This document provides guidelines for managing pregnancy in women with cardiac diseases. It begins by outlining the cardiovascular changes that normally occur during pregnancy, such as increased blood volume and cardiac output. It then discusses outcomes for different cardiac conditions, finding that outcomes are generally good for conditions involving left-to-right shunts but carry higher risks for cyanotic heart disease, Eisenmenger syndrome, and Marfan syndrome with aortic involvement. The document stresses the importance of risk stratification, counseling, and multidisciplinary care in managing these high-risk pregnancies.

1. Pregnancy Management
Guidelines in Women with
Cardiac Diseases
BY
Jameel Alata , MD
Consultant pediatric cardiologist,
KAAUH / KFSH&RC Jeddah, KSA.
32 ESC annual meeting in association with PACHDA
Cairo, 22-25 FEB 2005

2. •Introduction;
 Pregnancy in most women with heart disease has a
favourable maternal and fetal outcome.
 With the exception of patients with Eisenmenger
syndrome, pulmonary vascular obstructive disease, and
Marfan syndrome with aortopathy, maternal death during
pregnancy in women with heart disease is rare.
 However, pregnant women with heart disease do remain at
risk for other complications including heart failure,
arrhythmia, and stroke.

3. •Introduction;
 Women with congenital heart disease now
comprise the majority of pregnant women with
heart disease seen at referral centres.
 The next largest group includes women with
rheumatic heart disease.

4. •Introduction;
 Approximately 10% of all maternal deaths in the
United States can be attributed to cardiac disease.
 In one study of 1,000 pregnant women who had
various types of cardiac disease and were followed
by the same health-care team over a 10-year
period, more than 75% of the women had no
complications during pregnancy.
 The remaining 25%, the following complications
were seen most often:

5. •Introduction;
 Congestive heart failure, including pulmonary edema (12.3%)
 Cardiac arrhythmias (6%)
 Thromboembolism (1.9%)
 Angina (1.4%)
 Hypoxemia (0.7%)
 Infective endocarditis (0.5%)
 The overall maternal mortality rate in this group was 2.7%, and the
stillbirth and spontaneous abortion rate was 7.7%.

6. •Introduction;
 Cardiac disease covers a wide range of conditions,
including congenital heart disease, acquired
disease such as rheumatic valvular disease, and
coronary disease.
 It is estimated that 1% to 3% of women either
have cardiac disease entering pregnancy or are
diagnosed with cardiac disease while they are
pregnant.
 The frequency of specific types depends on the
patient population and local conditions.

7. •Introduction;
 Advances in the diagnosis and treatment of
congenital heart disease have increased the
survival rate of children affected with these
disorders.
 Pregnant women who have congenital heart
disease represent the largest number of patients
seen at some referral centers, comprising as many
as 70% to 80% of all the cardiac patients.
 It is estimated that 1 in 10,000 pregnancies is
associated with coronary heart disease, notably
myocardial infarction.

8. •Cardiovascular Physiology of Pregnancy;
 Normal pregnancy is associated with of 30 to 50
percent in blood volume and a corresponding
increase in cardiac output.
 These increases begin during the first trimester;
the levels peak by 20 to 24 weeks of pregnancy
and then are either sustained until term or
decrease.
 The heart rate increases by 10 to 20 beats per
minute, the stroke volume increases, and there is a
substantial reduction in systemic vascular
resistance, with decreases in blood pressure.

9. •Cardiovascular Physiology of Pregnancy;
 During labor, cardiac output increases; the blood
pressure increases with uterine contractions.
 Immediately after delivery, the cardiac filling
pressure may increase dramatically due to the
decompression of the vena cava and the return of
uterine blood into the systemic circulation.
 The cardiovascular adaptations associated with
pregnancy regress by approximately six weeks
after delivery.

10. •Cardiovascular Physiology of Pregnancy;
 Functional Murmurs develop in nearly all women
during pregnancy.
 Echocardiography is warranted when diastolic
murmurs, continuous murmurs, or loud systolic
murmurs (louder than grade 2 on the 6-point scale)
are detected or when murmurs are associated with
symptoms or an abnormal electrocardiogram

11. •Cardiovascular Physiology of Pregnancy;
 In normal pregnant women, serial echocardiography
usually demonstrates minor increases in the left and right
ventricular diastolic dimensions, which remain within the
normal range.
 Slight decrease in the left ventricular end-systolic
dimension and a minimal increase in the size of the left
atrium is also noted.
 The state of increased volume also results in increased
transvalvular flow velocities.
 Minor degrees of atrioventricular valve regurgitation are
normal.

