REVIEW ARTICLE

Mitral valve disease in pregnancy: outcomes

and management

Sarah Tsiaras MD* and Athena Poppas MD FACC†

*Teaching Fellow in Cardiovascular Diseases, Division of Cardiology, Warren Alpert Medical School at Brown University; †Associate Professor of
Medicine, Division of Cardiology, Warren Alpert Medical School at Brown University, Director, Echocardiography Laboratory, Rhode Island
Hospital, 593 Eddy Street, Providence RI

Summary: Young women may have asymptomatic mitral valve disease which becomes unmasked during the haemodynamic
stress of pregnancy. Rheumatic mitral stenosis is the most common cardiac disease found in women during pregnancy. The typical
increased volume and heart rate of pregnancy are not well tolerated in patients with more than mild stenosis. Maternal complications
of atrial fibrillation and congestive heart failure can occur, and are increased in patients with poor functional class and severe
pulmonary artery hypertension. Patients can be diagnosed by echocardiography and symptoms treated with beta-1 antagonists and
cautious diuresis. Patients with heart failure unresponsive to treatment can undergo percutaneous balloon mitral valvuloplasty.
Labour and delivery goals include reducing tachycardia by adequate pain control and minimized volume shifts. Mitral valve
regurgitation, even when severe, is usually very well tolerated in pregnancy as the increase in volume is offset by a decrease in
vascular resistance. On the other hand, patients with left ventricular dysfunction, moderate pulmonary hypertension or NYHA
functional class III-IV are at increased risk for heart failure and arrhythmias. They may need cautious diuresis and limitations on
physical activity during pregnancy, as well as invasive haemodynamic monitoring for labour and delivery. Vaginal delivery is
preferred and caesarean section reserved for obstetric indications.

Keywords: pregnancy, mitral valve stenosis, mitral regurgitation, heart disease, labour and delivery

INTRODUCTION patients with mitral stenosis due to restricted left ventricular

inflow and resultant increases in left atrial and pulmonary
The presence of valvular disease in pregnant women increases
pressures. Conversely, in patients with mitral regurgitation,
the risk of adverse maternal and fetal outcomes and poses a
the increase in intravascular volume ( preload) is offset by the
challenge to physicians. Mitral valve disease, specifically
decrease in systemic vascular resistance (afterload) and hence
mitral stenosis, is the most common valvular disease seen in
is generally well tolerated. The care of pregnant women with
pregnancy. The overall prevalence of rheumatic mitral stenosis
mitral valve disease requires an experienced, multidisciplinary
in developed countries is estimated at 1–2%,1 but in developing
team comprising obstetricians, cardiologists and anaesthesiolo-
countries it is much more prevalent and accounts for 40 –50%
gists. Ideally, care should begin with preconception counsel-
of the cardiac disease seen in pregnancy. In areas with large
ling, assessment and optimization of haemodynamic status,
immigrant populations, internists and obstetricians will likely
and should continue through gestation, labour and delivery.
continue to see pregnant women with rheumatic mitral
This article will address maternal and fetal outcomes in
stenosis.
women with mitral valve disease and the optimal management
The normal cardiovascular changes that occur during preg-
of these patients.
nancy are well described and include increased intravascular
volume, resting heart rate and cardiac output. The changes in
volume begin early in the first trimester and plateau by the
third trimester. Cardiac output increases by 30–60%; these MITRAL STENOSIS
changes are determined by a 30–40% increase in preload
early in pregnancy and maintained by a 10 –20% increase in Maternal outcomes
heart rate later in pregnancy.2 There is also decreased systemic The adverse maternal events in women with mitral stenosis
vascular resistance with a mild decrease in systolic blood include pulmonary oedema, arrhythmia and thromboembo-
pressure. Predictably, these changes are poorly tolerated in lism; the complication rate is related to the severity of mitral
stenosis and the patient’s functional class. A 2001 case-control
study that looked at the effect of valvular heart disease on
Correspondence to: Athena Poppas
maternal and fetal outcome in 46 pregnancies in 44 patients
Email: [email protected]
with mitral stenosis found a high incidence of maternal

Obstetric Medicine 2009; 2: 6 –10. DOI: 10.1258/om.2008.080002

 Tsiaras and Poppas. Mitral valve disease in pregnancy 7
................................................................................................................................................

