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conginital
cyanotic
decrease
pulmonary blood
flow
increase
pulmonary blood
flow
aquierd
reumatic heart
disease
non-cyanotic
mixed
L-RT shunt
obstructive
stenosis
TOF
TGA
TAPVR
VSD
Pulmonary
stenosi
tricusped
truncus
arteriosus
HLHS
ASD
aortic stenosis
PDA
coarctation of
aorta
atresia
endocardial
cushion defect
kawasaki disease
infections
( the common complication of them that they are increase the risk of cerebral
Decrease
pulmonary
blood flow Tetralogy
of fallot
Tricuspid
Atresia
Definition
*Due to abnormal
septation of truncus
arteriosus into the
aorta and pulmonary
artery .
*there is 4
abnormalities : VSD ,
pulmonary stenosis ,
overriding aorta and
hypertrophy of right
ventricle .
*most common
cyanotic congenital
heart disease .
* according the
severity of
pulmonary stenosis
it could appear
either in infancy or
at first or second
year of life .
Absence of tricuspid
valve lead to
hypoplastic of right
ventricle .
PDA and VSD is
important for
pulmonary blood flow
and survival .
VSD can occur as a
part of the disease
Treatment of hypoxic
spells :
-O2
-knee chest position
-morphine-sulphate
-a-adrenergic agonist
(phenylephrine )
*palliative shunt
surgery
*complete surgical
repair .
*Severe cyanosis
*single S2
*VSD murmur if VSD
present .
Presence of VSD is
important for the
baby , if it is small or
absent give
prostaglandin E1 , to
keep ductus
arteriosus open .
stages of surgery :
1-blalock taussing
procedure
2-bidirectional glenn
3-fontan procedure
Truncus
arteriosus
Definition
Transposition of
great artery
secondary to
abnormality of
septation of the
truncus arteriosus
characteristic
*most common
cyanotic losion that
present in the
newborn period .
*if there is no
mixing death occur
early in life .
*usually VSD, ASD
,PDA occur as part
of the disease .
If not diagnosed at
birth , the infant
may develop signs
of heart failure .
Clinical Investigation
picture
- cyanosis ( degree
of it depends on
the amount of
mixing.
-queit tachypnea
-single S2
-VSD murmur may
present
-may present with
HF .
- cyanosis ( degree
of it depends on
the amount of
pulmonary blood
flow )
-tachypnea
-cough
-peripheral pulses
are usually
pounding .
-single S2
-may be cystolic
ejection click
-systolic murmur
-signs of HF .
Treatment
- prostaglandin E1
to maintain ductul
patency .
-pallon atrial
septostomy .
-atrial switch
usually done at the
first 2 weeks of life
-anticongistive
drugs
-surgical repair :
VSD closure ,
placement of
conduit between
the RT.ventricle and
pulmonary artery .
Definition
Total
anomalies
pulmonary
venous
return
Disruption of the
development of
normal pulmonary
venous drainage
during 3ed week of
gastation , results
one of 4
abnormalities .
all of pulmonary
viens fail to connect
to the ledt atrium
and return
abnormally via RT
side of the heart .
they may have
supracardiac ,
infracardiac ,
cardiac , mixed
drainage .
characteristic
Clinical picture
Investigation
Treatment
Infant wighout
obstruction :
*ECG > cinsistant
with RT ventricular
volume overload
*CXR>
cardiomrgaly
*Echo
Infants with
obstruction :
*ECG> RT.axis
deviation and
RT.ventricular
hypertrophy
*CXR: normal or
mild enleargment
of the heart , and
varieng degree of
pulmonary edema
that appear as
pneumonia .
( snow man sign
on CXR )
Surgery repair :
common pulmonary
vie nary opend into
the left atrium .
Hypoplastic
left heart
syndrome
Failure of
development of
mitral or aortic valve
or the aortic arch .
Most common
cause of death
from cardiac
defects in the first
month of life .
-sign of HF
-pulses are weak or
absent
-S2 single and load
-no murmur
-cyanosis may be
minimal but low cardiac
output gives a grayish
color to the cool ,
mottled skin .
- prostaglandin E1 .
-correction acidosis
-surgical repair :
norwood procedure
, bidirectional glenn
and fontan
procedure )
Ventricul
ar septal
defect
Definition
characteristic
Clinical picture
Investigati
on
Treatment
Ventricular
septum contain 4
component :
-muscular
septum,
-endocardial
cushion septum,
-supracristal
septum,
-membranous
septum .
VSD occur when
any of these
component fails
to develop
normally.
