A case report : Pregnant women with Rheumatic

Heart Disease, Severe Mitral stenosis, Severe

Tricuspid Regurgitation, Severe Pulmonal
hypertension, Mild Pericard Effusion with
Functional NYHA Class III-IV, What should we do
in tertiary ?

*MOCH. ANDALAS , ** CUT MEURAH YENI, ***HERMAN

SUPRIADI
* Resident Obstetrics & Gynecology, Syiah Kuala University
** Department Obstetrics & Gynecology Division Feto Maternal, Syiah Kuala University
*** Chief Department Obstetrics & Gynecology, Syiah Kuala University

ABSTRACT

Background: Pregnancy with heart disease caused by rheumatic heart disease due to
streptococcal infection is still prevalent in the community and one of the most serious cases
that management needs so much. Professionals managed by multidisciplinary teams for
pregnant women with NYHA III-IV are one of the solutions to make a better mothers life, but
if pregnancy continues to grow and fetal well-being becomes another problem, it is usually a
poor baby outcome. The problem in Indonesia in general and especially in Aceh is pregnancy
of women with a history of rheumatic heart disease did not do initial examination when
knowing that she lost her periode in primary health care or hospital. Late arriving at the
doctor to do the examination and make the team of doctors confused in determining the
choice of solutions because the baby becomes larger and the right of life of the baby.

Objective: Pregnancy is strongly associated with life-threatening problems especially in

patients with NYHA III Pulmonary Hypertension. Mitral valve stenosis (the most common
and highest-risk lesion) is a serious case in both, especially for the mother like in this case.
Therefore hope and need the right decision to minimize the risk of death for the mother. We
discuss this to share and make a better solution if we have the next case.

Case: Thirty-year-old woman with G2P1 gestation 16-17 weeks, Intra uterine Singleton Live
fetus, Dyspnea due to CHF Nyha III-IV with Rheumatic Heart Disease, Severe Mitral
Stenosis, Severe Tricuspid Regurgitation, Pulmonary Hypertension, Pericard Effusion with
chief complaints was breathless since 3 months ago. His condition is getting worse. After
discussions with an obstetrician, anesthesiologist, cardiologist and ethical committee, we
decided that pregnancy should be stopped because pregnancy exacerbated the condition of
heart disease and made a bad prognosis for the mother.

Conclusion: Women with rheumatic heart disease should have pre-counseling when she
wants to conceive and this will make the incidence of morbidity and mortality of pregnant
and fetal mothers lower. Obstetricians, Cardiologists, Anesthesiologists and ethical teams
must work together to get the best solution for pregnant women with rheumatic heart disease.
Prevention priority programs include good premarital counseling, serious care for children
with upper respiratory infections, education and training for primary health care providers.

Keywords: Rheumatic heart disease, Pregnancy, Pulmonary Hypertension, Mitral

Stenosis, and Termination.

INTRODUCTION birth.The rate of maternal death in

pregnancies complicated by PAH is
Heart disease is an important cause of variously reported to be 20–60%. 4Women
maternal death and, surprisingly, rates are with VHD had a high rate of clinical
increasing . Interim analysis of the deterioration and a marked increase in
Registry Of Pregnancy and Cardiac morbid events during pregnancy, including
disease (ROPAC) reported a 100 times CHF, arrhythmias and need to either
higher maternal mortality rate in women initiate or increase cardiovascular drug
with heart disease compared to the general therapy or to hospitalize patients during
pregnant population. 1According to World pregnancy. An increased incidence of CHF
Health Organization (WHO) in patients with VHD is not surprising
approximately 500,000 individuals acquire owing to the marked hemodynamic
acute rheumatic fever each year, the changes normally occurring during
problem in Indonesia in general and gestation. 5
especially in Aceh is pregnancy of women
with a history of rheumatic heart disease
did not do initial examination when CASE REPORT
knowing that she lost her periode in
primary health care or hospital. Late 30-year-old woman G2P1, came to
arriving at the doctor to do the Pertamedika hospital with complaints of
examination and make the team of doctors breathless felt since 3 month ago.
confused in determining the choice of Shortness of breath experienced during
solutions because the baby becomes larger
mild activity and patient never get any
and the right of life of the baby. 2
Rheumatic heart still remains commonest treatment. Chest pain is also felt by the
etiological factor for heart disease patient spread to the back, heart
complicating pregnancy. It is because of palpitations, heartburn and nausea also
frequent and repeated streptococcal complained by the patient. Patient
infections in childhood especially in rural admitted four months pregnant and forgot
areas with poor sanitary conditions. In last menstrual periode, patient did ANC 1
many pregnant women, heart disease still
time in Obstetrician and from the
remains undiagnosed until complications
develop. Even after the diagnosis, many ultrasound examination, it has been 14
women do not comply with the weeks pregnancy which happen 2 weeks
instructions given by obstetrician for ago. Chief complaint become more severe
various reasons. Women having additional since the pregnancy. Patients are unable to
obstetrical complications further worsen perform daily activities. Even in the sitting
the prognosis.3 Pregnancy is strongly position patient still felt breathless and
associated with life-threatening problems
in patients with PAH. The risk of cardiac become more severe when the patient
failure during and after pregnancy sleeps on supine position. Patient admitted
increases and sudden cardiac arrest may awake oftenly at the night because it was
occur during cesarean section or soon after difficult to breathe. History of weight loss
is denied, whitish history is denied,
miction in normal range, constipation
history and already given laxative. In the
first pregnancy the patient also has
experienced shortness of breath and it is
said to be an asthma.

