CONGENITAL HEART

DEFECTS
1. Congenital heart defects are structural defects of the heart, great vessels,
or both that are present from birth. The incidence of CHD is 5 to 8/1,000 live
births.
2. Children with CHD are more likely to have associated defects, such as
tracheoesophageal fistula and chromosomal defects.
3. CHD is second only to prematurity as a cause of death on the first year of
life.

CLASSIFICATION
OF CHD
ETIOLOGY
The exact cause is unknown.
1. Result from faulty development of
the heart structure in the utero.
Acyanotic Heart Disease(Left to Right
2. Teratogenic effects, i.e. taking drugs,
Shunt)
which are dangerous to fetus especially
during the first trimester. When the 1. There is shunting of oxygenated blood
placental barrier is still incomplete and from the left side of the heart back to the
different body organs are developing. right side of the heart. It is true that only

oxygenated blood enters the systemic
circulation but because of the pressure of
the defect that permits passage of blood
from left-sided chamber to the left-sided
cardiac chamber oxygenated blood goes
back to the right side of the heart and
2. Thus in an effort of the heart to there is now less oxygen blood being
compensate for the diminished delivered to the body
amount of oxygenated blood being
delivered to the body, the heart now
pumps much harder than usual.
Eventually, the left sided of the heart
will “get tired” and so will not work
anymore (heart failure). At the same
time, the right of the heart will be
subjected to a pressure that is 3. In acyanotic disease, usually the first
greater than normal. This condition sign in children is refusal to feed or suck
will eventually lead to what is known because of easy fatigability when
as CONGESTIVE HEART FAILURE. sucking or feeding
4. Patent Ductus Arteriosus (PDA)
5. Atrial Septal Defects
6. Ventricular Septal Defects
7. Coarction of Aorta

Cyanotic Heart Disease (Right-to-

Left Shunt)
1. Indicates an abnormality that
permits some of the systemic
OTHER FOUR
venous return (unoxygenated
CLASSIFICATION
blood) to bypass the lungs and
OF CHD
enter general circulation directly.
2. Child frequently squats to
relieve dyspnea as the earliest 1. Defects with increase pulmonary
symptom in cyanotic disorder blood flow- PDA, atrial and ventricular
▪ Tetralogy of Fallot septal defects, and atrio ventricular
▪ Transposition of Great Arteries canal defect
2. Obstructive defects- coarctation of
aorta, aortic stenosis, pulmonic stenosis
3. Defects with decrease pulmonary
blood flow- tricuspid atresia and
tetralogy of fallot
4. Mixed defects- transposition of great
vessels, truncus arteriosus, hypolplastic
left heart syndrome.
 PATENT DUCTUS
ARTERIOSUS
➢There is an opening between the pulmonary artery and the aorta.
➢ Caused by failure of ductus to closed after birth.
➢ Remember: functional closure of the ductus normally occurs soon

ALTERED
PHYSIOLOGY
During fetal life, the ductus arteriosus
allows most of the right ventricular
blood to bypass the non-functioning
lungs by directing blood from the
pulmonary artery to the aorta.
SIGNS AND
SYMPTOMS
1. Continues machinery – like
murmur at the left intraclavicular
area – pathognomonic sign of PDA
2. Prominent radial pulses in the NB,
2. After the initiation of respiration, the
ductus arteriosus is no longer necessary.
It should functionally close within several
hours after birth and anatomically closed
within several weeks after birth and it
becomes ligamentum arteriosus.
3. When the ductus remains patent, the
oxygenated blood from the
higherpressure systemic circuit (aorta)
flows to the lower pressure pulmonary
circuit (pulmonary artery) through the
PDA.
4. The volume of blood that the heart
must pump to meet the demand of
peripheral tissues is increased. A greater
volume burden is placed on the lungs and
eventually on the left heart.

widened pulse pressure & bounding

pulse
3. limited growth and physical
activity Laboratory and diagnostic study
4. fatigue 1. Electrocardiogram (ECG) and chest x-ray –

5. hepatosplenomegaly may show ventricular enlargement

MANAGEMENT: ligate PDA at 3-4 years of age
6. Tachypnea & irritability
➢ PROGNOSIS: without surgery, life expectancy
7. child may have frequent
is shortened because of common complications
respiratory infections
COMPLICATIONS:
1. CHF
2. Endocarditis