12. •Cardiovascular Physiology of Pregnancy;
 During labour and delivery, pain and uterine
contractions result in additional increases in
cardiac output ( 15% with each contraction ) and
blood pressure.
 Immediately following delivery, relief of caval
compression and autotransfusion from the emptied
and contracted uterus produce a further increase in
cardiac output ( upto 45 % ).

13. •Other physiologic changes;
 Hypercoaguble state.
 Hypoalbumineamia.
 Insulin resistant state.
 Increased red c.ell mass
 Increased ESR.
 Increased renal blood flow ( 30 % ).
 Increased hepatic clearance of medications.

14. •Outcome of pregnancy with CHD

15. •Outcome ; LT to RT shunts
 In the absence of pulmonary hypertension, pregnancy,
labour and delivery are well tolerated 2nd to attenuation of
volume overload by peripheral vasodilation.
 However arrhythmias, ventricular dysfunction, and
progression of pulmonary hypertension may occur,
especially when the shunt is large or when there is pre-
existing elevation of pulmonary artery pressure.
 In ASDs, paradoxical embolisation may be encountered if
systemic vasodilatation and/or elevation of pulmonary
resistance promote transient right to left shunting.

16. •Outcome ;AS, COA & LVOTO
 The absence of symptoms antepartum is not sufficient
assurance that pregnancy will be well tolerated.
 Pregnant women with severe aortic stenosis have limited
ability to augment cardiac output, elevation of left
ventricular systolic and filling pressures& non-compliant,
hypertrophied ventricle is sensitive to falls in preload leads
to:
CHF , HYPOTENSION & or ISCHEMIA

17. •Outcome ;AS, COA & LVOTO
 In a compilation of many earlier small
retrospective series, 65 patients were followed
through 106 pregnancies with a maternal mortality
of 11% and a perinatal mortality of 4%.
 In 25 pregnancies managed recently, there was no
maternal mortality but deterioration of maternal
functional status occurred in 5 (20%).
 In the absence of prosthetic dysfunction or
residual aortic stenosis, patients with bioprosthetic
aortic valves usually tolerate pregnancy well.

18. •Outcome ;AS, COA & LVOTO
 Ross procedure reported favourable maternal and fetal
outcomes except in one woman who developed postpartum
left ventricular dysfunction.
 Pregnancy in a woman with a mechanical valve prosthesis
carries increased risk of valve thrombosis as a result of the
hypercoagulable state.
 The magnitude of this increased risk (3-14%) is greater if
subcutaneous unfractionated heparin rather than warfarin is
used as the anticoagulant agent.

19. Outcome ; COA
 Maternal mortality with uncorrected coarctation
has been reported as 3% in an early series.
 Aortopathy, or longstanding hypertension; aortic
rupture accounted for eight of the 14 reported
deaths and occurred in the third trimester as well
as in the postpartum period..
 More recently, a preliminary report described
encouraging maternal and fetal outcome in
87 pregnancies, with no maternal deaths and one
early neonatal death.

20. •Outcome ; COA
 The management of hypertension in uncorrected
coarctation is particularly problematic in
pregnancy because satisfactory control of upper
body hypertension may lead to excessive
hypotension below the coarctation site,
compromising the fetus.
 Intrauterine growth restriction and premature
labour and delivery are more common. Following
coarctation repair, the risk of dissection and
rupture is likely reduced but not eliminated.

21. •Outcome ; Pulmonary stenosis
 Mild pulmonic stenosis, or pulmonic stenosis that has been
alleviated by valvuloplasty or surgery, is well tolerated
during pregnancy and fetal outcome is favourable.
 Severe pulmonic stenosis may be asymptomatic , but may
precipitate right heart failure or atrial arrhythmias; such
condition should be considered for correction before
pregnancy.
 Even during pregnancy, balloon valvuloplasty may be
feasible .

22. Outcome; Cyanotic CHD
 Uncorrected or palliated pregnant patients with cyanotic
congenital heart disease such as tetralogy of Fallot, single
ventricle, etc, the usual pregnancy associated fall in
systemic vascular resistance and rise in cardiac output
exacerbate right to left shunting leading to increased
maternal hypoxaemia and cyanosis.
 Outcomes of 96 pregnancies in 44 women with a variety of
cyanotic congenital heart defects reported a high rate of
maternal cardiac events (32%, including one death).

23. Outcome; Cyanotic CHD
 Prematurity (37%), and a low live birth rate (43%).
 The lowest live birth rate (12%) was observed in those
mothers with an arterial oxygen saturation of 85%.
 Pregnancy risk is low in women who have had successful
correction of tetralogy without residuals.