Physical examination BP 90/60 mmgHG,

pulse 120 x / I, respiration 30x/minute,
body temperature 36,70C, conscious Figure 2 In Echochardiography examination found
awareness compos mentis.Jugular venous severe MS, severe TR,severe PH, mild pericardef
pressure increased, basal rhonkhi in both fusion , LA dilated, SEC (-) in LV, good LV function
EF 70%.
lungs, edema in both limbs. On Laboratory
examination Hb 12.1 gr / dl (12-14 gram /
dl), Leukocytes: 17,500 / ul, Platelets:
164,000 / ul, MCV / MCH / MCHC:
83/28/33, Ht: 36%, HbsAg: negative,
Sodium: 137 mmol / L, Potassium: 2.9
mmol / L, Chloride: 104 mmol / L,
Calcium: 7.6 mg / dl, Magnesium: 1.6 mg
/ dl.

Figure 3 ECG examination found: suspected right

ventricular hyperthrophy, right axis deviation, ST
depression segment inferior and lateral

Patients diagnosed with G2P1 ,16-17

weeks gestational age, Single Live Intra
uterine fetus, Dyspneuec CHF Nyha III-IV
. Figure 1 Thorax Roentgen Examination found
with Rheumatoid Heart Disease, Severe
Cardiomegaly with CTR 64% LAD / LVD / RVD, Mitral Stenosis, Tricuspid Severe
TrunculPolmunal prominent ec MS / MI on RHD Regurgitation, Severe Pulmonary
advance / VSD advance / HHD advance, Lung:
Lung marks grade 1 edema
Hypertension, mild Pericard Effusion,
Electrolyte Balance Disorder. Patients are
treated at ICCU. After getting treatment in
iccu for 4 days the patient is become more
stable and shortness of breath is reduced.
On 4 September 2017, obstetricians,
anesthesiologists, and cardiologists
decided that the patient's pregnancy must
be terminated because pregnancy
aggravated the condition of heart disease.
Termination of pregnancy using two
modalities which are misoprostol 50
ugram per oral and folley catheter. On 5
september 2017, conception born and there
is still remaining plasenta.continued with
misoprostol 3 x 200 mcg orally for 3 days.
By the USG on 8 September 2017, found
antefleksi uterine, positive endometrial
line, remains of conceptions not found,
conclusion being made that uterine within Figure 4 Haemodynamic changes during delivery
normal limits. Patients undergoing
treatment in iccu for 9 days.Patients feels
shortness of breath, heart palpitations, It remains high 2–5 days after delivery.
swelling legs has been decreased. Pregnancy induces a series of haemostatic
changes, with an increase in concentration
DISCUSSION of coagulation factors, fibrinogen, and
platelet adhesiveness, as well as
PHYSIOLOGY CARDIOVASCULAR IN diminished fibrinolysis, which lead to
PREGNANCY hypercoagulability and an increased risk of
thrombo-embolic events. In addition,
Pregnancy induces changes in the obstruction to venous return by the
cardiovascular system to meet the enlarging uterus causes stasis and a further
increased metabolic demands of the rise in risk of thrombo-embolism.
mother and fetus. They include increases Maternal glucose homeostasis may change
in blood volume and cardiac output (CO), and cholesterol levels increase in
adaptation to fetal–maternal needs SBP
and reductions in systemic vascular
and DBP increase 15–25% and 10–15%,
resistance and blood pressure (BP). Plasma respectively, during uterine contractions.
volume reaches a maximum of 40% above CO increases by 15% in early labour, by
baseline at 24 weeks gestation. A 30–50% 25% during stage 1, and by 50% during
increase in CO occurs in normal expulsive efforts.15 It reaches an increase
pregnancy. of 80% early post-partum due to
autotransfusion associated with uterine
involution and resorption of leg oedema.
In conclusion, the physiological
adaptations to pregnancy influence the
evaluation and interpretation of cardiac
function and clinical status.2
Level of

PATHOPHYSIOLOGY

Figure 3 Haemodynamic changes during pregnancy

 Figure 5 Physiological changes in pulmonary arterial hypertension (PAH) patients which occur in response to
pregnancy. NO: nitric oxide; CO: cardiac output; PAH-CHD: PAH associated with congenital heart disease;
LV: left ventricle; RV: right ventricle; PVR: pulmonary vascular resistance; SVR: systemic vascular resistance.

