Congenital heart diseases

BY. ABHIJIT
BHOYAR
M SC NURSING

INTRODUCTION
 Congenital heart disease (or defects) (CHD) is one of
the most common forms of congenital anomalies. It
involves
Chambers,
Valves,
Great vessels arising from the heart
The heart is a muscular organ about the size of a fist, located just
behind and slightly left of the breastbone. The heart pumps blood
through the network of arteries and veins called the cardiovascular
system.

 In most cases, the cause of CHD is not known.
 Some infants and children with CHD may appear
perfectly healthy, whereas others may be critically
ill.
 Most infants and children with CHD can be
successfully managed with medications and
surgeries.

ETIOLOGY AND INCIDENCE
 CHD affects 8 to 12 of every 1,000 neonates.
 Exact cause of CHD is unknown in 90% of cases.
The heart begins as a single cell and develops into a
four-chambered pumping system during the third
to eighth weeks of gestation

Conti…
 Associated factors for CHD include:
◦ Fetal or maternal infection during the first
trimester (rubella).
◦ Chromosomal abnormalities (trisomy 21, 18, 13).
◦ Maternal insulin-dependent diabetes.
◦ Teratogenic effects of drugs and alcohol
Teratogens are drugs,
chemicals, or even
infections that can cause
abnormal fetal

Conti…
 Syndromes that include CHD:
◦ Marfan's syndrome: mitral valve prolapse (MVP),
dilated aortic root.
◦ Turner's syndrome: aortic valve stenosis (AVS),
coarctation of the aorta (CoA).
◦ Noonan's syndrome: dysplastic pulmonary valve.
◦ William's syndrome: supravalvular pulmonary
stenosis (PS).

Conti..
◦ DiGeorge syndrome: interrupted aortic arch
(IAA), truncus arteriosus, transposition of great
arteries (TGA), tetralogy of Fallot (TOF).
◦ Down syndrome (trisomy 21): atrioventricular
(AV) canal defect, ventricular septal defect
(VSD). About 50% of children with Down
syndrome have a CHD.

CLASSIFICATION
 Congenital heart defects can be
classified into three categories:
Obstruction to blood
flow
Increased pulmonary
blood flow (acyanotic
lesions)
Decreased pulmonary
blood flow (cyanotic
lesions).
OBSTRUCTIVE
ACYNOTIC
CYNOTIC

CONTI..
Increased
pulmonary blood
flow (acyanotic):
• PDA.
• ASD.
• VSD.
• AV canal.
• PAPVR
Decreased
pulmonary blood
flow (cyanotic):
• TOF.
• TA.
• TAPVR.
• TGA.
• Truncus
arteriosus.
• HLHS.
• DORV.
Obstructive
lesions
• AS,
• CoA.
• PS

AORTIC STENOSIS
 Congenital AS may be
caused by a bicuspid
aortic valve with fused
commissures that does
not open completely.
 The result is turbulent
blood flow across the
aortic valve and into the
ascending aorta.
Aortic stenosis is a
narrowing of
the aortic valve
opening

Conti..
 AS is the most common form of left ventricular outflow
tract obstruction. It accounts for 3% to 6% of
congenital heart defects.
 AS may occur at any age, and it occurs more
commonly in boys than in girls.
 In most children, it is a progressive lesion that creates
LVOTO.
Left ventricular outflow tract
obstruction (LVOTO)

Pathophysiology
 Blood flows at an increased velocity across the
obstructive valve or stenotic area and into the
aorta.
 During systole, left ventricular pressure rises
dramatically to overcome the increased resistance
at the aortic valve

Conti..
 Myocardial ischemia may occur because of an
imbalance between the increased oxygen
requirements related to the hypertrophied left
ventricle (LV) and the amount of oxygen that can
be supplied.
 Left-sided heart failure results in an increased LV
end diastolic pressure that is reflected back to the
left atrium and pulmonary veins

Clinical Manifestations
IN NEONATE
 Severe congestive heart failure (CHF).
 Metabolic acidosis.
 Tachypnea.
 Faint peripheral pulses, poor perfusion, poor
capillary refill, cool skin.
 Poor feeding and feeding intolerance

Conti..
CHILD AND
ADOLESCENT
 Chest pain on exertion,
 Decreased exercise
tolerance.
 Dyspnea,
 Fatigue,
 Shortness of breath.
 Syncope,
 Light-headedness.
 Palpitations.
 Sudden death
Syncope (pronounced
“sin ko pea”) is the
medical term for
fainting or passing out.

