in idiopathic and peripartum cardiomyopa-
number of forms exist, and several types that are seen in pregnant thy.83 It is also possible that the stress of pregnancy may “unmask” an
women are discussed here. underlying cardiomyopathic process that might otherwise have mani-
fested later in life.84 This devastating disease can affect previously
healthy young women and can cause unexpected sudden death.85
Dilated Cardiomyopathy The clinical course of peripartum cardiomyopathy is frustratingly
In dilated cardiomyopathy, the cardiac chambers are severely dilated variable and difficult to predict.86 About 20% of patients have a dra-
and the left ventricle is diffusely hypokinetic. Left ventricular wall matic and fulminant downhill course and can be saved only with
tension is increased, and systolic pump function progressively declines. cardiac transplantation. Others, perhaps 30% to 50%, have partial
Consequently, cardiac output falls and filling pressures increase; both recovery with persistence of some degree of cardiac dysfunction. The
of these changes cause progressive dyspnea, edema, and fatigue. Serious rest show remarkable recovery.81 Apparently, the initial degree of left
ventricular arrhythmia develops in most cases. ventricular dysfunction does not predict the long-term outcome.87 A
Despite advances in treatment, the 5-year survival rate in patients recent study showed that cardiac function improved gradually over a
with dilated cardiomyopathy and symptomatic heart failure approaches 5-year period.88
50%. In some cases, however, improvement or even return to normal Women who recover from peripartum cardiomyopathy must be
has been noted. Both ACE inhibitors and β-adrenergic blocking agents informed that cardiomyopathy may recur with a subsequent preg-
(most notably carvedilol) have been shown to slow the deterioration nancy. For some time, this risk has been believed to be 50%.87 However,
of left ventricular function in patients with congestive heart failure and one report of four women who had peripartum cardiomyopathy with
occasionally to actually improve the left ventricular EF.75 In addition, a previous pregnancy but whose hearts remained normal clinically and
some of the patients who recover may have had unrecognized myocar- by echocardiography in a subsequent pregnancy indicated that the risk

19. CHAPTER 39 Cardiac Diseases 815
may be less.89 The largest study to date of patients with a history of
peripartum cardiomyopathy who subsequently became pregnant90
showed that heart failure recurred in 20% of those patients whose EF
had normalized after the previous pregnancy. None of these patients
died during the study period. However, heart failure recurred in 40%
LA
of the patients who had persistent left ventricular dysfunction after
their previous pregnancy, and the maternal mortality in this group was
19%. A study from Haiti of 15 women with peripartum cardiomyopa-
thy showed a recurrence rate of almost 50% during a subsequent AO
pregnancy.91 Therefore, the risk of recurrent heart failure is high in
women with peripartum cardiomyopathy, especially in those who do
not have complete recovery of left ventricular function.
Treatment is similar to that for other patients with heart failure.
Because ACE inhibitors are contraindicated during pregnancy hydrala- LV
zine is the vasodilator of choice. If cardiac dysfunction is severe,
anticoagulation is usually recommended, given the prothrombotic
tendency of pregnancy. Low-molecular-weight heparin (LMWH)
is probably preferred to unfractionated heparin (warfarin is not
recommended).86
A
Idiopathic Hypertrophic
Cardiomyopathy
Hypertrophic cardiomyopathy, which is usually inherited as an auto-
somal dominant trait with variable penetrance but sometimes is caused
by a spontaneous mutation, is being recognized with increasing fre- LA
quency. The phenotypes vary greatly. Left ventricular outflow tract
obstruction may or may not be present, and the hypertrophy may be
either symmetrical or asymmetrical. The chief symptoms are angina, AO
dyspnea, arrhythmia, and syncope. Sudden death is a feature mostly
confined to patients in whom the diagnosis is established in childhood
or youth, patients with a history of syncope or ventricular arrhythmia,
and patients with a family history of hypertrophic cardiomyopathy and
sudden death. Recent research has shown that certain specific genetic LV
defects place patients at great risk for sudden death.