morbidity but no mortality. Heart failure requiring hospitaliz- Fetal outcomes

ation occurred in 61% of women with moderate stenosis
Adverse fetal outcomes associated with mitral stenosis include
(mitral valve area [MVA] 1–1.5 cm2) and 78% of women with
preterm delivery, intrauterine growth retardation, low birth
severe stenosis. Of those with moderate or severe stenosis,
weight and fetal or neonatal death. The most likely explanation
deterioration in functional status was noted in 82% of class I
for these adverse events is uteroplacental insufficiency second-
and 61% of class II patients. Outcomes in patients with mild
ary to left heart obstruction. The large 2001 multicentre study
mitral stenosis were not statistically different from controls.3
by Siu et al. identified five predictors of neonatal events includ-
A Canadian cohort study that looked at 80 pregnancies in 74
ing: NYHA class .II; maternal left heart obstruction; smoking
women provides further evidence that severity of stenosis is an
during pregnancy; multiple gestations; and use of anticoagu-
important predictor of cardiac events. The overall adverse
lants during pregnancy. This places women with mitral stenosis
cardiac event rate was 35%, but varied based on the severity
and poor functional status among the most likely to experience
of the valvular disease; in patients with mild (MVA
adverse neonatal outcomes.5
.1.5 cm2), moderate (MVA 1.1–1.5 cm2) and severe (MVA
In a subsequent substudy, Siu et al. looked at 302 pregnancies in
1 cm2) stenosis, the adverse cardiac event rates were 26%,
women with heart disease compared with 572 normal pregnancies
38% and 67%, respectively. Twenty-five pregnancies (31%)
and found that neonatal events were more frequent in women with
were complicated by pulmonary oedema and nine pregnancies
all types of heart disease (18% versus 7% in controls), but specifi-
(11%) were complicated by arrhythmias (atrial fibrillation and
cally left heart obstruction, cyanosis and poor functional class.
SVT), but there were no incidences of stroke, need for invasive
Adverse outcomes were further increased in the presence of obste-
intervention, cardiac arrest or death. Independent predictors of
tric risk factors, specifically anticoagulant use, multiple gestations,
cardiac complications were moderate or severe stenosis (odds
maternal age and tobacco, emphasizing the importance of
ratio 3.4, 95% CI 1.2 –10) and a history of arrhythmia, pulmo-
smoking cessation and optimization of obstetric status prior to con-
nary oedema, transient ischemic attack (TIA) or stroke prior
ception. Lesion-specific risk was not addressed in this study.6
to pregnancy (odds ratio 6.8, 95% CI 1.8–25.9).4
There is limited data regarding the risk of adverse neonatal
A prospective multicentre study of 599 pregnancies in
outcome specifically in women with mitral stenosis. The case-
women with heart disease, including 48 with mitral stenosis,
control study by Hameed (46 pregnancies in 44 women with
identified four predictors of primary cardiac events that they
mitral stenosis) found that haemodynamically significant
defined as pulmonary oedema, sustained arrhythmia, stroke,
mitral stenosis increased the rate of IUGR and resulted in
cardiac arrest or death. The predictors were: (1) any prior
lower birth weights (Table 2).3 The rate of preterm birth and
cardiac event (heart failure, TIA, stroke or arrhythmia); (2) base-
stillbirth among women with mitral stenosis was higher
line NYHA class .II; (3) left heart obstruction defined by MVA
compared with controls, but was not statistically significant.
,2.0 cm2 or peak left ventricular outflow gradient .30 mmHg
by echocardiography; and (4) ejection fraction (EF) ,40%. They
found that the estimated risk of a primary cardiac event for
women with 0, 1 and  2 predictors was 5%, 27% and 75%
Management
respectively. They qualify this risk index with the statement Preconception
that in women with severe mitral stenosis, the model had a Patients with mild-moderate mitral stenosis may first become
lower discriminative accuracy. However, this risk index may symptomatic and be diagnosed during pregnancy when increased
be helpful when counselling women with mitral stenosis who plasma volume and resting heart rate stress a previously compen-
are considering pregnancy.5 A summary of maternal outcomes sated system. Ideally, however, women should be evaluated by a
in women with mitral stenosis is outlined in Table 1. multidisciplinary team of physicians prior to a planned