Diagnostic Evaluation
 Auscultation.
◦ Systolic ejection murmur heard best at right
upper sternal border, radiates to neck.
◦ Ejection click.
◦ S2 splits normally or narrowly.
 Electrocardiogram (ECG): left ventricular
hypertrophy (LVH) with a strain pattern may be
seen in severe cases.
Ejection
clicks are
high-
pitched
sounds
that occur
at the
moment of
maximal
opening of
the aortic
or
pulmonary
valves.

Conti..
 Chest X-ray: increased cardiac silhouette,
increased pulmonary vascular markings. A
prominent aortic knob may be seen occasionally
from poststenotic dilatation with valvular AS.
 Echocardiogram: two-dimensional
echocardiogram with Doppler study and color flow
mapping to visualize the anatomy and to estimate
the gradient across the valve and through the
aorta.

Management
NEONATE
 Stabilize with prostaglandin E1 (PGE1) infusion to maintain
cardiac output through the PDA.
 Inotropic support as needed.
 Intubation and ventilation as needed.
 Infective endocarditis prophylaxis (lifelong).
 Cardiac catheterization: aortic balloon valvuloplasty or
aortic balloon angioplasty.
 Surgical valvotomy, commissurotomy, or
myectomy/myotomy.

Child and Adolescent
 Medical management with close follow-up to
monitor increasing gradient across the aortic valve
or through the aorta.
 Restrict strenuous exercise and anaerobic exercise
(eg, weight lifting).
 Restrict participation in competitive sports

 Aortic balloon valvuloplasty or aortic balloon
angioplasty.
 Surgical intervention.
◦ Surgical valvotomy, commissurotomy, or myectomy/
myotomy.
◦ Aortic valve replacement.
 Mechanical prosthesis (St. Jude valve).
 Ross procedure (pulmonary autograft).

COMPLICATIONS
- CHF
- Pulmonary
edema
- Dizziness
- Light-
headedness
- Syncope
-Palpitations
-Arrhythmias
- Infective
endocarditis.
- Sudden
death.

COARCTATION OF THE
AORTA
 CoA is a discrete
narrowing or a long
segment hypoplasia of
the aortic arch, usually
in the juxtaductal
position.
 It accounts for 8% to
10% of congenital heart
defects

Pathophysiology
 The discrete narrowing or hypoplastic segment of
the aorta increases the workload of the left ventricle
(increased LV systolic pressure).
 In a neonate with critical CoA, lower body blood
flow occurs through the PDA (right-to-left shunting).
 In the older child, collateral vessels grow and
bypass the coarctation to perfuse the lower body.

 The neonate with critical CoA (ductal
dependent lesion):
◦ Asymptomatic until the PDA begins to close.
◦ After PDA closure: severe CHF, poor lower body
perfusion, tachypnea, acidosis, progressive
circulatory shock, absent femoral and pedal
pulses.

 The child or adolescent with CoA:
◦ Usually asymptomatic normal growth and
development.
◦ Hypertension in the upper extremities, with
absent or weak femoral pulses.
◦ Nosebleeds, headaches, leg cramps.

 Auscultation - varies; nonspecific systolic ejection
murmur.
 Chest X-ray- cardiomegaly and pulmonary edema
or pulmonary venous congestion.
 ECG varies; normal or right ventricular
hypertrophy (RVH) in infants and LVH in older
children.

 Two-dimensional echocardiogram with Doppler
study and color flow mapping identifies area of
aortic arch narrowing and associated lesions
(bicuspid aortic valve, VSD, PDA).
 Invasive studies (cardiac catheterization) usually
not needed to make the initial diagnosis; may need
aortic angiography to identify collateral vessels
before surgery.
 Cardiac magnetic resonance imaging may be
done to noninvasively assess the location and
degree of narrowing and identify collateral vessels.

Management
Critical Coarctation in the Neonate
 Medical management:
◦ Resuscitation and stabilization with PGE1 infusion:
monitor for complications related to PGE1 therapy (fever,
apnea).
◦ Intubation and ventilation as needed.
◦ Infective endocarditis prophylaxis (lifelong).
◦ Anticongestive therapy (digoxin and Lasix) and
inotropic support as needed.
◦ Assess renal, hepatic, and neurologic function.

 Balloon angioplasty may be indicated for infants who are a high surgical
risk.
 Surgical intervention: usually performed as soon as the diagnosis is made.
◦ Subclavian flap repair (Waldhausen procedure).
◦ End-to-end anastomosis.
◦ Dacron patch repair

Coarctation in the Child or
Adolescent
 Surgical intervention.
◦ End-to-end anastomosis.
◦ Dacron patch.
 Medical management for hypertension (beta-
adrenergic blockers).

