Pregnancy & Congenital Heart Disease

8/8/2019 Pregnancy & Congenital Heart Disease

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Pregnancy & Congenital HeartDisease (CHD)

Dr. Georges IBRAHIM

08/02/2007


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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 havechromosomal defects )


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Relative incidence at birth

percentageGender >disease

30.5VSD

9.8FASD

9.7FPDA

6.9Pulmonic stenosis

6.8MCoarctation of the Ao


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Relative incidence at birth

6.1MAortic stenosis

5.8Tetralogy of Fallot

4.2MComplete TGA

2.2Persistent truncus A

1.3MTricuspid atresia

16.5

FEbstein`s anomaly

All others


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Incidence of CHD : gender

In toto :

predominate in males

ASD, PDA, Ebstein`s anomaly :

more common in females


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Unoperated, adult survival expectedCommon

Congenital aortic valve disease

Coarctation of the aortaPulmonary valve stenosis

ASD (ostium secundum)

PDA


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Unoperated, adult survival expectedUncommon

Congenital complete heart block

Ebstein`s anomalyCongenitally corrected TGA

Dextrocardia

Sinus of Valsalva aneurysm, coronary arterial fistula, pulmonary

AV fistula

VSD

Fallot`s tetralogy


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Operated, adult survival expected

The same unoperated

Complete TGA (atrial switch operations )

Fontan procedure (& fontan-like operations )


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CV physiology during pregnancy

++++++Blood volume

+++/++++/++++Cardiac output

++/++++++Heart rate

=_=Systolic BP

__ __Diastolic BP

=+++Pulse pressure

_ __ _ __SVR


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Maternal outcome

Is determined by :

--nature of disease & surgical repair

-- cyanosis & Hb

-- PVR

-- functional capacity

CVdeterioration favorised by :

- exercise, heat, numidity, anemia, infections, and arrhythmias


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Maternal outcome

in general :

- good in most non-cyanoticcases

- unfavorable if cyanosis

impaired functional status

( CHF, arrhythmias, hypertension, angina, IE and

thromboembolic )


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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


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Tolerance of pregnancy in CHD

POORLYINTERWELL

NYHA IVNYHA II-IIINYHA I

R-L shunt,

Unrepaired cyan

Ebstein`s

anomaly

L-R shunt - PH

PHT/ P vascular

disease

Repaired TGA

Fontan repair

Repaired TF


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Tolerance of pregnancy in CHD

PS (severe) ?PS (mild to

moderate)

Marfan`s

CoarctationAo

PR,TR (evensevere if low

pressure)

AS, MS (severe)AS, MS(moderate)

AR, MR (mild tomoderate)


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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)


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PDA

Mild & moderate-sized :- risk ofIEduring delivery

Moderately restrictive :- decrease in SVRdecrease L-Rshunt

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)


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PDA post-op

No risk ofIE six months after division

Large PDA :

- depends on residua (PVR)

(LV function )


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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 ofIE

- Mild to moderate PR not a concern


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Coarctation of the aorta

Hypertension (comparatively low incidence of toxemia )

Increased risk ofaortic rupture ordissection

Increased risk ofcerebralhemorrhage

LVFexceptional under age 40 (except infants)

Risk ofIE if bicuspid aortic valve

angina

To be corrected prior to pregnancy

Limiting physical activity & controlling BP

Surgical correction during pregnancy successful

(severe uncontrolable hypertension orCHF )


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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 ofWillis ?

Procedure of choice : resection & end-to-end

anastomosis


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Congenital aortic valve disease(Bicuspid aortic valve)

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

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

Regurg : generally well tolerated even severe(provided good LV function )

High risk ofIE : prophylaxis during labor & delivery

Risk of aortic root dissection (bicuspid )


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Severe AS :

balloon dilatation (provided thin, mobile& not calcified )Mild to moderate AS & significant AR :

advice pregnancy before replacement (provided good LVfunction )

Severe AS and\or AR (replacement indicated ) : advice a tissue valve

Post-op : risk ofIE and aortic root dissection persist


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Severe aortic stenosis (pregnant patient) :(high risk of decompensation in the 2nd or3rd trimester)

early abortion :+ valvuloplasty or replacement

continuation of pregnancy :+

medicaltreatment, hemodynamic monitoring during laborand delivery and appropriate anesthesia

+balloon valvuloplasty or

+surgicalreplacement


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Fallot`s tetralogy

Pregnancy :

- exacerbate R-to-L shunt and cyanosis.

poor prognostic signs : HTC> 60%, SaO2


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Fallot`s tetralogy

to be corrected and revised if partially

repaired prior to pregnancy (outcome markedlyimproved by surgical repair )

Post-op :

- small risk especially ifRVoutflow obstructionis relieved without significant PR.

