Succesful Management in Pregnancy with Heart Disease

in Dr.Zainoel Abidin Hospital Banda Aceh : a Case Series

1
Mohd.Andalas 2Cut Meurah Yeni 3Fauzal Aswad 4Fitra Rizia

1,2 Divisionof Obstetrics and Gynecology Department of Syiah Kuala University-Dr.Zainoel Abidin Hospital
3Division of Cardiology Department of Syiah Kuala University-Dr.Zainoel Abidin Hospital
4Resident of Obstetrics and Gynecology Department of Syiah Kuala University-Dr.Zainoel Abidin Hospital

Abstract
The frequency of pregnancy complicated by maternal heart disease does not appear to have changed
over the years. Heart disease complicates approximately 1% of all pregnancies. In women with heart
disease, maternal mortality is reported to be much higher than average and the risk appears to be
increasing such that in western countries heart disease is the major cause of maternal death. The full
spectrum of structural heart disease including congenital heart disease (CHD), valvular heart disease
(VHD), and cardiomyopathy (CMP), and also ischemic heart disease (IHD) may be encountered in
pregnant women. We reported three cases, first a Mrs. 26 years old, 38 weeks gestational age, singleton
live head presentation with post mitral valve reconstruction five years ago. Second, Mrs. 33 years old,
36 weeks gestational age, twin pregnancy, mother with Congestive Heart Failure NYHA fc III-IV,
Rheumatoid Heart Disease, baby with Intrauterine Growth Restriction (IUGR). Third, Mrs. 32 years
old, 40 weeks gestational age with severe oligohydramnios (Amnionic Fluid Index 1,1) and IUGR,
mother with corrected tetralogy of Fallot five years ago. All patients conducted medical history,
physical examination, laboratory test and ultrasound examination. Additional examination as well as
echocardiography and Electrocardiography were conducted in all patients to analyze cardiac function.
After complete evaluation, first patient was plan to be performed elective Caesarean section, second
patient was plan to be performed emergency Caesarean section due to non reassuring fetal state after
hospitalized for two months, while the last patient has to underwent emergency Caesarean Section due
to non reassuring fetal state. Overall, outcome maternal heart disease increase rate of mortality and
mobidity maternal and also neonatal. Regarding a proper antenatal care followed by well management,
resulting in good outcome patient.
Keywords: mitral valve reconstruction, congestive heart failure, corrected tetralogy of fallot,
pregnancy

