NCM 109: Care of Mother & Child at Risk w/ Problems

Lesson 8: Care of Pediatric Client w/ Cardiovascular Disorder

Increased Pulmonary Blood Flow: VSD
CONGENITAL HEART DEFECTS
→ A ventricular septal defect (VSD) is a birth defect
1. Increased Pulmonary Blood Flow
of the heart in which there is a hole in the wall
a. Ventricular Septal Defect (septum) that separates the two lower chambers
b. Atrial Septal Defect (ventricles) of the heart. This wall also is called
c. Atrioventricular Septal Defect the ventricular septum.
d. Patent Ductus Arteriosus

2. Obstruction to Blood Flow

a. Pulmonary Stenosis
b. Aortic Stenosis
c. Coarctation of the Aorta

3. Defects with Mixed Blood Flow

a. Transposition of the Great Arteries

b. Total Anomalous Pulmonary Venous Return
c. Truncus Arteriosus
d. Hypoplastic Left Heart Syndrome

4. Defects with Decreased Pulmonary Blood

Flow

a. Tricuspid Atresia
b. Tetralogy of Fallot

5. Acquired Heart Disease

a. Rheumatic fever
b. Kawasaki Disease
c. Bacterial Endocarditis

INTRODUCTION
Respiratory distress in the setting of normal
peripheral perfusion and without overt cyanosis is the
least common manifestation of symptomatic
cardiovascular disease in the newborn. Particularly in
the absence of a murmur, the diagnosis of heart
disease is often delayed or missed entirely because Types of Ventricular Septal Defects
respiratory distress alone in an acyanotic infant with
An infant with a ventricular septal defect can have
normal perfusion is most often caused by lung disease
one or more holes in different places of the septum.
rather than intrinsic cardiac disease.
There are several names for these holes. Some
Furthermore, symptoms usually develop gradually common locations and names are:
over the first few days or weeks of life, and the
1. Conoventricular Ventricular Septal Defect
respiratory symptoms, such as tachypnea with
feeding, are often subtle. It may take several weeks or » In general, this is a hole where portions of the
more to recognize that the infant is growing poorly ventricular septum should meet just below the
and that heart disease may be the cause. pulmonary and aortic valves.

