Cardiovascular Disorders
BS. Nguyễn Hồng Anh
In an analysis o maternal mortality in the United States
between 2011 and 2013, deaths related to hemorrhage, hypertensive disorders, and embolism showed declining rates. In
contrast, deaths attributable to cardiovascular diseases are rising and are responsible or one ourth o all pregnancy-related
mortalities (Creanga, 2017; Petersen, 2019). Recent development o state-level maternal mortality review committees have
emphasized this issue and highlighted the relatively protracted
timeline o illness. Up to a year ater delivery, cardiac events
were among the leading causes o death or women in exas
in 2013 (exas Maternal Morbidity ask Force, 2020). Tese
disorders account also or signicant maternal morbidity and
are a common reason or intensive care unit admissions (Small,
2012).
Cardiovascular disease complicates 1 to 4 percent o pregnancies in the United States (American College o Obstetricians and Gynecologists, 2019). Te increasing prevalence is
likely multiactorial and includes the higher rates o obesity,
hypertension, and diabetes (Klingberg, 2017). According to the
National Center or Health Statistics, almost hal o adults aged
20 and older have at least one risk actor or cardiovascular
disease (Fryar, 2012). Another related reason is delayed childbearing. Last, as discussed subsequently (p. 927), an increasing number o women with congenital heart disease are now
becoming pregnant.
Te importance o heart disease and its adverse eect on
pregnancy morbidity and mortality led the American College
o Obstetricians and Gynecologists to create a ask Force on
Pregnancy and Heart Disease. Its purpose is to emphasize the
prevalence and eect o heart disease in pregnancy, provide
guidance or risk actor identication, outline common cardiovascular disorders, provide recommendations or management,
and develop a comprehensive interpregnancy plan. Multidisciplinary care is essential (Quinones, 2021).
PHYSIOLOGICAL CONSIDERATIONS
IN PREGNANCY
■ Cardiovascular Physiology
Te marked pregnancy-induced anatomical and unctional
changes in cardiac physiology can have a proound, negative
eect on underlying heart disease. Tese changes are discussed
in detail in Chapter 4 (p. 62), and some are listed in Table 52-1
(Clark, 1989). Importantly, cardiac output increases approximately 40 percent during pregnancy. Almost hal o this total
takes place by 8 weeks’ gestation and is maximal by midpregnancy (Capeless, 1989). Tis early rise stems rom augmented
stroke volume, which results rom lowered vascular resistance.
Later in pregnancy, resting pulse and stroke volume are even
higher because o greater end-diastolic ventricular volume that
results rom augmented pregnancy blood volume. Tese adaptations are even more proound in multietal pregnancies (Ghi,
2019). Intrinsic let ventricular contractility does not change,
and thus normal let ventricular unction is maintained during
pregnancy. Namely, pregnancy is not characterized by hyperdynamic unction or a high cardiac-output state.
Women with underlying cardiac disease may not always
accommodate these changes, and ventricular dysunction leads
to cardiogenic heart ailure. A ew women with severe cardiac
dysunction can experience evidence o heart ailure beore midpregnancy. In others, heart ailure may develop ater 28 weeks’
gestation, when pregnancy-induced hypervolemia and cardiac
output reach their maximum. In most, however, heart ailure
develops peripartum, when labor, delivery, and several common
obstetrical conditions add undue cardiac burdens. Some o the
latter include preeclampsia, hemorrhage and anemia, and sepsis.
■ Ventricular Function in Pregnancy
Ventricular volumes and mass accrue to accommodate pregnancy-induced hypervolemia. Tis is reected by greater
end-systolic and end-diastolic dimensions. At the same time,
however, septal thickness and ejection raction are unchanged.
Tis is because these alterations are accompanied by substantive ventricular remodeling—plasticity—which is characterized
by eccentric expansion o let-ventricular mass that averages 30
to 35 percent near term. All o these adaptations return to prepregnancy values within a ew months postpartum.
Certainly or clinical purposes, ventricular unction during
pregnancy is normal as estimated by the Braunwald ventricular unction graph (Fig. 4-9, p. 63). Tus, or given lling pressures, cardiac output is appropriate and allows eudynamic cardiac
unction during pregnancy. Esoteric changes in pregnancyrelated cardiac physiology continue to be claried. In nonpregnant subjects with a normal heart who sustain a high-output
state, the let ventricle undergoes longitudinal remodeling, and
echocardiographic unctional indices o its deormation show
normal values. In pregnancy, the ventricle instead undergoes
spherical remodeling, and the calculated indices that measure longitudinal deormation are depressed. Tus, normal nonpregnant
indices are likely less accurate when used to assess unction in
pregnant women because they do not account or the spherical
remodeling (Savu, 2012; Stewart, 2016).
Adjusting or these geometrical changes, Melchiorre and
coworkers (2016) studied normal cardiac echocardiographic
ndings in 559 nulliparas at our points during pregnancy
and again at 1 year postpartum. At term, signicant chamber
diastolic dysunction was present in 18 percent and impaired
myocardial relaxation was evident in 28 percent o the women.
Also, a signicant proportion o women demonstrated a drop
in stroke volume index and a tendency toward eccentric remodeling. Tese ndings suggest cardiovascular maladaptation to
the expanded volume demands in a substantial proportion o
apparently normal pregnancies. Signicant dyspnea at rest was
reported by 7.4 percent o the women at term, most o whom
had chamber diastolic dysunction. Cardiac unction and all
signs o dyspnea ully recovered at 1 year postpartum.
Cardiac magnetic resonance (MR) imaging increasingly is
used to evaluate cardiac structure and unction. Stewart and
associates (2016) perormed cardiac MR imaging studies in
23 women longitudinally across pregnancy and at 12 weeks postpartum. Compared with studies perormed at 12 to 16 weeks’
gestation, let ventricular mass grew signicantly or both normalweight and overweight women. Te calculated geometrical ratio
o let ventricular mass to let ventricular end-diastolic volume
demonstrated concentric remodeling throughout gestation,
which resolved by 12 weeks’ postpartum. Te right ventricle
also undergoes remodeling (Martin, 2017). Tese observations
likely mean that pregnancy causes a mixture o eccentric and
concentric ventricular remodeling.
DIAGNOSIS OF HEART DISEASE
Te physiological adaptations o normal pregnancy can induce
symptoms and alter clinical ndings that may conound the
diagnosis o heart disease. For example, in normal pregnancy,
unctional systolic heart murmurs are common, respiratory eort is accentuated, edema requently accrues in lower
extremities ater midpregnancy, and atigue and exercise intolerance oten develop. Some systolic ow murmurs can be loud,
and normal changes in the various heart sounds depicted in
Figure 52-1 may erroneously suggest cardiac disease. In contrast, clinical ndings that are more likely to suggest heart disease are listed in Table 52-2.
■ Diagnostic Studies
Noninvasive cardiovascular studies such as electrocardiography,
chest radiography, and echocardiography will provide the data
necessary or cardiac unctional evaluation in most women.
In the electrocardiogram (ECG), an average 15-degree letaxis deviation is ound as the diaphragm is elevated in advancing pregnancy. Other ndings are described in Figure 52-2
FIGURE 52-1 Normal cardiac examination findings in the pregnant woman. S1 = first sound; M1 = mitral first sound; S2 = second sound;
P2 = pulmonary second sound. (Data from Gei, 2001; Hytten, 1991.)
TABLE 52-2. Clinical Indicators of Heart Disease
During Pregnancy
Symptoms
Progressive dyspnea or orthopnea
Nocturnal cough
Hemoptysis
Syncope
Chest pain
Clinical Findings
Cyanosis
Clubbing of fingers
Persistent neck vein distention
Systolic murmur grade 3/6 or greater
Diastolic murmur
Cardiomegaly
Persistent tachycardia and/or arrhythmia
Persistent split second sound
Fourth heart sound
Criteria for pulmonary hypertension
Q wave in
lead DIII
Leftward deviation of the mean QRS axis
+90°
aVF
+90°
aVF
aVF aVF
0° DI 0° DI
DI DI
Reduction in
PR interval
Increase
in HR
FIGURE 52-2 Normal electrocardiograph (ECG) adaptations during
pregnancy, including a reduced mean PR interval, increased heart
rate, left axis deviation, inverted or flattened T waves, and a Q wave in
lead DIII. HR = heart rate. (Reproduced with permission from Angeli F,
Angeli E, Verdecchia P: Electrocardiographic changes in hypertensive
disorders of pregnancy, Hypertens Res 2014 Nov;37(11):973–975.)
(Angeli, 2014). Pregnancy does not alter voltage ndings.
Atrial and ventricular premature contractions are relatively
requent.
With radiography, anteroposterior (AP) and lateral chest
radiographs are useul, and when a lead apron shield is used,
etal radiation exposure is minimal. Gross cardiomegaly can
usually be excluded, but slight heart enlargement is poorly
detected because the heart silhouette normally is larger in pregnancy. Tis is accentuated urther with a portable posterioranterior chest radiograph.
Echocardiography is now widely used and permits accurate
diagnosis o most heart diseases during pregnancy. Some normal pregnancy-induced changes include a small increase in
the dimensions o all cardiac chambers, a slight but signicant
growth in let ventricular mass, and greater tricuspid and mitral
valve regurgitation (Grewal, 2014). O note, systolic unction
normally does not change and ejection raction is preserved.
Late geometric changes such as interventricular septum dimension (IVd) are likely to be abnormal beore changes in ejection
raction. Savu (2012) and Vitarelli (2011) and their colleagues
have provided normal echocardiographic parameters or pregnancy, which are listed in the Appendix (p. 1233). In some
situations, such as complex congenital heart disease, transesophageal echocardiography may be useul.
Cardiovascular MR imaging, compared with echocardiography, is associated with higher reproducibility and is less hindered by ventricular geometry and body habitus. It is useul or
assessment o the right ventricle and visualization o congenital
heart lesions and myocarditis (Herrey, 2019; Martin, 2017).
Ducas and coworkers (2014) have published normal reerence
values or pregnancy.
Exercise stress testing is an objective assessment o maternal unctional capacity. It is also useul to diagnose exerciseinduced arrhythmias. Stress testing can be perormed in women
with known heart disease prior to pregnancy or, i necessary, in
the asymptomatic pregnant women (Regitz-Zagrosek, 2018).
Dennis and associates (2019) have described ndings in normal
pregnant women using the 6-minute walk test.
Myocardial perusion studies using albumin or red cells
tagged with technetium-99m are rarely needed during pregnancy to evaluate ventricular unction. Tat said, the estimated etal radiation exposure rom nuclear medicine studies
o myocardial perusion is negligible (Chap. 49, p. 875). Cardiac catheterization with limited uoroscopy time also is sae
to perorm. During coronary angiography, the mean radiation exposure to the unshielded abdomen is 1.5 mGy, and
less than 20 percent o this reaches the etus (Regitz-Zagrosek,
2018). Shortening the uoroscopic time may help to minimize
radiation exposure (Raman, 2015; uzcu, 2015). In women
with clear indications, any minimal theoretical etal risk is
outweighed by maternal benets, and such studies should be
perormed as indicated.
■ Functional Classification of Heart Disease
Pregnancy is a stress test o cardiovascular reserve. However,
no clinically applicable test accurately measures unctional cardiac capacity. Te clinical classication o the New York Heart
Association (NYHA) is based on past and present disability and
is uninuenced by physical signs:
• Class I. Uncompromised—no limitation o physical activity:
Tese women do not have symptoms o cardiac insufciency
or experience anginal pain.
• Class II. Slight limitation o physical activity: Tese women
are comortable at rest, but with ordinary physical activity,
discomort in the orm o excessive atigue, palpitation, dyspnea, or anginal pain results.
• Class III. Marked limitation o physical activity: Tese women
are comortable at rest, but less than ordinary activity causes
excessive atigue, palpitation, dyspnea, or anginal pain.
• Class IV. Severely compromised—inability to perorm any physical activity without discomort: Symptoms o cardiac insu-
ciency or angina may develop even at rest. I any physical
activity is undertaken, discomort is increased.
At least our predictive systems detect and classiy heart
disease in pregnant women. Tese include CARPREG I and
II, ZAHARA, and the World Health Organization (WHO)
system (Wole, 2019). O these, the most comprehensive risk
stratication system is the modied WHO Risk Classication
o Cardiovascular Disease and Pregnancy (Table 52-3). Tis
classication system was validated in an international cohort o
2742 pregnant women with heart disease (van Hagen, 2016).
It is especially useul or assessing maternal risk and or preconceptional counseling. Lu (2015) and Pijuan-Domènech (2015)
and their colleagues concluded that the modied WHO classication provides the greatest predictive accuracy or cardiac
complications during pregnancy.
GENERAL PREGNANCY CONSIDERATIONS
■ Preconceptional Counseling
Women with severe heart disease will benet immensely rom
counseling beore pregnancy, and they usually are reerred or
maternal-etal medicine or cardiology consultation (Clark,
2012; Wole, 2019). Optimizing cardiac unction to mitigate
complications during pregnancy is the goal. Maternal mortality
rates generally correlate directly with unctional classication,
however, this relationship may change as pregnancy progresses.
Siu and coworkers (2001b) observed signicant worsening o
NYHA class in 4.4 percent o 579 pregnancies in which the
baseline class was I or II.
As described later, some women have lie-threatening cardiac abnormalities that can be reversed by corrective surgery,
and subsequent pregnancy becomes less dangerous. In other
cases, such as women with mechanical valves taking wararin,
etal teratogenic concerns predominate. Last, because many
congenital heart lesions are inherited as polygenic characteristics, some women with congenital heart lesions give birth to
similarly aected neonates. Tis risk varies widely based on the
specic abnormality (Table 52-4) (Lupton, 2002).