Recurrent Coarctation in the
Neonate or Child
 Balloon angioplasty in the cardiac
catheterization laboratory.
 Redo surgical intervention

Complications
 Systemic hypertension.
 CHF.
 Cerebral hemorrhage.
 Infective endocarditis.
 Left ventricular failure.
 Aortic aneurysm

PULMONARY STENOSIS
 The pulmonary valve opens
during systole to let blood flow
from the right ventricle into the
main PA.
 Obstruction to flow can occur
at three levels: subvalvular,
valvular, or supravalvular level.
 The most common cause of
RV outflow tract obstruction is
pulmonary valve stenosis.
 PS accounts for 8% to 12% of
CHDs.

Pathophysiology
• Critical PS in the neonate: blood flows into the right atrium, across a
patent foramen ovale (PFO) into the left heart;
• pulmonary blood flow comes from a left-to-right shunt through a PDA.
• Right ventricular pressure increases to pump blood across the
obstructive pulmonary valve.
• RVH develops in response to the increased pressure gradient across
the pulmonary valve.
Signs of right-sided heart failure include hepatic congestion,
neck vein distention, elevated central venous pressure (CVP).

C
L
I
N
I
C
A
L
M
A
N
I
F
E
S
T
A
T
I
O
N
S
• Hypoxia.
• Tachypnea.
• RV failure.
Critical PS
in the
Neonate
• Asymptomatic.
• Decreased exercise
tolerance, fatigue,
dyspnea.
• Chest pain.
Mild to
Moderate
PS in the
Child and
Adolescent

 Auscultation: systolic ejection murmur heard best at
the left upper sternal border; ejection click.
 ECG: varies; normal in mild cases and RVH in
moderate to severe cases.
 Chest X-ray: varies; may show right ventricular
enlargement; poststenotic dilatation of PA.
 Two-dimensional echocardiography with Doppler
study and color flow mapping to visualize the sites of
obstruction, observe the degree of RVH, and estimate
the pressure gradient across the valve.
 Cardiac catheterization is usually not needed for the
initial diagnosis.
D
I
A
G
N
O
S
T
I
C
E
V
A
L
U
A
T
I
O
N

NEONATE WITH CRITICAL PS
◦ Stabilize and improve oxygen saturations with PGE1
infusion.
◦ Intubation and ventilation as needed.
◦ Inotropic support as needed.
 Balloon pulmonary valvuloplasty.
 Blalock-Taussig shunt (Gore-Tex graft between the
subclavian artery and the PA to supply pulmonary blood
flow).
M
A
N
A
G
E
M
E
N
T

Child and Adolescent with PS
◦ Close follow-up to monitor and record RV to PA
gradient and assess RV function.
◦ Restrict strenuous exercise and participation in
competitive sports.
◦ Refer for intervention when RV pressure is
greater than two-thirds of the systemic
pressures.
M
A
N
A
G
E
M
E
N
T

 Balloon pulmonary valvuloplasty in the cardiac
catheterization laboratory.
 Surgical intervention:
◦ Valvotomy or valvectomy for dysplastic
pulmonary valve.
◦ Patch repair of the right ventricular outflow tract.
◦ Placement of an RV-to-PA conduit.
M
A
N
A
G
E
M
E
N
T

COMPLICATIONS
Cyanosis
(critical
PS in the
neonate).
Arrhythmia;
sudden
death.
Right
ventricular
failure
Infective
endocarditis.

PATENT DUCTUS ARTERIOSUS
 The ductus arteriosus is a normal fetal connection
between the left PA and the descending aorta.
 During fetal life, blood flow is shunted away from the
lungs through the ductus arteriosus and directly into
the systemic circulation.
 PDAs are common in premature neonates who weigh
less than 1,500 g.
 They account for 5% to 10% of CHDs, excluding
premature neonates.

P
A
T
H
O
P
H
Y
S
I
O
L
O
G
Y
During fetal life, the ductus arteriosus allows blood to
bypass the pulmonary circulation (fetus receives oxygen
from the placenta) and flow directly into the systemic
circulation
After birth, the ductus arteriosus is no longer needed.
Functional closure usually occurs within 48 hours after
birth. Anatomic closure is completed by age 2 to 3
weeks.
When the ductus arteriosus fails to close, blood from the
aorta (high pressure) flows into the low-pressure PA,
resulting in pulmonary overcirculation.
Increased pulmonary blood flow leads to a volume-
loaded LV.