Table 1 Maternal outcomes

Pulmonary Need for cardiac Cardiac arrest
Degree of stenosis oedema Arrhythmias medications Hospitalizations Stroke or death
Mild (.1.5 cm2) 19.7% (12/61) 8.2% (5/61) 5.3% (1/19) 10.5% (2/19) 0% (0/42) 0% (0/61)
Moderate (1.0 –1.5 cm2) 44.7% (21/47) 14.9% (7/47) 72.2% (13/18) 61.1% (11/18) 0% (0/29) 0% (0/47)
Severe (,1.0 cm2) 66.7% (12/18) 33.3% (6/18) 100% (9/9) 77.8% (7/9) 0% (0/9) 0% (0/18)

Combined data from Silversides and Hameed

Table 2 Fetal outcome in patients with valvular heart disease versus controls
IUGR Birth weight (g)
Degree of stenosis PTS CTRL P PTS CTRL P
Mild n¼19 16% 0% 0.6 3.135+419 3.288+531 0.3
Moderate n¼18 27% 0% 0.1 2.706+1.039 3.427+426 0.02
Severe n¼9 33% 0% 0.5 2.558+947 3.332+403 0.05
All n¼46 24% 0% ,0.001 2.845+818 3.372+486 0.02

Adapted from Hameed et al. 3

IUGR ¼ intrauterine growth restriction; PTS ¼ patients; CTRL ¼ controls
8 Obstetric Medicine Volume 2 March 2009
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pregnancy. They should be informed of the maternal and fetal an upward dose adjustment to maintain therapeutic levels.15
morbidity and mortality associated with their condition. Patients with a dilated left atrium are at increased risk for devel-
Assessment should include a thorough cardiac, obstetric and oping both atrial fibrillation and thrombus. However, because
family history, physical exam, CBC, EKG and echocardiogram. the majority of fibrillation episodes will be symptomatic in
Ideally, an echocardiogram should be obtained within six to 12 this population, routine anticoagulation based on left atrial
months prior to pregnancy. During pregnancy, echocardiographic dimensions alone is not currently recommended.16
gradients across the mitral valve will be falsely elevated secondary Endocarditis prophylaxis at the time of labour and delivery is
to increased cardiac output, and thus will overestimate the sever- a controversial topic in pregnant women with rheumatic mitral
ity of mitral stenosis (the gradient increases by the square root of stenosis. The ACC/AHA guidelines do not recommend pro-
the CO). Hence, MVA should be based on independent echocar- phylactic antibiotics in women with valvular disease under-
diographic measurements and calculations.7 going uncomplicated vaginal delivery or caesarian section.
Women with moderate to severe mitral stenosis (MVA Antibiotics are optional in women who have a prior history
1.5 cm2 or mean gradient 5 mmHg) who are symptomatic of endocarditis.17 A study from Ireland in 1980 demonstrated
(NYHA functional class II) should be evaluated for percuta- that the rate of bacteraemia following spontaneous vaginal
neous balloon mitral valvuloplasty (PBMV) prior to con- delivery in the absence of a known infection was 2.15%. This
ception.3,4,8 This is a class I indication for all patients from the compares with a peripheral blood bacterial isolation rate after
2006 The American College of Cardiology/American Heart dental extraction of 60–90%.18 More recent studies, however,
Association (ACC/AHA) guidelines. Furthermore, women have demonstrated a higher rate of bacteremia following
with at least moderate mitral stenosis have an increased risk labour and delivery, ranging from 5–19%.12 Given this data,
of pulmonary oedema and arrhythmias, and an increased the use of antibiotics is probably prudent for women who
fetal morbidity and mortality. experience prolonged rupture of the membranes prior to deliv-
ery. The American Heart Association recommends ampicillin
plus gentamicin at the initiation of labour or within 30
Medical management minutes of caesarean section, with a second dose of ampicillin
Medical therapy should be targeted towards decreasing heart or amoxicillin six hours later. Vancomycin may be substituted
rate, which will increase left ventricular filling time and for ampicillin if allergies preclude its administration.
thereby decrease left atrial pressure. Many women with mode-
rate to severe stenosis will do well with a limitation of physical
Percutaneous balloon mitral valvuloplasty
activity, avoiding the increase in heart rate and blood pressure