Risk Factors
According to the American College o Obstetricians and
Gynecologists (2019), our maternal risk actors are linked to
TABLE 52-3. World Health Organization (WHO) Risk Classification of Cardiovascular Disease and Pregnancy with
Management Recommendations
Risk Category Associated Conditions
WHO 1—Morbidity or mortality risk no higher than
general population
Uncomplicated, small, or mild:
Pulmonary stenosis
Patent ductus arteriosus
Mitral valve prolapse with no more than trivial mitral regurgitation
Successfully repaired simple lesions:
Ostium secundum atrial septal defect
Ventricular septal defect
Patent ductus arteriosus
Total anomalous pulmonary venous drainage
Isolated ventricular extrasystoles and atrial ectopic beats
• Cardiology consultation once or twice during pregnancy. Local hospital care suitable
WHO 2—Small increase in risk of maternal mortality
and moderate increase in morbidity risk
If otherwise uncomplicated:
Unoperated atrial or ventricular septal defect
Repaired Fallot tetralogy
Most arrhythmias
Turner syndrome without aortic dilation
• Cardiology consultation each trimester. Local hospital care suitable
WHO 2 or 3—Intermediate increase in maternal
mortality risk and moderate to severe rise in
morbidity risk
Mild left ventricular impairment
Hypertrophic cardiomyopathy
Native or tissue disease not considered WHO 1 or 4
Marfan syndrome without aortic dilation
Repaired coarctation
Prior heart transplantation
• Cardiology consultation bimonthly. Care at referral hospital
WHO 3—Significantly increased risk of maternal
mortality and severe increase in morbidity risk
Mechanical valve
Systemic right ventricle
Post-Fontan operation
Unrepaired cyanotic heart disease
Other complex congenital heart disease
Moderate left ventricular impairment
Prior peripartum cardiomyopathy with no residual effect
Moderate mitral stenosis
Severe asymptomatic aortic stenosis
Moderate aortic dilation (40–50 mm)
Ventricular tachycardia
• Cardiology consultation monthly or bimonthly. Care at tertiary-care hospital
WHO 4—Very high risk of maternal mortality or
severe morbidity; pregnancy contraindicated
and termination discussed
Pulmonary arterial hypertension
Severe systemic ventricular dysfunction (NYHA III–IV or LVEF <30%)
Prior peripartum cardiomyopathy with residual effects
Severe left heart obstruction
Severe aortic dilation
Severe coarctation
Fontan procedure with residual complications
• Pregnancy contraindicated
• If pregnancy occurs, cardiology consultation monthly. Care at tertiary-care hospital
Summarized from European Society of Gynecology, 2018; Nanna, 2014; Thorne, 2006
TABLE 52-4. Risks for Fetal Heart Lesions Related to
Affected Family Members
Congenital Heart Disease in
Fetus (%)
Cardiac Lesion
Previous
Sibling
Affected
Father
Affected
Mother
Affected
Aortic stenosis 2 3 15–18
Pulmonary stenosis 2 2 6–7
Ventricular septal defect 3 2 10–16
Atrial septal defect 2.5 1.5 5–11
Patent ductus arteriosus 3 2.5 4
Coarctation of the aorta NS NS 14
Fallot tetralogy 2.5 1.5 2–3
Marfan syndrome NS 50 50
NS = not stated.
cardiovascular disease morbidity and mortality: (1) race/ethnicity, with morbidity highest in Arican-American women; (2)
age, with increased morbidity in women older than 40 years;
(3) hypertension o all varieties; (4) obesity, with higher morbidity associated with increasing degrees o obesity (Ackerman,
2019). o these, social and health disparities could be added
because lower income, ood and housing insecurity, and lack o
childcare are directly linked to underutilization o prenatal care
(Gadson, 2017). In turn, lack o prenatal care is a risk actor or
maternal morbidity and mortality (Howland, 2019).
■ Antepartum Care
In most instances, management involves a team approach with
an obstetrician, cardiologist, anesthesiologist, and other specialists as needed. With complex lesions or other high-risk cases,
evaluation by a multidisciplinary team is recommended early in
pregnancy. Both prognosis and management are inuenced by
the type and severity o the specic lesion and by the maternal
unctional classication. In some, pregnancy termination may
be advisable.
With rare exceptions, women in NYHA class I and most in
class II negotiate pregnancy without morbidity. Special attention is directed toward both prevention and early recognition
o heart ailure. O specic risks, inection with sepsis can precipitate decompensation. Also, bacterial endocarditis is a deadly
complication o valvular heart disease (p. 934). Each woman is
instructed to avoid contact with persons who have respiratory
inections, including the common cold, and to immediately
report any evidence or inection. Pneumococcal vaccine, i not
previously administered, and yearly inuenza vaccine are recommended (Chap. 10, p. 188).
Cigarette smoking is prohibited. Illicit drug use may be particularly harmul, an example being the cardiovascular eects
o cocaine or amphetamines. In addition, intravenous drug use
raises the inective endocarditis risk.
Fortunately, gravidas in NYHA class III and IV are uncommon today. In the Canadian study, only 3 percent o the
approximately 600 pregnancies were complicated by NYHA
class III heart disease, and no women had class IV when rst
seen (Siu, 2001b). I a woman chooses pregnancy, she must
understand the risks and is encouraged to be compliant with
planned care. In some women, prolonged hospitalization or
bed rest is oten necessary.
■ Labor and Delivery
In general, vaginal delivery is preerred, and labor induction
is usually sae (Turman, 2017). From the large Registry on
Pregnancy and Cardiac Disease, Ruys and associates (2015)
compared pregnancy outcomes between 869 women who
had a planned vaginal delivery and 393 gravidas who had a
planned cesarean delivery. Planned cesarean delivery conerred
no advantage or maternal or neonatal outcome. Similar results
were reported rom Brigham and Women’s Hospital (Easter,
2020).
Cesarean delivery is usually limited to obstetrical indications, and considerations are given or the specic cardiac
lesion, overall maternal condition, and availability o experienced anesthesia personnel and hospital capabilities. Some o
these women tolerate major surgical procedures poorly and are
best delivered in a unit experienced with management o complicated cardiac disease. Occasionally, pulmonary artery catheterization may be needed or hemodynamic monitoring (Chap.
50, p. 883). In our experiences, however, invasive monitoring
is rarely indicated.
Based on her review, Simpson (2012) recommends cesarean
delivery or women with the ollowing: (1) dilated aortic root
>4 cm or aortic aneurysm; (2) acute severe congestive heart
ailure; (3) recent myocardial inarction; (4) severe symptomatic aortic stenosis; (5) need or emergency valve replacement
immediately ater delivery; and (6) wararin administration
within 2 weeks o delivery due to etal risk or intracerebral
hemorrhage because the etal liver takes up to 2 weeks to
metabolize wararin. Although we agree with most o these,
we have some caveats. For congestive heart ailure, we preer
aggressive medical stabilization o pulmonary edema ollowed
by vaginal delivery i possible.
During labor, the mother with signicant heart disease
should be kept in a semirecumbent position with a lateral tilt.
Vital signs are taken requently between contractions. Elevations in pulse rate much above 100 beats per minute (bpm)
or respiratory rate above 24 breaths per minute, particularly
when associated with dyspnea, may suggest impending ventricular ailure. For evidence o cardiac decompensation, intensive
medical management must be instituted immediately. Delivery
itsel does not necessarily improve the maternal condition and,
in act, may worsen it. Moreover, emergency cesarean delivery
may be particularly hazardous. Clearly, both maternal and etal
status must be considered in the decision to hasten delivery
under these circumstances.
Analgesia and Anesthesia
Relie rom pain and rom apprehension is important. Although
intravenous analgesics provide satisactory pain relie or some
women, continuous epidural analgesia is recommended or
most. Te major problem with conduction analgesia is maternalCardiovascular Disorders 921
CHAPTER 52
hypotension (Chap. 25, p. 473). Tis is especially dangerous in
women with intracardiac shunts in whom ow may be reversed.
Hypotension can also be lie-threatening i there is pulmonary
arterial hypertension or aortic stenosis. In these, ventricular
output is dependent on adequate preload. In women with these
conditions, narcotic regional analgesia; low-dose, slow-inusion
epidural; or general anesthesia may be preerable.
For vaginal delivery in women with only mild cardiovascular
compromise, epidural analgesia given with intravenous sedation oten sufces. Tis minimizes intrapartum cardiac output
uctuations and allows orceps or vacuum-assisted delivery.
Incrementally dosed subarachnoid blockade is approached
especially cautiously in women with signicant heart disease
due to associated hypotension. For cesarean delivery, epidural
analgesia is preerred by most clinicians with caution or its use
with pulmonary arterial hypertension (p. 932).
Intrapartum Heart Failure
From the Nationwide Inpatient Sample, one ourth o cases o
heart ailure during pregnancy were encountered intrapartum
(Mogos, 2018). Women with underlying cardiovascular disorders are at higher risk (Schlichting, 2019). Te physiological
stress and uid shits associated with labor and delivery explain
this risk. Catecholamine release due to pain and second stage
Valsalva increase let ventricular work, which can precipitate
heart ailure (Anthony, 2016).
Obstetrical complications can either advance or precipitate heart ailure. Preeclampsia is common and may provoke
aterload ailure (Vaught, 2018). Findings rom the Registry
on Pregnancy and Cardiac Disease indicate that women with
preexisting heart disease who develop preeclampsia have a
30-percent risk o developing heart ailure during pregnancy
(Ruys, 2014). Obesity is another common coactor (Vonck,
2019). Also, high-output states caused by hemorrhage and
acute anemia elevate cardiac workload and magniy the physiological eects o compromised ventricular unction. Similarly, inection and sepsis increase cardiac output and oxygen
utilization and depress myocardial unction.
Chronic hypertension with superimposed preeclampsia is
the most requent cause o heart ailure in pregnancy in numerous populations. Many o these women have preexisting concentric let ventricular hypertrophy (Ambia, 2017, 2018). In
some, mild antecedent undiagnosed hypertension causes covert
cardiomyopathy, and when superimposed preeclampsia develops, together they may cause otherwise inexplicable peripartum heart ailure. Obesity is requently comorbid with chronic
hypertension, and it also is associated with ventricular hypertrophy (Kenchaiah, 2002).
Cardiovascular decompensation during labor may mani-
est as pulmonary edema with hypoxia or as hypotension, or
both. Te proper therapeutic approach depends on the specic
hemodynamic status and the underlying cardiac lesion. Continuous pulse oximetry and invasive blood pressure monitoring
with an arterial line is helpul in some cases (Easter, 2020) In
general, pulmonary edema due to uid overload is oten best
treated with aggressive diuresis. I precipitated by tachycardia,
heart rate control with β-blocking agents is preerred. However, unless the underlying pathophysiology is understood and
the cause o the decompensation is clear, empirical therapy may
be hazardous. Details o heart ailure management and specic
cardiovascular disorders are discussed below.
■ Puerperium
Women who have shown little or no evidence o cardiac compromise during pregnancy, labor, or delivery may still decompensate postpartum (Mogos, 2018). Fluid mobilized into the
intravascular compartment and reduced peripheral vascular
resistance place higher demands on myocardial perormance.
Tereore, meticulous care is continued into the puerperium
(Sliwa, 2018; Zeeman, 2006). Postpartum hemorrhage, anemia, inection, and thromboembolism are much more serious
complications with heart disease. Indeed, these actors oten
act in concert to precipitate postpartum heart ailure. In addition, sepsis and severe preeclampsia cause or worsen pulmonary
edema because o endothelial activation and capillary-alveolar
leakage. For example, women with pregnancy-associated hypertensive diseases have a greater risk or readmission or heart
ailure within 90 days o delivery (Nizamuddin, 2019).
For puerperal tubal sterilization ater vaginal delivery, the
procedure can be delayed up to several days to ensure that the
mother has normalized hemodynamically and that she is aebrile, not anemic, and ambulatory. For those desiring uture
ertility, contraception is crucial (Abarbanell, 2019; Sobhani,
2019). Detailed contraceptive advice is available in the U.S.
Medical Eligibility Criteria or Contraceptive Use guidelines
(Curtis, 2016). Tese are discussed in Chapter 38 (p. 664).
HEART FAILURE
Primary structural or unctional cardiac disorders can lead to
this clinical syndrome caused by impaired ventricular unction.
Mitral stenosis, pulmonary hypertension, and peripartum cardiomyopathy are some examples. Chronic pressure overload
leads to ventricular dilation and impaired unction over time.
Associated perinatal mortality rates are high (Bright, 2021).
■ Diagnosis
Heart ailure is best thought o as chronic underlying ventricular
myopathy with episodic worsening causing clinical symptoms
(Packer, 2019). Tus, ailure can have a gradual onset or may
present as acute “ash” pulmonary edema. In pregnant women,
onset is most likely at the end o the second or beginning o
the third trimester and peripartum (Ruys, 2014). O symptoms,
dyspnea is universal and others are orthopnea, palpitations, substernal chest pain, nocturnal cough, and a sudden decline in
the ability to complete usual duties. Clinical ndings include
persistent basilar rales, hemoptysis, progressive edema, tachypnea, and tachycardia. According to Malhamé and associates
(2019), serum levels o brain natriuretic peptide (BNP) are variably elevated (Appendix, p. 1231). Cardiomegaly and pulmonary edema are hallmark radiographic ndings. Acutely, there is
usually systolic ailure. Echocardiography may show an ejection
raction <0.45 or a ractional shortening <30 percent, or both,
and an end-diastolic dimension >2.7 cm/m2 (Hibbard, 1999).922 Medical and Surgical Complications
Section 12
Coincidental diastolic ailure also may be ound, depending on
the underlying cause (Redeld, 2016). Last, the cause o ventricular dysunction—such as valvular abnormalities or concentric
hypertrophy—may become apparent.