Small to Moderate-Sized PDA
 Usually asymptomatic.
Large PDA
 CHF,
 Tachypnea,
 Frequent respiratory tract infections.
 Poor weight gain, failure to thrive.
 Feeding difficulties.
 Decreased exercise tolerance.
C
L
I
N
I
C
A
L
M
A
N
I
F
E
S
T
A
T
I
O
N

 Auscultation: continuous murmur heard best at
left upper sternal border. Hyperactive precordium
with large PDAs.
 Wide pulse pressure; bounding pulses.
 Chest X-ray: varies; normal or cardiomegaly with
increased pulmonary vascular markings.
 ECG: varies; normal or LVH.
study and color flow mapping to visualize the PDA
with left-to-right blood flow.
 Cardiac catheterization is not needed for the
initial diagnosis.
D
I
A
G
N
O
S
T
I
C
E
V
A
L
U
A
T
I
O
N

 In the symptomatic premature neonate:
indomethacin given I.V.
◦ Monitor growth and development.
◦ Reassess for spontaneous PDA closure.
◦ Increase caloric intake as needed for normal weight
gain.
◦ Diuretics: furosemide (Lasix), spironolactone
(Aldactone).
◦ Infective endocarditis prophylaxis for 6 months after
surgery or coil occlusion
M
A
N
A
G
E
M
E
N
T

 Cardiac catheterization:
◦ For small PDAs coil occlusion.
◦ For larger PDAs a closure device may be used.
 Surgical management through PDA ligation
M
A
N
A
G
E
M
E
N
T

C
O
M
P
L
I
C
A
T
I
O
N
CHF, pulmonary edema.
Infective
endocarditis.
Pulmonary hypertension/pulmonary
vascular occlusive disease.
Recurrent pneumonia.

ATRIAL SEPTAL DEFECT
 ASD is an abnormal communication between the
left and right atrias.
 ASDs account for 9% of CHDs.

T
Y
P
E
S
O
F
A
S
D
Ostium
secundum
• The most common type of ASD;
abnormal opening in the middle of
the atrial septum
Ostium
primum
• Abnormal opening at the bottom of
the atrial septum; increased
association with cleft mitral valve
and atrioventricular defects.
Sinus
venosus
• Abnormal opening at the top of the
atrial septum; increased association
with partial anomalous pulmonary
venous return.

Blood flows from the higher-pressure
left atrium across the ASD into the
lower-pressure right atrium (left-to-right
shunt).
Increased blood return to the right heart
leads to right ventricular volume
overload and right ventricular dilation.
Increased pulmonary blood flow leads
to elevated pulmonary artery pressures.
P
A
T
H
O
P
H
Y
S
I
O
L
O
G
Y

 Usually asymptomatic.
 Clinical symptoms vary depending on type of
associated defects:
◦ CHF
◦ Frequent upper respiratory infections (URIs).
◦ Poor weight gain.
◦ Decreased exercise tolerance

 Auscultation: soft systolic ejection murmur heard
best at the left upper sternal border; widely split, fixed
second heart sound.
 Chest X-ray: varies; normal to right atrial and
ventricular dilation, increased pulmonary markings.
 ECG: varies; right axis deviation and mild RVH or
right bundle-branch block.

study and color flow mapping to identify the site of the
ASD and associated lesions and document left-to-
right flow across the atrial septum.
 Cardiac catheterization usually not needed for initial
diagnosis; performed if defect can be closed using an
atrial occlusion device (device can be used only in
ostium secundum defects).

Management
◦ Monitor and reassess (spontaneous closure rate is
small but may occur up to age 2).
◦ Treatment with anticongestive therapy (digoxin and
Lasix) may be necessary if signs of CHF are
present.
surgery or atrial occlusion devise is used.

 Cardiac catheterization for placement of an atrial
occlusion device for ostium secundam defects.
 Surgical intervention:
◦ Primary repair: suture closure of the ASD.
◦ Patch repair of the ASD.

Complications
 CHF (rare).
 Embolic stroke.
 Pulmonary hypertension.
 Atrial arrhythmias.

VENTRICULAR SEPTAL DEFECT
 A VSD is an abnormal communication between the
right and left ventricles.
 It is the most common type of congenital heart defect,
accounting for approximately 25% of all CHDs.
 VSDs vary in the size (small and restrictive to large
and nonrestrictive defect), number (single versus
multiple), and type (perimembranous or muscular).

Pathophysiology
 Blood flows from the high-pressure left ventricle
across the VSD into the low-pressure right
ventricle and into the PA, resulting in pulmonary
overcirculation.
 A left-to-right shunt because of a VSD results in
increased right ventricular pressure and increased
PA pressure.
 The increased pulmonary venous return to the left
side of the heart results in left atrial dilation.