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NCM 109: Care of Mother & Child at Risk w/ Problems
Lesson 8: Care of Pediatric Client w/ Cardiovascular Disorder
2. Perimembranous Ventricular Septal Defect Classification
» This is a hole in the upper section of the
ventricular septum.
3. Inlet Ventricular Septal Defect
» This is a hole in the septum near to where the
blood enters the ventricles through the tricuspid
and mitral valves. This type of ventricular septal
defect also might be part of another heart defect
called an atrioventricular septal defect (AVSD).
4. Muscular Ventricular Septal Defect
» This is a hole in the lower, muscular part of the
ventricular septum and is the most common type
of ventricular septal defect.
Causes and Risk Factors
The causes of heart defects (such as a ventricular
septal defect) among most babies are unknown. Some
babies have heart defects because of changes in their
genes or chromosomes.
Heart defects also are thought to be caused by a
combination of genes and other risk factors, such as
the things the mother comes in contact with in the
environment or what the mother eats or drinks or the
medicines the mother uses.
Diagnosis
A ventricular septal defect usually is diagnosed after a
baby is born.
The size of the ventricular septal defect will influence
what symptoms, if any, are present, and whether a
doctor hears a heart murmur during a physical
examination. Signs of a ventricular septal defect might
be present at birth or might not appear until well after
birth. If the hole is small, it usually will close on its
own and the baby might not show any signs of the
defect. However, if the hole is large, the baby might
have symptoms, including:
 Shortness of breath
 Fast or heavy breathing
 Sweating
 Tiredness while feeding
 Poor weight gain
Small VSD: (less than 0.5 cm) close spontaneously.
Large VSD: (usually greater than 1 cm) 90% require
surgical intervention
Diagnosis
During a physical examination the doctor might hear a
distinct whooshing sound, called a heart murmur. If
the doctor hears a heart murmur or other signs are
present, the doctor can request one or more tests to
confirm the diagnosis. The most common test is an
echocardiogram, which is an ultrasound of the heart
that can show problems with the structure of the
heart, show how large the hole is, and show how
much blood is flowing through the hole.
Treatments
Treatments for a ventricular septal defect depend on
the size of the hole and the problems it might cause.
Many ventricular septal defects are small and close on
their own; if the hole is small and not causing any
symptoms, the doctor will check the infant regularly
to ensure there are no signs of heart failure and that
the hole closes on its own. If the hole does not close
on its own or if it is large, further actions might need
to be taken.
Depending on the size of the hole, symptoms, and
general health of the child, the doctor might
recommend either cardiac catheterization or open-
heart surgery to close the hole and restore normal
blood flow. After surgery, the doctor will set up
regular follow-up visits to make sure that the
ventricular septal defect remains closed. Most
children who have a ventricular septal defect that
closes (either on its own or with surgery) live healthy
lives.
Surgical Management:
Catheter Procedure:
- Insert a catheter into the femoral vein and
threads it to the heart’s septum.
- The device is positioned so that it plugs the hole
between the ventricles.
Open-heart Surgery:
- Rarely used in VSD
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NCM 109: Care of Mother & Child at Risk w/ Problems
Lesson 8: Care of Pediatric Client w/ Cardiovascular Disorder
NURSING DIAGNOSIS:
Decrease in cardiac output associated with heart
malformations.
NURSING GOAL:
To improve cardiac output.
NURSING INTERVENTION:
- Observe the quality and strength of the heartbeat,
peripheral pulses, skin color and warmth.
- Assess the degree of cyanosis (mucous
membranes, clubbing)
- Monitor signs of CHF (anxiety, tachycardia,
tachypnea, shortness of breath, periorbital
edema, oliguria, hepatomegaly)
NURSING DIAGNOSIS:
Impaired gas exchange related to pulmonary
congestion
NURSING GOAL:
Improve gas exchange
NURSING INTERVENTION:
- Monitor the quality and rhythm of breathing.
- Adjust the position of the child w/ fowler position
Preoperative and Postoperative Care
- Avoid child of an infected person.
- Prophylactic antibiotic are often required to - Give adequate rest
prevent infectious endocarditis. - Give oxygen as indicated
- The child should be assessed postoperatively for Increased Pulmonary Blood Flow: ASD
dysrhythmia, since edema in the septum may
interfere with condition.

Medicines
Some children will need medicines to help strengthen
the heart muscle, lower their blood pressure, and help
the body get rid of extra fluid.

Nutrition
Some babies with a ventricular septal defect become
tired while feeding and do not eat enough to gain An atrial septal defect is a birth defect of the heart in
weight. To make sure babies have a healthy weight which there is a hole in the wall (septum) that divides
gain, a special highcalorie formula might be the upper chambers (atria) of the heart.
prescribed. Some babies become extremely tired
while feeding and might need to be fed through a
feeding tube.

Medical Management:
Digoxin (Lanoxin)
 Increase the strength of heart’s contractions.
Furosemide (Lasix)
 Decrease the amount of fluid in circulation
and in the lungs.
Bisoprolol (concor)
 Keep the heartbeat regular.

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NCM 109: Care of Mother & Child at Risk w/ Problems
Lesson 8: Care of Pediatric Client w/ Cardiovascular Disorder
Incidence
→ Sex: Female-Male = 3:1
→ 1/1500 live births
→ Ostium secundum counts for 7% of CHD’s
→ 100% ASDs ≤3mm in diameter
→ Most of the secundum are associated with
Holt-Oram’s syndrome
Holt-Oram’s Syndrome- a disorder that affects bones
in the arms and hands (the upper limbs) and also may
cause heart problems.