■ Management
Pulmonary edema rom heart ailure usually responds promptly
with diuretic administration to reduce preload. Recall that
urosemide (Lasix) is a potent venodilator in addition to its
diuretic action. Hypertension is common in pregnancy, and
aterload reduction is accomplished with hydralazine, niedipine, or another vasodilator. Angiotensin-converting enzyme
inhibitors are withheld until ater delivery because o marked
etal eects (Chap. 8, p. 150). β-blocking agents lower mortality rates in the setting o heart ailure, and carvedilol (Coreg) is
commonly used in pregnancy. Last, digoxin provides inotropic
support and is associated with decreased hospitalizations. With
chronic heart ailure, the incidence o associated thromboembolism is high, and thereore prophylactic heparin is oten recommended.
Let ventricular assist devices are now employed more
requently or acute and chronic heart ailure treatment. A
ew reports describe their use during pregnancy (Hamdan,
2017; Makdisi, 2017). Extracorporeal membrane oxygenation
(ECMO) was reported to be liesaving in a woman with ulminating peripartum cardiomyopathy, and it may be used in
women with pulmonary hypertension (Meng, 2017; Pacheco,
2018).
SURGICALLY CORRECTED HEART DISEASE
Most clinically signicant congenital heart lesions are repaired
during childhood. Tose requently not diagnosed until adulthood include atrial septal deects, pulmonic stenosis, bicuspid
aortic valve, and aortic coarctation (Brickner, 2014). In some
cases, the deect is mild and does not require repair. In others,
a signicant anomaly is amenable to corrective surgery, per-
ormed ideally beore pregnancy. Rarely, surgical corrections
are necessary during pregnancy.
■ Valve Replacement Before Pregnancy
Numerous reports describe subsequent pregnancy outcomes
in women who have a prosthetic mitral or aortic valve placed
beore pregnancy. From one review, the overall estimated
maternal mortality rate was 1.2 percent (Lawley, 2015). Using
the Registry o Pregnancy and Cardiac Disease, the maternal
mortality rate was 1.4 percent in women with a mechanical
heart valve and 1.5 percent in women with a tissue heart valve
(van Hagen, 2015). Compared with the general maternal mortality rate measured per 100,000 births, this risk is more than
50-old higher (Chap. 1, p. 4).
Te type o valve, either mechanical or bioprosthetic, is
paramount. From the just-described cohort, mechanical heart
valve thrombosis complicated 4.7 percent. Only 58 percent o
women with a mechanical heart valve had a pregnancy ree o
serious adverse events compared with 79 percent o patients
with a tissue heart valve (Table 52-5) (van Hagen, 2015). A
study o 417 women showed that pregnancy loss occurred in
61 and 15 percent with mechanical and bioprosthetic valves,
respectively (Batra, 2018). Anticoagulation is a requisite with
mechanical valves because o thrombosis risks, and its complications are described in the next section. Tus, pregnancy is
undertaken only ater serious consideration or women with a
mechanical valve.
Bouhout and associates (2014) reported the outcomes o
27 pregnancies in 14 women who underwent an aortic valve
replacement prior to pregnancy. Seven o the 27 pregnancies
occurred in ve women with a mechanical valve. Complications in this group included two embolic myocardial inarctions and one each o miscarriage, postpartum hemorrhage,
placental abruption, and preterm birth. In the bioprosthetic
group, there were nine miscarriages.
Adverse maternal and etal outcomes plague also women
with a mechanical mitral valve. In one report o 28 pregnancies, Vause and colleagues (2017) described severe maternal
morbidity and mortality in 57 percent. A review o pregnancy
outcomes in 800 women reported similar results (Steinberg,
2017).
Porcine tissue mitral valves are saer during pregnancy, primarily because thrombosis is rare, and anticoagulation is not
required (see able 52-5). However, valvular dysunction with
cardiac deterioration poses a serious risk. Another drawback is
that bioprostheses are less durable than mechanical ones, and
valve replacement longevity averages 10 to 15 years. Batra and
coworkers (2018) concluded that pregnancy accelerated the
risk or subsequent replacement.
Anticoagulation
Tis is critical or women with mechanical valves. Unortunately, wararin is the most eective anticoagulant or preventing maternal thromboembolism but causes harmul etal
eects. As seen in Table 52-6, anticoagulation with heparin
is less hazardous or the etus, however, the risk o maternal
thromboembolic complications is increased (Steinberg, 2017).
As a compromise, some use heparin in early pregnancy, and
then transition to wararin in the second trimester.
Wararin is teratogenic and causes miscarriages, stillbirths,
and etal malormations (Chap. 8, p. 156). In one study o
TABLE 52-5. Selected Outcomes in Pregnancies
Complicated by Heart-Valve Replacement
Outcome
Mechanical
Valve (n = 212)
Tissue Valve
(n = 134)
Maternal mortality 3 (1.4) 2 (1.5)
Heart failure 162 (7.5) 1 (8.2)
Thrombotic complication 13 (6.1) 1 (0.7)
Hemorrhagic complication 49 (23) 7 (5.1)
Pregnancy loss <24 weeks 33 (15.6) 2 (1.5)
Stillbirth 6 (2.8) 0 (0)
Preterm birth <37 weeks 29 (18) 24 (19)
Data presented as n (%).Cardiovascular Disorders 923
CHAPTER 52
TABLE 52-6. Maternal and Fetal Composite Outcomes
in 800 Women with a Mechanical Heart
Value Receiving Anticoagulation
Treatment
Composite Adverse Outcome (%)
Maternala Fetalb
VKA 5 39
LMWH 16 14
LMWHc followed by VKA 16 16
UFHc followed by VKA 16 34
aMaternal death, valve failure, thromboembolism.
bMiscarriage, fetal death, congenital malformation.
cDuring first trimester.
LMWH = low-molecular-weight heparin; UFH = unfractionated heparin; vitamin K antagonist.
71 women given wararin throughout pregnancy, the rates o
miscarriage were 32 percent; stillbirth, 7 percent; and embryopathy, 6 percent (Cotruo, 2002). Te risk was highest when
the mean daily dose o wararin exceeded 5 mg. Similarly,
the American College o Cardiology and the American Heart
Association estimate that the risk o embryopathy is dose
dependent, and the risk is <3 percent i the wararin dose is
≤5 mg/d (Nishimura, 2014). Women treated with <5 mg/d
o wararin had etal risks similar to the low-molecular-weight
heparin (LMWH) regimen (Steinberg, 2017). I the dosage is
>5 mg/d, the risk o embryopathy exceeds 8 percent.
Anticoagulation or mechanical valves using low-dose unractionated heparin (UFH) is denitely inadequate and carries a
high associated maternal mortality rate (Chan, 2000; IturbeAlessio, 1986). Even ull anticoagulation with either UFH
or one o the LMWHs is associated with valvular thrombosis (Leyh, 2002, 2003; Rowan, 2001). However, compliance
with dosing and therapeutic monitoring may have contributed
(McLintock, 2014). Tereore, i ull anticoagulation with doseadjusted UFH or LMWH is used, meticulous monitoring is recommended. Te activated partial thromboplastin time (aP)
should be at least 2 times control or anti-Xa levels should be 0.8
to 1.2 U/mL at 4 to 6 hours postdose (Nishimura, 2014).
Recommendations for Anticoagulation
Several dierent treatment options exist, although all are principally based on consensus opinion, and none is completely ideal.
Wararin and LMWH are associated with ewer valve thromboses and thereore avored over subcutaneous UFH (D’Souza,
2017). In addition, therapeutic subcutaneous UFH dosing is
difcult to achieve due to lower peak plasma concentrations in
pregnant women, especially with advancing gestation (Barbour,
1995; Brancazio, 1995). One guideline rom the American College o Cardiology and the American Heart Association oers
dierent treatment options based on trimester and baseline war-
arin dose (Nishimura, 2014). Figure 52-3 displays a treatment
algorithm based on these guidelines. All recommendations also
include aspirin 75 to 100 mg orally daily.
Vaginal or cesarean delivery is ideally scheduled to allow controlled discontinuation o anticoagulation and partial reversal
o its eects. Tis also permits administration o regional anesthesia, which requires a degree o coagulation to avoid epidural
hematoma ormation (Chap. 25, p. 478). I delivery intervenes
while the anticoagulant is still eective, and extensive bleeding is encountered, protamine sulate is given intravenously
to reverse heparin eects. Patients should be counseled that in
these situations regional anesthesia may not saely be possible.
Following vaginal delivery, anticoagulant therapy with war-
arin or heparin may be restarted 6 hours later, usually with no
problems. Following cesarean delivery, ull anticoagulation is
withheld, but the optimal duration is unclear. Te American
College o Obstetricians and Gynecologists (2018a) recommends resuming UFH or LMWH 6 to 12 hours ater cesarean delivery. At Parkland Hospital, however, we wait at least
24 hours ollowing a major surgical procedure given inherent
bleeding risks. Following rst-trimester dilation and curettage
heparin is begun immediately.
Wararin, LMWH, and UFH are compatible with breast-
eeding. Tey do not accumulate in breast milk and thus do
not induce anticoagulant eects in the newborn (Briggs, 2022).
■ Cardiac Surgery During Pregnancy
Although usually postponed until ater delivery, valve replacement or other cardiac surgery during pregnancy may be liesaving. Several reviews conrm that such surgery is associated
with major maternal and etal morbidity (Liu, 2021). Elassy
and associates (2014) described 23 women who underwent
urgent open cardiac surgery or severe valve malunction.
wo women and 10 etuses—all at a gestational age below
28 weeks—died beore hospital discharge. Only six etuses
were delivered at term. In a review o 154 women undergoing
bypass surgery during pregnancy, the maternal mortality rate
was 11 percent, and the etal loss rate was 33 percent (Jha,
2018). o optimize outcomes, Chandrasekhar and coworkers (2009) recommend that surgery be elective when possible,
pump ow rate should remain >2.5 L/min/m2, perusion
pressure should exceed 70 mm Hg, and hematocrit should be
kept >28 percent.
■ Pregnancy after Heart Transplantation
Many successul pregnancies have ollowed cardiac transplantation (D’Souza, 2018; Macera, 2018). Current recommendations rom the International Society o Heart and Lung
ransplantation do not discourage pregnancy in stable heart
transplant recipients who are more than 1 year posttransplant
(Costanzo, 2010). Obviously, a highly specialized level o care
and multidisciplinary team is necessary.
Te transplanted heart appears to respond normally to
pregnancy-induced alterations (Cowan, 2012). Despite this,
complications are common during pregnancy. O 103 pregnancies in 57 heart recipients rom the National ransplantation
Pregnancy Registry, almost hal developed hypertension, and
11 percent suered at least one rejection episode during pregnancy (Coscia, 2010). Tey were usually delivered by cesarean
at a mean o 37 weeks’ gestation. Lie expectancy ollowing
heart transplantation is known to be limited. At ollow-up,924 Medical and Surgical Complications
Section 12
FIGURE 52-3 Algorithm for anticoagulation in gravidas with a mechanical heart valve. INR = international normalized ratio; IV = intravenous;
LMWH = low-molecular-weight heparin; aPTT = activated partial thromboplastin time; UFH = unfractionated heparin. (From Elkayam, 2020;
Nishimura, 2014; Roeder, 2011.)
at least 16 women had died more than 2 years postpartum. In
another small study, pregnancy itsel did not worsen long-term
survival rates (Dagher, 2018).
VALVULAR HEART DISEASE
Rheumatic ever is uncommon in the United States because o
less crowded living conditions, penicillin availability, and evolution o nonrheumatogenic streptococcal strains. Still, it remains
the chie cause o serious mitral valvular disease in women o
childbearing age in the nonindustrialized world (Liaw, 2021;
van Hagen, 2018).
■ Mitral Stenosis
Rheumatic endocarditis causes most mitral stenosis lesions. Te
normal mitral valve surace area is 4.0 cm2, and when stenosis
narrows this to <2.5 cm2, symptoms usually develop. Te contracted valve impedes blood ow rom the let atrium to the
ventricle.
With more severe stenosis, the let atrium dilates, let
atrial pressure is chronically elevated, and signicant pulmonary hypertension develops (Table 52-7) (Galiè, 2016).
Tese women have a relatively xed cardiac output, and thus
the increased preload o normal pregnancy and other actors
that raise cardiac output may cause ventricular ailure andCardiovascular Disorders 925
CHAPTER 52
pulmonary edema. Cardiac ailure develops or the rst time
during pregnancy in one ourth o women with mitral stenosis
(Caulin-Glaser, 1999). Te resulting pulmonary venous hypertension and pulmonary edema create symptoms o dyspnea,
atigue, palpitations, cough, and hemoptysis. Te classic murmur may not be heard in some women, and this clinical picture
at term may be conused with idiopathic peripartum cardiomyopathy (Cunningham, 1986, 2019).
Also with signicant stenosis, tachycardia shortens ventricular
diastolic lling time and elevates the mitral gradient. Tis too
may lead to pulmonary edema, and thereore, sinus tachycardia
is oten treated prophylactically with β-blocking agents. Atrial
tachyarrhythmias, including brillation, are common in mitral
stenosis and are treated aggressively. Atrial brillation predisposes
to mural thrombus ormation and cerebrovascular embolization
that can cause stroke (Chap. 63, p. 1132). Atrial thrombosis can
also develop despite a sinus rhythm (Hameed, 2005).
Pregnancy Outcomes
In general, complications are directly associated with the degree
o valvular stenosis. Women with a mitral-valve area <2 cm2
are at greatest risk (Siu, 2001b). In one study o 273 gravidas with mitral stenosis, 43 percent developed heart ailure,
and almost a ourth o these women required admission (van
Hagen, 2018). For women with severe stenosis, hal developed
heart ailure, and one woman died. Fetal-growth restriction was
more common in women with a mitral valve area <1.0 cm2.
Prognosis also is related to maternal unctional capacity. Among 486 pregnancies complicated by rheumatic heart
disease—predominantly mitral stenosis—8 o 10 maternal deaths
were in women in NYHA classes III or IV (Sawhney, 2003).