 Long-standing pulmonary overcirculation causes a
change in the pulmonary arterial bed, leading to
increased pulmonary vascular resistance.
 High pulmonary vascular resistance (PVR) can
reverse the blood flow pattern that leads to a right-to-
left shunt across the VSD (Eisenmenger's syndrome),
resulting in cyanosis.
 Once this develops, the child is no longer a candidate
for surgical repair.

 Small VSD”usually asymptomatic; high spontaneous
closure rate during the first year of life.
 Large VSDs.
◦ CHF: tachypnea, tachycardia, excessive sweating
associated with feeding, hepatomegaly.
◦ Frequent URIs.
◦ Poor weight gain, failure to thrive.
◦ Feeding difficulties.
◦ Decreased exercise tolerance

 Auscultation: harsh systolic regurgitant murmur
heard best at the lower left sternal border (LLSB);
systolic thrill felt at LLSB, narrowly split S2.
 Chest X-ray: varies; normal or cardiomegaly and
increased pulmonary vascular markings. Pulmonary
vascular markings are directly proportionate to the
amount of left-to-right shunting.
 ECG: varies; normal to biventricular hypertrophy.

study and color flow mapping to identify the size,
number, and sites of the defects, estimate
pulmonary artery pressure, and identify associated
lesions.
 Cardiac catheterization usually not needed for
initial diagnosis; may be needed to calculate the size
of the shunt or to assess PVR. May be performed if
defect can be closed using a ventricular occlusion
device (device can be used only in muscular

Management
Small VSD
◦ Usually no anticongestive therapy is needed.
surgical implantation of a ventricular occlusion
device.
 Cardiac catheterization for placement of a ventricular
occlusion device for muscular defects.
 Surgical intervention is usually not necessary.

Moderate to Large VSD
 Medical Management:
◦ CHF management: digoxin and diuretics
(furosemide, spironolactone) and afterload
reduction.
◦ Avoid oxygen; oxygen is a potent pulmonary
vasodilator and will increase blood flow into the PA.
◦ Increase caloric intake: fortify formula or breast
milk to make 24 to 30 cal/oz formula; supplemental
nasogastric feeds as needed.
◦ Infective endocarditis prophylaxis for 6 months
after surgery/ventricular device occluder.

 Cardiac catheterization for placement of a ventricular
occlusion device for muscular defects.).
 Refer for surgical intervention.
◦ Usually repaired before age 1.
◦ One-stage approach: preferred surgical plan; patch
closure of VSD.
◦ Two-stage approach: first surgery is to band the PA
to restrict pulmonary blood flow; second surgery is to
patch close the VSD and remove the PA band.

CHF.
Frequent
URIs.
Infective
endocarditi
s
Failure to
thrive
Eisenmenger'
s syndrome
Pulmonary
hypertension
.
Aortic
insufficienc
y
COMPLICATIONS

TETRALOGY OF FALLOT
 TOF is the most common complex congenital heart
defect; it accounts for 6% to 10% of all CHDs.
 The four abnormalities of TOF include the following:
A large, nonrestrictive VSD.
Aortic override.
Pulmonary stenosis (right ventricular outflow tract
obstruction).
Right ventricular hypertrophy.

Pathophysiology
• Degree of cyanosis depends on the size of the
VSD and the degree of right ventricular outflow
tract obstruction (RVOTO).
• Obstruction of blood flow from the right ventricle to the PA
results in deoxygenated blood being shunted across the
VSD and into the aorta (right-to-left shunt causes
cyanosis).
• RVOTO can occur at any or all of the following
three levels: pulmonary valve stenosis,
infundibular stenosis, or supravalvular stenosis.

• The right ventricle becomes hypertrophied as a
result of the increased gradient across the RVOT.
• Minimal RVOTO results in a pink TOF variant, with
the physiology behaving more like a large,
nonrestrictive VSD.

 Cyanosis.
◦ Neonate may have normal oxygen saturations; as the
infant grows, the RVOTO increases and the oxygen
saturation falls.
◦ Neonate with unacceptably low oxygen saturation needs
PGE1 infusion to maintain ductal patency and adequate
oxygen saturation.
◦ Cyanosis may initially be observed only with crying and
with exertion.

 Polycythemia.
 Decreased exercise tolerance.
 A common clinical manifestation years ago was
squatting, a posture characteristically assumed
by older children to increase systemic vascular
resistance and to encourage increased
pulmonary blood flow. Squatting is rarely seen
currently because TOF is now surgically repaired
during the first year of life.