Introduction In the presence of maternal heart

The frequency of pregnancy
complicated by maternal heart disease does
not appear to have changed over the years.
Heart disease complicates approximately
1% of all pregnancies. In women with heart
disease, maternal mortality is reported to be
much higher than average and the risk
appears to be increasing such that in
western countries heart disease is the major
cause of maternal death.4
disease, the circulatory changes of
pregnancy may result in decompensation or
death of the mother or fetus.
The prevalence of pregnancy
complicated by rheumatic heart disease
(RHD) has decreased in developed
countries and the former ratio of 3:1 for
RHD to congenital heart disease (CHD) in
patients with cardiac disease complicating
pregnancy is now essentially reversed.
Although rheumatic diseases are reported to
be almost eradicated in developed
countries, they still continue to contribute
significantly to maternal morbidity and
mortality in the developing world.4,5
On the other hand, the first
successful replacement of heart valve in
human was reported in 1960.1 Since then,
prosthetic heart valves (PHV) have been
developed into remarkably useful devices.
A large number of PHVs are being
implanted every year around the world, and
many of them in women of childbearing age
who desire to have children.1,2,3
For patients with mechanical heart
valve, lifelong anticoagulation is
1
mandatory. However in pregnant women,
anticoagulation management is a complex
issue. Pregnancy is a hypercoagulable
state, due to increase in fibrinogen, factors
VII, VIII and X, von Willebrand factor and
relative decrease in protein S activity, stasis
and venous hypertension.5 This further
increases the already existing risk of
thrombo-embolic complications (TEC) in
these patients. This state of
hypercoagulability extends into the
postpartum period too and requires a
persistently higher maintenance dose of
warfarin.6 Similarly, increase in total blood
volume affects the distribution of heparin
and low molecular weight heparin
(LMWH). The presence of placental
heparinase further contributes to
unpredictable changes in the quantum of
medication required. Thus, optimal
anticoagulation therapy is considered
essential, but the appropriate choice of
agent among the options available
(warfarin, heparin or LMWH) is highly
debatable.3,4
There is controversy about the
safest anticoagulant regimen during
pregnancy. Anticoagulation is essential for
women with a mechanical valve and for
those with a bioprosthetic who are in atrial
fibrillation or have a history of
thromboembolism. Antiplatelet agents do
not offer protection. Heparin carries a high
risk of fetal loss from retroplacental
haemorrhage as well as maternal bleeding
events, the various regimens are non
standardised, hard to control, and heparin's
efficacy in preventing arterial
thromboembolism is not established.5
Coumarin derivatives are alleged to
increase fetal wastage by bringing a risk of
warfarin embryopathy during the first
trimester as well as a continuing risk of
central nervous system damage throughout
pregnancy.1,4
Uncorrected cyanotic heart disease
carries a high risk in pregnancy for both
mother and fetus: a review of 57
pregnancies in women with uncorrected
tetralogy of Fallot showed a fetal mortality
of 22% and a maternal mortality of 7%.5
The high fetal mortality was caused by poor
fetal growth, leading to a high incidence of
abortion, prematurity, and small-for-dates
babies. Pregnancy in patients with
surgically corrected tetralogy of Fallot is
increasing.
Case Report
We reported three cases, first a
woman 26 years old, nulliparous, with term
pregnancy (38 weeks gestational age)
singleton live head presentation with post
mitral valve reconstruction. Patient was
referred from OBGYN for emergency
Caesarean Section. Patient did antenatal
care routinely at OBGYN and got
anticoagulation therapy. Ultrasound
examination showed that the baby was in
good condition. Patient has been operating
mitral valve reconstruction in 2012. Patient
came with no contraction, no vaginal
discharge and no water broke. Fetal
movement was active. Patient was in stabile
haemodynamic.
Second patient was a woman 33
years old, multiparous with term 36-37
weeks gestational age, twin pregnancy,
head-transverse lie presentation both alive,
monochorionic diamniotic, mother with
CHF NYHA fc III-IV, Rheumatoid Heart
Disease, baby with IUGR. Patient was
referred from Outside Hospital with 33-34
weeks gestational age. Patient did ANC at
OBGYN every month during this
pregnancy. Last ultrasound examination
showed that this pregnancy was twin
pregnancy with Intra Uterine Growth
Restriction. Patient came with chief
complaint dyspnea and cough that felt
every night since three weeks pregnancy.
Dyspnea was especially felt at night and
made her can not sleep. She slept with some
pillow. Dyspnea was felt all day long and
made her can not did her daily activity and
sometimes she woke up suddenly at night
because of hard to breath until she looked
for medication.
Patient has been hospitalized
more than 20 times for three until seven
days. Patient has been hospitalized and has
gotten blood tranfussion. These symptoms