This is a challenge for patients with PAH, cardiovascular demands. The development
who have compromised cardiac function of these symptoms on minimal exercise or
due to an increase in right ventricular at rest early in pregnancy when further
afterload and a reduced ability to increase increases in cardiac output will be required
cardiac output in response to stress, be it is an ominous sign.6 Pregnancy drastically
on exercise or during pregnancy. In severe stresses the circulation in women with
PAH, the resting cardiac output may be severe mitral stenosis. The increased blood
low and less than 3.0 l/min. In some volume, heart rate, and cardiac output raise
patients, the cardiovascular demands of left atrial pressure to a level that causes
pregnancy cannot be met. This will be severe pulmonary congestion, leading to
reflected by increasing breathlessness or progressive
syncope as the patient struggles to meet
exertional dyspnea, orthopnea, paroxysmal
nocturnal dyspnea, and pulmonary edema.7

MANAGEMENT

It is important that a woman with

PAH who becomes pregnant is referred to
a PAH specialist centre that has experience
in managing PAH in pregnancy. If a
woman with PAH becomes pregnant,
counselling should be offered and
therapeutic abortion seriously considered.
Patients should also be advised that PAH
can worsen during the post-partum period.
If a therapeutic abortion is accepted, it is
recommended that this occurs before 22 Figure 6 Clasification WHO for maternal
weeks of gestation. 8 cardiovascular risk
 should be avoided as pain-relief during
vaginal birth because of its ability to cause
vasoconstriction of the pulmonary
vasculature.8
Induction of labor with PGE2
intracervical gel has been found safe and
effective. 9 We quote an absolute mortality
risk of around 20% but higher in patients
who have significant exercise limitation,
WHO Class III and evidence of right heart
dysfunction. Progesterone-only
Figure 7 Conditions in which pregnancy risk WHO IV contraceptives such as cerazette (oral
daily), nexplanon (implant) and depot-
For women with PAH who decide to carry provera (every 3 months) are safe and
on with their pregnancy, regular close effective if taken as prescribed and reduce
follow-up at a PAH centre is the pro-thrombotic effects of the combined
recommended and, in some cases, elective oral contraceptive pill The Mirena intra-
hospitalisation for optimisation of uterine system is highly effective and
medication leading up to delivery may be helpful in women with heavy periods. The
advised. There is an increasing body of risk of vaso-vagal events, which can be
evidence on the successful and safe use of dangerous in PH, mandates that these
PAH-specific therapies during pregnancy, devices be inserted in a hospital
such as CCBs, prostacyclin and its environment.6 The NYHA class in most
analogues, and phosphodiesterase type-5 cases of severe PAH was III or worse in
inhibitors. Hospitalisation of women with later pregnancy.10
PAH in the second trimester is sometimes
appropriate due to the increased risk of Post-natal care for the mother and
premature labour and of haemodynamic neonate
complications. There are practical
considerations regarding the different Post-partum monitoring of women with
modes of delivery for women with PAH. PAH is very important as most deaths
A vaginal birth is usually associated with occur in this period and monitoring should
less blood loss, fewer infections, less continue for several days to weeks
thromboembolic risk, and less abrupt following delivery. The highest risk of
haemodynamic changes compared with a mortality is during the first 4 weeks after
caesarean section. However, a prolonged delivery with the majority of deaths due to
labour can be detrimental, with right ventricular failure There is a high
haemodynamic changes that can be chance that babies born to women with
problematic for the mother, including an PAH will be premature, being delivered
increase in CO and venous return. Further during gestational weeks 32–36.
disadvantages that are associated with Pregnancy in PAH is an extremely
vaginal births include labour-induced sensitive topic. Pregnancy is not
acidosis, hypercapnia or hypoxia that can recommended in women with PAH.8
lead to increases in pulmonary arterial
pressure. Women should be monitored by
electrocardiogram at all times throughout PRECOUNCELLING
labour and should have regular
measurements of pulse oximetry, central RHD is the most common heart disease in
venous pressure, and intra-arterial blood pregnancy. Feto maternal mortality and
pressure taken . Nitrous oxide (N2O) morbidity is high in NYHA grade III &
IV. Heart disease complicating pregnancy wants to conceive and this will make the
is a high risk situation and demands incidence of morbidity and mortality of
special attention throughout pregnancy, so pregnant and fetal mothers lower.
early booking prevents and decreases the
Obstetricians, Cardiologists,
maternal and fetal morbidity and
mortality.3 Anesthesiologists and ethical teams must
work together to get the best solution for
PROGNOSIS pregnant women with rheumatic heart
disease. Prevention priority programs
Mortality in women with heart disease include good premarital counseling,
during pregnancy is high when serious care for children with upper
complicated with infective endocarditis.9 respiratory infections, education and
Pulmonary arterial hypertension (PAH) is training for primary health care providers.
a progressive disease that preferentially
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