Management
Limited physical activity is generally recommended in women
with mitral stenosis. I symptoms o pulmonary congestion
develop, activity is urther reduced, dietary sodium is restricted,
and diuretics are given. Also, β-blocker drug therapy slows the
ventricular response to activity. I new-onset atrial brillation
develops, intravenous verapamil, 5 to 10 mg, is given, or electrocardioversion is perormed. For chronic brillation, digoxin,
a β-blocker, or a calcium-channel blocker can slow ventricular
response. Terapeutic anticoagulation is indicated with persistent brillation, let atrial thrombus, and/or a history o embolism (Nanna, 2014).
Surgical intervention is considered or women with symptomatic severe mitral stenosis. Other candidates are those with
mitral-valve area 1.5 to 2.0 cm2 complicated by recurrent systemic
embolization or severe pulmonary hypertension. Balloon valvuloplasty is preerred i the valve is pliable (Bui, 2014). In one review
o 71 pregnant women with severe mitral stenosis and heart ailure who underwent percutaneous valvuloplasty, 98 percent were
either NYHA class I or II by the time o delivery (Esteves, 2006).
At a mean o 44 months, the total event-ree maternal survival
rate was 54 percent. However, eight women required another surgical intervention. All o the 66 newborns who were delivered at
term had normal growth and development.
Labor and delivery are particularly stressul or women with
symptomatic mitral stenosis. Pain, exertion, and anxiety cause
tachycardia with possible rate-related heart ailure. Epidural
analgesia or labor is ideal. Fluid overload should be avoided,
and these women are best managed on the “dry” side. As
shown in Figure 52-4 uterine contractions raise cardiac output
by increasing circulating blood volume. Abrupt expansion in
preload may elevate pulmonary capillary wedge pressure and
cause pulmonary edema. Wedge pressures rise immediately
postpartum. One hypothesis or this suggests that the loss o
the low-resistance placental circulation couples with venous
“autotransusion” rom a now-empty, contracted uterus and
rom the lower extremities and pelvis (Clark, 1985). Tus, pulmonary edema may maniest immediately postpartum.
TABLE 52-7. Major Cardiac Valve Disorders
Type Cause Pathophysiology Pregnancy
Mitral stenosis Rheumatic valvulitis LA dilation and passive
pulmonary hypertension
Atrial fibrillation
Heart failure from fluid overload,
tachycardia
Mitral insufficiency Rheumatic valvulitis
Mitral valve prolapse
LV dilation
LV dilation and eccentric
hypertrophy
Ventricular function improves with
afterload decrease
Aortic stenosis Congenital bicuspid valve LV concentric hypertrophy,
decreased cardiac output
Moderate stenosis is tolerated; severe
is life-threatening with decreased
preload, e.g., obstetrical hemorrhage
or regional analgesia
Aortic insufficiency Rheumatic valvulitis
Connective tissue disease
Congenital
LV hypertrophy and dilation Ventricular function improves with
afterload decrease
Pulmonary stenosis Rheumatic valvulitis
Congenital
Severe stenosis associated
with RA and RV
enlargement
Mild stenosis usually well tolerated;
severe stenosis associated with right
heart failure and atrial arrhythmias
LA = left atrium; LV = left ventricle; RA = right atrium; RV = right ventricle.926 Medical and Surgical Complications
Section 12
Most preer vaginal delivery in women with mitral stenosis.
Elective induction is reasonable so that labor and delivery are
attended by a scheduled, experienced team. With severe stenosis and chronic heart ailure, insertion o a pulmonary artery
catheter may help guide management.
■ Mitral Insufficiency
A trivial degree o mitral insufciency is ound in most normal
patients. But i mitral valve leaets align improperly during
systole, abnormal degrees o mitral regurgitation can develop.
Tis is eventually ollowed by let ventricular dilation and
eccentric hypertrophy (see able 52-7). Acute mitral insu-
ciency is caused by chordae tendineae rupture, papillary muscle
inarction, or leaet peroration rom inective endocarditis.
Chronic mitral regurgitation, in contrast, may derive rom
rheumatic ever, connective tissue diseases, mitral valve prolapse, or let ventricular dilation o any etiology—or example, dilated cardiomyopathy. Less common causes include a
calcied mitral annulus, possibly some appetite suppressants,
and in older women, ischemic heart disease. Mitral valve vegetations—Libman-Sacks endocarditis—are relatively common
in women with antiphospholipid antibodies (Shro, 2012).
Tese sometimes coexist with systemic lupus erythematosus
(Chap. 62, p. 1114).
In nonpregnant patients, symptoms rom mitral valve
insufciency are rare, and valve replacement is seldom indicated unless inective endocarditis develops. During pregnancy, mitral regurgitation is similarly well tolerated, probably
because the lowered systemic vascular resistance yields less
regurgitation. In the report by van Hagen and associates
(2018), o 117 women with moderate or severe regurgitation,
23 percent developed heart ailure during pregnancy. Occasionally tachyarrhythmias or severely depressed systolic unction require treatment.
■ Mitral Valve Prolapse
Tis diagnosis implies the presence o a pathological connective
tissue disorder—oten termed myxomatous degeneration—which
may involve the valve leaets, the annulus, or the chordae tendineae. Mitral insufciency may develop. Most women with
mitral valve prolapse are asymptomatic and are diagnosed during routine examination or echocardiography. Te ew women
with symptoms have anxiety, palpitations, atypical chest pain,
dyspnea with exertion, and syncope (Guy, 2012).
Pregnant women with mitral valve prolapse rarely have
cardiac complications. Hypervolemia may even improve
alignment o the mitral valve, and women without pathological myxomatous degeneration generally have excellent pregnancy outcomes (Leśniak-Sobelga, 2004). For women who
are symptomatic, β-blocking drugs diminish sympathetic
tone, relieve chest pain and palpitations, and reduce the risk
o lie-threatening arrhythmias.
■ Aortic Stenosis
Since the decline in incidence o rheumatic disease, congenital
bicuspid valve is now the most requent cause o aortic stenosis
in young women in the United States (Carabello, 2017). A normal aortic valve has an area o 3 to 4 cm2, with a pressure gradient <5 mm Hg. I the valve area is <1 cm2, ow is severely
obstructed, and progressive pressure overload on the let ventricle develops. Concentric let ventricular hypertrophy ollows,
and i it is severe, end-diastolic pressures become elevated,
ejection raction decreases, and cardiac output is reduced (see
able 52-7). Severity is estimated by peak velocity o ow across
the aortic valve. Mild stenosis has a peak velocity o 2–2.9 m/s,
moderate 3-3.9 m/s, and severe ≥4 m/s. Corresponding mean
pressure gradients are <20, 20–39, and ≥40 mm Hg, respectively (Carabello, 2017). Characteristic maniestations develop
late and include chest pain, syncope, heart ailure, and sudden
death rom arrhythmias. In asymptomatic patients, the mortality rate is 1 percent per year, but with symptoms it increases
to 25 percent per year. Tus, valve replacement is indicated or
symptomatic patients.
Pregnancy
In one single-center study, aortic stenosis accounted or 15 percent o cases o congenital heart disease in 178 pregnant women
(Kim, 2019). Tat said, clinically signicant aortic stenosis is
inrequent during pregnancy. Mild to moderate degrees o stenosis are well tolerated. Severe disease is lie-threatening and
carries a 6-percent mortality risk (Lin, 2017). Te principal
underlying hemodynamic problem is the xed cardiac output
associated with severe stenosis. During pregnancy, several common events acutely lower preload urther and thus aggravate the
xed cardiac output. Tese include vena caval occlusion rom
the gravid uterus, regional analgesia, sepsis, and hemorrhage.
From the earlier-cited Canadian study, complication rates were
higher i the aortic valve area measured <1.5 cm2 (Siu, 2001b).
Management
For asymptomatic women with aortic stenosis, no treatment except
close observation is required. Management o a symptomatic
Pulmonary capillary wedge pressure
(mm Hg)
25
A B
Time-arbitrary units
C D E
5 0
10
15
20
A C
E
B
D
FIGURE 52-4 Mean pulmonary capillary wedge pressure measurements (red graph line) in eight women with mitral valve stenosis. Shaded yellow and blue boxes are mean (± 1 SD) pressures in
nonlaboring normal women at term. A. First-stage labor. B. Second-stage labor 15 to 30 minutes before delivery. C. Postpartum 5
to 15 minutes. D. Postpartum 4 to 6 hours. E. Postpartum 18 to
24 hours. (Data from Clark, 1985, 1989.)Cardiovascular Disorders 927
CHAPTER 52
woman is undertaken in conjunction with a cardiologist and
includes strict limitation o activity and cautious use o diuretics. I symptoms persist, surgical intervention or preterm
delivery may be considered. Catheter-based valvuloplasty is
associated with risks to both the mother and etus and shows
poor long-term efcacy (Pessel, 2014). Namely, the aortic valve
can again narrow or new aortic regurgitation may develop. Te
alternative surgical approach—valve replacement—is associated with signicant risk o etal demise due to the hypotension associated with cardiac bypass. ranscatheter aortic valve
replacement (AVR) has been perormed in low-risk nonpregnant women, but there is little experience with this procedure
during pregnancy (Hodson, 2016; Mack, 2019). Accordingly,
the American College o Cardiology, the American Heart Association, and the European Society o Cardiology recommend
delaying conception until ater surgical correction or severe
aortic stenosis (Nishimura, 2014; Regitz-Zagrosek, 2018). For
those with uncorrected severe symptomatic aortic stenosis,
cesarean delivery is preerred. For asymptomatic women with
severe stenosis, care is individualized. In nonsevere stenosis,
vaginal delivery is preerred (Regitz-Zagrosek, 2018).
For women with critical aortic stenosis, intensive monitoring during labor is essential. Pulmonary artery catheterization may be helpul because o the narrow margin separating
uid overload rom hypovolemia. Women with aortic stenosis are dependent on adequate end-diastolic ventricular lling
pressures to maintain cardiac output and systemic perusion.
Abrupt drops in end-diastolic volume may result in hypotension, syncope, myocardial inarction, and sudden death. Tus,
avoiding diminished ventricular preload and maintaining cardiac output are key. During labor and delivery, aected women
are best managed on the “wet” side. Tis provides a margin o
saety in intravascular volume in anticipation o possible hemorrhage. In women with a competent mitral valve, pulmonary
edema is rare.
During labor, narcotic or low-dose, slow-inusion epidural
analgesia seems ideal and avoids potentially hazardous hypotension. Easterling and coworkers (1988) studied the eects
o epidural analgesia in ve women with severe stenosis and
demonstrated immediate and proound eects rom decreased
lling pressures. Xia and colleagues (2006) emphasize slow
administration o dilute local anesthetic agents into the epidural space. In hemodynamically stable women, orceps or vacuum delivery is used only or standard obstetrical indications.
In those experiencing dizziness, shortness o breath, or tachycardia with pushing, an operative vaginal delivery is preerred.
■ Aortic Insufficiency
Aortic valve regurgitation or insufciency allows diastolic ow
o blood rom the aorta back into the let ventricle. Frequent
causes o insufciency are rheumatic ever, connective tissue
abnormalities, and congenital lesions (Carabello, 2017). With
Maran syndrome, the aortic root may dilate and create regurgitation (p. 936). Acute insufciency may also develop with
bacterial endocarditis or aortic dissection. Last, aortic and
mitral valve insufciency have both been linked to the appetite suppressants enuramine and dexenuramine and to the
ergot-derived dopamine agonists cabergoline and pergolide
(Schade, 2007; Zanettini, 2007). With chronic insufciency,
let ventricular hypertrophy and dilation develop. Slow-onset
atigue, dyspnea, and pulmonary edema ollows ventricular
dilation with subsequent rapid deterioration (see able 52-7).
Aortic insufciency is generally well tolerated during
pregnancy. Like mitral valve insufciency, reduced vascular
resistance is thought to improve hemodynamic unction. I
symptoms o heart ailure develop, diuretics are given, and bed
rest is encouraged.
■ Pulmonic Stenosis
Tis lesion is usually congenital and may be associated with Fallot tetralogy or Noonan syndrome (Chikwe, 2017). Te greater
hemodynamic burden o pregnancy can precipitate right-sided
heart ailure or atrial arrhythmias in women with severe stenosis. Surgical correction ideally is done beore pregnancy, but i
symptoms progress, a balloon valvuloplasty may be necessary
antepartum (Galal, 2015; Siu, 2001a).
In studying pregnancy outcomes, Drenthen and associates
(2006) ound inrequent cardiac complications in 81 pregnancies in 51 Dutch women with pulmonic stenosis. Te NYHA
classication worsened in two women, and nine experienced
palpitations or arrhythmias. No changes in pulmonary valvular
unction or other adverse cardiac events were reported. However, noncardiac complication rates were signicant—17 percent had preterm delivery, 15 percent had hypertension, and
4 percent developed thromboembolism.
CONGENITAL HEART DISEASE
Te incidence o congenital heart disease in the United States
approximates 1.9 percent o live births, and hal o these are
moderate to severe orms (Lin, 2017). With modern surgeries, approximately 90 percent o those born with congenital
heart disease survive to childbearing age, and it is now the most
common type o heart disease encountered during pregnancy
(Hopkins, 2018). Specically, analysis rom the United States
Nationwide Inpatient Sample database showed a linear rise in
the prevalence o congenital heart disease between 2000 and
2010—rom 6.4 to 9.0 per 10,000 women admitted or delivery (Tompson, 2015).
Te odds o obstetrical and perinatal complications are
increased two- to threeold in women with congenital heart disease compared with unaected women (Ramage, 2019). Also,
the maternal mortality rate was higher or women with congenital heart disease at 178 per 100,000 deliveries compared
with 7 per 100,000 deliveries in unaected gravidas (Tompson, 2015).
■ Atrial Septal Defects
A patent oramen ovale (PFO) is a persisting incompetence o
the ossa ovale, and this ap has the potential to open under
increased hydrostatic pressure. O all adults, approximately
one ourth have this deect (Silvestry, 2015). Te small risk o
PFO-related stroke, discussed subsequently, is likely higher in928 Medical and Surgical Complications
Section 12
pregnant than in nonpregnant women and mainly attributed to
normal pregnancy hypercoagulability (Chen, 2016). Preventive
PFO repair is not recommended (Kernan, 2014).