When the disease is first detected in older adults, the course is more
benign and sudden death is rare. Left ventricular hypertrophy is often
apparent on clinical examination and ECG and is invariably present
on the echocardiogram. The echocardiographic findings are often
diagnostic and include marked thickening of the ventricular septum,
usually with less thickness of the other walls of the left ventricle (asym- B
metrical hypertrophy), and abnormal systolic anterior movement of FIGURE 39-17 Transesophageal echocardiographic images of
the mitral valve (Fig. 39-17). The internal dimensions of the left hypertrophic cardiomyopathy. A, During diastole the anterior
ventricle are normal to small, and its contractility is increased. leaflet of the mitral valve (arrow) is in a normal position. B, During
An important feature in many cases is obstruction of the space systole the leaflet (arrow) is pulled by Venturi forces into the left
between the ventricular septum and the anterior leaflet of the mitral ventricular outflow tract, causing obstruction to outflow. AO, aorta;
valve. This space constitutes the left ventricular outflow tract. Outflow LA, left atrium; LV, left ventricle.
obstruction by the anterior mitral valve leaflet is worsened by increased
inotropy, decreased heart size, and diminished peripheral vascular
resistance. The normal fall in peripheral vascular resistance that There is a complex interplay between the hemodynamics of the
accompanies pregnancy tends to increase outflow tract obstruction, cardiomyopathy and those of pregnancy, neither of which is constant.
although this effect may be compensated for by the physiologic increase Exacerbation of symptoms93 and even sudden death94 have been
in blood volume. In addition, vena caval obstruction in late pregnancy reported during pregnancy in women with obstructive cardiomyopa-
and blood loss at delivery, both of which may result in hypotension, thy. Treatment is aimed at avoiding hypovolemia, maintaining venous
can have a similar deleterious effect. Outflow tract obstruction may return, and diminishing the force of myocardial contraction by avoid-
also be worsened by the increases in circulating catecholamine levels ing anxiety, excitement, and strenuous activity.
frequently encountered during labor and delivery. The Valsalva maneu- Because left ventricular diastolic compliance can be greatly reduced
ver during the second stage of labor may greatly diminish heart size in this disease, excessive or too rapid volume repletion can induce
and increase outflow tract obstruction. Despite all these problems, pulmonary edema. β-Adrenergic blockade is considered first-line
however, most pregnant women with hypertrophic cardiomyopathy do pharmacologic therapy for symptomatic hypertrophic cardiomyopa-
tolerate labor and delivery.92 thy and can be continued or instituted during pregnancy. The dose

20. 816 CHAPTER 39 Cardiac Diseases
should be the minimal effective dose needed to avoid excessive slowing disorder and premature CAD. In women with CAD or severe dyslip-
of the fetal heart. idemia, oral contraceptives may be detrimental.102 In addition, spasm
Esmolol can be given intravenously if the patient first presents with of anatomically normal coronary arteries leading to myocardial
severe symptoms. Volume replacement and vasopressor therapy may infarction has been reported.103 Finally, as mentioned earlier, women
be needed, along with β-adrenergic blockers. Calcium channel block- with HIV infection may develop dyslipidemia on HAART, which can
ers, such as verapamil, have been shown to be effective in reducing increase the risk of CAD.100
symptoms, but they must be used cautiously because they can cause Spontaneous coronary artery dissection is quite rare and occurs
pulmonary edema in severe cases. Nifedipine, because of its vasodilator chiefly in young women during or soon after pregnancy.104,105 Treat-
properties, is best avoided. ment has included placement of a stent, emergency coronary bypass
Vaginal delivery is almost always appropriate in the absence of an operation, and thrombolysis.106-109 Although coronary artery dissection
obstetric indication for abdominal delivery. Impaired venous return is is very uncommon, it is extremely important to consider this diagnosis
highly undesirable in hypertrophic cardiomyopathy and can be ame- whenever a woman presents with severe chest pain in the peripartum
liorated by managing the second stage of labor with the patient in the period. If the coronary artery dissection remains undetected, massive
left lateral decubitus position. myocardial infarction and even death can occur. If the diagnosis is
made expediently, however, outcome appears to be quite good, and
long-term survival is expected.110
Acquired Immunodeficiency Syndrome
Myocarditis or cardiomyopathy is frequently discovered on postmor-
tem examination of patients with acquired immunodeficiency Management of Stable
syndrome (AIDS).95,96 Symptomatic myocardial disease, although Angina Pectoris
considerably less common, also occurs. If patients with full-blown
AIDS are screened for cardiac involvement (e.g., by echocardiography), Women with CAD who experience angina pectoris only at high levels
cardiac or pericardial involvement is found in almost 75% of the of exertion should be treated with β-adrenergic blocking drugs, aspirin,
cases.97 In some cases, these abnormalities are transient.98 Myocarditis and lipid-lowering agents. In this setting, the likelihood of significant
is usually caused by opportunistic infection, but in some cases hybrid- complications during pregnancy, labor, or delivery is low. If there is
ization studies have proved direct AIDS infection. Clinical findings any question regarding the severity of myocardial ischemia, however,
range from occult ventricular dysfunction to severe uncompensated stress testing should be performed before pregnancy is attempted.
