Pregnancy & Congenital Heart

Disease (CHD)

Dr. Georges IBRAHIM

08/02/2007
Incidence of CHD
 0.8 % of live births
 + nonstenotic bicuspid aortic valve
 + mitral valve prolapse
 + PDA in small preterm infants

 10 times more often in stillborn babies

 Early spontaneous abortions ( many have
chromosomal defects )
Relative incidence at birth

percentage > Gender disease

30.5 VSD
9.8 F ASD
9.7 F PDA
6.9 Pulmonic stenosis
6.8 M Coarctation of the Ao
Relative incidence at birth

6.1 M Aortic stenosis

5.8 Tetralogy of Fallot
4.2 M Complete TGA
2.2 Persistent truncus A
1.3 M Tricuspid atresia
F Ebstein`s anomaly
16.5 All others
Incidence of CHD : gender

: In toto
predominate in males

: ASD, PDA, Ebstein`s anomaly

more common in females
Unoperated, adult survival expected
Common

Congenital aortic valve disease

Coarctation of the aorta
Pulmonary valve stenosis
ASD (ostium secundum)
PDA
Unoperated, adult survival expected
Uncommon

Congenital complete heart block

Ebstein`s anomaly
Congenitally corrected TGA
Dextrocardia
Sinus of Valsalva aneurysm, coronary arterial fistula, pulmonary
AV fistula

VSD
Fallot`s tetralogy
Operated, adult survival expected

…The same unoperated

Complete TGA ( atrial switch operations )
Fontan procedure ( & fontan-like operations )
CV physiology during pregnancy

+++ ++ + Blood volume

++/+++ +++/++ + Cardiac output
+++/++ ++ + Heart rate
= _ = Systolic BP
_ __ _ Diastolic BP
= ++ + Pulse pressure
__ ___ _ SVR
Maternal outcome
: Is determined by
nature of disease & surgical repair --
cyanosis & Hb --
PVR --
functional capacity --

: CV deterioration favorised by
exercise, heat, numidity, anemia, infections, and -
arrhythmias
Maternal outcome

: in general
good in most non-cyanotic cases -

unfavorable if cyanosis -
impaired functional status
CHF, arrhythmias, hypertension, angina, IE and (
) thromboembolic
CHD - Fetal outcome

Is determined by maternal
cyanosis --
functional capacity --

Fetal wastages (20% noncy vs 45% cy )

Low birth w.& prematurity (correlate with Hb )
CHD ( 10 % ) ( VSD: 22%, AS: 20% , Fallot: 3-17%)
Other physical & mental abnormalities
Tolerance of pregnancy in CHD

POORLY INTER WELL

NYHA IV NYHA II-III NYHA I

R-L shunt, Ebstein`s L-R shunt - PH

Unrepaired cyan anomaly

PHT/ P vascular Repaired TGA Repaired TF

disease Fontan repair
Tolerance of pregnancy in CHD

? PS (severe) PS (mild to
moderate)

PR,TR (even
Marfan`s severe if low
pressure)
Coarctation Ao
AS, MS (severe) AS, MS AR, MR ( mild to
(moderate) moderate)
ASD ( ostium secundum )
Majority female, reproductive life
Tolerate well usually
) PHT rare in childbearing age (
Paradoxical embolus
Acute blood loss  increase L-R shunt

: Postop
atrial arrhythmias & mitral regurgitation -
(if repair after young adulthood)
PDA
: Mild & moderate-sized
risk of IE during delivery -
: Moderately restrictive
decrease in SVR  decrease L-R shunt -
shunt reversal if PHT
risk of HF ( > Age 30 ) -

: Non restrictive (+ PV disease & R-L shunt)

decrease in SVR  increase R-L shunt -
lower uterine oxygen saturation  potential harm to the (
)fetus
PDA post-op

No risk of IE six months after division

: Large PDA
depends on residua ( PVR ) -
) LV function (
Isolated PS
Well tolerated ( even severe occasionally )
IE prophylaxis advisable

: Severe PS
Should be corrected prior to conception -
Balloon dilatation during pregnancy efficacious -
) progressive RV failure despite drug therapy (

: Post-op
Low risk of IE -
Mild to moderate PR not a concern -
Coarctation of the aorta
Hypertension (comparatively low incidence of toxemia )
Increased risk of aortic rupture or dissection
Increased risk of cerebral hemorrhage
LVF exceptional under age 40 (except infants)
Risk of IE if bicuspid aortic valve
angina

 To be corrected prior to pregnancy

 Limiting physical activity & controlling BP
 Surgical correction during pregnancy successful
) severe uncontrolable hypertension or CHF (
Coarctation post-op
: Risk of pregnancy depends on
relief of the isthmic obstruction -
reduction of systemic BP -
surgical technique -
presence of bicuspid AV -

Risk of gestational rupture of aneurysm of circle of

? Willis

Procedure of choice : resection & end-to-end

anastomosis
Congenital aortic valve disease
(Bicuspid aortic valve)

Low incidence among women, cardiac defects in 20% of infants

Stenosis : mild to moderate generally well tolerated
severe (+ dyspnea, angina) high risk

Regurg : generally well tolerated even severe

) provided good LV function(

High risk of IE : prophylaxis during labor & delivery

Risk of aortic root dissection ( bicuspid )
Congenital aortic valve disease

: Severe AS
 balloon dilatation ( provided thin, mobile& not calcified )
: Mild to moderate AS & significant AR
 advice pregnancy before replacement ( provided good LV
) function
: Severe AS and\or AR ( replacement indicated )
 advice a tissue valve

Post-op : risk of IE and aortic root dissection persist

Congenital aortic valve disease

Severe aortic stenosis ( pregnant patient) :

