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heart disease

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

A) Cyanotic congenital heart disease

( the common complication of them that they are increase the risk of cerebral

thromboembolism and cerebral abscess)

Decrease
pulmonary
blood flow Tetralogy
of fallot

Tricuspid
Atresia

Definition

characteristic Clinical picture Investigation Treatment

*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

-Hypoxic (tet) spells .


rapid and deep
breathing
- cyanosis ( degree of
it depends on
pulmonary stenosis)
-right ventricle
impulse
-single S2

*ECG > RT.axis


deviation and
RT.ventricular
hypertrophy
*CXR> Boot shaped
heart sign
*Echo .

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 .

*ECG > L.ventricle


hyprertrophy , sup
QRS axis .
Rt. Atrial
hypertrophy.
*CXR> normal or
mild enlargement
of the heart.
*Echo

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

Cyanotic congenital heart disease


increase
pulmonary
blood flow Transposition
of great
artery

Truncus
arteriosus

Definition
Transposition of
great artery
secondary to
abnormality of
septation of the
truncus arteriosus

Result from failure


of septation of the
truncus arteriosus
during first 3-4 week
of gastation .
single arterial trunk
arises from the
heart with a large
VSD immediately
below the trunk
valve .

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

*ECG > RT.axis


deviation and
RT.ventricular
hypertrophy
*CXR> egg on a
string sign
*Echo

- prostaglandin E1
to maintain ductul
patency .
-pallon atrial
septostomy .
-atrial switch
usually done at the
first 2 weeks of life

*ECG > combined


ventricular
hypertrophy and
cardiomegaly .
*CXR> displased
pulmonary artery .
*Echo

-anticongistive
drugs
-surgical repair :
VSD closure ,
placement of
conduit between
the RT.ventricle and
pulmonary artery .

Cyanotic congenital heart disease


Mixed

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

The most important


determinant of
presentation is the
presence or absence of
obstruction to the
pulmonary venous
drainage . infant without
obstruction have
minimal cyanosis and
nay be asymptomatic .
-hyperactive RT ,
ventricular impulse .
-widely split S2 .
-systolic ejection
murmur at the left
sternal border .
- mid-diastolic murmurs
at the lower left sternal
border
-poor of growth
infants with obstruction
present with cyanosis ,
marked tachypnea , and
dyspnea , and sighn of
RT. Side heart failure
including hepatomegaly .

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 .

*ECG > RT.


Ventricular
hypertrophy ,
decreased left
ventricular forces .
*CXR>
cardiomegaly with
pulmonary vonous
congestion or
pulmonary edema
.
*Echo

- prostaglandin E1 .
-correction acidosis
-surgical repair :
norwood procedure
, bidirectional glenn
and fontan
procedure )

B) ACyanotic congenital heart disease


Left to
right
shunt

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 : little shunt so it may


asymptomatic but have a loud
murmur .
Had 2 important sign :
(important in OSCE)
1- thrill at left lower sternal
border .
2- loud pan systolic murmur ar
left lower sternal border .
moderate to large VSD : results
in pulmonary over circulation
and HF .
Presentation is usually early
and can present at 1 week of
age with sign and symptoms of
Heart failure :
Breathlessness during feeding
Poor feeding
Sweating during feeding
,Failure to thrive
,Tachypnoea
,Tachycardia
Hepatomegaly
-Recurrent chest infection one
of the presentation
*large shunt increase flow

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.

Small : no need for


treatment , up to 90%
it will close
spontaneously .
initial treatment of
moderate to large VSD
include ; diuretics ,
digoxin and afterload
reduction .
most of it close
surgically
All children with VSD
must be given
antibiotic prophylaxis
to prevent bacterial
endocarditis

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
.

across mitral valve so middiastolic murmur at the apex


may be heard ( Soft or no
systolic murmur ).
* Parasternal Thrill
*there may found splitting of S2
and intensity of P2.
-even with large ASD infants are
rarly symptomatic .
-prominent RT.ventricular
impulse ( at the left lower
sternal border)
2 most important sign :
(important in OSCE)
1-systolic ejection murmur .
Soft murmur at upper left
sternal edge .
2-fixed split S2 .
-large shunt may result in a middiastolic murmur at left lower
sternal border .

Small PDA asymptomatic


moderate to large PDA : shunts
can produce the symptoms of
heart failure .
exam. Finding :
2 most important sign :
(important in OSCE)

*ECG > RT.axis


deviation and
RT.ventricular
hypertrophy .
*CXR>
cardiomegaly
and prominent
pulmonary
artery .

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

In Preterm Baby: I/V


indomethacin or Oral
Brufen if no
contraindication to
these medications
If Failed Medical
:Treatment

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 .

1-widened pulse pressure


(Bounding pulse , or collapsing
pulse )
2-continuous , machine like
murmur ( can be heard over
mid-clavicular area .
-large shunts can cause middiastolic murmur over mitral
area ( at the apex ) and hyper
dynamic pericardium .
-thrill may be palpable splitting
S2 and intensity of P2.
-FTT
-symptom of HF usually
develop.
-symptoms may be earliar and
more severe with significant
arterio-ventricular valve
insufficiency.
-presence of murmur varies
depending on the amount of
shunting .
-if there is large VSD , single S2
may be found .
- poor growth of child.

ventricular
hypertrophy
*CXR>full
pulmonary
artery
silhouette , and
increase
pulmonary
vasculature .

Small PDA can be


closed in Cardiac Cath
Lab with COIL at 1
year
If Large and S/S of
Failure to thrive or
Pulmonary HTN
Surgical Ligation at 3-5
month

*ECG > L. axis


deviation and
combined
ventricular
hypertrophy.
*CXR>
cardiomegaly
with increased
vascularity .
*Echo (
diagnostic test )

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

ACyanotic congenital heart disease


Obstructi
ve
stenosis

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 pulmonary stenosis :


asymptomatic .
moderate to severe stenosis :
exertional dyspnea , easy
fatigability .
newborn with severe stenosis
may be cyanotic because right to
left shunt at the atrial level .
- systolic ejection murmur at the
second left intercostal space with
radiates to the back .
-thrill may be present .
-S2 may be widely split .
- in severe stenosis there may be
RT.ventricular impulse .
-valvular stenosis may result click
that varies with respiration .

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 to moderate stenosis :


asymptomatic .
severe stenosis : exertional
dyspnea , exertional chest pain
and syncope.
newborn with critical stenosis
may present with symptoms of
HF .
- systolic ejection murmur at the
right second intercostal space
with radiates to the neck .
-systolic ejection click often is
heard .
-thrill may be present at the .
-the aortic component of S2 may
be decreased in intensity

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

Symptoms develop when aortic


ampula of the ductus closes .
In infantile coarctation
symptoms : poor feeding ,
respiratory distress , shock may
develop before 2 weeks of life .
classicaly the femoral pulses are
weeeker and delayed compared
with the right radial pulse ( radiofemoral delay )
- there may be no murmur but S3
is often present .
older children may be
asymptomatic of have leg
discomfort during exercise ,
headache , or epistaxis .
Arterial hypertension in the
arms with low blood pressure in
the lower extremities is classic
and weeker pulse in femoral
artery ( radio-femoral delay )
the murmur is typically best
heard in the left inter-scapular
area of the back .
if there is abnormal aortic valve ,
there is systolic ejection murmur
and systolic ejection click .

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

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