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NCM 109: Care of Mother & Child at Risk w/ Problems
Lesson 8: Care of Pediatric Client w/ Cardiovascular Disorder
Increased Pulmonary Blood Flow: AVSD Tests to diagnose atrioventricular canal defect
→ An atrioventricular septal defect (AVSD) is a heart may include:
defect in which there are holes between the
chambers of the right and left sides of the heart,
and the valves that control the flow of blood
between these chambers may not be formed
correctly.
Diagnosis
Atrioventricular canal defect might be diagnosed in a
baby before birth during a pregnancy ultrasound or
special heart imaging.
After birth, symptoms of complete atrioventricular
canal defect are usually noticeable within the first
weeks. When listening to a baby's heart, a health care
provider might hear a whooshing sound. The sound is
called a heart murmur.
ɷ Pulse oximetry. A sensor placed on the fingertip
records the amount of oxygen in the blood. Too
little oxygen may be a sign of a heart or lung
problem.
ɷ Electrocardiogram. Also called an ECG or EKG, this
noninvasive test records the electrical activity of
the heart. Sticky patches with sensors are placed
on the chest. Wires connect the patches to a
computer, which displays results.
ɷ Echocardiogram. Sound waves are used to create
pictures of the heart in motion. An
echocardiogram can reveal a hole in the heart or
heart valve issues. It also shows how blood flows
through the heart.
ɷ Chest X-ray. A chest X-ray shows the condition of
the heart and lungs. It can show if the heart is
enlarged, or if the lungs contain extra blood or
other fluid. These could be signs of heart failure.
ɷ Cardiac catheterization. A thin, flexible tube called
a catheter is inserted into a blood vessel in the
groin and up to the heart. A dye injected through
the catheter makes the heart structures show up
more clearly on X-rays. During the procedure, a
health care provider can measure pressure in
different parts of the heart.
Complete defect
» The condition affects all of the heart's chambers.
Symptoms of a complete atrioventricular canal
defect usually develop in the first weeks of life.
Symptoms are generally similar to those of heart
failure. They may include:
 Blue or gray skin color due to low oxygen levels
 Difficulty breathing or rapid breathing
 Excessive sweating
 Fatigue
 Irregular or rapid heartbeat
 Lack of appetite
 Poor weight gain
 Swelling in the legs, ankles and feet
 Wheezing
Partial defect
» The condition affects only the two upper heart
chambers. Symptoms of a partial atrioventricular
canal defect may not appear until early
adulthood. The symptoms may be due to
complications such as heart valve problems, high
blood pressure in the lungs or heart failure.
Symptoms may include:
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NCM 109: Care of Mother & Child at Risk w/ Problems
Lesson 8: Care of Pediatric Client w/ Cardiovascular Disorder
 Fatigue and weakness
 Nausea and lack of appetite
 Persistent cough or wheezing
 Rapid or irregular heartbeat, also called an
arrhythmia
 Reduced ability to exercise
 Shortness of breath
 Swelling in the legs, ankles and feet
 Chest pressure or pain
Risk factors
Things that might increase the risk of atrioventricular
canal defect include:
1. Genetics. Congenital heart disease appears to run in
families. It's associated with many genetic syndromes.
For instance, children with Down syndrome often
have heart problems present at birth.
2. German measles, also called rubella. Having rubella
during pregnancy may affect how the baby's heart
develops while in the womb.
3. Diabetes. Poorly controlled diabetes during
pregnancy may affect a baby's heart development.
Gestational diabetes generally doesn't increase the
risk of congenital heart disease.
4. Alcohol use. Drinking alcohol while pregnant has
been linked to an increased risk of heart defects in the
baby.
5. Smoking. If you smoke, quit. Smoking during
pregnancy increases the risk of congenital heart
defects in the baby.
6. Some medicines. Taking certain medications while
pregnant may cause heart problems and other birth
defects in the baby. Always tell your health care
provider about the medications you take.
Treatment for atrioventricular canal defects
Complete AV canal defects require surgery, usually
within the first two or three months of life. The
surgeon will close the large hole with one or two
patches. The patches are stitched into the heart
muscle, and as the child grows, the tissue grows over
the patches.
More than one surgery may be needed. Other
surgeries depend on whether the defect is partial or
complete and what other heart problems exist.
 For a partial atrioventricular canal defect, surgery
to repair the mitral valve is needed so that the
valve will close tightly. If repair isn't possible, the
valve might need to be replaced.
 For a complete atrioventricular canal defect,
surgeons separate the large single valve between
the upper and lower heart chambers into two
valves. If this isn't possible, the mitral and
tricuspid valves might need to be replaced.
Possible complications of atrioventricular canal
defect include:
1. Enlargement of the heart. Increased blood flow
through the heart forces it to work harder than
normal, causing it to become larger.
2. High blood pressure in the lungs. This condition is
also called pulmonary hypertension. A hole in the
heart lets oxygen-rich blood mix with oxygen poor
blood. The lungs get too much blood. Pressure
builds up in the lungs.
3. Respiratory tract infections. A hole in the heart
can lead to repeated lung infections.
4. Heart failure. If atrioventricular canal defect is not
treated, the heart can't pump enough blood to
meet the body's needs.
Treatment greatly improves the outlook for children
with atrioventricular canal defect. But complications
may still occur later in life. They may include:
ʘ Breathing problems due to lung damage
ʘ Irregular heart rhythms
ʘ Leaky heart valves, also called valve
regurgitation
ʘ Narrowing of the heart valves
Increased Pulmonary Blood Flow: PDA
→ Patent ductus arteriosus is a persistent opening
between the two main blood vessels leaving the
heart. Those vessels are the aorta and the
pulmonary artery.
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NCM 109: Care of Mother & Child at Risk w/ Problems
Lesson 8: Care of Pediatric Client w/ Cardiovascular Disorder
blood vessel to the heart. They insert a plug or coil
CAUSES into the heart through the catheter to close the PDA
Patent ductus arteriosus causes may include genetic and stop patent ductus arteriosus blood flow.
disorders or a family history of the condition. Providers typically don’t perform cardiac
Other PDA causes may include: catheterization on premature babies, though older
German measles during pregnancy: Babies born to babies and children can have this procedure.
mothers who had rubella (German measles) during
pregnancy may have a higher risk of a PDA.
Neonatal respiratory distress syndrome: Babies whose
lungs didn’t get enough lubricating substance
(surfactant) before birth may develop neonatal
respiratory distress syndrome, a breathing problem.
These babies may also develop a PDA.
SYMPTOMS
PDA symptoms vary according to patent ductus
arteriosus types. Small PDAs may not cause any
symptoms other than a heart murmur.