*most common
congenital heart
disease .
*peri-membranous
VSD are the most
common of all VSD.
*large VSD are not
symptomatic at
birth , at first 6 to 8
weeks of life it
decrease , the
amount of shunt
increase , and
symptoms may
develop .
Small VSD
normal in ECG
and CXR .
large VSD:
*ECG > left
atrial and
ventricular
hypertrophy .
*CXR>
cardiomegaly ,
enlargement of
L.Ventricle ,
increase in
pulmonary
blood flow and
increase in
pulmonary
artery
silhouette.
Atrial
septal
defect
Failure of septum
growth , or
excessive
reabsorption of
tissue lead to ASD
.
Patent
ductus
arteriosus
Failure of normal
closure of ductus
arteriosus .
which is
important to
keep open in fetal
There is 3 types :
*septum secundom
defect with the
hole in the region
of the foramen
ovale , is the most
common type .
*septum premium
( partial
arterioventricular
defect ) common in
down syndrome .
* the least
common ASD is the
sinus venous defect
.
Medical management
rarely recommended .
if a shunt is still
present at around 3
years , closure usually
recommended.
many secondum ASD
closed by surgical
device , while
premium and sinus
venous defect require
surgical closure .
Small VSD
normal in ECG
and CXR .
large VSD:
*ECG > vary
from normal to
evidence of left
life , it allows
blood to flow
from pulmonary
artery to the
aorta .
Endocard
ial
cushion
defect
Also called
arterioventricular canal
defect , may br
complete or
partial.
Failure of the
septum to fuse
with the
endocardial
results in
abnormal arterioventricualr valves
as well .
the complete
defect defect
results in
* complete
endocardial
cushion defect are
most commonly in
a child with down
syndrome .
ventricular
hypertrophy
*CXR>full
pulmonary
artery
silhouette , and
increase
pulmonary
vasculature .
Initial management
includes diuretics ,
digoxin , afterload
reduction for
treatment for
treatment of HF .
surgical repair is
required .
premium ASD ,
posterior or inlet
VSD , amd cleft in
the anterior
leaflet of the
mitral and septal
leaflet of the
tricuspid valve
.there may be
arterioventricular valve
insufficiency
Definition
Pulmona
ry
stenosis
Failure of development
in early gestation of the
three leaflet of the
valve , insufficient
reapportion of
infundibular or
insufficient canalization
of the peripheral
pulmonary arteries.
Can be valvular ,
supvavular and
supravalvular .
characte
ristic
Clinical picture
Investigation
Treatment
Mild stenosis:
normal in ECG and
CXR .
moderate to
severe :
*ECG > Rt. axis
deviation and Rt.
ventricular
hypertrophy .
*CXR> heart size
may be normal ,
dilatation of the
main pulmonary
artery .
*Echo
Usually Nothing do
with mild stenosis .
Balloon vavoplasty
.
surgical repair
require if balloon
unsuccessful .
Aortic
stenosis
Failure of development
of the three leaflet of
the valve , insufficient
reapportion of tissue
around the valve .
Can be valvular ,
supvavular and
supravalvular .
* secundom
defect with
the hole in
the region of
the foramen
ovale . is the
most
common
ASD .
* the least
common
ASD is the
sinus venous
defect .
Mild stenosis:
normal in ECG and
CXR .
moderate to
severe :
*ECG > L.
ventricular
hypertrophy .
*CXR> L.
ventricular
hypertrophy ,
dilatation of
ascending aorta or
aortic knop .
*Echo
The degree of
aortic stenosis
frequently
progresses with
growth and age.
Aortic insufficiency
usually develop .
serial follow up
with Echo is
indicated .
Balloon vavoplasty
.
surgical repair
require if balloon
unsuccessful .
Coarcta
tion of
the
aorta
During development of
aortic arch area near
the insertion of the
ductus arteriosus fails
to develop correctly ,
resulting in narrowing
of the aortic lumen .
*Usually
juxta-ductal
in position
Done by Arwa Al-ahmadi , reviewed by shada albogami and Arwa Alahmadi (1436 H )
In infantile
coarctation
marked
cardiomegaly , Rt
ventriculat
hypertrophy and
pulmonary edema
in older children :
l.ventricular
hypertrophy and
midly enlarged
heart .
hypertrophy
Rib-notching may
also be seen in
older children ( > 8
years )
*echo
*IV infusion of
prostaglandin E1 . ,
inotropic agent ,
diuretics and other
supportive care .
balloon
angioplasty
surgical repair
most commonly
perform