NURSING
▪ ADDITIONAL INFORMATION: said to be the
most common anomaly in children born to
MANAGEMENT
mothers exposed to rubella during pregnancy

1. Provide family teaching about treatment

options for PDA
▪ Some PDA close spontaneously others
can close surgically or non surgically
▪ surgical closure is accomplished by one
or two methods
• Left thoracotomy with pulmonary
bypass
In premature neonates, and some
• Visual-assisted thorascopic surgery,
other neonates, PDA sometimes can
which eliminates the need for
be closed using prostaglandin
thoracotomy
synthetase inhibitors (e.g.,
indomethacin), which stimulate
closure of the ductus arteriosus.

2. Provide preoperative and post-

operative care
 ATRIAL SEPTAL
DEFECT (ASD)
➢ is an abnormal opening in the septum between the left atrium and the
right atrium allowing blood to shunt from the left to right
➢ Condition caused by delayed or incomplete closure or foramen ovale
or atrial septum

SIGNS AND
SYMPTOMS
Symptoms vary with the size of the defect
1. history of frequent respiratory infection
2. dyspnea on mild exertion
3. easy fatigability
4. slow weight gain
5. Systolic ejection murmur may be
auscultated, usually most prominent at the
second intercostals space.

Laboratory and Diagnostic Study

Findings
1. Echocardiography with Doppler
generally reveals the enlarged right
side of the heart and increased
pulmonary circulation.
2. Cardiac catheterization NURSING
demonstrates the separation of the MANAGEMENT
right atrial septum and the
increased oxygen saturation in the
right atrium 1. Provide family teaching about
treatment options for atrial septal
defect.
▪ Surgical closure or patch
requires cardiopulmonary bypass
& usually is performed during the
school-age years.

Closure via cardiac

catheterization at some
centers

2. Provide preoperative and

postoperative care.
 VENTRICULAR SEPTAL
DEFECT (VSD)
➢Is an abnormal opening in the ventricular septum between the right
and left ventricles allowing shunting of oxygenated blood in right
ventricle
➢ Results from inadequate development of septal tissue during fetal life,
the most common of all congenital HD.
➢ The degree of this defect may vary from a pinhole between the right
& left ventricles to an absent septum.

TYPES

1. Low-septum – defect is small, may

spontaneously close, no treatment is
needed
2. High-septum – larger defect, does
not close spontaneously (Silastic – a
plastic patch to close the defect is
applied early to prevent pulmonary
hypertension)

SIGNS AND
SYMPTOMS

1. bradycardia

2. slowing of growth pattern

3. feeding difficulties, increased

fatigue

Laboratory and Diagnostic Study

4. frequent respiratory infections
findings
1. Echocardiography with Doppler
5. excessive sweating
ultrasound or magnetic resonance
imaging reveals right ventricular
6. Failure to thrive
hypertrophy and possible pulmonary
artery dilatation from the increase blood
flow
NURSING
2. ECG shows right ventricular
MANAGEMENT
hypertrophy.

1. Provide family teaching about

treatment options for ventricular
septal defect

a. Some ventricular septal defects b. Others are closed with a Dacron

close spontaneously patch requiring cardiopulmonary
bypass. This is recommended for
children with large defects, pulmonary
arterial hypertension, heart failure,
recurrent respiratory tract infections, &
failure to thrive
c. It is important to note that surgery is
complex & that pulmonary artery
banding may be done as a palliative
procedure for infants who are poor
surgical candidates.
 ATRIOVENTICULAR
CANAL DEFECT (AV)
1. An AV defect results from the incomplete fusion of endocardial
cushions
2. It consists of a low atrial septal defect that is continuous with a high
ventricular septal defect & clefts of the mitral & tricuspid valves, creating
a large central AV valve that allows blood to flow between all heart
chambers

PATHOPHYSIOLOGY
1. Alterations in hemodynamic depend on
the severity of the defect & the child’s
pulmonary vascular resistance, which is
usually high in the neonate'
2. Once pulmonary vascular resistance
drops, left-to-right shunting occurs,
increasing pulmonary blood flow &
CLINICAL
MANIFESTATION
1. The child usually has moderate to severe
heart failure
2. Mild cyanosis that increase with crying
3. Characteristics murmur exists
4. Patients are at high risk for developing
pulmonary vascular obstructive disease.

resulting in pulmonary vascular LABORATORY

AND
engorgement and, possibly heart failure. DIAGNOSTIC STUDY
FINDINGS
1. Echocardiography confirms the
diagnosis.