Larger PDAs may cause: Patent ductus arteriosus surgery: Surgeons make an
o Rapid breathing. incision in the side of the chest. They close the PDA
o Shortness of breath (dyspnea). with stitches (sutures) or a metal clip.
o Sweating during feedings.
o Fatigue or tiredness. Closing the PDA gets blood flow back to normal. After
o Feeding and eating problems. closure via a cardiac catheterization, your child will
o Poor weight gain or growth. take antibiotics for six months to prevent heart
o Fast pulse or heart rate. infection (endocarditis). Your healthcare provider will
discuss the necessary follow-up care with you.

Adults who have PDA closure procedures will also take

antibiotics for six months and should see a
cardiologist for follow-up care.

PDA Treatment

Cardiac catheterization: During cardiac

catheterization, experts insert a thin, flexible tube
(catheter) into the groin and thread it up through a

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NCM 109: Care of Mother & Child at Risk w/ Problems
Lesson 8: Care of Pediatric Client w/ Cardiovascular Disorder
Nursing Assessment
Assessment should focus on: Symptoms
1. Activity and rest. The nurse should assess for
weakness, fatigue, dizziness, a sense of pulsing,
and even sleep disorders.
2. Circulation. Circulatory assessment should include
history trigger conditions, history of heart
murmurs and palpitations, BP, and pulse pressure.
3. Food and fluids. The nurse should assess for
dysphagia and changes in body weight.
Nursing Interventions
Patent ductus arteriosus necessitates careful
monitoring, patient and family teaching, and
emotional support.
! Signs and symptoms. Watch carefully for signs of
PDA in premature infants.
! Monitoring. Frequently assess vital signs, ECG,
electrolyte levels, and intake and output.
! Adverse effects of indomethacin. If the infant
receives indomethacin for ductus closure, watch
for possible adverse effects, such as diarrhea,
jaundice, bleeding, and renal dysfunction.
! Preoperative instructions. Before surgery,
carefully explain all treatments and tests to
parents, including the child, and tell them about
expected IV lines, monitoring equipment, and
postoperative procedures.
! Postoperative procedures. Immediately after
surgery, the child may have a central venous
pressure catheter and an arterial line in place, so
careful assessment of vital signs, intake and
output, and arterial and venous pressures are
needed, as well as pain relief.
Obstruction to Blood Flow: Pulmonary
Stenosis
→ Pulmonary valve stenosis is a type of heart valve
disease that involves the narrowing of the
pulmonary valve, which controls the flow of blood
from the heart's right ventricle into the
pulmonary artery to carry blood to the lungs.
Pulmonary valve stenosis symptoms depend on how
much blood flow is blocked. Some people with mild
pulmonary stenosis do not have symptoms. Those
with more-severe pulmonary stenosis may first notice
symptoms while exercising.
Pulmonary valve stenosis symptoms may include:
*A whooshing sound called a heart murmur that can
be heard with a stethoscope.
*Fatigue.
*Shortness of breath, especially during activity.
*Chest pain.
*Fainting.
Babies with pulmonary valve stenosis may have blue
or gray skin due to low oxygen levels.
Causes
Pulmonary valve stenosis usually results from a heart
problem present at birth. The exact cause is unclear.
The pulmonary valve doesn't develop properly as the
baby is growing in the womb. The pulmonary valve is
made of three thin pieces of tissue called flaps, also
called cusps. The cusps open and close with each
heartbeat. They make sure blood moves in the right
direction.
In pulmonary valve stenosis, one or more of the cusps
may be stiff or thick. Sometimes the cusps may be
joined together. That means they are fused. So the