24. Outcome; Cyanotic CHD
 For Atrial repair (Mustard or Senning procedure)
43 pregnancies in 31 women described in recent
reports, showed one late maternal death.
 There was a 14% incidence of maternal heart
failure, arrhythmias, or cardiac deterioration.
 Few recipients of the current repair of choice for
complete transposition the arterial switch
procedure have yet reached reproductive age.

25. Outcome; Cyanotic CHD
 The Fontan operation eliminates cyanosis and
volume overload .
 A recent review of 33 pregnancies in 21 women
showed 15 (45%) term pregnancies with no
maternal mortality although two women had
cardiac complications .
 The incidence of first trimester miscarriage was
high (39%).
 Since the 10 year survival rate following the
Fontan operation is only 60-80%, it is important
to discuss prognosis during preconception
counselling.

26. Outcome; Marfan
 Medial aortopathy resulting in dilatation,
dissection, and valvar regurgitation risks are
increased in pregnancy because of haemodynamic
stress and perhaps hormonal effects.
 Recently 45 pregnancies in 21 patients reported
no increase in obstetrical complications or
significant change in aortic root size in the patients
with normal aortic roots.

27. Outcome; Marfan
 The eight patients with a dilated aortic root
(> 40 mm) or prior aortic root surgery, three of
their nine pregnancies were complicated by either
aortic dissection (two) or rapid aortic dilatation
(one).
 In contrast, women with little cardiovascular
involvement and with normal aortic root diameter
may tolerate pregnancy well.
 Serial echocardiography should be used to identify
progressive aortic root dilatation and prophylactic
B-blockers should be administered.

28. Outcome; L-TGA
 Potential problems in pregnancy include
dysfunction of the systemic Right ventricle and/or
increased Systemic AtrioVentricular valve
regurgitation with heart failure, Atrial arrhythmias,
andAV- block.
 41 patients, there were 105 pregnancies with 73%
live births and no maternal mortality, although
seven patients developed either heart failure,
endocarditis, stroke, or myocardial infarction.

29. Outcome; Eisenmenger
 A recent review of outcome of 125 pregnancies in
patients with Eisenmenger syndrome, primary
pulmonary hypertension, and secondary
pulmonary hypertension showed;
maternal mortality of 36%, 30%, and 56%,
respectively.
 The overall neonatal mortality was 13%
 The preponderance of complications occurs at
term and during the first postpartum week.

30. Outcome; Eisenmeger
 Preconception counselling should stress the
extreme pregnancy associated risks.
 Termination of pregnancy should always be
offered to such patients, as should sterilisation.
 The vasodilation associated with pregnancy will
increase the degree of right to left shunting in
patients with Eisenmenger syndrome, resulting in
worsening of maternal cyanosis with poor fetal
outcome

31. Outcome; RHC Disease
 Mitral stenosis is the most common rheumatic
valvar lesion encountered during pregnancy.
 Patients with mild to moderate mitral stenosis,
who are asymptomatic before pregnancy, may
develop atrial fibrillation and heart failure during
the ante- and peripartum periods.

32. Outcome; RHC Disease
 Earlier studies showed that mortality rate
increased with worsening antenatal
maternal functional class.
 A more recent study found no mortality but
described substantial morbidity from heart
failure and arrhythmia.

33. Outcome; RHC Disease
 Pregnant women whose dominant lesion is
rheumatic aortic stenosis have a similar outcome
to those with congenital aortic stenosis.
 Severe aortic or mitral regurgitation is generally
well tolerated during pregnancy although
deterioration in maternal functional class has been
observed.

34. Outcome;
Peripartum Cardiomyopathy
 Unexplained left ventricular systolic dysfunction,
confirmed echocardiographically, presenting
during the last antepartum month or in the first
five postpartum months.
 The relapse rate during subsequent pregnancies is
substantial in women with evidence of persisting
cardiac enlargement or left ventricular
dysfunction.
 It remains unclear whether pregnancy is safe in
those with recovery of systolic function.

35. Management
 Risk stratification.
 Counseling.
 Antepartum management.
 Multidisiplenary, high risk units.
 Labour and delivery.

36. Risk stratification
 The data required for risk stratification can
be acquired readily from a thorough
cardiovascular history and examination,
12 lead ECG, and transthoracic
echocardiogram.
 In patients with cyanosis, arterial oxygen
saturation should be assessed by
percutaneous oximetry.

37. •Risk stratification
 Low risk
 1- Small left to right shunts.
 2- Repaired lesions without residual cardiac dysfunction.
 3- Isolated mitral valve prolapse without significant
regurgitation.
 4-Bicuspid aortic valve without stenosis.
 5-Mild to moderate pulmonic stenosis.
 6- Valvar regurgitation with normal ventricular systolic
function.