An atrial septal deect (ASD) is a true hole in the septum.
Te secundum-type deect accounts or 70 percent, and associated mitral valve myxomatous abnormalities with prolapse
are common. Most ASDs are typically asymptomatic until the
third or ourth decade o lie (Lin, 2017). I an ASD is discovered in adulthood, most recommend repair.
Pregnancy with an unrepaired ASD is well tolerated unless
pulmonary hypertension has developed, but this is uncommon
(Bredy, 2018). Medical treatment during pregnancy is indicated or congestive heart ailure or an arrhythmia. Te risk o
endocarditis with an ASD is negligible.
With the potential to shunt blood rom right to let, a paradoxical embolism is possible. A venous thrombus enters the systemic arterial circulation through the ASD, causing an embolic
stroke (Bredy, 2018). For this reason, lters should be placed
on intravenous access sites. In a gravida with an ASD but without current venous thromboembolism (VE), the decision to
add anticoagulant prophylaxis to counter this potential embolism risk is problematic. However, or a pregnant woman with
an ASD who is immobile or has another risk actor or thromboembolism, compression stockings and prophylactic heparin
are reasonable.
■ Ventricular Septal Defects
Tese lesions close spontaneously during childhood in 90 percent o cases. O the our main ventricular septal deect (VSD)
types, most deects are paramembranous. Tis location is well
below the outlet valves yet above the ventricular musculature.
Te degree o associated let-to-right shunt and physiological
derangements are related to lesion size. In general, i the deect
measures <1.25 cm2, pulmonary hypertension and heart ailure
do not develop. I the eective deect size exceeds that o the
aortic valve orice, symptoms rapidly develop. For these reasons, most children undergo surgical repair beore pulmonary
hypertension develops. Adults with unrepaired large deects
develop let ventricular ailure and pulmonary hypertension and
have a high incidence o bacterial endocarditis (Brickner, 2014).
Pregnancy is well tolerated with small- to moderate-sized
VSD shunts. However, i pulmonary arterial pressures reach
systemic levels, ow is reversed or bidirectional—Eisenmenger
syndrome (p. 929). I this cyanotic condition develops, the
maternal and etal mortality rates are signicantly higher, and
thus pregnancy is generally not advisable (Lin, 2017). Bacterial endocarditis is more common with unrepaired deects, and
antimicrobial prophylaxis is oten required (p. 934). As shown
in able 52-4, 16 percent o ospring born to these women
also have a VSD.
■ Atrioventricular Septal Defects
An atrioventricular (AV) septal deect is characterized by a
common, ovoid AV junction. Tese account or approximately
3 percent o all congenital cardiac malormations and are distinct rom isolated ASDs or VSDs. Tis deect is associated with
aneuploidy, Eisenmenger syndrome, and other malormations
(Foeller, 2018). Compared with simple septal deects, complications are more requent during pregnancy. In a review o
48 pregnancies in 29 aected women, complications included
persistent deterioration o NYHA class in 23 percent, signi-
cant arrhythmias in 19 percent, and heart ailure in 2 percent
(Drenthen, 2005). Congenital heart disease was identied in
15 percent o the ospring.
■ Persistent (Patent) Ductus Arteriosus
Te ductus connects the proximal let pulmonary artery to
the descending aorta just distal to the let subclavian artery.
Functional closure o the ductus rom vasoconstriction occurs
shortly ater term birth. Te physiological consequences with
its persistence are related to its size. Most signicant lesions
are repaired in childhood. However, in women with an unrepaired ductus, pulmonary hypertension, heart ailure, or cyanosis will develop i systemic blood pressure alls and blood
ow reverses rom the pulmonary artery into the aorta (Foeller,
2018). A sudden blood pressure decline at delivery—such as
with regional analgesia or hemorrhage—may lead to atal collapse. Tereore, hypotension is avoided but treated vigorously
i it develops. Prophylaxis or bacterial endocarditis is indicated at delivery or unrepaired deects (p. 934). As shown in
able 52-4, the incidence o inheritance approximates 4 percent.
■ Cyanotic Heart Disease
Cyanosis develops when congenital heart lesions produce rightto-let shunting o blood past the pulmonary capillary bed. Te
classic and most commonly encountered lesion in adults and
during pregnancy is the Fallot tetralogy (Foeller, 2018). Tis
is characterized by a large VSD, pulmonary stenosis, right
ventricular hypertrophy, and an overriding aorta that receives
blood rom both the right and let ventricles. Te magnitude
o the shunt varies inversely with systemic vascular resistance.
Hence, during pregnancy, when peripheral resistance decreases,
shunt ow increases and cyanosis worsens.
Generally, women with cyanotic heart disease do poorly
during pregnancy. Tose with concomitant Eisenmenger syndrome are at greatest risk (p. 929). With uncorrected Fallot
tetralogy, maternal mortality rates approach 10 percent. For
etal outcome, there is a relationship between chronic hypoxemia, polycythemia, and complications such as miscarriage and
perinatal morbidity. When hypoxemia is intense enough to
stimulate a rise in hemoglobin concentration >20 g/dL, pregnancy wastage is virtually 100 percent (Lin, 2017).
Although not all cyanotic lesions are repairable, with satis-
actory surgical correction beore pregnancy, maternal and etal
outcomes are much improved. In a review o 197 pregnancies in
99 women with surgically corrected Fallot tetralogy, pregnancy
was usually well tolerated, and no mothers died (Cauldwell,
2017). Still, almost 9 percent o pregnancies were complicated
by adverse cardiac events including new-onset or worsening
arrhythmias and heart ailure (Balci, 2011; Kamiya, 2012).
Some women with Ebstein anomaly, characterized by a
malpositioned and malormed tricuspid valve, may reachCardiovascular Disorders 929
CHAPTER 52
reproductive age. Te right ventricle is small and the right
atrium is severely dilated. Right-sided heart ailure is common.
Tese women are very preload dependent, and pregnancyinduced hypervolemia can worsen the tricuspid regurgitation
(Kanoh, 2018). Arrhythmias also are common, especially
Wol-Parkinson-White syndrome (p. 936). Vaginal delivery
seems preerable in most cases. In the absence o cyanosis,
heart ailure, or signicant arrhythmias, aected women usually tolerate pregnancy well (Sa, 2016).
■ Pregnancy after Surgical Repair
Transposition of the Great Vessels
Pregnancy ollowing arterial switch operation or transposition is associated with good outcomes. A major concern is
atal arrhythmias that usually are precipitated by exercise (Lin,
2017). Earlier studies cited a relatively high rate o heart ailure
and arrhythmias, but more recent studies have avorable pregnancy outcomes (rigas, 2014). In one study o 20 pregnancies, there were three cases o heart ailure (Horiuchi, 2019).
Stoll and coworkers (2018) reported no adverse cardiac events
in 25 pregnancies.
O other deects, repaired truncus arteriosus and doubleoutlet right ventricle with subsequent successul, although
eventul, pregnancies have been described (Drenthen, 2008;
Hoendermis, 2008). Ironically, preconceptional counseling did little to dissuade these women rom childbearing
(Cauldwell, 2016).
Single Functional Ventricle
With hypoplastic let heart syndrome, most aected women are
now expected to survive into adulthood (Davis, 2018). Frequently, these women become pregnant, and those who have
undergone a Fontan repair carry a particularly higher risk or
complications. In brie, this procedure involves diverting blood
via a surgical anastomosis rom the vena cava to the pulmonary
artery without passing through the right ventricle. Blood ows
passively to the pulmonary vasculature. Preload drives circulation in the Fontan circuit, and thus patients are sensitive to
volume changes (Moroney, 2018).
From their review o 255 pregnancies in 133 women,
Garcia-Ropero and colleagues (2018) reported 115 miscarriages
(45 percent) and 19 elective terminations (7 percent). Cardiac
complications included arrhythmias in 8 percent and heart ailure in 4 percent o pregnancies. Among 133 live births, there
were 68 preterm births (59 percent) and 7 perinatal deaths
(6 percent). Postpartum venous thromboembolism also is common (Moroney, 2020).
Similar complications attend a maternal systemic right ventricle, that is, one in which the right ventricle rather than the
let pumps blood to the systemic circulation (Khan, 2015).
■ Eisenmenger Syndrome
Tis describes secondary pulmonary hypertension that arises
rom any cardiac lesion. Te most common underlying deects
are ASD, VSD, and persistent ductus arteriosus (Fig. 52-5).
Initial left-to-right shunt
A B
Pulmonary
arteriole
Left
atrium
Left
atrium
Right
atrium
Right
atrium
Right
ventricle
Right ventricle
hypertrophy
Narrowed pulmonary
arterioles result and
lead to pulmonary
hypertension
Left
ventricle
Left
ventricle
Pulmonary
artery
Pulmonary
artery
Ultimate right-to-left shunt
FIGURE 52-5 Eisenmenger syndrome due to a ventricular septal defect (VSD). A. Substantial left-to-right shunting through the VSD leads
to morphological changes in the smaller pulmonary arteries and arterioles. Specifically, medial hypertrophy, intimal cellular proliferations,
and fibrosis lead to narrowing or closure of the vessel lumen. These vascular changes create pulmonary hypertension and a resultant reversal of the intracardiac shunt (B). With sustained pulmonary hypertension, extensive atherosclerosis and calcification often develop in the
large pulmonary arteries. Although a VSD is shown here, Eisenmenger syndrome may also develop in association with a large atrial septal
defect or persistent ductus arteriosus.930 Medical and Surgical Complications
Section 12
Te syndrome develops when pulmonary vascular resistance
exceeds systemic resistance and leads to concomitant right-tolet shunting. Patients are asymptomatic or years, but eventually pulmonary hypertension becomes severe enough to cause
this shunting.
Pregnant women with Eisenmenger syndrome tolerate hypotension poorly, and death usually is caused by right ventricular
ailure with cardiogenic shock. In a review o 73 pregnancies,
Weiss and associates (1998) cited a 36-percent maternal death
rate. Tree o 26 deaths were antepartum, and the remainder
o women died intrapartum or within a month o delivery. In
another study o 13 gravidas, one mother died 17 days ater
delivery, and there were ve perinatal deaths (Wang, 2011).
Last, in a series o 11 pregnancies in China, our mothers died
(Duan, 2016). Given such poor outcomes or both mother and
etus, Eisenmenger syndrome is considered to be an absolute contraindication to pregnancy (American College o Obstetricians and
Gynecologists, 2019; Foeller, 2018; Meng, 2017).
PULMONARY HYPERTENSION
Normal resting mean pulmonary artery pressure is 12 to
16 mm Hg. Most dene pulmonary hypertension in nonpregnant
individuals as a resting mean pulmonary pressure >25 mm Hg
(Franco, 2019). Pulmonary vascular resistance in late normal
pregnancy approximates 80 dyne/sec/cm−5, which is 34-percent
less than the nonpregnant value o 120 dyne/sec/cm−5
(Clark, 1989).
Physiologically, pregnancy is associated increased cardiac
output, and in healthy gravidas, pulmonary vascular resistance
decreases to help accommodate this (Clark, 1989). With pulmonary hypertension, the sti pulmonary vasculature does not
allow the normal all in pulmonary vascular resistance. Tus,
the normal increased cardiac output actually leads to urther
pulmonary artery pressure elevation and then eventually to
right heart ailure. With ailure, the interventricular septum
bulges letward to impair let ventricular diastolic lling, which
compromises cardiac output (Gei, 2014; Pieper, 2011).
Te current clinical classication system, shown in
Table 52-8, contains ve groups o disorders that cause pulmonary hypertension (Galiè, 2016). Important prognostic and
therapeutic distinctions separate group 1 pulmonary arterial
hypertension and the other groups. Group 1, which is more
common in nonpregnant women, indicates that a specic
disease aects pulmonary arterioles. It includes idiopathic or
primary pulmonary arterial hypertension as well as those cases
secondary to a known cause such as connective tissue disease.
For example, approximately a third o women with scleroderma
and 10 percent with systemic lupus erythematosus have pulmonary arterial hypertension (Franco, 2019). Other causes
in young women are human immunodeciency virus (HIV)
inection, sickle-cell disease, and thyrotoxicosis.
In pregnant women, group 2 disorders are the most common. Tese are secondary to pulmonary venous hypertension
caused by let-sided atrial, ventricular, or valvular disorders. A
typical example is mitral stenosis discussed earlier (p. 924). In
contrast, groups 3 through 5 are seen inrequently in young
otherwise healthy women.
■ Diagnosis and Prognosis
Symptoms may be vague, and dyspnea with exertion is the
most requent. With group 2 disorders, orthopnea and nocturnal dyspnea are also usually present. Angina and syncope
occur when right ventricular output is xed, and they suggest
advanced disease. Chest radiography oten shows enlarged pulmonary hilar arteries and attenuated peripheral markings. It
may also disclose parenchymal causes o hypertension. Noninvasive echocardiography can provide an estimate o pulmonary
artery pressures, although cardiac catheterization remains the
standard or measurement. In studies o pregnant women who
underwent both echocardiography and cardiac catheterization,
pulmonary artery pressures were signicantly overestimated by
echocardiography in approximately a third o cases (Herrera,
2020; Wylie, 2007).
Regardless o the etiology, the nal common pathway o
pulmonary hypertension is right heart ailure and death. Te
average survival length ater diagnosis is <4 years (Krexi, 2015).
Tat said, longevity depends on the severity and cause o pulmonary hypertension at discovery. As discussed later, some disorders respond to medical interventions, which may improve
quality o lie. Preconceptional and contraceptive counseling
are imperative (American College o Obstetricians and Gynecologists, 2019).
■ Pregnancy
Te maternal mortality rate is appreciable in aected women,
and this is especially so with idiopathic pulmonary arterial
hypertension (Martin, 2019). In the past, the ability to accurately identiy causes and assess disease severity were oten poor.