valve doesn't open fully. The smaller opening makes it
harder for blood to leave the lower right heart
chamber. Pressure increases inside the chamber. The
increased pressure strains the heart. Eventually the
lower right heart chamber wall gets thicker.
Risk factors
Things that may increase the risk of pulmonary valve
stenosis include:
1. German measles, also called rubella. Having
German measles during pregnancy increases the
risk of pulmonary valve stenosis in the baby.
2. Noonan syndrome. This condition is caused by
altered DNA. It can lead to many problems with
the heart's structure and function.
3. Rheumatic fever. This complication of strep throat
can cause permanent damage to the heart and
heart valves. It increases the risk of developing
pulmonary valve stenosis later in life.
4. Carcinoid syndrome. This condition occurs when a
rare cancerous tumor releases certain chemicals
into the bloodstream. It causes shortness of
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NCM 109: Care of Mother & Child at Risk w/ Problems
Lesson 8: Care of Pediatric Client w/ Cardiovascular Disorder
breath, flushing and other symptoms. Some
people with this syndrome develop carcinoid
heart disease, which damages heart valves.
Complications
Possible complications of pulmonary stenosis include:

Infection of the lining of the heart, called infective

endocarditis. People with heart valve problems, such
as pulmonary stenosis, have an increased risk of
developing bacterial infections that affect the inner
lining of the heart.
Surgical valvotomy- is direct incision of the fused
Irregular heartbeats, called arrhythmias. People with leaflets of the valve during open-heart surgery. During
pulmonary stenosis are more likely to have irregular this procedure an incision, called a midline
heartbeats. Unless the stenosis is severe, irregular sternotomy, is made down the middle of the chest. A
heartbeats due to pulmonary stenosis usually aren't heart lung machine is used to support the patient
life-threatening. while the heart is opened and the pulmonary valve is
repaired.
Thickening of the heart muscle. In severe pulmonary
stenosis, the lower right heart chamber must pump Surgical repair is preferred when there is more
harder to force blood into the pulmonary artery. The extensive narrowing in addition to the pulmonary
strain on the heart causes the muscular wall of the valve itself, often as a result of narrowing below
ventricle to thicken. The condition is called right and/or above the valve.
ventricular hypertrophy.
In these situations, a patch may be needed to enlarge
Heart failure. If the right ventricle can't pump narrowed areas in addition to valve repair. The results
properly, heart failure eventually develops. Symptoms from this procedure are also excellent. Hospital length
of heart failure include fatigue, shortness of breath, of stay is short (3 or 4 days) and complications are
and swelling of the legs and belly area. rare. Mild residual narrowing is common but will not
result in long-term problems. Valve leakage may also
Pregnancy complications. The risks of complications occur but is very well tolerated without the need for
during labor and delivery are higher for those with valve replacement.
severe pulmonary valve stenosis than for those
without it.
Treatment
Balloon angioplasty
During the procedure catheters (thin plastic tubes) are
placed into the large blood vessels in the legs and
gently guided to the heart. The catheter tip is placed
across the pulmonary valve and the balloon tip is
inflated. The balloon gently dilates the narrowed area.
This procedure achieves a good result in most patients
and no further treatment is ever needed. Patients
with Noonan and Williams syndromes and patients
with a very small pulmonary valve are less likely to
achieve good results from the procedure.