SURGICAL

TREATMENTS
1. Palliative: Pulmonary artery
NURSING
banding is occasionally done in
MANAGEMENT
small infants with severe symptoms
2. Complete repair: Surgical repair
consists of the septal defects &
reconstruction of the AV valve 1. Provide family teaching
tissue (either repair of the mitral about treatment options for
valve cleft or fashioning of the two AV canal defect
valves) 2. Provide preoperative and
post-operative care

Post-operative Complication
1. Heart block
2. CHF
3. Mitral regurgitation
4. Pulmonary hypertension
Prognosis
1. Operative mortality is less than
5%
2. Potential later problem is mitral
regurgitation which require valve
replacement
 COARCTATION OF THE
AORTA
1. Is a defect that involves a localized narrowing of the aorta
2. There are three types of coarctation of aorta, depending on location.
a. Preductal or infantile coarctation of aorta is proximal to the insertion of the
ductus arteriosus; constriction exists between the subclavian artery & ductus
arteriosus
b. Postductal coarctation of aorta is distal to the ductus arteriosus
c. Justductal coarctation of aorta is at the insertion of the ductus arteriosus

2. Increase pressure in the upper

PATHOPHYSIOLOGY portion or the body because increase

1. Coarctation of aorta is
characterized by increased pressure
proximal to the defect and decreased
pressure to it.
Clinical Manifestations
1. The child may be asymptomatic or any
experience the classic difference in blood
pressure & pulse quality between the
upper & lower extremities
a. Absent femoral pulse – pathogonomic
sign
b. BP is higher in the upper extremities –
normally, systolic BP is 20-40 mmHg
higher in the lower extremities than
upper extremities because of peripheral
vascular resistance, the reverse is true in
COA.
pressure in the subclavian artery;
decreased blood pressure in the lower
extremities
3. Restricted blood flow through the
narrowed aorta increases the
pressure on the left ventricle & causes
dilatation of the proximal aorta & left
ventricular hypertrophy which may
lead to left-sided heart failure
4. Eventually collateral vessels
develop to bypass the coarctated
segment & supply circulation to the
lower extremities.
c. Because of higher BP in the upper
extremities:
• Headache & epistaxis are common
• Pulse in the upper extremities are rapid
& bounding
d. Because of lower BP in the lower
extremities:
• Leg pains on exertion • Cold feeT •
Muscle spasms • Weak pulse, delayed or
absent e. A systolic murmur may be
heard over the left anterior chest &
between the scapula posterior

➢ Laboratory and Diagnostic

study findings

NURSING
1. ECG, echocardiography, and MANAGEMENT
chest radiography may reveal an
enlarge left side of the heart
resulting from back pressure 1. Provide family teaching about
2. The radiograph may also treatment options for coarctation of
demonstrate rib notching from
aorta
enlarged collateral vessels.
a. Repair involves surgical removal of
the stenotic area or enlargement of
the constricted section with a graft;
bypass surgery is not necessary
because repair takes place outside of
the heart
b. Nonsurgical repair via balloon
angioplasty is performed.
 AORTA STENOSIS

1. Is a defect that primarily involves an obstruction to the left ventricular

outflow of the valve
2. Narrowing or stricture of the aortic valve, causing resistance to blood
flow in the left ventricle, decrease cardiac output, left ventricular
hypertrophy & pulmonary vascular congestion.