Tus, although most severe cases o idiopathic pulmonary
arterial hypertension had the worst prognosis, it was erroneously assumed that all types o pulmonary hypertension were
equally dangerous. With widespread use o echocardiography,
less-severe lesions with a better prognosis are now discernible.
Te maternal mortality rate or pulmonary hypertension has
improved. In one study, it was 25 percent during the decade
ending in 2007 compared with 38 percent or the decade ending in 1996 (Bédard, 2009). Importantly, almost 80 percent
o the deaths were during the rst month postpartum. Meng
and associates (2017) reported mortality rates o 23 percent
with group 1 and 5 percent with the other groups. Mortality
risk correlates positively with advancing pulmonary hypertension severity, which is characterized by Eisenmenger syndrome,
severe hypertension, and higher NYHA class (Keepanasseril,
2019; Sun, 2018). In an audit o 47 pregnancies in women with
pulmonary hypertension at Parkland Hospital, the maternal
mortality rate was 9 percent (Herrera, 2020). O our deaths,
three women had severe hypertension. A striking example o
right ventricular hypertrophy in one o these women who died
is shown in Figure 52-6.
Pregnancy is contraindicated with severe disease. Tis is
especially true in women with pulmonary arterial changes,
which develop in most group 1 cases. With milder disease rom
other causes—group 2 being the most common—the prognosis is better. With the more requent use o echocardiography
and pulmonary artery catheterization in young women withCardiovascular Disorders 931
CHAPTER 52
TABLE 52-8. Comprehensive Clinical Classification of Pulmonary Hypertension of the European Society of Cardiology
and the European Respiratory Society
1. Pulmonary arterial hypertension
Idiopathic
Heritable
Drug and toxin induced
Associated with connective tissue disease, HIV infections, portal hypertension, congenital heart diseases, schistosomiasis
I’ Pulmonary venoocclusive disease and/or pulmonary capillary hemangiomatosis
Idiopathic
Heritable
Drugs, toxins and radiation induced
Associated with connective tissue disease, HIV infection
I” Persistent pulmonary hypertension of the newborn
2. Pulmonary hypertension due to left heart disease
Left ventricular systolic dysfunction
Left ventricular diastolic dysfunction
Valvular disease
Congenital/acquired left heart inflow/outflow tract obstruction and congenital cardiomyopathies
Congenital/acquired pulmonary vein stenosis
3. Pulmonary hypertension due to lung diseases and/or hypoxia
Chronic obstructive pulmonary disease
Interstitial lung disease
Other pulmonary diseases with mixed restrictive and obstructive pattern
Sleep-disoriented breathing
Alveolar hypoventilation disorder
Chronic exposure to high altitude
Developmental lung diseases
4. Chronic thromboembolic pulmonary hypertension/other pulmonary artery obstructions
Chronic thromboembolic pulmonary hypertension
Other pulmonary artery obstructions, i.e., tumors, arteritis, pulmonary stenosis, parasites
5. Pulmonary hypertension with unclear and/or multifactorial mechanisms
Hematological disorders: chronic hemolysis, myeloproliferative disorders, splenectomy
Systemic disorders: sarcoidosis, pulmonary histiocytosis, neurofibromatosis
Metabolic disorders: glycogen storage disease, Gaucher disease, thyroid disorders
Others: fibrosing mediastinitis, chronic renal failure
HIV = human immunodeficiency virus.
Adapted from Galiè, 2016.
FIGURE 52-6 Cross-sectional display of right cardiac dysmorphology in a pregnant woman who died from undiagnosed primary
pulmonary hypertension. There is prominent dilation of the right
ventricle with right ventricular hypertrophy (arrow).
heart disease, we have identied women with mild to moderate pulmonary hypertension who tolerate pregnancy, labor, and
delivery well (Herrera, 2020).
■ Management
reatment o symptomatic pregnant women includes limiting
activity and avoiding supine position later in gestation. Diuretics, supplemental oxygen, and pulmonary vasodilator drugs are
standard therapy or symptoms. Some recommend anticoagulation. Several reports describe the successul use o intravenous
pulmonary artery vasodilators (Foeller, 2018; Franco, 2019).
Prostacyclin analogues that can be administered parenterally
include epoprostenol (Flolan) and treprostinil (Remodulin),
whereas iloprost (Ventavis) is inhaled. Each has been used
in pregnancy. Inhaled nitric oxide is an option that has been932 Medical and Surgical Complications
Section 12
employed in cases o acute cardiopulmonary decompensation.
As reviewed by Običan and Cleary (2014), phosphodiesterase-5 inhibitors, such as sildenal (Viagra), cause vasodilation
o both the pulmonary and systemic vascular beds and have an
inotropic eect on the hypertrophic right ventricle. Tis also
has been used to advantage during pregnancy (Meng, 2017).
Bosentan—an endothelin-receptor antagonist, and riociguat—
a soluble guanylate cyclase stimulator, are teratogenic in mice
and contraindicated in pregnancy (Franco, 2019).
During labor and delivery, supplemental oxygen is given to
maintain >90 percent saturation. Tese women are at greatest
risk when venous return and right ventricular lling are diminished. o avoid hypotension, assiduous attention is given to
epidural analgesia induction, uid therapy, and blood loss
prevention and treatment at delivery (Martin, 2019; Meng,
2017).
CARDIOMYOPATHIES
Te American Heart Association denes these as a heterogeneous group o myocardial diseases associated with mechanical
and/or electrical dysunction (Narula, 2017). Aected women
usually have inappropriate ventricular hypertrophy or dilation.
Cardiomyopathies stem rom varied causes, and the most requent is genetic. O the two major divisions, primary cardiomyopathies are solely or predominantly conned to the heart
muscle. Examples are hypertrophic cardiomyopathy, dilated
cardiomyopathies, and peripartum cardiomyopathy. Secondary
cardiomyopathies result rom generalized systemic disorders that
produce pathological myocardial involvement. Diabetes, systemic lupus erythematosus, chronic hypertension, and thyroid
disorders are representative conditions.
■ Hypertrophic Cardiomyopathy
Tis disorder aects approximately 1 in 500 adults (Herrey, 2014).
Characterized by cardiac hypertrophy, myocyte disarray, and interstitial brosis, the condition in up to 60 percent o aected patients
is caused by mutations in genes that encode cardiac sarcomere
proteins. In such cases, inheritance is autosomal dominant, and
genetic screening is complex (Cirino, 2019; Elliott, 2014). Other
genetic and nongenetic etiologies underlie 5 to 10 percent o cases,
and the cause is unknown in approximately 25 percent. Te resulting myocardial muscle abnormality is typied by let ventricular
myocardial hypertrophy with a pressure gradient against let ventricular outow. Diagnosis is established by echocardiographic
identication o a hypertrophied and nondilated let ventricle in
the absence o other cardiovascular conditions.
Most aected women are asymptomatic, but dyspnea, anginal
or atypical chest pain, syncope, and arrhythmias may develop.
Complex arrhythmias may progress to sudden death, which is
the most requent cause o death. Asymptomatic patients with
runs o ventricular tachycardia are especially prone to sudden
death. Symptoms usually worsen with exercise.
Studies that include more than 700 pregnancies in 500 women
indicate an overall relatively good prognosis (Schauelberger,
2019). In a systematic review with 237 women with hypertrophic cardiomyopathy who had a combined 408 pregnancies,
the maternal mortality rate was 0.5 percent (Schinkel, 2014).
Worsening o symptoms or other complications developed in
29 percent, and 26 percent delivered preterm.
Management is similar to that or aortic stenosis (p. 926).
Controlling heart rate and avoiding preload and aterload reduction are therapy basics. Strenuous exercise is prohibited during
pregnancy. Abrupt positional changes are avoided to prevent
reex vasodilation and decreased preload. I symptoms develop,
especially angina, β-adrenergic or calcium-channel blocking
drugs are given. Drugs that evoke diuresis or reduce vascular resistance are generally not used because they decrease preload. But
i these are necessary, women should be closely monitored. Te
delivery route is determined by obstetrical indications. Choice o
anesthesia is controversial, and some consider general anesthesia
the saest. Regional analgesia can be used with careully titrated,
adequate intravascular volume to maintain let ventricular lling
pressure. Neonates rarely demonstrate inherited lesions at birth.
■ Dilated Cardiomyopathy
Tis is characterized by let and/or right ventricular enlargement
and reduced systolic unction in the absence o coronary, valvular, congenital, or systemic disease known to cause myocardial
dysunction. Although there are many known inherited and
acquired causes o dilated cardiomyopathy, the etiology remains
undened in approximately hal o cases (Schauelberger, 2019).
Some result rom viral inections, including myocarditis and HIV.
Other causes, which are potentially reversible, include alcoholism, cocaine abuse, coronavirus disease 2019 (COVID-19),
and thyroid disease.
In aected gravidas, the rate o major adverse cardiovascular
events in pregnancy ranges rom 25 to 40 percent (American College o Obstetricians and Gynecologists, 2019; Grewal, 2009).
Heart ailure and arrhythmias are the most common, and women
with preexisting moderate or severe let ventricular dysunction or
NYHA unctional class III or IV are at greatest risk. Dilated cardiomyopathy is managed with therapy or standard heart ailure
and or the specic underlying etiology (Bozkurt, 2016).
■ Peripartum Cardiomyopathy
Tis disorder is similar to other orms o nonischemic dilated
cardiomyopathy except or its unique relationship with pregnancy. Peripartum cardiomyopathy shares a genetic predisposition with both amilial and sporadic idiopathic dilated
cardiomyopathy (Cunningham, 2019; Ware, 2016). It is a
diagnosis o exclusion ollowing a concurrent evaluation or
peripartum heart ailure.
Although the term peripartum cardiomyopathy has been
used widely, until recently, little evidence supported a unique
pregnancy-induced cardiomyopathy. Pearson (2000) reported
ndings o a workshop o the National Heart, Lung, and Blood
Institute that established the ollowing diagnostic criteria:
Development o cardiac ailure in the last month o pregnancy or within 5 months ater delivery (Fig. 52-7),
• Absence of an identiable cause for the cardiac failure,
• Absence of recognizable heart disease prior to the last month
o pregnancy, andCardiovascular Disorders 933
CHAPTER 52
• Left ventricular systolic dysfunction demonstrated by classic
echocardiographic criteria. Tese include depressed ejection
raction or ractional shortening along with a dilated let
ventricle.
Te incidence o peripartum cardiomyopathy varies considerably and depends on the population studied and the diligence
o the search or a cause. Te average requency in the United
States is 1 per 1000 to 4000 births (Cruz, 2018; Cunningham,
2019).
Te etiology o peripartum cardiomyopathy remains
unknown, and proposed causes include viral myocarditis,
abnormal immune response to pregnancy, aberrant response to
the greater hemodynamic burden o pregnancy, hormonal interactions, malnutrition, inammation, and apoptosis. Te current
theory regarding pathophysiology is that o a “two-hit hypothesis” (Cruz, 2018; Ricke-Hoch, 2020). In this regard, peripartum cardiomyopathy aects genetically susceptible women who
have one o several cardiac gene mutations to include TTNC1,
TTN, and STAT3. Pregnancy at term is urther characterized by
prodigious secretion o prolactin by the maternal pituitary. At
the same time, the placenta secretes high levels o the antiangiogenic molecule soluble ms-like tyrosine kinase (sFlt-1). Although
a number o putative triggering events have been hypothesized,
a 16-kDa prolactin ragment—vasoinhibin—acts to cause myocardial damage with clinically apparent ventricular dysunction.
Tis is made worse by high levels o sFlt-1, which is superabundant in women with preeclampsia, multietal pregnancy,
or both. Bromocriptine therapy has been evaluated because it
inhibits prolactin secretion, and preliminary studies support its
use (Haghikia, 2019). For the same reason, it is also reasonable
to proscribe breasteeding.
Hypertensive disorders requently coexist with peripartum
cardiomyopathy, and another proposed mechanism links peripartum cardiomyopathy to preeclampsia (Cunningham, 2019).
Antiangiogenic actors are known to be associated with preeclampsia and can induce peripartum cardiomyopathy in susceptible mice. Tus, cardiomyopathy may be precipitated by
antiangiogenic actors in a genetically predisposed host because
o insufcient proangiogenic actors.
Ntusi and coworkers (2015) analyzed the clinical eatures
o women with peripartum cardiomyopathy compared with
those with hypertensive heart ailure. All women with peripartum cardiomyopathy became symptomatic in the postpartum
period, whereas 85 percent o women with hypertensive heart
ailure developed symptoms antepartum. Peripartum cardiomyopathy was signicantly linked with twin gestation, smoking,
and echocardiographic abnormalities. In contrast, hypertensive
heart ailure patients more oten had a amily history o hypertension, hypertension and preeclampsia in a prior pregnancy,
and let ventricular hypertrophy.
Management
Management o peripartum cardiomyopathy is the same as that
o heart ailure, and described earlier (p. 922). Terapy is aimed
at volume overload, aterload reduction, rhythm control, and
inotropic support (Davis, 2020). In addition, β-blocker therapy is used to decrease mortality rates. Anticoagulation with
LMWH is considered when the ejection raction reaches 30
to 35 percent because o the increased risk or let ventricular
thrombi (Bauersachs, 2016; Bozkurt, 2016).
In one pilot study, bromocriptine resulted in higher rates o
let ventricular recovery at 6 months and lower mortality rates
(Sliwa, 2010). A larger study o 115 women showed no di-
erence in ull restoration o let ventricular unction between
those treated and untreated with bromocriptine (Haghikia,
2013). Another investigation o 63 women showed no dierence in let ventricular unction improvement with short-term
versus long-term bromocriptine therapy (Hilker-Kleiner,
2017). Te use o bromocriptine as adjuvant therapy is considered experimental at this time.