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NCM 109: Care of Mother & Child at Risk w/ Problems
Lesson 8: Care of Pediatric Client w/ Cardiovascular Disorder

Obstruction to Blood Flow: AORTIC STENOSIS

→ Aortic stenosis occurs when the aortic valve
opening becomes narrowed, resulting in left
ventricular outflow obstruction. The most
common cause is calcification of the valve leaflets,
and is most often seen in older adult patients. Can aortic stenosis be treated with medication?

No pill can cure or even improve your aortic stenosis.

But there are some medications that may help you

control your symptoms and lower the chance of
having certain complications. Among the medicines
your doctor might prescribe are: ACE inhibitors, which
can open blood vessels more fully.

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NCM 109: Care of Mother & Child at Risk w/ Problems
Lesson 8: Care of Pediatric Client w/ Cardiovascular Disorder
How is aortic stenosis managed?
The treatment for severe AS is aortic valve
replacement. This is accomplished either through the
open-heart surgery SAVR (surgical aortic valve
replacement) or the less invasive procedure called
TAVI or TAVR (transcatheter aortic valve implantation
or transcatheter aortic valve replacement).
11. Monitor the patient’s vital signs, weight, and
intake and output for signs of fluid overload.
12. Evaluate patient’s activity tolerance and degree of
fatigue.
13. Monitor the patient for chest pain that may
indicate cardiac ischemia.
14. Regularly assess the patient’s cardiopulmonary
function.
15. Observe the patient for complications and adverse
reactions to drug therapy.
Coarctation of the Aorta

Coarctation of the aorta is a birth defect in which a

part of the aorta is narrower than usual.
Signs and Symptoms
Nursing Interventions: *Labored or rapid breathing.
1. Assist the patient in bathing, if necessary. *Weak femoral artery pulse (taken in the groin area)
2. Provide a bedside commode because using a *Heavy sweating.
commode puts less stress on the heart than using a *Poor growth.
bedpan. *Pale or gray appearance.
3. Offer diversional activities that are physically *Heart murmur: extra heart sound heard when the
undemanding. doctor listens with a stethoscope.
4. Alternate periods of rest to prevent extreme fatigue
and dyspnea.
5. To reduce anxiety, allow the patient to express his
concerns about the effects of activity restrictions on
his responsibilities and routine.
6. Keep the patient’s legs elevated while he sits in a
chair to improve venous return in the heart.
7. Place the patient in an upright position to relieve
dyspnea.
8. Administer oxygen as needed to prevent tissue
hypoxia.
9. Keep the patient in a low sodium diet. Consult with
a dietitian to ensure that the patient receives foods
that he likes while adhering to the diet restrictions.
10. Allow the patient to express his fears and concerns
about the disorder, it’s impact on his life, and any
impending surgery.