stayed along in this pregnancy and made the
patient finally referred to RSUDZA at 32
weeks gestational age and diagnosed with
heart failure. Patient felt her fetal
movement was decreased since two days
before admission. There was no contraction
and no water broke. From previous history,
we know that patient was used to get
dyspnea one week after delivery in her
previous pregnancy. Patient has been
hospitalized in Intensive Care Unit Outside
hospital for one week after delivered her
first baby but patient did not know the cause
and the history of the medication he got.
After delivery the second and third baby,
patient also got the same condition but she
just only looked for herbal medicine. From
obstetrical history, patient has previous
Caesarean Section due to failed of
induction. Patient was well educated.
Third patient was a woman 32
years old referred by another health care
due to oligohydramnios and IUGR in 9
months pregnancy. Last US examination in
policlinic this morning said that this was
prolonged pregnancy, baby was IUGR,
oligohydramnios and this pregnancy had to
be terminated. Patient came with no
contraction, no bloody show and no water
broke. Fetal movement was felt active.
Patient have been corrected for her TOF in
2013 in Cipto Mangunkusumo Hospital.
There was no symptom of palpitation, chest
pain, and orthopnea. There was three times
abortion in previous obstetric history.
Additionally, the patient was conducted
Caesarean Section due to transverse lie.
This was the fifth pregnancy for her.
All patients conducted medical
history, physical examination, laboratory
test and ultrasound examination. Additional
examination as well as echocardiography
and ECG was conducted in both patients to
analyze cardiac function. After complete
evaluation, first patient was plan to be
performed elective Caesarean section,
second patient was plan to be performed
emergency Caesarean section due to non
reassuring fetal state after hospitalized for
two months, while the last patient has to
underwent emergency Caesarean Section
due to non reassuring fetal state. From first
patient, born baby with body weight 3100
g, Apgar Score 9/10, rooming in. From
second patient, born baby I with body
weight 2000 g, Apgar Score 8/9 and baby II
with body weight 1500 g, Apgar Score 7/8.
Both of these baby were admitted to NICU
and have been hospitalized for two weeks.
From third patient, born female baby with
body weight 1800 g, Apgar Score 8/9,
asymmetrical IUGR and were also admitted
to NICU.
Discussion
Pregnancy related heart failure and
rheumatic heart disease
Pregnancy produces significant
cardiovascular and hemodynamic changes,
which in patients with structural heart
disease, may lead to decompensation.
Congestive heart failure is a serious
problem of cardiac decompensation and is
often associated with maternal death. In our
series maternal mortality due to rheumatic
heart disease was 2% and the majority of
deaths were due to pulmonary edema. All
the women were in NYHA class III– IV.
There was no death due to infective
endocarditis as routine endocarditis
prophylaxis was given to all women during
labor.4,5
Pregnancy outcome is strongly
influenced by the maternal functional status
and the potential for successful outcome is
determined by the maternal functional
status in which patient enters pregnancy.
Both maternal morbidity and mortality are
high in pregnant women with poor function
(functional class III and IV). Hsieh et al. in
their series reported that out of the total
maternal deaths 75% were in patients with
NYHA classes III and IV. In our series also,
eight of 10 maternal deaths (80%) occurred
in patients with NYHA class III and IV.
Higher perinatal morbidity and mortality
was seen in patients with poor functional
status.4,5,6
The maternal and perinatal outcome
in patients with rheumatic heart disease
depends mainly on the functional cardiac
status of the mother at the time of
pregnancy. The risk of maternal morbidity
and mortality is greater in those patients
with functional classes III and IV. Perinatal
outcome is also poorer in these patients.4,5,6
Pregnancy related valvular prosthetic
The problem of prosthetic heart
valves in pregnancy is many faceted. The
physician is concerned with the welfare of
two individuals, either of whom may be
adversely affected by the heart disease or its
specific therapy. She must be on the alert
for the occurrence of congestive heart-
failure, embolie phenomena, carditis,
upper-respiratory tract infection, anemia,
and emotional upset, all of which may alter
the course of events in less than 24 hours in
any stage of gestation. Finally, one of the
most perplexing aspects of the management
of these patients concerns the use of
anticoagulants. Although in this patient an
embolism did occur during the immediate
post-operative period, her gestational
period was not complicated by any medical
problems. Because it is recognized that
systemic embolization is a constant danger
after insertion of prosthetic valves, it is
advisable to maintain these patients on
continuous anticoagulant therapy.1
Fig 1. Echocardiography showed mild pulmonary hypertension and prosthetic valve with
ejection fraction 58 %