heart failure. Rarely, even Kaposi sarcoma has been detected in the Similarly, a woman who previously sustained a myocardial infarction
heart or pericardium. Pericardial effusion, often occult, is one of the but recovered without heart failure, significant left ventricular dysfunc-
more common cardiac manifestations and suggests a worse progno- tion, or unstable angina pectoris can also be advised that her pregnancy
sis.99 Malignant lymphoma involving the myocardium and endocar- and labor should be relatively uncomplicated.
dium has been reported as well. In the current era of highly-active The major indications that pregnancy and labor would pose a sig-
antiretroviral therapy (HAART), myocardial involvement has become nificant risk to a woman with ischemic heart disease are the presence
considerably less common in patients with human immunodeficiency of overt heart failure, significant enlargement or dysfunction of the left
virus (HIV) infection. However, some classes of antiretroviral ventricle, and ischemia at rest or provoked by mild exertion.
drugs appear to cause dyslipidemia, and this can lead to an increased
risk of coronary artery disease (CAD) and subsequent myocardial
infarction.100
Severe Myocardial Ischemic
Cardiac failure ranks low on the list of problems faced by the physi- Syndromes: Unstable Angina
cian managing pregnancy complicated by AIDS. Nevertheless, physi- The diagnosis of severe ischemia may be confirmed if angina occurs
cians need to be on guard lest severe dilated cardiomyopathy, at rest or with mild exertion. This unstable angina frequently, but not
myocarditis, or cardiac tamponade develop, and the pregnant woman necessarily, follows a period of classic stable angina pectoris. Unstable
must be treated appropriately to prevent transmission of HIV to her angina is a clear warning of the imminence of a major ischemic event,
offspring (see Chapter 38.) such as acute myocardial infarction or a fatal ventricular arrhythmia.
Starting a pregnancy under these circumstances is not advisable, and
aggressive treatment (including coronary angiography followed by
Coronary Artery Disease percutaneous coronary intervention or coronary artery bypass surgery)
is recommended. If the outcome is satisfactory, pregnancy can then be
Because premenopausal women enjoy substantial protection against considered.
coronary atherosclerosis,15 ischemic heart disease is rarely relevant to In some women with CAD, the clinical picture is less dramatic but
obstetric practice. However, CAD may be found in women of child- a treadmill exercise test demonstrates that profound and dangerous
bearing age when other risk factors, such as insulin-dependent diabe- ischemia can be precipitated by minimal exertion. If the treadmill test
tes, smoking, or severe dyslipidemia, overwhelm the natural protection provokes an abnormal response at a low level of exercise, and particu-
they should normally enjoy. Lupus erythematosus, especially when larly if this response is accompanied by either angina pectoris or a fall
treated with steroidal agents, may precipitate premature CAD. Coro- in blood pressure, the woman is at high risk for a serious and possibly
nary atherosclerosis appears in a significant proportion of patients who fatal myocardial ischemic event and must not undertake pregnancy
have received a cardiac transplant101 and may be observed in familial unless the myocardium can be revascularized.
lipid disorders. In the latter instance, the exact nature of the lipid Pregnant women who develop unstable ischemia require aggressive
disorder must be defined by detailed analysis of the patient’s lipid treatment in an intensive care unit. If the ischemia proves intractable,
chemistry and lipoproteins to enable the physician to provide an percutaneous coronary intervention (e.g., stenting) or bypass graft
accurate forecast of the risk that the infant would inherit the lipid surgery will be necessary.111 If possible, the coronary bypass operation

21. CHAPTER 39 Cardiac Diseases 817
should be performed without cardiopulmonary bypass to help decrease nary and systemic congestion, and reduced cardiac output (during
the risk to the fetus.112 exercise or, in severe cases, at rest as well). The combined effects of
inadequate cardiac output and congestion are dyspnea, fatigue, and
edema. In the later stages of heart failure, these changes lead to progres-
Myocardial Infarction sive dysfunction of vital organs, principally the liver and kidneys. The
Acute myocardial infarction complicates about 1 in 10,000 pregnan- prognosis of severe uncorrectable heart failure is quite poor, and preg-
cies.113 The highest incidence appears to occur in the third trimester nancy is absolutely contraindicated.