PATHOPHYSIOLOGY
3. Left atrial pressure increase; this
1. A stricture in the aortic outflow tract
causes increased pressure in the
causes resistance to ejection of blood from
pulmonary veins, which results in
the left ventricle
pulmonary vascular congestion
2. Left ventricular pressure increases to
(pulmonary edema)
overcome resistance of the obstructed

valve and allow blood to flow into the

aorta, eventually producing left ventricular

4. Myocardial ischemia may
hypertrophy
develop as the increased oxygen
demands of the hypertrophied left
ventricle

➢ Clinical Manifestations
1. The child with severe defect
demonstrates
a. Faint pulse NURSING
MANAGEMENT
b. Hypotension
c. Tachycardia
d. Poor feeding pattern 1. Provide family teaching about
treatment option for aortic
2. The child may experience signs
stenosis
of exercise intolerance, chest pain &
a. If the child’s symptoms warrant,
dizziness when standing for long
surgical aortic valvulotomy or
prosthetic valve replacement is
necessary
b. Balloon angioplasty can be
used to dilate the narrow valve 2.
Provide preoperative and
3. A systolic ejection murmur may be
postoperative care
hear best at the second intercostal

space
4. There is characteristics murmur 5.
Patients are at risk for bacterial
endocarditis, coronary insufficiency,
and ventricular dysfunction
➢ Laboratory and Diagnostic Study
Findings
1. ECG or echocardiography reveals left
ventricular hypertrophy
2. Cardiac catheterization demonstrates
the degree of the stenosis
 PULMONIC STENOSIS

1. Is a defect that involves obstruction of blood flow from the right

ventricle
2. Narrowing at the entrance to the pulmonary artery
3. Pulmonary artreia is the extreme form of pulmonic stenosis. There is
total fusion of the commissures & no blood flow to the lungs.

PATHOPHYSIOLOGY
3. If PS is severe, CHF occurs &
1. When pulmonic stenosis present,
systemic venous engorgement will
resistance to blood flow causes right
be noted
ventricular hypertrophy

2. Right atrial pressure increase & this may

4. An associated defect PDS
result in reopening of the foramen ovale,
partially compensates for the
shunting of unoxygenated blood into the
obstruction by shunting blood from
left atrium & systemic cyanosis.
the aorta to the pulmonary artery &
into the lungs

➢ Clinical Manifestations
1. The child may be asymptomatic
or may have mild cyanosis or heart
failure TREATMENT
2. A systolic murmur may be heard
over the pulmonic area; a thrill may
be heard if stenosis is severe 1. Nonsurgical Treatment:
3. In severe cases, decreased a. Balloon angioplasty in the cardiac
exercise tolerance, dyspnea, catheterization laboratory to dilate
precordial pain, and generalized the valve
cyanosis may occur 2. Surgical Treatment
a. In infants, transventricular
(closed) valvotomy (Brock
procedure)
b. In children, pulmonary valvotomy
with cardiopulmonary by pass

➢ Laboratory and Diagnostic

Study Findings

1. ECG or echocardiography reveals

right ventricular hypertrophy.
2. Cardiac catheterization
demonstrates the degree of the
stenosis.
 TRICUSPID ATRESIA

1. The tricuspid valve failed to develop; consequently there is no

communication from the right atrium to the right ventricle.
2. Blood flows through an atrial septal defect (ASD) or a patent foramen
ovale to the left side of the heart & through a VSD to the right ventricle &
out of the lungs.
3. The condition is of often associated with pulmonic steno

PATHOPHYSIOLOGY 2. The patent ductus arteriosus allows

blood to flow to the pulmonary artery
1. At birth the presence of a patent
into the lungs for oxygenation.
foramen ovale (or other atrial septal
3. A VSD allows blood to enter the
opening) permit blood flow across the
right ventricle & pulmonary artery for
septum into the left atrium.
oxygenation.
4. Pulmonary blood flow is usually
diminished.