Prognosis
Approximately hal o women suering rom peripartum cardiomyopathy recover baseline ventricular unction within
6 months o delivery. Tis rate is lower in obese women (Davis,
2018). In a group o 100 women with newly diagnosed peripartum cardiomyopathy, 72 percent had a let ventricular ejection
raction ≥50 percent at 1 year postpartum (McNamara, 2015).
Recovery to this level occurred in almost 90 percent o women
whose baseline ejection raction was at least 30 percent. Tis
is compared with <40 percent in women whose baseline ventricular ejection raction was <30 percent. Recovery was also
related to the baseline let ventricular end-diastolic diameter.
Event-ree survival at 1 year occurred in 93 percent. Six women
experienced nine major events that included our deaths, our
let ventricular assist device implantations, and one heart transplantation. Li and colleagues (2016) also ound that a baseline
let ventricular ejection raction <34 percent and a BNP level
>1860 pg/mL were associated with an approximately three-
old greater risk o persistent let ventricular systolic dysunction. Te mortality rate approaches 5 to 10 percent at 1 year
in women with persistent cardiac ailure (American College o
Obstetricians and Gynecologists, 2019).
Long-term ollow-up was reported in 28 women with a
median surveillance o 91 months (Ersbøll, 2018). Although
FIGURE 52-7 Peripartum cardiomyopathy with mild pulmonary edema. Chest radiograph of a woman with an abnormally
enlarged heart and mild perihilar opacification consistent with
dilated cardiomyopathy.934 Medical and Surgical Complications
Section 12
most women were asymptomatic, compared with controls, they
had lower ejection ractions, less maximal exercise capacity, and
subtle diastolic dysunction.
Subsequent Pregnancy
From the largest studies on the topic, approximately a third
o women with a history o peripartum cardiomyopathy will
suer relapse with worsening o symptoms and deterioration o
let ventricular unction during another pregnancy (Elkayam,
2014a). Te risk o relapse in women with persistent let ventricular dysunction is substantially higher than in those who
have recovered normal ventricular unction beore a subsequent
pregnancy (Hilker-Kleiner, 2017). However, normalization o
let ventricular unction does not guarantee an uncomplicated
pregnancy, because approximately 20 percent o these women
are at risk or deterioration in that unction (Codsi, 2018).
■ Other Cardiomyopathy Types
Arrhythmogenic right ventricular dysplasia is a unique cardiomyopathy dened histologically by progressive replacement
o right ventricular myocardium with adipose and brous tissue. It has an estimated population prevalence o 1 in 5000,
predisposes to ventricular tachyarrhythmias, and is a cause o
sudden death, particularly in younger individuals (Agir, 2014).
Studies o pregnancies in aected women reported symptoms,
including heart ailure, in 18 to 33 percent o pregnancies
(Schauelberger, 2019). Based on their systematic review, Krul
and coworkers (2011) counsel against pregnancy.
Restrictive cardiomyopathy is probably the least common
type. Tis inherited cardiomyopathy is characterized by a ventricular lling pattern in which worsening myocardial stiness
raises ventricular pressure precipitously and allows only a small
lling volume (Elliott, 2008). Pregnancy is not advised because
o the severe clinical course and poor prognosis in general.
Takotsubo cardiomyopathy is a rare orm o acute reversible
let ventricular apical wall ballooning (Krat, 2017). It is considered to be “stress induced” and appears to be more common
with preeclampsia and cesarean delivery. Myocardial inarction
must be excluded (Oindi, 2019).
INFECTIVE ENDOCARDITIS
Persons at greatest risk or endocarditis are those with congenital heart lesions, intravenous drug use, degenerative valve
disease, and intracardiac devices (Karchmer, 2018). Subacute
bacterial endocarditis usually stems rom a low-virulence bacterial inection superimposed on an underlying structural lesion.
Tese are usually native valve inections. Organisms that cause
indolent endocarditis are most oten viridans-group streptococci or Staphylococcus or Enterococcus species. Among intravenous drug abusers and those with catheter-related inections,
Staphylococcus aureus predominates. With prosthetic valve
inections, Staphylococcus epidermidis is a requent cause. Streptococcus pneumoniae and Neisseria gonorrhoeae may occasionally
cause acute, ulminating disease. Others have reported other
Neisseria species, group B streptococcus, and Escherichia coli
endocarditis during pregnancy or peripartum.
■ Diagnosis and Management
Inective endocarditis symptoms vary and oten develop insidiously. Fever, oten with chills, is seen in 80 to 90 percent o
cases; a murmur is heard in up to 85 percent; and anorexia,
atigue, and other constitutional symptoms are common
(Karchmer, 2018). Clinical clues are anemia, proteinuria, and
maniestations o embolic lesions that include petechiae, ocal
neurological changes, chest or abdominal pain, and extremity
ischemia. In some cases, heart ailure develops. Symptoms may
persist or several weeks beore the diagnosis is ound, and a
high index o suspicion is necessary.
Diagnosis is made using the Duke-Li classication, which
combines microbiology and imaging criteria with ve minor
criteria (Iung, 2019). Echocardiography may be diagnostic, but
lesions <2 mm or those on the tricuspid valve may be missed.
I uncertain, transesophageal echocardiography is accurate and
inormative. Importantly, a negative echocardiographic study
does not exclude endocarditis.
reatment is primarily medical, and ascertainment o the
inecting organism and its sensitivities is imperative or antimicrobial selection. Guidelines or appropriate antibiotic
treatment are published by proessional societies and updated
regularly (Habib, 2015; Karchmer, 2018). Recalcitrant bacteremia and heart ailure due to valvular dysunction are a
ew reasons or which persistent inection may require valve
replacement (Iung, 2019).
■ Pregnancy
Inective endocarditis is uncommon during pregnancy and
the puerperium. In an earlier period, the incidence o endocarditis at Parkland Hospital approximated 1 in 16,000 births
(Cox, 1988). With the current opioid use disorder epidemic,
endocarditis may become more requent (Prasad, 2019).
Associated maternal and etal mortality rates are rom 25 to
35 percent (Dagher, 2021; Habib, 2015). In one review, risk
actors were intravenous drug use (14 percent), congenital heart
disease (12 percent), and rheumatic heart disease (12 percent).
Te most common pathogens were streptococcal (43 percent)
and staphylococcal (26 percent) species. Among 51 pregnancies, the maternal mortality rate was 11 percent (Kebed, 2014).
■ Endocarditis Prophylaxis
For years, patients with any heart valve problem were given periprocedural antibiotics or endocarditis prophylaxis. Currently,
however, recommendations are more stringent. Te American
Heart Association recommends prophylaxis or dental procedures in those with: (1) a prosthetic valve or prosthetic material
used in a valve repair, (2) prior endocarditis, (3) unrepaired
cyanotic heart deect or repaired lesion with residual deect at
prosthetic sites, and (4) valvulopathy ater heart transplantation (Nishimura, 2017). In the absence o pelvic inection, the
American College o Obstetricians and Gynecologists (2018b)
does not recommend endocarditis prophylaxis or either vaginal or cesarean delivery, except with the lesions cited above.
Pregnant women at highest risk or endocarditis are
those with cyanotic cardiac disease, prosthetic valves, or both.Cardiovascular Disorders 935
CHAPTER 52
Table 52-9 shows prophylactic regimens or women not already
receiving intrapartum antimicrobial therapy or another indication that would also provide coverage against endocarditis.
Tese are administered as close to 30 to 60 minutes beore the
anticipated delivery time as is easible.
ARRHYTHMIAS
Both preexisting and new-onset cardiac arrhythmias are oten
encountered during pregnancy, labor, delivery, and the puerperium. Tey are usually benign and their incidence in pregnancy
appears to be increasing (MacIntyre, 2018). Te mechanism(s)
responsible or the higher incidence are not clear. From some
studies, estradiol and progesterone are proarrhythmic. Estrogen augments the number o adrenergic receptors in the myocardium, and adrenergic responsiveness seems to be greater
in pregnancy (Enriquez, 2014). Perhaps the normal but mild
hypokalemia o pregnancy and/or the physiological rise in heart
rate serves to induce arrhythmias. Alternatively, detection o
arrhythmias may be greater because o the requent visits in
routine prenatal care.
■ Bradyarrhythmias
Slow heart rhythms, including complete heart block, are compatible with a successul pregnancy outcome (Keepanasseril,
2015). Some women with complete heart block have syncope
during labor and delivery, and occasionally temporary cardiac
pacing is necessary. In our experiences and rom others, women
with permanent articial pacemakers usually tolerate pregnancy
well (Hidaka, 2011). With xed-rate devices, cardiac output
apparently is increased by augmented stroke volume.
Patients with pacemakers or other electrical implants require
special precautions during surgery. Stray current may be interpreted as an intracardiac signal by the implanted device and
lead to pacing changes. In addition, myocardial burns may
result rom conduction o electrosurgical current to the pacing
electrode rather than to the grounding pad. With these devices,
preventive steps include cardiology consultation; bipolar electrosurgery or Harmonic scalpel use rather than monopolar
current; i needed, minimal monopolar settings; continuous
cardiac and pulse oximetry monitoring; contingency plans
or arrhythmias; and close proximity o active (electrosurgical
pencil) and return (electrosurgical grounding pad) electrodes
(Crossley, 2011).
■ Supraventricular Tachycardias
Te most common arrhythmia seen in reproductive-aged women
is paroxysmal supraventricular tachycardia (SVT). Te prevalence
during pregnancy is 24 cases per 100,000 hospital admissions,
and approximately 20 percent will experience symptomatic exacerbations during pregnancy (Enriquez, 2014). Interestingly, the
mean heart rate o pregnant women with paroxysmal SV is 184
bpm compared with 166 bpm in nonpregnant aected women
(Yu, 2015). In one study, approximately hal o women with
paroxysmal SV had an initial onset during pregnancy (Bánhidy, 2015). Notably, maternal paroxysmal SV was associated
with a twoold higher risk o septal cardiac deects, particularly
secundum atrial septal deects, in their ospring.
For acute SV treatment, vagal maneuvers, which include
Valsalva maneuver, carotid sinus massage, bearing down, and
immersion o the ace in ice water, raise vagal tone and block
the atrioventricular node. Intravenous adenosine is a shortacting endogenous nucleotide that also blocks AV nodal conduction. Our experiences are similar to those o others in that
adenosine is sae and eective or cardioversion in hemodynamically stable gravidas (Page, 2015). ransient etal bradycardia
has been described with adenosine (Dunn, 2000).
I pharmacological therapy is ineective or contraindicated,
the American College o Cardiology and the American Heart
Association recommend synchronized cardioversion in pregnant
women with hemodynamically unstable SV (Page, 2015).
Although electrical cardioversion with standard energy settings is
not contraindicated in pregnancy, vigilance is important. Barnes
and colleagues (2002) described a case in which direct current
cardioversion led to a sustained uterine contraction and etal bradycardia. As an aside, pregnancy has no eect on the operation
o implantable cardioverter-debrillator devices (Boulé, 2014).
I cardioversion ails or is unsae because o concurrent thrombus, then long-term anticoagulation and heart rate control with
medication are necessary (DiCarlo-Meacham, 2011). Other treatment options recommended by the American College o Cardiology and the American Heart Association (Page, 2015) include:
• Intravenous metoprolol or propranolol when adenosine is
ineective or contraindicated,
TABLE 52-9. Single-Dose Antibiotic Prophylaxis for Infective Endocarditis in High-Risk Patients
American College of Obstetricians and Gynecologists (2018b)
Standard (IV): ampicillin 2 g or cefazolin or ceftriaxone 1 g
Penicillin-allergic (IV): cefazolin or ceftriaxone 1 g or clindamycin 600 mg
Oral: amoxicillin 2 g
American Heart Association/European Society of Cardiology (Karchmer, 2018)
Standard: amoxicillin 2 g PO or ampicillin 2 g IV or IM
Penicillin-allergic: clarithromycin or azithromycin 500 mg PO; cephalexin 2 g PO; clindamycin
600 mg PO, IV, or IM; or cefazolin or ceftriaxone 1 g IV or IM
IM = intramuscularly; IV = intravenously; PO = per os (orally).
Cefazolin or ceftriaxone given 30 minutes, and all others given 1 hour prior to procedure.936 Medical and Surgical Complications
Section 12
• Intravenous verapamil when adenosine and β-blocking
agents are ineective or contraindicated,
• Intravenous procainamide,
• Intravenous amiodarone for potentially life-threatening SVT
and when other therapies are ineective or contraindicated.
Atrial brillation and atrial utter rarely present or the rst
time during pregnancy. A new-onset atrial brillation should
prompt a search or underlying etiologies that include cardiac
anomalies, hyperthyroidism, pulmonary embolism, drug toxicity, and electrolyte disturbances (MacIntyre, 2018). Major
complications include embolic stroke. When associated with
mitral stenosis, pulmonary edema may develop in later pregnancy i the ventricular rate is increased. Unstable patients are
treated with cardioversion and rate control.
Pregnancy may predispose otherwise asymptomatic women
with Wolf-Parkinson-White (WPW) syndrome to exhibit arrhythmias. In a study o women with asymptomatic or mildly symptomatic disease, hal developed SV or the rst time, and the
other hal experienced an increase in their attack rate (Kounis,
1995). In some patients, accessory pathway ablation may be indicated. Patients with Ebstein anomaly are prone to have WPW
syndrome. Driver and associates (2015) have provided a review.
■ Ventricular Tachycardia
Tis orm o arrhythmia is uncommon but potentially atal in
healthy young women without underlying heart disease. Brodsky and coworkers (1992) described seven pregnant women
with new-onset ventricular tachycardia and reviewed 23
reports. Most o these women were not ound to have structural heart disease. In 14 cases, tachycardia was precipitated by
physical exercise or psychological stress. Abnormalities ound
included two cases o myocardial inarction, two o prolonged
Q interval, and one o anesthesia-provoked tachycardia. Tey
concluded that pregnancy events precipitated the tachycardia
and recommended β-blocking agents or control. As previously discussed (p. 934), arrhythmogenic right ventricular dysplasia will result occasionally in ventricular tachyarrhythmias.