 Hypercyanotic spells (formerly known as Tet
spells): a life-threatening hypoxic event with a
dramatic decrease in oxygen saturations.
◦ Typical hypoxic spells occur in the morning soon
after awakening; during or after a crying episode;
during or after a feeding; during painful procedures
such as blood draws.
◦ Typical scenario includes tachypnea, irritability, and
increasing cyanosis, followed by flaccidity and loss of
consciousness.

◦ Home treatment for the caregiver: soothe the infant and
place him or her in a knee-chest position; notify the
health care provider immediately.
◦ Hospital treatment includes knee-chest position,
sedation (morphine), oxygen, beta-adrenergic blockers
(propranolol, esmolol) to relax the infundibulum, and
administration of medications to increase systemic
vascular resistance (phenylephrine).
◦ Hypercyanotic spells usually prompt the cardiologist to
refer for surgical intervention.

 Auscultation: harsh systolic ejection murmur heard
best at the upper left sternal border (RVOT murmur);
single second heart sound; during a hypercyanotic spell
the murmur disappears.
 Chest X-ray: varies; normal or decreased pulmonary
vascular markings. The heart may appear “boot shaped”•
because of a concave main PA with an upturned apex
resulting from RVH.
 ECG: varies; normal or RVH.

 Two-dimensional echocardiogram with Doppler study
and color flow mapping to identify the structural
abnormalities, estimate the degree of RVOTO and
assess the coronary artery pattern.
 Cardiac catheterization is usually not needed for the
initial diagnosis. May be performed before surgical
intervention to identify the location and number of VSDs,
the PVR, the degree of RVOTO, and the presence of
any coronary abnormalities.

Management
 Medical Management:
◦ Monitor oxygen saturation level.
◦ Monitor growth and development.
◦ Monitor for hypercyanotic spells (many spells go
unnoticed by parents).
◦ Restrict strenuous activity and participation in
competitive sports.
 Balloon pulmonary angioplasty (rarely).

 Surgical intervention: Palliative versus definitive
repair.
◦ Potential obstacles for one-stage repair: abnormal
coronary artery distribution (left anterior descending
arises from right coronary artery and crosses RVOT);
multiple VSDs; hypoplastic branch pulmonary
arteries; small infant weighing less than 5.5 lb (2.5
kg).

◦ Palliative surgery: modified Blalock-Taussig shunt
(BT shunt); tube Gore-Tex graft between the left
subclavian artery and the PA: increased pulmonary
blood flow results in higher oxygen saturations.
◦ Definitive surgery: patch closure of VSD, relief of
right ventricular outflow tract obstruction; with or
without transannular patch across the pulmonary
valve.

Long-Term Follow-Up
 Assess RV outflow tract, monitor degree of
pulmonary insufficiency.
 Monitor RV function and exercise tolerance.
 Monitor for arrhythmias.

Complications
 Hypoxia.
 Hypercyanotic spells.
 Polycythemia.
 CHF: rare; associated with pink TOF.
 Right ventricular dysfunction.
 Ventricular arrhythmias.
 Infective endocarditis

TRANSPOSITION OF THE GREAT
ARTERIES
 Transposition of the great
arteries (TGA) occurs when
the PA arises off the left
ventricle and the aorta
arises off the right ventricle.
 It accounts for 5% to 10%
of CHDs.
 Associated lesions include
VSD, ASD, PDA, PS, and
CoA

Pathophysiology
 This defect results in two parallel circulations:
◦ The right atrium receives deoxygenated blood from
the inferior vena cava (IVC) and superior vena cava
(SVC); blood flow continues through the tricuspid
valve into the right ventricle and is pumped back to
the aorta.
◦ The left atrium receives richly oxygenated blood from
the pulmonary veins; blood flow continues through
the mitral valve into the left ventricle and is pumped

 To sustain life, there must be an accompanying defect
that allows mixing of deoxygenated blood and
oxygenated blood between the two circuits, such as
PDA, ASD, PFO, or VSD.
 Neonates born with tga with an intact ventricular system
are usually more cyanotic and sicker than neonates
born with TGA with a VSD.

 Symptoms evident soon after birth; clinical
scenario is influenced by the extent of
intercirculatory mixing.
 Cyanosis.
 Tachypnea.
 Metabolic acidosis.
 CHF.

 Auscultation: varies; no murmur, or a murmur related to
an associated defect.
 Chest X-ray: varies; neonate chest X-ray usually normal;
cardiomegaly with a narrow mediastinum (egg-shaped
cardiac silhouette) and increased pulmonary markings;
or decreased pulmonary markings with pulmonary
stenosis.
 ECG: RVH or biventricular hypertrophy.
 Two-dimensional echocardiogram.

Management
Medical Management
 Stabilize with PGE1 infusion.
 Treat pulmonary overcirculation with digoxin and
diuretics as needed.
 Intubate and ventilate as needed.