➢ Clinical Manifestation
1. Cyanosis
2. Tachycardia
3. Dyspnea
4. Older children have signs of chronic
hypoxemia with clubbing
➢ Laboratory and diagnostic study
1. Echocardiography reveals the defect

➢ Therapeutic management
1. For the neonate whose pulmonary
SURGICAL
blood flow depends on the patency of TREATMENT
the ductuc arteriosus, a continous
infusion of prostaglandin E is started at
0.1 of body weight per unit surgical
intervention. 1. Palliative treatment:
a. This is the placement of a shunt
(pulmonary-to-systemic artery
anastomosis) to incraese blood flow
to the lungs.
b. If ASD is small, an atrial septostomy
is performed during catheterization.

c. Children with pulmonary blood flow

require pulmonary artery banding to
lessen the volume of blood to the lungs.
d. A bidirectional Glen shunt
(cavopulmonary anastomosis) may be
performed at 4 to 9 months as a second
stage
2. Modified Fontan procedure
a. Systemic venous return is directed to
the lungs without a ventricular pump
through surgical connections between
the right atrium & the pulmonary artery.
 TRANSPOSITION OF THE
GREAT (ARTERIES)
VESSELS
➢ Is a congenital heart defect in which the two major vessels that carry blood
away from the heart – the aorta and the pulmonary artery are switched
(transposed).
➢ The condition is the second most common cyanotic heart defect.

PATHOPHYSIOLOG
Y
CAUSES
1. The cause of most congenital heart
defects is unknown.
2. Factors in the mother that may 1. In normal hearts, blood that returns
increase the risk of this condition include: from the body goes through the right side
a. Age over 40 b. Alcoholism c. Diabetes of the heart and pulmonary artery to the
d. Poor nutrition during pregnancy lungs to get oxygen. The blood then
(prenatal nutrition) e. Rubella or other
comes back to the left side of the heart
viral illness during pregnancy
and travels out the aorta to the body.
2. In transposition of the great vessels,
the blood goes to the lungs, picks up
oxygen, and then goes right back to the
lungs without ever going to the body.
3. Blood from the body returns to the
heart and goes back to the body without
ever picking up oxygen in the lungs.
➢ Signs and Symptoms
1. Cyanosis
2. Low Apgar score at birth
3. Fatigability
4. Slow weight gain
5. Clubbing of the fingers and toes
6. Tachypnea
7. Failure to thrive
➢ Exams and Tests Tests often include the following:

1. The health care provider may detect a a. Cardiac catheterization

heart murmur while listening to the chest b. Chest x-ray

with a stethoscope. The baby's mouth c. ECG

and skin has blue color. d. Echocardiogram (if done before

birth, it is called a fetal

echocardiogram) e. Pulse oximetry
(to check blood oxygen level)

TREATMENT

1. The baby will immediately

receive a medicine called
Prostaglandin E through an IV
(intravenous line). This medicine
2. A procedure using cardiac
helps keep the ductus arteriosus
catheterization (balloon atrial
open, allowing some mixing of the
septostomy) may be needed to create a
two blood circulations.
large hole in the atrial septum to allow
blood to mix.
3. A surgery called an arterial switch
procedure is used to permanently
correct the problem within the baby's
first week
of life. This surgery switches the great
arteries back to the normal position and
keeps the coronary arteries attached to
the aorta
 TOTAL ANOMALOUS PULMONARY
VENOUS RETURN/ TOTAL
ANOMALOUS PULMONARY VENOUS
DRAINAGE
1. Rare defect characterized by failure of the pulmonary veins to join the left
atrium, instead the pulmonary veins are abnormally connected to the systemic
venous circuit via the right atrium or various veins draining toward the right
atrium, such as the superior vena cava.
2. The abnormal attachment results in mixed blood being returned to the right
atrium & shunted from the right to the left through an atrial septal defect.

CLASSIFICATION PATHOPHYSIOLOG
Y
1. Supracardiac – attachment above the
diaphragm, such as to the superior vena
cava (most common)
2. Cardiac – direct attachment to the
heart, such as the right atrium or 1. The right atrium receives all the blood
coronary sinus. that normally would flow into the left
3. Intradiaphragmatic – attachment
atrium.
below the diaphragm, such as to the
2. As a result, the right side of the heart
inferior vena cava (most severe form)
hypertrophies, whereas the left side,
especially the left atrium, may remain
small.
3. An associated ASD or patent foramen
ovale allows systemic venous blood to
shunt from the higher-pressure right
4. As a result , the oxygen saturation of the atrium to the left atrium & into the left side
blood in both sides of the heart is the of the heart
same. 5. If the pulmonary blood flow is
large, pulmonary venous return is also
large, & the amount of saturated blood is
relatively high. 6. If there is obstruction to
pulmonary venous drainage, pulmonary
venous pressure rises, & the pulmonary
interstitial C edema develops & eventually
contributes to CHF, infradiaphragmatic
TAPV is associated with obstruction to
pulmonary venous drainage & is a surgical
emergency

