CONGENITAL

HEART
DISORDERS
SUBMITTED BY: LAPUT, HANNAH C.
BSN 2 – F

WMSU

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Congenital Heart Disorders
•About 8% of term newborns are born
with a congenital cardiovascular
abnormality (Fulton & Freed, 2008). This
rate is even higher in preterm infants.
•Affect equal numbers of male and
female infants, but specific defects
show a tendency toward sex
differences.
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Congenital Heart Disorders
•The usual case of CHDs is failure of a
heart structure to progress beyond an
early stage of embryonic development.
•Maternal rubella is an example of an
infection known to lead to CHDs
•Atrial and ventricular septal defects can
also be familial.

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Classification
•Formerly, congenital heart disorders
were classified based on the
physical sign of cyanosis, or these
disorders were classified as either
cyanotic or acyanotic disorders.

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Classification
Acyanotic heart disease
• Involves heart or circulatory
anomalies that involve
either a stricture to the flow
of blood or a shunt that
moves blood from the
arterial to the venous
system (oxygenated to
unoxygenated blood, or left-
to-right shunts).
Cyanotic heart disease
• Occurs when blood is
shunted from the venous to
the arterial system as a
result of abnormal
communication between
the two systems
(deoxygenated blood to
oxygenated blood, or right-
to-left shunts).
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Classification
Acyanotic heart disease Cyanotic heart disease
• These disorders cause the • Occurs when blood is
heart to function as an shunted from the venous to
ineffective pump and make the arterial system as a
the child prone to heart result of abnormal
failure. communication between
the two systems
(deoxygenated blood to
oxygenated blood, or right-
to-left shunts).

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ACYANOTIC
CHDs

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Ventricular Septal Defect
(VSD)
• Most common type of congenital cardiac disorder
seen, accounts for about 30% of all instances of
congenital heart disease, or about 2 in every 1000
live births.
• With this defect, an opening is present in the
septum between the two ventricles. Because
pressure in the left ventricle is greater than that in
the right ventricle, blood shunts from left to right
across the septum.

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Ventricular Septal Defect
(VSD)
• This impairs the effort of the heart because
blood that should go into the aorta and out
to the body is shunted back into the
pulmonary circulation, resulting in right
ventricular hypertrophy and increased
pressure in the pulmonary artery.

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Assessment
• A VSD may not be evident at birth. With
incomplete opening of the alveoli, there is
still high pulmonary artery resistance, so little
blood is shunted through the defect.
• At about 4 to 8 weeks of age, as shunting
begins, the infant demonstrates easy fatigue,
and a loud, harsh pansystolic murmur
becomes evident.
• A thrill (vibration) also may be palpable.
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Diagnosis
•Examination by echocardiography
with colour flow Doppler or MRI
•ECG will also reveal right ventricular
hypertrophy.

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Therapeutic Management
• Up to 85% of VSDs are so small they close
spontaneously (Sondheimer, Yetman, &
Miyamoto, 2008).
• Those that are moderate in size may be
closed during cardiac catheterization.
• Larger ones (over 3 mm) require open heart
surgery. This is usually scheduled before 2
years of age to prevent pulmonary artery
hypertension.
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Atrial Septal Defect (ASD)
• An ASD is an abnormal communication
between the two atria, allowing blood to
shift from the left to the right atrium.
• More common in girls than boys.
• Blood flow is from left to right because of the
stronger contraction of the left side of the
heart. This causes an increase in the volume
in the right side of the heart.

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Atrial Septal Defect (ASD)
• There are two types of ASDs: ostium primum
(ASD1), where the opening is at the lower end of
the septum, and ostium secundum (ASD2), where
the opening is near the centre of the septum.

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Assessment
• A harsh systolic murmur is heard over the second or
third interspace because of the extra amount of
shunted blood that crosses the pulmonic valve.
• As the volume of blood crossing it causes the
pulmonic valve to close consistently later than the
aortic valve, the second heart sound will be
auscultated as split (fixed splitting).
• Echocardiography with colour flow Doppler will
generally reveal the enlarged right side of the heart
and the increased pulmonary circulation.

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Therapeutic Management
• Surgery to close the defect is done electively
between 1 and 3 years of age.
• Closure is important because without it, a child is at
risk for infectious endocarditis and eventual heart
failure.
• It is particularly important that ASDs be repaired in
girls, because they can cause emboli during
pregnancy.
• With uncomplicated surgery, children can expect a
normal quality and length of life.
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Patent Ductus Arteriosus
(PDA)
• The ductus arteriosus is an accessory foetal
structure that connects the pulmonary artery to
the aorta. If it fails to close at birth blood will
shunt from the aorta to the pulmonary artery
because of the increased pressure in the aorta.
• The shunted blood returns to the left atrium of
the heart, passes to the left ventricle, out to the
aorta, and shunts back to the pulmonary artery.