Percutaneous balloon mitral valvuloplasty (PBMV) is the treat-
associated with exercise. However, in the case-control study by
ment of choice for isolated, non-calcified mitral stenosis and
Hameed et al., 50% of patients with mitral stenosis required
should ideally be performed preconception in symptomatic
cardiac medications during their pregnancy. These included
patients. The indications are congestive heart failure (CHF),
beta-blockers, calcium channel blockers, digoxin, heparin,
NYHA Class .II and MVA ,1.5–1.7 cm2 and contraindica-
hydralazine and aspirin.3 Although ACE inhibitors are effective
tions include left atrial thrombi, .2þ mitral regurgitation
afterload-reducing agents in the non-pregnant population, they
(MR), and subvalvular stenosis. Complications of the procedure
are contraindicated in pregnancy secondary to their teratogenic
are rare, the most common being mitral regurgitation, which
effects. Beta-blockers are the cornerstone of medical therapy in
occurs in 12% of patients.19 A randomized trial of 60 patients
patients with haemodynamically significant mitral stenosis.
with severe mitral stenosis found PBMV to be comparable to
The resultant decrease in heart rate allows left ventricular
open surgical commissurotomy both initially and at three
filling at lower left atrial and hence pulmonary pressures.
years follow-up.20
Beta-1 selective agents are recommended to minimize the
PBMV has been well established in several case series as a
beta-2-mediated effects of uterine relaxation. At least two
treatment option for pregnant women with class III and IV
studies have demonstrated an increase in IUGR in women
heart failure who are refractory to medical management. The
treated with atenolol during pregnancy, so metroprolol is the
immediate short-term success rate approaches 100% with few
drug of choice.9,10 In a study of 25 pregnant women with symp-
adverse maternal or fetal events. Esteves et al. recently reported
tomatic, moderate to severe mitral stenosis (MVA ¼ 1.1 cm2),
on a cohort of 71 women who underwent PBMV in Brazil. All
treatment with beta-blockers resulted in improvement in or
of the women were in class III or IV heart failure despite
stabilization of NYHA functional class to II in 92%.11
maximal medical therapy and had echocardiographic findings
Chronic diuretics should be used with caution to avoid hypo-
of severe mitral stenosis. PBMV resulted in significant decreases
perfusion of the placenta, but they have been used successfully
in mean left atrial (LA) pressure (25.3 + 8.1 to 12 +6.1 mm Hg,
in women with acute pulmonary oedema due to peripartum
P ,0.001), mean diastolic mitral gradient (18.0 + 7.0 to 3.9 +
cardiomyopathy and mitral stenosis without adverse risk to
3.1 mmHg, P , 0.01) and mean pulmonary artery pressure
the fetus.12 Sodium restriction and modest fluid restriction
(38 + 15 to 24 + 11 mmHg, P , 0.05). The mean MVA increased
should be recommended in symptomatic women. In women
from 0.9 to 2.0 cm2. Thromboembolic events occurred in two of
who develop atrial fibrillation, digoxin has been shown to
the 71 patients, but with no permanent vascular or neurological
improve rate control and has a long safety record in preg-
sequelae. At the conclusion of the pregnancy, 98% of women
nancy.13 Atrial fibrillation confers an increased risk of throm-
were in NYHA functional class I or II.21
boembolism; therefore, subcutaneous unfractionated heparin
in doses of 10–20,000 units twice-daily adjusted to target a
partial thromboplastin time (PTT) 1.5 times normal should Mitral valve surgery
be initiated.14 Conversely therapeutic, adjusted-dose low- If PBMV is not available, open mitral valve commissurotomy
molecular-weight heparin has been used with careful monitor- can be safely and quickly performed, but does require general
ing of antifactor Xa levels as most pregnant patients will require anaesthesia with the attendant risks. Several case series and
 Tsiaras and Poppas. Mitral valve disease in pregnancy 9
................................................................................................................................................