- post-op electrophysiological sequlae

infrequent if repair at a young age


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Ebstein`s anomaly of the TV

RVF : IncreasedCO

Atrial tachyarrhythmias : one-third of nonpregnant

(rapid ventricular rate in response to AF or flutter)

Paradoxical emboli and hypoxemia :IncreasedR-Lshunt byIncrease in RV filling pressure

Risk of IE

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

*** successful pregnancy reported in the majority ofpatients


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Ebstein`s anomaly post-op

Surgery :

improve RV functioneliminate the risk of paradoxical emboli &

bypass tracts

reduce the risk ofIE& supraVarrhythmias


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VSD

Isolated : well tolerated

(risk ofIE)(some CHF & arrhythmias reported )

With PHT :

(marked reduction in BPshunt reversal )

Post-op :

(electrophysiological sequelae exceptional )

(offspring : 22% CHD, 50% of them VSD )


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Congenital complete heart block

Uncommon

Asymptomatic : uneventful pregnancy

(provided QRS not prolonged & satisfactory rateresponse to exercise )

Stokes-Adams attacks : occasionally occur duringpregnancy

Post-op ( occasionally ) :- dual-chamber PMpreferable


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Marfan`s syndrome

High maternal morbidity

Inheritance 50%

Majority : CV manifestation during pregnancy (AR, HF,Ao dissection)

Aortic root < 40 mm :In general, favorable outcomePeriodic follow up, prophylactic beta blockers

(recommended) & limitedphysicalactivity

Preconception counseling : Dilatation of the aorta

advise against conceptionduring pregnancy : Significant aortic dilatation

therapeuticabortion

surgicalintervention

During delivery : Significant cardiac complicationabdominaldelivery


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Eisenmengers syndrome

high risk for maternalmorbidity and

mortality ( mort 38% ).

poor fetaloutcome.

( fetal loss, prematurity, itrauterine growth retardation, and

perinatal death )

to be advised against pregnancy,

abortion indicated.


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Eisenmengers 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 laborpreferred 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 orsegmental epidural )


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Complex cyanotic CHD

Cyanosis before surgeryabnormalities of gynecological

endocrinology that may influence fertility

high riskif uncorrected or partially

corrected.

Hb andSaO2best predictors for fetal

outcome.

pregnancy discouraged, early

interruption indicated.


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Complex cyanotic CHD - postop

Successful Fontan repairadequate

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

Postop cTGA late postop sequellae the rule( electrophysiological sequellae, RV progressive systolicdysfunction & AR )


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Should not become pregnant

* Cyanotic CHD

* Pulmonary hypertension* Marfan`s syndrome


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Prenatal care

Anxiety

Diuretics :judiciously for HF

Exercise : moderate isotonic

Dry & cool atmosphere therapeutic

Pathological anemia addressed

Meticulous leg care

Passive standing avoidedSupine position minimized

Oxygen administration open to question

Anticoagulation at least 2rd tri & 1 monthspp in high risk


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Labor & deliverymild unoperated & successfully operated

The same as for normal pregnant

IE prophylaxis :

Routine delivery recommended( low incidence ofbactremia in uncomplicated vaginal delivery )

Episiotomy & vacuum extraction indicated

(from the onset of labor through the 3rd or 4th postpartal day)


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Labor & deliveryfunctionally 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 )


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Labor & deliveryfunctionally important CHD

Antibiotic prophylaxis

O2 therapyHemodynamic & blood gas monitoring

( strongly recommended )

hypotension avoided (increasing R-L shunt)

regional anesthesia with caution( recommended : inhalation a, nerve blocks, intrathecal

morphine )


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Contraception

Barrier methods (condoms, diaphragm withspermicides )

Tubal ligation : safe even in high risk

Levonorgestrel implants : safe & efficacious , fluidretention modest

Low estrin : safe & nonthrombogenic

IUD : low risk ofIE, but induce excessive bleeding

Progestin injections : not recommended if HF, fluidretention


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Pregnancy & Congenital Heart Disease

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