CLINICAL
MANIFESTATION

1. Cyanosis in early life

2. Cardiac failure

Surgical Treatment
1. Corrective repair in early infancy – the
pulmonary vein is anastomosed to the
back of the left atrium, the ASD is
closed, & the anomalous pulmonary vein
venous connection is ligated.
➢ Postoperative complication
1. Re-obstruction
2. Bleeding
3. Dysrhythmias
4. Pulmonary artery hypertension
5. Heart failure
 TRANCUS ARTERIOSUS

1. Is the failure of normal septation & division of the embryonic bulbar

trunk into the pulmonary artery & aorta, which results in development of
a single vessels that overrides both ventricles.
2. Blood from both ventricles mixes in the common great artery, which
leads to desaturation & hypoxemia.

TYPES PATHOPHYSIOLOGY
1. Blood ejected from the left & right
1. Type I – a single pulmonary trunk arises
ventricles enters the common artery &
near the base of the truncus & divides into
flows either into the lungs or the aortic
the left & right pulmonary arteries.
arch, so that pulmonary & systemic
2. Type II – the left & right pulmonary
circulations are mixed.
arteries arise separately but in close
2. Blood flows is distributed to the
proximity at the same level from from the
pulmonary & systemic circulations
back of the truncus.
according to the relative resistance of
3. Type III – the pulmonary arteries arise
each system.
independently from the sides of the
3. Pressure in both ventricles is high &
truncus.
blood flow to the lungs is markedly
increased.

CLINICAL
MANIFESTATIONS
1. Severe pulmonary edema & heart
failure
2. Marked cyanosis, especially on
exertion
3. Left ventricular hypertrophy,
dyspnea, marked activity
intolerance & growth retardation
4. Loud systolic murmur best heard
at the lower left sternal border &
radiating throughout the chest.
LABORATORY &
SURGICAL
MANAGEMENT
1. Early repair is performed in the
first month of life. It involves closing
the VSD so that the truncus
arteriosus receives the outflow
from the left ventricle, & existing
the pulmonary arteries from the
aorta & attaching them to the right
ventricle by means of homograft.
2. Without surgery children die
within 1 year.

DIAGNOSTIC STUDY
FINDINGS

1. Echocardiography
 HYPOPOLASTIC LEFT
HEART SYNDROME
1. Is a disorder that results in underdevelopment of the left of the heart &
involves various left-sided defects, commonly including severe
coarctation of aorta, severe aortic valvular stenosis or atresia.
2. The left ventricle, aortic valve, mitral valve, & ascending aorta usually
are small or hypoplastic.

PATHOPHYSIOLOGY
2. The amount of blood flow to the
1.. An ASD or patent foramen ovale allows
pulmonary & systemic circulations
saturated blood from the left atrium to mix
depends on the relationship
with desaturated blood from the right
between the pulmonary & systemic
atrium & to flow through the right ventricle
vascular resistances. The coronary
& out into the pulmonary artery, the blood
& cerebral vessels receive blood
flows both to the lungs & through the
retrograde flow through the
ductus areriosus into the aorta & out to the
hypoplastic ascending aorta.
body.

➢ Clinical Manifestations
1. Usually evident by the first 2
weeks after birth.
a. Mild cyanosis NURSING
MANAGEMENT
b. Pulmonary edema
c. Single second heart sound
d. Soft systolic ejection murmur 1. Provide family teaching about

treatment options for HLHS.
➢ Laboratory & diagnostic study a. Surgical repair (Norwood
1. Echocardiogaphy reveals the
procedure) is performed in
defect
stages & has a mortality greater
than 25%
b. cardiac transplant has a
mortality greater than 25%

c. without intervention, the defect

is fatal; neonates rarely survive
longer than 1 month.
d. Infants are maintained on
prostaglandin E1, which will delay
the closure of the ductus
arteriosus until a decision about
treatment is reached.