and in older (>33 years) multigravidas. The maternal mortality rate is The critical clinical features that enable physicians to diagnose and
high (about 20%), and death usually occurs at the time of infarction monitor the course of heart failure are body weight, jugular venous
or during labor and delivery.114 Coronary angiography in this popula- pressure, the S3, cardiac size, radiologic evidence of pulmonary conges-
tion demonstrated atherosclerosis in about 40% of cases, coronary tion, pulmonary rales, and peripheral edema. Echocardiography is an
thrombosis without atherosclerosis in 20%, and coronary dissection in extremely useful tool for evaluating left ventricular function and prog-
16%, but 30% had normal coronary arteries.114 Treatment of acute nosis in heart failure115 and should be performed without delay if heart
myocardial infarction during pregnancy should include use of heparin failure is suspected. Circulating B-type natriuretic peptide (BNP) is
and β-blockers (unless acute heart failure is present). The use of increased in congestive heart failure. Serum BNP levels provide an
thrombolytics in pregnancy is controversial, because there is increased effective, inexpensive, and quickly available test for heart failure. The
risk of maternal hemorrhage. Therefore, percutaneous coronary inter- degree of BNP increase correlates with the severity of heart failure.116
vention (with stenting) is probably the procedure of choice. Obviously, The presence of heart failure greatly limits physical activity and
this exposes the fetus to radiation, and so extensive lead shielding warrants several or all of the following treatments:
should be used.
A remote myocardial infarction, followed by recovery without Continuous, usually escalating courses of diuretic drugs
angina, major left ventricular dysfunction, or heart failure, should have β-Adrenergic receptor blockers
little influence on pregnancy or labor. Patients should wait a year after ACE inhibitors (or, if these are not well tolerated, angiotensin
an infarction before undertaking pregnancy. In many cases, coronary receptor blockers)—note that these agents are contraindicated
arteriography should be done first so that, if critical coronary stenoses during pregnancy and should be replaced by hydralazine
are found, myocardial revascularization can be performed. Severe left Salt restriction
ventricular damage and heart failure are contraindications to pregnancy. Digoxin
For remote myocardial infarction without evidence of ischemia,
heart failure, or severe left ventricular dysfunction, simple electrocar- If heart failure is first discovered during pregnancy, episodes of
diographic monitoring suffices during labor. If a large myocardial cardiac decompensation that do not respond to adjustment of orally
infarction has occurred during pregnancy, then arterial blood pressure, administered medicine necessitate admission to an intensive care unit.
central venous pressure, pulmonary arterial and pulmonary wedge There, the effects of treatment on cardiac output, pulmonary arterial
pressure, and cardiac output should be monitored invasively. Monitor- pressure, systemic venous pressure, and pulmonary wedge pressure,
ing should be continued until after the completion of labor, because along with the maternal and fetal ECGs, can be monitored. If the
maternal preload abruptly increases with the birth, after which sub- hemodynamic parameters and clinical condition indicate continuing
stantial loss of blood can accompany delivery of the placenta. deterioration despite maximal medical therapy, emergency abdominal
delivery may be necessary.
Heart Failure Asymptomatic Left
Chronic heart failure is a syndrome that develops when the heart Ventricular Dysfunction
cannot meet the metabolic requirements of the normally active indi- There is a remarkable lack of correlation between symptoms of heart
vidual. It may be defined as ventricular dysfunction causing dyspnea, failure and objective evidence of left ventricular dysfunction.117 For
fatigue, and sometimes arrhythmia. The lesion may be an intrinsic example, patients with chronic heart disease after myocardial infarc-
myocardial abnormality. Examples include myocarditis, the various tion may have a considerably enlarged and extremely hypokinetic ven-
cardiomyopathies, ischemic heart disease, other specific myocardial tricle and yet be relatively free from symptoms. For this reason, any
disorders (e.g., amyloidosis), and metabolic abnormalities (e.g., myx- woman who has sustained myocardial damage should have left ven-
edema). The myocardial response to chronic pressure overload is con- tricular function assessed by echocardiography before deciding on
centric hypertrophy with increased thickness of the ventricular walls; pregnancy.