I unstable, emergency cardioversion is indicated, and standard
adult energy settings are adequate (Lin, 2015).
■ Prolonged QTInterval
Te long QT syndrome is the most requent inherited channelopathy (MacIntyre, 2018). Tis conduction anomaly may predispose individuals to a potentially atal ventricular arrhythmia
known as torsades de pointes. wo studies comprised o 502
pregnant women with long Q syndrome both reported a
signicant rise in cardiac events postpartum but not during
pregnancy (Rashba, 1998; Seth, 2007). Te normal elevation
in heart rate during pregnancy may be partially protective.
Paradoxically, β-blocking agents—preerably propranolol—
lower the risk o torsades de pointes in patients with long Q
syndrome and should be continued throughout pregnancy
and the puerperium (Ishibashi, 2017). Importantly, many
medications, including some used during pregnancy, such
as the erythromycins and many antiemetics, may predispose
to Q prolongation. For opioid-addicted women, the use o
methadone to treat withdrawal symptoms may be problematic
(Bogen, 2017). Last, Cuneo and colleagues (2020) observed an
eightold risk or stillbirth in women with long Q syndrome
(Chap. 35, p. 624).
DISEASES OF THE AORTA
■ Aortic Dissection
Maran syndrome and coarctation are two aortic diseases that
place the pregnant woman at greater risk or aortic dissection (Russo, 2017). Indeed, hal o dissection cases in young
women are related to pregnancy (O’Gara, 2004). Other risk
actors are bicuspid aortic valve and urner, Noonan, LoeysDietz, or Ehlers-Danlos syndrome (Cauldwell, 2019b; Russo,
2018). Pregnancy-related cardiac guidance or women with
urner syndrome is outlined in Chapter 3 (p. 37). Although
the mechanism(s) involved are unclear, the initiating event is a
tear in the intimal layer o the aorta, ollowed by hemorrhage
into the media, and nally rupture.
In most cases, aortic dissection presents with severe chest
pain described as ripping, tearing, or stabbing. Diminution or
loss o peripheral pulses coupled with a recently acquired aortic insufciency murmur is an important physical nding. Te
dierential diagnosis o aortic dissection in pregnancy includes
myocardial inarction, pulmonary embolism, pneumothorax,
aortic valve rupture, and obstetrical catastrophes such as placental abruption and uterine rupture.
More than 90 percent o patients with aortic dissection have
an abnormal chest radiograph. Aortic angiography is the most
denitive method or diagnosis conrmation. However, sonography, computed tomography, and MR imaging are used more
requently depending on the clinical urgency.
Initial medical treatment is given to lower blood pressure.
Proximal dissections most oten need to be resected, and the
aortic valve replaced i necessary. Distal dissections are more
complex, and many may be treated medically. Among nonpregnant patients with abdominal aortic aneurysms <5.5 cm, survival is not improved by immediate elective repair compared
with surveillance and delayed repair. Karthikesalingam and
associates (2016) suggest that the size threshold or aneurysm
repair should be revisited.
■ Marfan Syndrome
Tis autosomal dominant connective tissue disorder has an
incidence o 1 per 3000 to 5000 individuals and is without
racial or ethnic predilection (Azizad-Pinto, 2017). As discussed
in Chapter 62 (p. 1121), Maran syndrome is caused by any
o more than 1000 mutations in the brillin (FBN1) gene. It
is characterized by generalized tissue weakness that can result
in dangerous cardiovascular complications. All tissues are
involved, and other requent deects include joint laxity and
scoliosis. Progressive aortic dilation causes aortic valve insufciency, and inective endocarditis or mitral valve prolapse with
insufciency may be comorbid. Aortic dilation and dissecting
aneurysm are the most serious abnormalities. Early death is due
to either a dissecting aneurysm or to valvular insufciency and
heart ailure.Cardiovascular Disorders 937
CHAPTER 52
During pregnancy, the primary concern with Maran syndrome is aortic dissection (Curry, 2014; Russo, 2017). A
study using the Nationwide Inpatient Sample rom 2003 to
2010 described 339 deliveries in women with Maran syndrome. Tere was one maternal death and six (1.8 percent)
aortic dissections (Hassan, 2015). From the United Kingdom,
Cauldwell and colleagues (2019a) described 258 pregnancies
in 151 aected women. Although no women died, 1.9 percent
had an aortic dissection.
Te aortic root usually measures approximately 2 cm, and
during normal pregnancy, it expands slightly. With Maran
syndrome, aortic root repair is recommended at diameters
o 4.0 to 4.5 cm (Azizad-Pinto, 2017). Te guidelines o the
American College o Cardiology, the American Heart Association, and the American Association o Toracic Surgeons advise
prophylactic aortic repair in women considering pregnancy i
the diameter o the ascending aorta exceeds 4 cm (Hiratzka,
2010). Te guidelines o the European Society o Cardiology advise repair o the aorta at diameters >4.5 cm (RegitzZagrosek, 2018). Surgical repair is also considered using a
ormula indexed to height because shorter patients have dissection at a smaller diameter (Bradley, 2014).
For pregnant women with known thoracic aortic root or
ascending aortic dilation, monthly or bimonthly echocardiographic measurements o the ascending aortic dimensions are
recommended to detect expansion (American College o Obstetricians and Gynecologists, 2019). Prophylactic β-blocking
agents have become standard or gravidas with Maran syndrome because they reduce hemodynamic stress on the ascending aorta and slow the dilation rate. Ideally, pregnant women
with aortic aneurysms are delivered at acilities in which cardiothoracic surgery is available. Vaginal delivery with regional
analgesia and an assisted second stage seem sae or women with
an aortic root diameter <4 cm.
Cesarean delivery may be considered or values between 4.0
and 4.5 cm. When the aortic root measures >4.5 cm, elective
cesarean delivery is recommended, and direct replacement o
the proximal aorta with a prosthetic grat can be considered
(Regitz-Zagrosek, 2018). Successul aortic root replacement
during pregnancy has been described, but the surgery has also
been associated with etal hypoxic-ischemic encephalopathy
(Seeburger, 2007). Several case reports describe emergency
cesarean deliveries in women with acute type A dissections that
were repaired successully at the time o delivery (Guo, 2011;
Haas, 2011; Papatsonis, 2009).
o evaluate obstetrical outcomes, investigators or one study
o 63 women with Maran syndrome analyzed their 142 pregnancies. O 111 pregnancies progressing past 20 weeks’ gestation, 15 percent delivered preterm, and 5 percent had preterm
prematurely ruptured membranes (Meijboom, 2006). Tere
were eight perinatal deaths, and hal o the neonatal survivors
were subsequently diagnosed with Maran syndrome.
■ Aortic Coarctation
In this relatively rare lesion, the aorta is abnormally narrowed
and is oten accompanied by abnormalities o other large arteries. A ourth o aected patients have a bicuspid aortic valve,
and another 10 percent have cerebral artery aneurysms. Other
associated lesions are persistent ductus arteriosus, septal deects,
and urner syndrome. Te collateral circulation arising above
the coarctation remodels and expands, oten strikingly, to cause
localized erosion o rib margins by hypertrophied intercostal
arteries. ypical ndings include hypertension in the upper
extremities but normal or decreased pressures in the lower
extremities. Some have described diagnosis during pregnancy
using MR imaging (Sherer, 2002; Zwiers, 2006). Jimenez-Juan
and associates (2014) ound that aortic diameter measured by
MR imaging and the risk o adverse events during pregnancy
were inversely correlated. O note, no adverse outcomes occurred
i the minimum diameter at the coarctation exceeded 15 mm.
Major complications with aortic coarctation include congestive heart ailure ater long-standing severe hypertension, bacterial endocarditis o an associated bicuspid aortic valve, and
aortic rupture. Antihypertensive therapy using β-blocking drugs
is usually required because hypertension may worsen in pregnancy. Aortic rupture is more likely late in pregnancy or early
puerperium. Cerebral hemorrhage rom circle o Willis aneurysms
also may occur. According to the World Health Organization,
severe coarctation should preclude pregnancy (Foeller, 2018).
In one study o outcomes rom 188 pregnancies, a third
o women had hypertension that was related to signicant
coarctation gradients, and one woman died rom dissection
at 36 weeks’ gestation (Beauchesne, 2001). In 700 deliveries
complicated with coarctation, hypertensive complications o
pregnancy were increased three- to ourold (Krieger, 2011).
Importantly, almost 5 percent o women with coarctation had
an adverse cardiovascular outcome—maternal death, heart
ailure, arrhythmia, cerebrovascular or other embolic event—
compared with only 0.3 percent o controls. O women with
coarctation, 41 percent underwent cesarean delivery compared
with 26 percent o controls.
Congestive heart ailure demands vigorous eorts to improve
cardiac unction and may warrant pregnancy interruption. In
this setting, some authors recommend that resection o the
coarctation be undertaken to protect against the possibility o
a dissecting aneurysm and aortic rupture. Tis poses signicant
perusion risk, especially or the etus, because all the arterial
collaterals must be clamped or variable periods.
ISCHEMIC HEART DISEASE
Pregnant women with coronary artery disease commonly have
the classic risk actors o amily history, diabetes, smoking,
hypertension, hyperlipidemia, and obesity (ripathi, 2019).
Te rate o ischemic heart disease is estimated to be 10 cases per
100,000 pregnancy and postpartum hospitalizations (Smilowitz, 2018; ripathi, 2019). Although relatively rare, the risk o
acute myocardial inarction is approximately threeold higher
in pregnant women compared with nonpregnant women o
similar age (Elkayam, 2014b).
■ Acute Coronary Syndrome
Myocardial inarction (MI) is the end result o an acute coronary
syndrome. It can result rom coronary artery atherosclerosis,938 Medical and Surgical Complications
Section 12
dissection, embolism, spasm, or arteritis (American College o
Obstetricians and Gynecologists, 2019; Cauldwell, 2020). Te
mortality rate with MI in pregnancy is higher compared with
age-matched nonpregnant women. In a Nationwide Inpatient
Sample study totaling 859 pregnancies complicated by acute
MI, the death rate was 5.1 percent (James, 2006). Women who
sustain an inarction <2 weeks beore delivery are at especially
high risk o death due to the greater myocardial demand o
labor and delivery (Esplin, 1999).
In a systematic review o 150 cases, most women developed an acute MI during the third trimester or postpartum
(Elkayam, 2014b). Approximately three ourths presented with
S segment-elevation MI (SEMI). Te leading mechanisms
o acute inarction included spontaneous coronary dissection (43
percent) and atherosclerotic disease (27 percent). Signicant
complications included heart ailure (38 percent), recurrent
angina or inarction (19 percent), and ventricular arrhythmias
(12 percent). Te maternal and etal mortality rates were 7 and
5 percent, respectively.
Diagnosis o acute coronary syndrome during pregnancy
does not dier rom that in nonpregnant patients and is based
on clinical presentation, characteristic ECG changes, and evidence o myocardial necrosis reected by elevated serum highsensitivity troponin I levels (Pacheco, 2014). Consider that
troponin I levels are greater, however, in preeclamptic and
hypertensive women compared with normotensive gravidas
(Ravichandran, 2019).
With spontaneous coronary artery dissection, establishing
the diagnosis requires an increased index o suspicion in the
women presenting with chest pain (Codsi, 2016). For this
condition, coronary angiography is considered the diagnostic
gold standard and should be expeditiously perormed i acute
coronary syndrome—either MI or unstable angina—is present
(Hayes, 2018).
reatment o acute MI is similar to that or nonpregnant
patients (Pacheco, 2014). Several reports describe successul
percutaneous transluminal coronary angioplasty and stent placement during pregnancy (American College o Obstetricians and
Gynecologists, 2019). Cardiopulmonary resuscitation may be
required, as described in Chapter 50 (p. 897). I the inarct has
healed sufciently, cesarean delivery is reserved or obstetrical
indications, and epidural analgesia is ideal or labor.
■ Pregnancy with Prior Ischemic Heart Disease
Ischemic heart disease is characteristically progressive, and
because it is usually associated with hypertension or diabetes, pregnancy in most o these women seems inadvisable. In
an earlier review o 30 pregnancies in women who had sustained an inarction remote rom pregnancy, none o the women
died, our had congestive heart ailure, and our had worsening angina during pregnancy (Vinatier, 1994). Pombar and
coworkers (1995) evaluated outcomes o women with diabetes-associated ischemic heart disease and inarction. Tree had
undergone coronary artery bypass grating beore pregnancy.
O 17 women, eight died during pregnancy. Certainly, pregnancy raises cardiac workload, and these investigators concluded that ventricular perormance should be assessed using
ventriculography, radionuclide studies, echocardiography, or
coronary angiography beore conception. Without signicant
ventricular dysunction, pregnancy will likely be tolerated. For
the woman who becomes pregnant beore these studies are per-
ormed, echocardiography is done. Exercise tolerance testing
may be indicated, and radionuclide ventriculography exposes
the etus to minimal radiation (Chap. 49, p. 875).
COMMON OBSTETRICAL MEDICATIONS
Common medications used in pregnancy require special consideration in women with cardiac conditions. erbutaline
causes vasodilation and tachycardia, which can be dangerous in
patients with mitral and aortic stenosis. Niedipine or hypertension control or tocolysis results in hypotension that can
have negative consequences or patients with aortic stenosis,
pulmonary hypertension, and Eisenmenger syndrome. Postpartum hemorrhage should be managed aggressively, and methylergonovine generally is a sae choice. Prostaglandin F2α may
cause pulmonary shunting and bronchospasm, which results in
elevation o pulmonary artery pressure. Consideration is given
to the vasodilatory eects o hydralazine in conditions such as
hypertrophic cardiomyopathy. Hydralazine also causes tachycardia. Te β-blocking agent labetalol is a sae choice unless
there is ventricular ailure. In general, oxytocin and magnesium
sulate have minimal cardiac eects
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