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Early

managementof

congenitalheart

diseases1Jameel A.

AL-AtaConsultant

&

assistant

professor

ofpediatrics

&

pediatric

cardiology.Introduction2Outcome

of

CHD

has

improved

mainly

due

toimproved

Surgical

&

Interventional

care,

speciallyforneonates.In

KSA

overall

CHD

surgical

mortality

in

4largecenters

is

3—6

%.Pre-surgical

morbidity

&

mortality

remains

highfor

many

differentreasons.Introduction3Poor

early

recognition.(

pre

,

natal

&

postnatal

).Delayed

presentation.None

familiarity

of

pathophysiology

and

naturalhistory

of

CHD.Delayed

initiation

of

treatment.Limited

NICU

/

PICU

facilities.Limited

PGE

availability

.Limited

medivac

services.Others.Pediatricians

can makethe

differenceby

;4Early

recognition.Categorizing

into

type

&

severity.Timely

initiation

of

proper

medical

treatment.Timely

referral

for

interventional

or

surgicaltreatment.=

EARLY

MANAGEMENTEarly management

of

secondum

Atrial

Septal

Defect

;5Confirm

DX

and

size

of

ASD.Most

pts

will

not

need

medicaltreatment.Assure

parents

and

inform

them

of

high

likelihoodof

spontaneous

closure.Watch

for

development

of

PHTN

at

F/U.Look

for

none

cardiac

associations.ASDNo

limitation

of

activity.SBE

prophylaxis

not

usuallyrecommended.Screen

the

family.Follow

every

6—12months.Refer

for

intervention

or

surgery

at

age

3-5

y.

ifsize

remains

>

5

mm.6Early management

of VSD

;7Confirm

DX

,

type

of

VSD

&

size.Examine

for

presence

or

development

ofcoarctation

or

aortic

insufficiency.Medical

therapy

(

diuretics

+/-

ACE

)usuallyneeded

for

>

5mm

defects.Digoxin

not

usuallyneeded.Treat

respiratory

infections

aggressively.89VSD10Ensure

optimum

caloricintake.High

risk

of

development

of

PHTN.Large

VSDs

can

be

silent.

(

PHTN

)No

limitation

of

activity.SBE

prophylaxis

is

amust.VSD11Follow

monthly

<

4

m.o

&

every

2—4

m.

for

age>4m.o.Refer

to

surgery

or

intervention

if

;FTT

,CHF

2)

PHTN

3)

AI

4)Endocarditis. (

usual

age6—12

months)Small

<

5mm

muscular

&

Pm

VSDs

have

a

goodchance

for

spontaneous

closure,

so

assure

parentsbut

follow

the

Pm

VSD

for

AI.Inlet

&

Sub

arterial

VSDs

do

notclosespontaneously.Early management

of

PDA12Confirm

DX

and

size.Spontaneous

closure

is

the

rule

in

the

1styear

of

life

,

so

assureparents.Limitation

of

activity

not

needed.Medical

therapy

(

diuretics

+/-

ACE

)

can

beneeded

usually

if

size

>

2

mm.131415PDA16Large

PDA

>

3

mm

act

like

largeVSDs.Look

for

associations

cardiac

or

noncardiac.Small

PDAs

can

be

referred

forinterventionif

still

patent

at

age

>

1

year

whethersymptomatic

ornot.Early management

of

aortic

stenosis

;17Confirm

DX

and

severity.Look

for

aortic

insufficiency

and

otherassociations.Mild

to

moderate

AS.

do

not

requiremedicaltherapy.

Avoid

hypotensive

agents.Assure

strict

6

m.

f/u by

echocardiography

forgrading

of

severity

&

LVH

+function.Limit

activity

only

if

moderate

to

severe

stenosis

,no

need

to

limit

usual

daily

activity

but

onlystrenuous

exercise

and

competitive

sports.AS

.

;18Strict

SBE

prophylaxis

&

dentalhygiene.Admit

the

child

with

AS.

and

chest

pain

&

obtainurgent

cardiacconsultation.Refer

for

balloon

valvuloplasty

if

severe

except

forsub

aortic

stenosis

which

should

be

referred

earlierto

prevent

aorticinsufficiency.Critical

AS

is

an

emergency

that

presents

withCHF

&

may

PDA

dependant.Early management

of

Pulmonary

stenosis19Confirm

DX

&severity.Look

forassociations.Even severe

PS

usually

does

not

requiremedicaltherapy.Limitation

of

activity

is

usually

not

required.PS

.

;20SBE

prophylaxis

is

controversial.Yearly

F/U

for

mild

to

moderate

PS

&

6

m.

formoderate

to

severe

byechocardiography.Refer

for

balloon

valvuloplasty

ifsevere.Critical

PS

can

present

with

RV

failure

&

orcyanosis

and

may

be

PDA

dependant.Early management

of

Tetralogy

of

Fallot21A

surgical

cyanotic

CHD

where

our

role

isto

get

the

child

to

surgery

safely

at

ag

6-9

mExcellent

physical

growth.CHF

is

rare.Accept

saturation

>

70%

in

room

airPrevent

aneamia.Prevent

dehydration.

(

no

LASIX

).TOF22Prevent

endocarditis.Advice

to

avoid

highaltitudePrevent

and

treat

hypercyanotic

spells.Refer

to

earlier

than

6

m

if

developedspells.23Early management

of

PDA

dependant

CHD24Severe

acute

cyanosis

<

70

%

or

circulatorycollapse

in

the

1st

week

of

life

indicate

cyanotic

orLt

sided

obstruction

PDA

dependant

CHDrespectively.Examples

are

pulmonary

atresia

and

d-TGA

forcyanotic

and

critical

COA

or

IAA.

ForobstructiveLt

sided

CHD.The

PDA

provides

the

needed

PBF,

MIXING

,orSBF.

for

these

lesions.2526PDA

dependant

CHD27Our

aims

in

these

pts

are

;Keep

ductal

patency

by

PGE

through

asecurevenous

line.Maintane

saturation

75—80

%

in

RA

even

ifventilated

to

avoid

induction

of

CHF

2nd

toincreased

PBF

with

decreasingPVR.Avoid

pulmonary

vasodilation.PDA

dependant

CHD28Avoid

fluid

overload

.Avoid

infection.Early

intervention

or

surgery within

2-7

d.

Provide

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