the response to chronic volume overload is dilation (eccentric hyper-
trophy). Contractile power is eventually diminished with either type
of overload, resulting in decreased pump function of the heart. Causes Cardiac Transplantation
include valvular disease, systemic and pulmonary hypertension, and Some women with advanced heart failure become successful recipients
congenital malformations. The clinical manifestations result in part of a cardiac transplant. Successful pregnancy and delivery in patients
from the abnormal loading conditions and in part from the damaged with cardiac transplantation have been reported.78 Medical treatment
myocardium. after transplantation is complex because of the immunosuppressive
drug regimen, the frequent endomyocardial biopsies, and the uncer-
tain long-term prognosis. Women should delay pregnancy for at least
Manifestations 1 year after cardiac transplantation, by which time the risk of acute
The principal manifestations of heart failure are caused by increased rejection and the intensity of immunosuppression and biopsy surveys
left and right ventricular diastolic pressure, which engenders pulmo- are considerably less.

22. 818 CHAPTER 39 Cardiac Diseases
Disturbances of
Cardiac Rhythm
Isolated supraventricular and ventricular extrasystoles are very
common, and no treatment is necessary. Pre-conception counseling is
simplified by a clear appreciation of several general principles.
Arrhythmia that occurs in the absence of organic heart disease is
almost always benign and is therefore not an indication for pharma-
cologic treatment unless the woman finds the symptoms intolerable.
Reassuring her of the benign nature of this symptom is often all that
is required. Sustained symptomatic arrhythmia, however, requires
treatment, which can be pharmacologic or procedural (e.g., transcath-
eter ablation of an anomalous conduction pathway, insertion of an
implantable cardiac defibrillator).
Pregnancy and labor should be safe except in the group with
sustained ventricular arrhythmia, with its attendant risk of cardiac
arrest and need for vigorous treatment. Pharmacologic treatment for
serious arrhythmia is likely to include newly introduced agents, such
as amiodarone or sotalol, for which there is at best limited knowledge
of potentially unfavorable effects on the fetus. Ideally, pregnancy
should be postponed until the arrhythmia has been eliminated or at
least controlled, preferably by nonpharmacologic means. If antiar-
rhythmic drugs must be used, whenever possible they should be those
that have been used for several decades, allowing prediction of the fetal
risk.
High-grade atrioventricular conduction disturbance, especially if it
is symptomatic, is treated by artificial pacing, which should not influ-
ence pregnancy, labor, or the fetus. Electrical cardioversion or defibril-
lation of the mother’s heart does not disturb or damage the fetal
heart.118
It is clearly desirable to evaluate disturbances of cardiac rhythm and FIGURE 39-18 Aortic aneurysm. Aortogram showing an aneurysm
conduction before pregnancy, proceeding to full electrophysiologic of the ascending aorta (AO) with regurgitation of contrast through an
testing if indicated. This plan avoids exposing a fetus to potentially incompetent aortic valve (arrows) into the left ventricle (LV). (From
Shabetai R, Adolph RJ: Principles of cardiac catheterization. In Fowler
toxic antiarrhythmic agents and the radiation associated with electro-
NO [ed]: Cardiac Diagnosis and Treatment. Hagerstown, MD: Harper
physiologic investigation.
& Row, 1980, p 106.)
Marfan Syndrome
Marfan syndrome is variably expressed and inherited as an autosomal Deficiency of elastic tissue is the cause of myxomatous degenera-
dominant trait. If it is left untreated, life expectancy is reduced by half tion of the aortic and mitral valves and cystic medial necrosis of the
in those who exhibit the classic syndrome. The basic defect is one of aorta (Figs. 39-18 and 39-19). This abnormality translates to large
connective tissue, particularly fibrillin, and connective tissue weakness aneurysms of the aortic root, multiple aneurysms elsewhere along the
in the aorta causes the dangerous complications, most notably aortic course of the aorta and great vessels, and severe aortic and mitral
dissection.119 regurgitation with resulting heart failure. Surgery is indicated for
Symptoms and signs may include dyspnea and chest pain, an aortic rapidly expanding aneurysm or if dissection is evident. Pregnancy is
diastolic murmur, and a midsystolic click. The best diagnostic test, and poorly tolerated under these conditions, and labor may precipitate
apparently the most critical one for determining the outcome of preg- rupture of an aneurysm or aortic dissection.