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Patent Ductus Arteriosus
(PDA)
•This causes increased pressure in the
pulmonary circulation from the extra
shunted blood; this leads to right
ventricle hypertrophy and ineffective
heart action.

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Assessment
• Twice as common in girls as boys and occur at a
higher incidence at higher altitudes.
• In preterm infants, the incidence may be as high
as 20% to 60%.
• On physical examination, the child usually has a
wide pulse pressure (the difference between
systolic and diastolic blood pressures).
• A typical continuous “machinery” murmur can be
heard at the upper left sternal border or under
the left clavicle in older children.
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Assessment
•ECG is generally normal, although it may
show ventricle enlargement if the shunt
is large.
•Echocardiography provides good
visualization of the patent ductus.

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Therapeutic Management
• One reason that the ductus arteriosus remains
open in foetal life is stimulation by
prostaglandins, particularly PGE1, from the
placenta and the low oxygen level of foetal blood.
• After birth, when the PGE1 level falls and the
oxygen level increases, the ductus arteriosus is
stimulated to close.
• If it does not close spontaneously, an infant may
be prescribed IV indomethacin or ibuprofen,
prostaglandin inhibitors.
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Therapeutic Management
• If indomethacin is given, assess for possible side
effects, including reduced glomerular filtration,
impaired platelet aggregation, and diminished
gastrointestinal and cerebral blood flow.
• If medical management fails, the disorder can
be closed by insertion of Dacron-coated
stainless-steel coils by interventional cardiac
catheterization when the child is 6 months to 1
year of age.

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Therapeutic Management
• Exceptionally large defects can be closed
surgically by ductal ligation. (Major surgery,
but not open-heart)
• If surgery is not done, the child is at risk for
heart failure and infectious endocarditis
developing from the recirculating blood and
potential stasis in the pulmonary artery.

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Pulmonary Stenosis
• Pulmonary stenosis is narrowing of the
pulmonary valve or the pulmonary artery just
distal to the valve.
• Accounts for about 10% of congenital heart
anomalies.
• Inability of the right ventricle to evacuate
blood by way of the pulmonary artery
because of the obstruction leads to right
ventricular hypertrophy.
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Assessment
• Infants with pulmonary artery stenosis may be
asymptomatic or have signs of right-sided heart
failure.
• If the narrowing is severe, cyanosis may be
present.
• A typical systolic ejection murmur can be heard,
usually loudest at the upper left sternal border.
• A thrill may be present in the upper left sternal
area or at the suprasternal notch.

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Assessment
• The second heart sound may be widely split
because of late closure of the pulmonary
valve.
• An ECG or echocardiography will reveal right
ventricular hypertrophy.

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Therapeutic Management
• Management of the defect depends on the
severity of the stenosis and the child’s age.
• Balloon angioplasty by way of cardiac
catheterization is the procedure of choice.

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Aortic Stenosis
• Stenosis, or stricture, of the aortic valve
prevents blood from passing freely from the
left ventricle of the heart into the aorta.
• Because the heart cannot force blood
through the strictured valve, increased
pressure and hypertrophy of the left ventricle
occur

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Aortic Stenosis
• If the left ventricular pressure becomes
acute, pressure in the left atrium also
increases, resulting in back-pressure in
pulmonary veins and possibly pulmonary
oedema.

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Assessment
• Most children with aortic stenosis are
asymptomatic, but physical assessment generally
reveals a typical murmur, a rough systolic sound
heard loudest in the second right interspace.
• A thrill may be present, particularly at the
suprasternal notch.
• If severe, decreased cardiac output evidenced by
faint pulses, hypotension, tachycardia, and
inability to suck for long periods may be present.

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Assessment
• When the child is active, chest pain similar to
angina occurs, because the coronary arteries
receive inadequate blood.
• Sudden death can occur when the amount of
oxygen needed by the heart muscle on
exertion far exceeds what is available
• ECG or echocardiography will reveal left
ventricular hypertrophy.

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Therapeutic Management
• Stabilization with a beta-blocker or a calcium
channel blocker may be necessary to reduce
cardiac hypertrophy before the defect is
corrected.
• Balloon valvuloplasty is the surgical
treatment of choice.

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Therapeutic Management
• Surgery that involves dividing the stenotic
valve or dilating an accompanying constrictive
aortic ring can be used for severe defects.
• Some children will need artificial valve
replacement for correction.
• If a prosthetic valve is used, children generally
continue to receive anticoagulation or
antiplatelet therapy and antibiotic prophylaxis
against endocarditis.
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Coarctation of the Aorta
• Narrowing of the lumen of the aorta due to a
constricting band.
• Occurs more frequently in boys than in girls
and is the leading cause of congestive heart
failure in the first few months of life.