Cardiac catheterization
 Balloon atrial septostomy (Rashkind) is indicated for
severe hypoxia to create or improve atrial level mixing.
Surgical Management
 Arterial switch operation procedure of choice:
◦ Ideally performed during the first week of life.
◦ The aorta and PA are switched back to their
anatomically correct ventricle above the level of the
valve.

◦ Coronary arteries are transferred to the new aorta.
◦ Associated lesions are also repaired at this time.
 Rastelli operation”performed for TGA, VSD, and PS.
◦ Repaired during the first year of life.
◦ VSD patch repaired to include LV to aortic outflow
continuity with pulmonary blood flow provided via an
RV to PA homograft.

 Atrial switch operation: Mustard or Senning procedure:
◦ Rerouting of atrial blood flow: RA, mitral valve, LV, PA
and LA tricuspid valve,’ RV, Ao
◦ Restores oxygenated blood into the systemic system
and deoxygenated blood guided to the pulmonary
system.
◦ Disadvantages:
 RV is left as the systemic ventricle”will develop RV
dysfunction.
 Increased incidence of atrial dysrhythmias and baffle
obstruction

Complications
 Severe hypoxia.
 Multiorgan ischemia.
 Arrhythmias.
 RV dysfunction.
 Coronary artery obstruction leading to
myocardial ischemia or death

TRICUSPID ATRESIA
 Tricuspid atresia involves
the absence of the
tricuspid valve and
hypoplasia of the right
ventricle.
 Associated defects such
as an ASD, VSD, or PDA
are necessary for survival.
Tricuspid atresia accounts
for 1% to 3% of CHDs.

Pathophysiology
 With TA, systemic venous return enters the
right atrium and cannot continue into the RV;
blood flows across an atrial septal opening into
the left atrium.
 Pulmonary blood flow occurs through a PDA or
VSD.

 Cyanosis.
 Tachypnea.

 Auscultation: murmurs vary depending on the
associated lesions; single second heart sound.
 Chest X-ray: pulmonary vascular markings related to the
amount of pulmonary blood flow (usually decreased);
normal to slightly increased cardiac silhouette.
 ECG: superior axis; right and left atrial hypertrophy; LVH.
 echocardiogram
 Cardiac catheterization may be necessary to delineate
anatomy.

Management
Medical Management
 Stabilize with PGE1 infusion.
 Intubate and ventilate as needed.
 Infective endocarditis prophylaxis
(lifelong).

Surgical
Management
 First surgery neonate:
◦ BT shunt indicated if pulmonary blood flow is insufficient.
◦ Pulmonary artery band indicated if pulmonary blood flow is
excessive.
◦ No treatment is required if pulmonary blood flow is balanced.
 Second surgery: ages 6 to 9 months:
◦ Bidirectional Glenn shunt: end-to-side anastomosis of the SVC to
the right PA.
 Third surgery: ages 18 months to 3 years:
◦ Fontan completion: IVC to PA connection (extracardiac conduit or
intracardiac baffle).
A Blalock-Taussig (BT) shunt is a small
tube, only a few millimeters wide, that is
used to create a pathway for blood to go
from the arterial circulation to the lungs. It
is used to treat congenital heart defects
that affect blood flow to the lungs.

Complications
 CHF.
 Persistent pleural effusion (especially after Stage II
and Stage III repairs).
 Thrombus formation in the systemic venous
system.
 Rarely, heart block.

NURSING CARE OF THE CHILD WITH
CONGENITAL HEART DISEASE
Nursing Assessment
 Obtain a thorough nursing history.
 Discuss the care plan with the health care team
(cardiologist, cardiac surgeon, nursing case manager,
social worker, nutritionist).
 Discuss the care plan with the patient, parents, and other
caregivers.
 Measure and record height and weight. Plot on a growth
chart.
 Record vital signs and oxygen saturations.
◦ Measure vital signs at a time when the infant/child is
quiet.
◦ Choose appropriate-size blood pressure (BP) cuff.

 Assess and record:
◦ Skin color: pink, cyanotic, mottled.
◦ Mucous membranes: moist, dry, cyanotic.
◦ Extremities: check peripheral pulses for quality and
symmetry; dependent edema; capillary refill; color and
temperature.
 Assess for clubbing (cyanotic heart disease).
 Assess chest wall for deformities; prominent precordial
activity.