nancy, is the echocardiogram. More than 90% of patients have evi- If a woman with Marfan syndrome chooses to become pregnant,
dence of MVP, and 60% have echocardiographic evidence of aortic therapy is directed at markedly limiting physical activity, preventing
root dilatation.120 hypertensive complications, and decreasing the pulsatile forces on the
Pregnancy is particularly dangerous for patients with this syn- aortic wall with the use of a β-blocker. Long-acting β-blockers
drome, because there appears to be a high risk of aortic rupture and are indicated before, during, and after pregnancy in women with
dissection, especially if dilatation of the aortic root is present.47 Women Marfan syndrome.122 Once the aortic root diameter reaches 50 to
with an aortic diameter exceeding 40 mm are at greatest risk of death 55 mm, most authorities recommend prophylactic aortic valve and
during pregnancy.121 The physician should also make sure the woman root replacement because of the high risk of aortic dissection. Abdomi-
understands the 50% risk of genetic transmission of Marfan syndrome nal delivery is recommended to avoid the hemodynamic stress of
to her children. labor.

23. CHAPTER 39 Cardiac Diseases 819
requirement for lifelong anticoagulation and the resultant small
increase in risk of bleeding.
It is important to note that the anticoagulant of choice with a
mechanical valve is warfarin, not heparin. Although heparin is clearly
X safer for the fetus,125 it is not equivalent to warfarin in preventing
A
thromboembolic complications (especially during the prothrombotic
state of pregnancy). This has been shown in several studies and appears
AO
LV to be most striking in the case of single tilting-disk mechanical pros-
theses.8,24 Therefore, many experts agree that women who require valve
surgery before pregnancy should receive a bioprosthetic valve—even
though repeat surgery will be necessary in the future—because these
LA valves have a much lower thromboembolic risk and do not usually
require systemic anticoagulation. Women with normally functioning
biologic valve replacements tolerate pregnancy well.
Management of the last group (i.e., women with mechanical cardiac
valves who become pregnant) is the most difficult. A woman with a
FIGURE 39-19 Marfan syndrome. The echocardiogram shows a mechanical prosthetic heart valve should be counseled strongly that
markedly dilated aortic root, measuring 7.4 cm in diameter (normal, pregnancy is risky, primarily because of the risk of embolic phenom-
≤3.5 cm). A, annulus diameter (7.43 cm); AO, aorta; LA, left atrium;
ena. If the patient decides to proceed with pregnancy, warfarin is supe-
LV, left ventricle.
rior to heparin for preventing thromboemboli with mechanical valves.
However, warfarin is teratogenic and carries a 4% to 10% risk of war-
farin embryopathy.126,127 This risk appears to be dose dependent as
well.23 For these reasons, the U.S. manufacturer of Coumadin (warfa-
rin) states that the drug is absolutely contraindicated during preg-
Pregnancy in Patients with nancy. Although warfarin is used during pregnancy in Europe (after
Artificial Heart Valves the first 12 weeks) and is recommended until the 35th week of preg-
nancy, American physicians face a particularly difficult dilemma
Pregnancy in women with artificial heart valves is one of the most because of the manufacturer’s contraindication (even though this con-
complex and challenging areas where cardiology and obstetrics inter- tradicts guidelines from acknowledged expert panels).2,10,11 In addition,
sect, and it could be the topic of an entire chapter itself. The discussion many pregnant women would prefer to put themselves rather than the
here covers three basic groups of patients: fetus at risk. Therefore, subcutaneous or intravenous heparin is used
during pregnancy in many American women with mechanical heart
1. Women contemplating pregnancy who are likely to need a valve valves, even though thromboembolic risk is higher.127,128 During treat-
replacement in the medium- to long-term future, such as women ment with heparin or LMWH, the activated partial thromboplastin
with moderate aortic or pulmonic stenosis, severe but asymp- time (or anti-Xa heparin levels) must be monitored frequently. In