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Coarctation of the Aorta
• There are two locations in which this
commonly occurs.
• Pre-ductal: t constriction occurs between
the subclavian artery and the ductus
arteriosus
• Postductal: constriction is distal to the
ductus arteriosus

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Coarctation of the Aorta
• Because it is difficult for blood to pass
through the narrowed lumen of the aorta,
blood pressure increases proximal to the
coarctation and decreases distal to it.
• Elevated upper-body blood pressure
produces headache and vertigo.
• Irritability may be the main clue that these
symptoms are present.

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Coarctation of the Aorta
• Epistaxis and cerebrovascular accident can
occur from this dangerously elevated blood
pressure.

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Assessment
• If the coarctation is slight, absence of palpable
femoral pulses may be the only symptom.
• Children who have an obstruction proximal to
the left subclavian artery may have absent
brachial pulses as well.
• As children with coarctation of the aorta grow
older, they may experience leg pain on exertion
because of the diminished blood supply to
their lower extremities.

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Assessment
• Collateral arteries enlarge and may be seen
on the ribs as obvious nodules as the child
grows older.
• On examination, the blood pressure in the
arms will be at least 20 mm Hg higher than in
the legs, a reversal of the normal pattern.
• Echocardiography, ECG, MRI, or x-ray
examination of older children will reveal left-
sided heart enlargement.
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Therapeutic Management
• Management of coarctation of the aorta is by
interventional angiography or surgery.
• With surgery, the narrowed portion of the
aorta is removed and the new ends of the
aorta are anastomosed.
• Many infants with coarctation of the aorta
require therapy with digoxin and diuretics in
the time before surgery can be performed.

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 PRIORITIZED NURSING PROBLEM FOR CONGENITAL HEART DISEASES
Nursing Diagnosis
• Decreased cardiac output related
to structural factors of congenital
heart defect.
Nursing Intervention
• Assess heart rate and blood
pressure.
• Inspect fluid balance and weight
gain. Weigh patient regularly prior
to breakfast.
• Provide adequate rest periods
• Position child in semi-Fowler’s
position.
• Administer oxygen therapy as
prescribed
• Administer medications as
prescribed (Digoxin, Alprostadil)
Rationale
• Most patients have compensatory
tachycardia and significantly low
blood pressure in response to
reduced cardiac output.
• Compromised regulatory
mechanisms may result in fluid
and sodium retention; Weight is an
indicator of fluid balance.
• Rest decreases metabolic rate,
decreasing myocardial and oxygen
demand.
• Upright position is recommended
to reduce preload and ventricular
filling when fluid overload is the
cause; Facilitates lung expansion.
• The failing heart may not be able
to respond to increased oxygen
demands. Oxygen saturation need
to be greater than 90%.
• Increases contractility of the heart
and force of contraction. Maintain
open PDA when needed for blood
flow.
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 PRIORITIZED NURSING PROBLEM FOR CONGENITAL HEART DISEASES
Nursing Diagnosis
• Activity intolerance related to
imbalance between oxygen supply
and demand.
Nursing Intervention
• Assess dyspnea on exertion, skin
colour changes during rest and
when active.
• Allow for rest periods between
care; disturb only when necessary
for care and procedures.
• Provide neutral environmental
temperature; when bathing infant,
expose only the area being bathed
and keep the infant covered to
prevent heat loss.
• Assist parents to plan for care and
rest schedule.
• Inform of activity or exercise
restrictions and to set own limits
for exercise and activity.
Rationale
• Indicates hypoxia and increased
oxygen need during energy
expenditure.
• Promotes rest and conserves
energy.
• Avoids hot or cold extremes which
increase oxygen and energy needs.
• Provides for rest and prevents
overexertion, minimizes energy
expenditure.
• Prevents fatigue while engaging in
activities as nearly normal as
possible
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 PRIORITIZED NURSING PROBLEM FOR CONGENITAL HEART DISEASES
Nursing Diagnosis
• Compromised family coping
related to situational and
developmental crises of family and
child.
Nursing Intervention
• Encourage expression of feelings
and provide factual information
about infant/child.
• Clarify any misinformation and
answer questions regarding
disease process.
• Suggest and reinforce appropriate
coping behaviours, support family
decisions.
• Instruct parents in nutritional and
activity needs and/or limitations
and approaches that will assist in
establishing an effective pattern.
• Refer family for additional support
and counselling if indicated.
Rationale
• Reduces anxiety and enhances
family’s understanding of the
condition.
• Prevents unnecessary anxiety
resulting from inaccurate
knowledge or beliefs.
• Promotes behaviour change and
adaptation to care for infant/child.
• Assists in coping with effects and
special needs of infant/child with a
cardiac defect.
• Referral supplies more assistance
with coping than is available from
nursing personnel.
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