 Assess respiratory pattern.
◦ Before disturbing the child, stand back and count the
respiratory rate.
◦ Loosen or remove clothing to directly observe chest
movement.
◦ Assess for signs of respiratory distress: increased respiratory
rate, grunting, retractions, nasal flaring.
◦ Auscultate for crackles, wheezing, congestion, stridor.
 Assess heart sounds.
◦ Determine rate (bradycardia, tachycardia, or normal for age)
and rhythm (regular or irregular).

 Assess fluid status.
◦ Daily weights.
◦ Strict intake and output (number of wet diapers; urine output).
 Assess and record the child's level of activity.
◦ Observe the infant while feeding. Does the infant need
frequent breaks or does he or she fall asleep during feeding?
Assess for sweating, color change, or respiratory distress
while feeding.
◦ Observe the child at play. Is play interrupted to rest? Ask the
parent if the child keeps up with peers while at play.
◦ Assess and record findings relevant to the child's
developmental level: age-appropriate behavior, cognitive

Nursing Diagnoses
 Impaired Gas Exchange related to altered pulmonary blood
flow or pulmonary congestion
 Decreased Cardiac Output related to decreased myocardial
function
 Activity Intolerance related to hypoxia or decreased myocardial
function
 Imbalanced Nutrition: Less Than Body Requirements related to
excessive energy demands required by increased cardiac
workload
 Risk for Infection related to chronic illness

Nursing Interventions
Goal- Relieving Respiratory Distress
 Position the child in a reclining, semi-upright position.
 Suction oral and nasal secretions as needed.
 Identify target oxygen saturations and administer oxygen as
prescribed.
 Administer prescribed medications and document response to
medications (improved, no change, or worsening respiratory status).
◦ Diuretics.
◦ Bronchodilators.
 May need to change oral feedings to nasogastric feedings because

Improving Cardiac Output
 Organize nursing care and medication schedule to provide
periods of uninterrupted rest.
 Provide play or educational activities that can be done in bed
with minimal exertion.
 Maintain normothermia.
 Administer medications as prescribed.
◦ Diuretics (furosemide, spironolactone):
 Give the medication at the same time each day. For older
children, do not give a dose right before bedtime.

 Monitor the effectiveness of the dose: measure and record
urine output.
◦ Digoxin:
 Check heart rate for 1 minute. Withhold the dose and notify
the physician for bradycardia (heart rate less than 90
beats/minute [bpm]).
 Lead II rhythm strip may be ordered for PR interval
monitoring. Prolonged PR interval indicates first-degree
heart block (dose of digoxin may be withheld).
 Give medication at the same time each day. For infants and
children, digoxin is usually divided and given twice per day.

 Monitor serum electrolytes. Increased incidence of digoxin
toxicity associated with hypokalemia.
◦ Afterload-reducing medications (captopril, enalapril):
 When initiating medication for the first time: check BP
immediately before and 1 hour after dose.
 Monitor for signs of hypotension: syncope, light-
headedness, faint pulses.
 Withhold medication and notify the physician according to
ordered parameters.

Improving Oxygenation and Activity
Tolerance
 Place pulse oximeter probe (continuous monitoring or measure
with vital signs) on finger, earlobe, or toe.
 Administer oxygen as needed.
 Titrate amount of oxygen to reach target oxygen saturations.
 Assess response to oxygen therapy: increase in baseline
oxygen saturations, improved work of breathing, and change in
patient comfort.
 Explain to the child how oxygen will help. If possible, give the
child the choice for face mask oxygen or nasal cannula oxygen.

Providing Adequate Nutrition
 For the infant:
◦ Small, frequent feedings.
◦ Fortified formula or breast milk (up to 30 cal/oz).
◦ Limit oral feeding time to 15 to 20 minutes.
◦ Supplement oral feeds with nasogastric feedings as needed to provide
weight gain (ie, continuous nasogastric feedings at night with ad-lib by-
mouth feeds during the day).
 For the child:
◦ Small, frequent meals.
◦ High-calorie, nutritional supplements.
◦ Determine child's likes and dislikes and plan meals accordingly.

◦ Allow the parents to bring the child's favorite foods to the
hospital.
 Report feeding intolerance: nausea, vomiting, diarrhea.
 Document daily weight (same time of day, same scale, same
clothing).
 Record accurate inputs and outputs; assess for fluid retention.
 Fluid restriction not usually needed for children; manage
excess fluid with diuretics.

Preventing Infection
 Maintain routine childhood immunization schedule.
 Administer yearly influenza vaccine.
 Administer RSV immunization for children younger than age 2
with complex CHD and those at risk for CHF or pulmonary
hypertension.
 Prevent exposure to communicable diseases.
 Good hand washing.
 Report fevers.
 Report signs of URI: runny nose, cough, increase in nasal

Congenital heart diseases

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