tomatic mitral or aortic regurgitation with normal myocardial addition, the dose must be adjusted as the patient gains weight during
function, or mild to moderate mitral stenosis pregnancy. There is some evidence that LMWH is superior to unfrac-
2. Women who wish to become pregnant but have severe valve tionated heparin in nonpregnant patients with mechanical prosthe-
disease that must be addressed expediently, including those with ses,129 and this approach has been used successfully in a number of
severe aortic or mitral stenosis, severe mitral regurgitation with pregnant patients.130,131 However, in a recent small study of enoxaparin
cardiac dysfunction, or severe aortic regurgitation with cardiac in pregnant women with prosthetic heart valves, two of eight patients
dysfunction developed prosthetic valve thrombosis leading to maternal and fetal
3. Women with mechanical valve replacements who become death.132 It is unclear but possible that these women did not receive
pregnant. adequate dosing of enoxaparin. Randomized trials have not been per-
formed, and more information is needed before LMWH can be recom-
The only group for whom no management controversy exists is the mended over unfractionated heparin in this setting.133
first.123 Without question, women who are likely to need valve surgery Tables 39-6 and 39-7 show two currently proposed protocols for
in several years should be strongly encouraged to complete their child- anticoagulation in the pregnant woman with a mechanical heart valve.
bearing as quickly as possible—so that they will not become members Most authorities recommend using heparin through the first trimester,
of the second or third group. although continuous use of warfarin until week 35 is an option in
Women in the second group require valve replacement before preg- high-risk patients (those with first-generation tilting-disk prostheses
nancy. In most adult patients younger than 70 years of age (but not in in the mitral position).10,11 The joint American College of Cardiology
women who wish to become pregnant), mechanical prosthetic valves (ACC)/American Heart Association (AHA) Guidelines for the Man-
would be favored over biologic prostheses, because biologic valves have agement of Patients with Valvular Heart Disease2 stress the impor-
a shorter life span and deteriorate much more quickly than mechanical tance of discussing the risks and benefits of various anticoagulation
valves. This difference appears even more pronounced in younger approaches with the patient, because she must be a full partner in her
patients; it was once thought that pregnancy itself hastened biologic medical care. She must be informed that if she chooses to change from
valve deterioration, but this does not appear to be true.124 In any case, warfarin to heparin during the first trimester (or for the entire preg-
younger patients with biologic valves will almost certainly require nancy), she has a higher risk of both thrombosis and bleeding, and any
repeat surgery. With mechanical valves, however, the patient faces the risk to her jeopardizes the baby as well. Table 39-6, from Elkayam and

24. 820 CHAPTER 39 Cardiac Diseases
TABLE 39-6 RECOMMENDED APPROACH FOR ANTICOAGULATION PROPHYLAXIS IN WOMEN WITH
PROSTHETIC HEART VALVES (PHV) DURING PREGNANCY
Higher Risk* Lower Risk†
Warfarin (INR 2.5-3.5) for 35 wk SC UFH (mid-interval aPTT, 2.0-3.0) or LMWH (pre-dose anti-Xa ≈ 0.6)
followed by for 12 wk
UFH (mid-interval aPTT >2.5) or LMWH (pre-dose anti-Xa ≈ 0.7) + followed by
ASA 80-100 mg qd Warfarin (INR 2.5-3.0) for 35 wk
then
SC UFH (mid-interval aPTT 2.0-3.0) or LMWH (pre-dose anti-Xa ≈ 0.6)
OR OR
UFH (aPTT 2.5-3.5) or LMWH (pre-dose anti-Xa ≈ 0.7) for 12 wk SC UFH (mid-interval aPTT 2.0-3.0) or LMWH (pre-dose anti-Xa ≈ 0.6)
followed by throughout pregnancy
Warfarin (INR 2.5-3.5) to 35th wk
then
UFH (mid-interval aPTT >2.5) or LMWH (pre-dose anti-Xa ≈ 0.7) +
ASA 80-100 mg qd
aPTT, activated partial thromboplastin time; ASA, acetylsalicylic acid; INR, international normalized ratio; LMWH, low-molecular-weight heparin; SC,
subcutaneous; UFH, unfractionated heparin.
*First-generation PHV (e.g., Starr-Edwards, Bjork-Shiley) in the mitral position, atrial fibrillation, history of thromboembolism on anticoagulation.