38. •Risk stratification
 Intermediate risk
 1- Unrepaired or palliated cyanotic congenital heart disease
 2- Large left to right shunt
 3-Uncorrected coarctation of the aorta
 4- Mitral or aortic stenosis
 5- Mechanical prosthetic valves
 6- Severe pulmonic stenosis
 7- Moderate to severe systemic ventricular dysfunction
 8- History of peripartum cardiomyopathy with no residual ventricular
dysfunction

39. •Risk stratification
 High risk
 1- New York Heart Association (NYHA) class III or IV symptoms
 2-Severe pulmonary hypertension
 3- Marfan syndrome with aortic root or major valvar involvement
 4-Severe aortic stenosis
 5- History of peripartum cardiomyopathy with residual ventricular
dysfunction

40. Counselling
 In counselling, the following six areas should be
considered:
 The underlying cardiac lesion,
 Maternal functional status,
 The possibility of further palliative or corrective
surgery,
 Additional associated risk factors,
 Maternal life expectancy ,
 Ability to care for a child,
 The risk of congenital heart disease in offspring.

41. Antepartum Management
 Issues are:
 Congestive heart failure,
 Arrhythmias,
 Thrombosis,
 Emboli, and
 Adverse effects ofAnticoagulants.

42. CHF
 Activity limitation is helpful and in severely
affected women with NYHA class III or IV
symptoms, hospital admission by mid second
trimester may be advisable.
 Pregnancy induced hypertension,
hyperthyroidism, infection, and anaemia should be
identified early and treated vigorously.

43. CHF
 For patients with important mitral stenosis,
the use of blockers or digoxin for control
of heart rate should be considered.
 Also offer empiric treatment with blockers
to patients with coarctation and to Marfan
patients.

44. Arrhythmias
 Arrhythmias in the form of premature atrial or
ventricular beats are common in normal
pregnancy.
 Sustained tachyarrhythmias such as atrial flutter or
atrial fibrillation should be treated promptly.
 Electrical cardioversion is safe in pregnancy.
 Digoxin and blockers are antiarrhythmic drugs of
choice in view of their known safety profiles.
Quinidine, adenosine, sotalol, and lidocaine are
also "safe“. ( avoid teratogens and Amiodarone )

45. Anticoagulation
 For pregnant women with mechnical valves
mainly.
 Warfarin more effective than Heparine , but
embryopathic.
 Should be stopped at least 2 wks before
labour to avoid fetal brain bleeding.

46. Labour & delivery
 Vaginal delivery is recommended with very few
exceptions.
 The only cardiac indications for caesarean section
are aortic dissection, Marfan syndrome with
dilated aortic root, and failure to switch from
warfarin to heparin at least two weeks before
labour.
 Preterm induction is rarely indicated, but once
fetal lung maturity is assured a planned induction
and delivery in high risk situations will ensure
availability of appropriate staff and equipment.

47. Labour & Delivery
 Invasive haemodynamic monitoring during labour
and delivery,is commonly utilised (intra-arterial
monitoring)with or without (concurrent
pulmonary artery catheterisation).
 Heparin anticoagulation is discontinued at least
12 hours before induction, or reversed with
protamine if spontaneous labour develops, and can
usually be resumed 6-12 hours postpartum.

48. Labour & delivery
 SBE prophylaxis:
 Centres with extensive experience in caring
for pregnant women with heart disease
utilise endocarditis prophylaxis routinely, as
an uncomplicated delivery cannot always be
anticipated.
 Not AHA recommended if no infection site.

49. Labour & delivery
 Epidural anaesthesia with adequate volume
preloading is the technique of choice.( but
can increase CHF & pulm oedema )
 Epidural fentanyl is particularly
advantageous in cyanotic patients with
shunt lesions as it does not lower peripheral
vascular resistance.

50. Labour & delivery
 Labour is conducted in the left lateral decubitus
position.
 Instrumentation to shorten 2nd stage is indicated.
 Patients at intermediate or high risk may require
monitoring for a minimum of 72 hours
postpartum.
 Patients with Eisenmenger syndrome require
longer close postpartum observation, since
mortality risk persists for up to seven days.

51. Conclusion
 Women who have survived congenital heart
disease into adulthood often have a strong desire
to become pregnant.
 Optimum care of these potentially complicated
pregnancies can only be achieved by a combined
approach by cardiologists and obstetricians in
specialist centres with an understanding of the
obstetric and cardiac complications that can arise.