Fig 2. ECG examination of patient 1

Fig 3. Ultrasound examination of patient 1

Fig 4. Echocardiography showed moderate Mitral Stenosis, severe Mitral Regurgitation due
to Rheumatoid Heart Disease, severe Tricuspid Regurgitation severe, severe Pulmonary
hypertension with ejection fraction 57 %

Fig 5. ECG examination of patient 2

Fig 6. Ultrasound examination of patient 2

 Fig 7. Echocardiography showed mild atrial regurgitation, with ejection fraction 47%, and
septal dischinetic

Fig 8. ECG examination of patient 3 showed Right Bundle Branch Block and anteroseptal
ischaemic

Fig 9. Ultrasound examination of patient 3 showed Systolic Diastolic Umbilical Artery that
currently use to determine fetal health and growth restricted fetus
 The administration of
anticoagulants to pregnant patients has been
the subject of numerous reviews, most of
which have concluded that coumarin
derivatives may have a deleterious effect on
the fetus. Heparin, with a molecular weight
of 20,000, apparently does not cross the
placental barrier and does not affect the
fetus. In an extensive review of 92 cases,
Villasanta found an 18.4% perinatal
mortality rate in those patients receiving
coumarin derivatives alone or in
conjunction with heparin. However, no
congenital physical anomalies could be
related to the drugs, and the most frequent
fetal lesion found was hemorrhage or
maceration or both. Eight of these patients
received anticoagulants in the first trimester
of pregnancy, none prior to the eighth week
of gestation.2,3,5
The risk of complications during
pregnancy in patients with mechanical heart
valve depends on the patient’s symptoms,
cardiac function, and her functional
capacity as well as on the type of valve
prosthetic, its position and function.3,4,6
1. Patient factors
There is an increased haemodynamic load
during pregnancy, labor and delivery. The
published experience indicates that most
patientsthat were asymptomatic or only
mildly symptomatic before conception,
tolerate this haemodynamic burden well.
However, cardiac decompensation may
occur, especially in patients with impaired
LV function and/or possible
patientprosthetic mismatch. In addition, an
increased incidence of arrhythmia is
reported during pregnancy and may add to
patient discomfort. Thus, it is not surprising
that decreased functional capacity,
pulmonary oedema and death are not
uncommon in pregnant women with
mechanical valves.Patients with prosthetic
heart valves and markedly impaired LV
function that are moderately or severely
symptomatic (New York Heart
Association, class III and IV) are best
advised against pregnancy.4,6,7
Residual tricuspid incompetence
often co-exists in patients with prosthetic
heart valves. The reported incidence of fetal
loss in mother suffering from tricuspid
incompetence severe enough to require
diuretics is around 73%.8 This risk is
significantly higher when compared with
fetal loss in pregnancies in which the
mother did not exhibit tricuspid
incompetence.7
2. Prosthetic related factors
The most common cause of
maternal death in patients with mechanical
heart valves is the device thrombosis. In
addition, there is also a high incidence of
thromboembolic events in these patients,
ranging from 7% to 23%.10,11 Mechanical
prosthetic in aortic position have a lower
thromboembolic risk than in mitral
position. Also, the relatively older
prosthetic have a higher thromboembolic
risk than the subsequent generation valves.
However, thrombosis of these newer
valves, including those in aortic position,
are not unknown.5,7
3. Drug therapy
Fetal complications related to
maternal anticoagulant therapy are
teratogenicity and fetal loss. The incidence
of abortion or fetal wastage (resulting from
retroplacental haemorrhage, congenital
malformations, etc) in these patients is
high, with reported rates ranging between
23% and 50%. Maternal risk of
haemorrhage while on anticoagulation is
estimated at around 2.5%, with majority of
such episodes (almost 80%) occurring in
association with delivery. Moreover, in
addition to anticoagulants, the use of other
cardiovascular drugs during pregnancy may
also adversely affect the fetal outcome.
Cardiac drugs that are relatively safe during
pregnancy include heparin, propranolol
(and other beta blockers), verapamil,
digoxin and few antihypertension drugs