reviews have suggested that pregnancy does not confer an since pregnancy after mechanical mitral valve replacement is
increased risk of maternal morbidity and mortality. However, complicated by the concomitant use of anticoagulation.
a review of the period from 1984 to 1996 including 58 patients Angiotension-converting enzyme inhibitors are contraindicated
with native valve disease found a maternal mortality rate of 9%, in pregnancy and should be avoided. If vasodilatory agents are
approximately two- to four-fold higher than the non-pregnant required for symptomatic relief, hydralazine and nitrates can
population. Fetal or neonatal mortality was significant at be used. As mentioned above, the risks of cardiovascular
29%.22 In an older, non-randomized study comparing PBMV surgery during pregnancy are significant, and surgery should
with open commissurotomy, de Souza et al. 23 found that be reserved for patients failing maximal medical therapy. In the
PBMV resulted in significantly less fetal morbidity and mor- patient with severe regurgitation and reduced ventricular ejec-
tality (neonatal mortality rate 1/21 in the PBMV group and tion fraction or Class III and IV functional status, labour and
8/24 in the surgical group, P ¼ 0.025). If surgery is required delivery should take place with carefully titrated epidural anaes-
prior to delivery, cardiopulmonary bypass should be done at thesia and invasive haemodynamic monitoring.
normal temperature and high flow and pressure for as brief a
time period as possible. In patients with mixed stenosis and
regurgitation, mitral valve replacement is the only treatment CONCLUSIONS
of choice but, given then higher maternal and fetal morbidity
Mitral stenosis is the most common cardiac condition affecting
and mortality, should be reserved for symptomatic patients
women during pregnancy and is poorly tolerated due to the
who have failed aggressive, in-house medical therapy.
increased intravascular volume, cardiac output and resting
heart rate that predictably occur during pregnancy. Patients at
Labour and delivery high risk for complications include those with class II –IV
Cardiovascular loading conditions are dramatically altered symptoms with mitral stenosis, III and IV symptoms with
during labour and delivery and are predominately driven by mitral regurgitation, severe pulmonary hypertension defined
pain and anxiety. The first stage of labour results in a 20 –30% as 75% of systemic pressures or left ventricular dysfunction
increase in cardiac output; uterine contractions cause an defined as EF ,40%.16 Moreover, the severity of the stenosis
additional 20% increase of cardiac output and blood pressure. is an important predictor of adverse maternal outcomes, includ-
Following delivery, relief of vena caval compression and auto- ing congestive heart failure and atrial arrhythmias. Patients
transfusion from the emptied and contracted uterus cause with mild mitral stenosis appear to do as well as controls.
additional rises in cardiac output. An older study in healthy Close follow-up and medical management with beta-1 selective
women found that epidural anaesthesia resulted in a significant antagonists and cautious use of diuretics is recommended for
blunting of the heart rate and cardiac output.24 Furthermore, con- symptomatic relief in patients with mitral stenosis and heart
tinuation into the postpartum period may provide increased failure symptoms. PBMV has been performed successfully in
venous capacitance to accommodate the extra blood volume, women who fail medical therapy and is recommended over
thereby reducing the risk of pulmonary oedema. open commissurotomy. The goal of labour and delivery is to
In patients with mitral stenosis, the primary treatment goal minimize tachycardia by controlling pain with the use of epi-
during labour is the avoidance of tachycardia and excess dural anaesthesia. Vaginal delivery can be safely performed,
preload. Therefore, carefully titrated epidural analgesia with with caesarian section reserved for patients with obstetric indi-
narcotics should be recommended for pain relief and prompt cations. In contrast to the negative impact of the haemodynamic
treatment of the anticipated hypotension by the judicious use changes of pregnancy on mitral stenosis, the decreased systemic
of crystalloids and pure vasoconstrictors such as phenyl- vascular resistance is haemodynamically favourable for
ephrine. In patients with severe mitral stenosis, Swan– Ganz patients with mitral regurgitation. Mitral regurgitation with a
catheterization for haemodynamic monitoring can allow optim- normal ejection fraction is well tolerated during pregnancy.
ization of filling pressures and should be continued for 12–24 Patients with mitral valve disease should be evaluated precon-
hours postpartum. For these patients, the second stage of ception when possible and should be followed closely by a
labour and Valsalva efforts should be reduced and shortened team of specialists, including obstetricians, cardiologists and
by instrumental delivery.12 In this way, vaginal delivery can anaesthesiologists, throughout the pregnancy and early post-
be safely accomplished in women with mitral stenosis, with partum period.
caesarean section reserved for obstetric indications. This was
the primary mode of delivery in the series by Hameed et al. 3
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