†
Second-generation PHV (e.g., St. Jude Medical, Medtronic-Hall) and any mechanical PHV in the aortic position.
Reproduced with permission from Elkayam U, Bitar F: Valvular heart disease and pregnancy. Part II: Prosthetic valves. J Am Coll Cardiol 46:403, 2005.
TABLE 39-7 RECOMMENDATIONS OF THE gestation. If labor occurs while a patient is taking warfarin, cesarean
SEVENTH ACCP CONSENSUS section is recommended to avoid fetal cerebral hemorrhage during
CONFERENCE ON vaginal delivery.2
All patients with prosthetic heart valves require antibiotic prophy-
ANTITHROMBOTIC THERAPY FOR
laxis for dental and surgical procedures and at delivery. Prevention of
PROPHYLAXIS IN PATIENTS WITH
prosthetic valve endocarditis is essential, because the mortality rate can
MECHANICAL HEART VALVES reach 40%. The patient who experiences endocarditis with a prosthetic
1. Aggressive adjusted-dose UFH, q12h SC throughout valve must receive aggressive antibiotic therapy and often will require
pregnancy; mid-interval aPTT time maintained at >2× control valve replacement. Obviously, the risk to the fetus is exorbitant.
levels, or anti-Xa heparin level maintained at 0.35 to 0.70 IU/mL
OR
2. LMWH throughout pregnancy, in doses adjusted according to
weight or as necessary to maintain a 4-h postinjection anti-Xa
Cardiac Surgery
OR
heparin level of about 1.0 IU/mL during Pregnancy
3. UFH or LMWH, as above, until the 13th week; then change to
warfarin until the middle of the third trimester, then restart
Whenever possible, any woman who requires cardiac surgery should
UFH or LMWH therapy until delivery undergo the procedure before becoming pregnant. Nevertheless, as
explained previously, in rare instances a patient may require surgery
ACCP, American College of Chest Physicians; aPTT, activated partial during pregnancy. Valvular surgery has been performed successfully
thromboplastin time; LMWH, low-molecular-weight heparin; SC, during pregnancy for many years, and patients have also undergone
subcutaneous; UFH, unfractionated heparin.
coronary artery bypass surgery and emergency aortic dissection repair.
Data from Bates SM, Greer IA, Hirsh J, Ginsberg JC: Use of
antithrombotic agents during pregnancy: The seventh ACCP Cardiac surgery during pregnancy does not appear to increase the
conference on antithrombotic and thrombolytic therapy. Chest maternal mortality risk.135,136 There is, however, a 10% to 15% risk of
126:627S, 2004. fetal mortality because of the nonpulsatile blood flow and hypotension
associated with conventional cardiopulmonary bypass. Therefore,
whenever possible, cardiac surgery should be performed without car-
Bitar,11 stratifies treatment options by risk of thrombosis and recom- diopulmonary bypass. In addition, hypothermia should be avoided,
mends consideration of continuing warfarin throughout the preg- because this appears to be especially dangerous to the fetus. In one
nancy (until week 35) for patients with first-generation valves in the study, fetal mortality was decreased by half when normothermic perfu-
mitral position, atrial fibrillation, or a history of previous embolic sion was used instead of hypothermic perfusion.136 Hypothermia
events. The treatment options in Table 39-7, from the American College stimulates uterine contractions and impairs oxygen delivery to the
of Chest Physicians134 are simpler, but they do not differentiate on the fetus, mandating careful monitoring of the uterus and the fetal heart.
basis of the type of mechanical valve or its position (aortic versus The deleterious effect of hypothermia on umbilical blood flow has
mitral). Consultation and close follow-up during pregnancy with an been documented by transvaginal ultrasonography.137
experienced cardiologist is strongly recommended, as well as meticu- Experimental studies suggest that fetal survival can be improved by
lous attention to blood coagulation testing. Because preterm labor the use of pulsatile perfusion, but results are not yet clear. If bypass is
occurs frequently in this group,24 warfarin should be replaced by thera- required for cardiac surgery at an immature gestational age, high-flow
peutic doses of subcutaneous heparin beginning at the 35th week of high-pressure normothermic perfusion should be instituted.138 If pos-

25. CHAPTER 39 Cardiac Diseases 821
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