such as labetolol, methyldopa, hydralazine,
nifedipine and prazosin. Amiodarone is
associated with fetal hypothyroidism and
intrauterine growth restriction. It should be
reserved only for cases with refractory
arrhythmias.4,5,7,8
Heparin (both UFH and LMWH)
does not cross the placenta, and does not
cause teratogenicity. On the contrary,
warfarin readily crosses the placenta.
Vitamin K acts as a co-factor for
carboxylation of glutamic acid residues of
osteocalcin and matrix Gla protein, which
modulate calcium deposition. Oral
anticoagulants when used during the first
trimester, may thus cause a failure in the
synthesis of osteocalcin and Gla matrix
protein resulting in nasal hypoplasia and
stippling seen on X-ray of proximal
epiphyseal growth areas (Chondroplasia
punctata). Exposure during the second and
third trimesters may lead to central nervous
system and eye abnormalities (optic
atrophy, cataract, blindness,
microphthalmia, intraventricular
haemorrhage, microcephaly,
hydrocephalus, seizures, and
6,8
growth/mental retardation).
Pregnancy after surgical correction of
tetralogy of Fallot
Surgical repair of tetralogy of Fallot
has a low perioperative mortality and
survivors are usually much improved
symptomatically. A small incidence of
sudden death has been reported in the years
after repair, the two most likely
mechanisms being the development of
defects in conduction and ventricular
tachyarrhythmias. Specific cardiovascular
changes accompanying pregnancy include
a reduction in systemic vascular resistance
and a diminution in venous return from
supine hypotension. These factors, together
with anaesthesia and blood loss, may
represent a greater than normal risk in
patients with tetralogy of Fallot, but to date
no large series has assessed this risk. In our
case there is no serious complication during
pregnancy or at delivery.11,12
Women with corrected tetralogy of
Fallot are at increased risk of
cardiovascular and obstetric event during
pregnancy. Most even are well treatable.
Cardiovascular and off spring event
outcomes are strongly related with the use
of cardiac medication before pregnancy. In
one cohort, 19% of pregnancies ended in
spontaneous abortion, which is slighty
higher in healthy women. This data in line
with our series which patient in this case has
three abortion in previous history.13
The most important obstetric events
were post partum hemorrhage and
oligohydramnios based on rupture of
membranes which occurred more
frequently in population based on previous
study. No direct explanation was found, no
relationship with anticoagulation therapy
was detected. In our case, patient come to
hospital due to decrease of amniotic fluid.
This result match with previous data.13
The use of maternal cardiac
medication before pregnancy was the most
important predictor of offspring outcome.
Maternal hemodynamic abnormalities as
well as direct effects of maternal
cardiovascular medication may undermine
placental blood flow and induce placental
insufficiency with subsequent intrauterine
growth restriction resulting in children born
small gestational age (SGA) as well as in
premature birth. The strong association
between maternal cardiovascular events
and SGA points in this direction. Palliative
surgery before correction appears to
influence offspring outcome negatively.
Longstanding right ventricular pressure
loading and hypoxia in women with a later
age at correction may have resulted in more
hemodynamic compromise and endothelial
dysfunction, compromising placental
perfusion and fetal well-being. Neonatal
outcome in this case also match with the
theory where born baby with IUGR in this
case.11,13
Conclusion
Pregnancy produces significant
cardiovascular and hemodynamic changes,
which in patients with structural heart
disease, may lead to decompensation.
Congestive heart failure is a serious
problem of cardiac decompensation and is
often associated with maternal death.
Pregnancy outcome is strongly influenced
by the maternal functional status and the
potential for successful outcome is
determined by the maternal functional
status in which patient enters pregnancy.
Medicational state before pregnancy related
cardiovascular therapy strongly associated
with fetomaternal outcomes.
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