(high risk of decompensation in the 2nd or 3rd trimester)

:  early abortion
valvuloplasty or replacement +

:  continuation of pregnancy
medical treatment, hemodynamic monitoring during labor +
and delivery and appropriate anesthesia
balloon valvuloplasty or +
surgical replacement +
Fallot`s tetralogy

: Pregnancy
.exacerbate R-to-L shunt and cyanosis -

poor prognostic signs : HTC > 60%, SaO2 <80%, RV

hypertension, syncopal episodes

: Labor & delivery

,sudden decrease in SVR  intense cyanosis -
syncope and death
Fallot`s tetralogy

to be corrected and revised if partially

repaired prior to pregnancy ( outcome markedly
) improved by surgical repair

: Post-op
small risk especially if RV outflow obstruction -
.is relieved without significant PR
post-op electrophysiological sequlae -
infrequent if repair at a young age
Ebstein`s anomaly of the TV
RVF : Increased CO
Atrial tachyarrhythmias : one-third of nonpregnant
)rapid ventricular rate in response to AF or flutter(
Paradoxical emboli and hypoxemia :Increased R-L
shunt by Increase in RV filling pressure
Risk of IE

Outcome depends on severity of TR, RVF, and cyanosis ***

successful pregnancy reported in the majority of ***
patients
Ebstein`s anomaly post-op

: Surgery
improve RV function
& eliminate the risk of paradoxical emboli
bypass tracts
reduce the risk of IE & supraV arrhythmias
VSD

Isolated : well tolerated

) risk of IE (
) some CHF & arrhythmias reported (
: With PHT
) marked reduction in BP  shunt reversal (
: Post-op
) electrophysiological sequelae exceptional(
) offspring : 22% CHD, 50% of them VSD(
Congenital complete heart block
Uncommon
Asymptomatic : uneventful pregnancy
provided QRS not prolonged & satisfactory rate (
) response to exercise

Stokes-Adams attacks : occasionally occur during

pregnancy

: Post-op ( occasionally )
dual-chamber PM preferable -
Marfan`s syndrome
High maternal morbidity
Inheritance 50%
Majority : CV manifestation during pregnancy (AR, HF,Ao dissection)
Aortic root < 40 mm :In general, favorable outcome
Periodic follow up, prophylactic beta blockers
limited physical activity & )recommended(
Preconception counseling : Dilatation of the aorta
advise against conception
during pregnancy : Significant aortic dilatation
therapeutic abortion
surgical intervention
During delivery : Significant cardiac complication
abdominal delivery
Eisenmenger’s syndrome
high risk for maternal morbidity and
.mortality ( mort 38% )

.poor fetal outcome

fetal loss, prematurity, itrauterine growth retardation, and (
) perinatal death

,to be advised against pregnancy

.abortion indicated
Eisenmenger’s syndrome

: If a patient decide to proceed to term

Close follow-up -
Restriction of physical activity -
Anticoagulation -
Hospitalization for any sign of premature uterine activity -
Early elective hospitalization recommended -
Spontaneous labor preferred to induction -
BP, ECG, blood gas monitoring essential -
High concentration O2 may be helpful -
Vaginal delivery most tolerate ( + forceps & vacuum extraction) -
Cesarean section ( GA with minimal negative inotropic or -
segmental epidural )
Complex cyanotic CHD
Cyanosis before surgery abnormalities of gynecological
endocrinology that may influence fertility

high risk if uncorrected or partially

.corrected
Hb and SaO2 best predictors for fetal
.outcome
pregnancy discouraged, early
.interruption indicated
Complex cyanotic CHD - postop

Successful Fontan repair 

adequate homodynamic reserve
if good ventricular function but other variables may (
) ,,influence outcome; atrial arrhythmias, thromboembolic

Postop cTGA 
late postop sequellae the rule
electrophysiological sequellae, RV progressive systolic (
) dysfunction & AR
Should not become pregnant

Cyanotic CHD *
Pulmonary hypertension *
Marfan`s syndrome *
Prenatal care

Anxiety
Diuretics : judiciously for HF
Exercise : moderate isotonic
Dry & cool atmosphere therapeutic
Pathological anemia addressed
Meticulous leg care
Passive standing avoided
Supine position minimized
Oxygen administration open to question
Anticoagulation at least 2rd tri & 1 months pp in high risk
Labor & delivery
mild unoperated & successfully operated

The same as for normal pregnant

: IE prophylaxis
Routine delivery recommended ( low incidence of
) bactremia in uncomplicated vaginal delivery
Episiotomy & vacuum extraction indicated
)from the onset of labor through the 3rd or 4th postpartal day(
Labor & delivery
functionally important CHD

: Induced vaginal delivery

) forceps & vacuum 2nd stage +( -

: Cesarean section
obstetrical reasons -
preterm women on coumadin -
deteriorating maternal status -
about twice the blood loss as vaginal delivery (
,risks of wound and uterine infection &
thrombophlebitis and potential postoperative
) complications
Labor & delivery
functionally important CHD

Antibiotic prophylaxis
O2 therapy
Hemodynamic & blood gas monitoring
) strongly recommended (
hypotension avoided (increasing R-L shunt)

regional anesthesia with caution

recommended : inhalation a, nerve blocks, intrathecal (
) morphine
Contraception

Barrier methods ( condoms, diaphragm with

) spermicides
Tubal ligation : safe even in high risk
Levonorgestrel implants : safe & efficacious , fluid
retention modest
Low estrin : safe & nonthrombogenic
IUD : low risk of IE, but induce excessive bleeding
Progestin injections : not recommended if HF, fluid
retention
Thank you