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NCM 109: Care of Mother & Child at Risk w/ Problems
Lesson 8: Care of Pediatric Client w/ Cardiovascular Disorder
Prostaglandin E1 is a potent dilator of arterial ductal
tissue and therefore useful in the early treatment of
aortic coarctation.
Digoxin (causes the heart to pump stronger but at
a slower rate) and diuretics (to remove fluid)
6. Surgery usually includes removing the area of the
aorta that is narrow and reconnecting
(anastomosis) the ends…usually done by 2 years
old… may need balloon angioplasty later on to re-
open the artery if narrowing occurs again, which is
a complication that can happen later on.
Educating about treatments and possible
complications:
*Aorta can narrow again at some point and will
require a procedure to re-open
*Hypertension: some child can continue to experience
hypertension long-term and will need medications to
control
*Long-term follow-up care with a cardiologist
Defects with Mixed Blood Flow:
Transposition of the Great Arteries
Dextro-Transposition of the Great Arteries or d-TGA is
a birth defect of the heart in which the two main
arteries carrying blood out of the heart – the main
pulmonary artery and the aorta – are switched in
position, or “transposed.”

Nursing Management
1. Monitoring for signs and symptoms of heart
failure (decrease cardiac output or fluid volume
overload): fluid in lungs with crackles, difficulty
breathing, increased respiratory rate, fatigue,
trouble feeding
2. Monitoring and reporting blood pressure and
pulse differences in upper and lower extremities
3. Mild cases…may go undetected until later in
childhood when abnormal blood pressures or
pulses are detected in the upper vs. lower
extremities…sometimes a heart murmur may be
present (systolic) that is located on the back on
the left side near the shoulder blades
“interscapular”
4. If CoA is severe in a newborn, the patient may be
started on a prostaglandin infusion to keep the
ductus arteriosus open until surgery. This will
help:
- decrease the work load on the left ventricle
- help blood to flow to the lower extremities
5. In addition, other medications may be ordered
prior to surgery to help the heart’s function:

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NCM 109: Care of Mother & Child at Risk w/ Problems
Lesson 8: Care of Pediatric Client w/ Cardiovascular Disorder

Defects with Mixed Blood Flow: Total

Anomalous Pulmonary Venous Return
(TAPVR)

Total anomalous pulmonary venous return (TAPVR) is

a birth defect of the heart. In a baby with TAPVR,
oxygen-rich blood does not return from the lungs to
the left atrium. Instead, the oxygen-rich blood returns
to the right side of the heart. Here, oxygen-rich blood
mixes with oxygen-poor blood.

The primary objective of performing Pulmonary artery

banding (PAB) is to reduce excessive pulmonary blood
flow and protect the pulmonary vasculature from
hypertrophy and irreversible (fixed) pulmonary
hypertension. More recently, PAB has played a role in
the preparation and "training" of the left ventricle (LV)
in patients with dextro-transposition of the great
arteries (d-TGA) who are evaluated for a delayed
arterial switch procedure.

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NCM 109: Care of Mother & Child at Risk w/ Problems
Lesson 8: Care of Pediatric Client w/ Cardiovascular Disorder
Prenatal diagnosis: Anomalous pulmonary venous
return can be diagnosed before birth by a fetal
echocardiogram or heart ultrasound as early as 18
weeks into the pregnancy. This test is done when
there is a family history of congenital heart disease or
when a question is raised during a routine prenatal
ultrasound.
The diagnosis of total anomalous pulmonary venous
return may be suspected when a doctor hears a
typical heart murmur. They may see evidence of
overload on the right side of the heart.
Oxygen saturations may be low in children without
pulmonary venous obstruction. An electrocardiogram
(ECG) may be helpful to show if there are problems
with enlargement of the heart.
A chest X-ray will show enlargement of the right side
of the heart. In newborns with TAPVR and obstructed
pulmonary venous return, chest X-ray may show signs
of pulmonary edema (fluid in the lungs).
The diagnosis of TAPVR is made by echocardiogram
(echo). This study will show the abnormal connection
of the pulmonary veins.
Echocardiography (echo) will also show if the right
atrium and right ventricle are larger than normal. It
can show the size of and flow across the atrial septal
defect (ASD).
Sometimes cardiac catheterization is needed to make
a diagnosis of TAPVR.
Cardiac catheterization will define the abnormal
connection of all pulmonary veins. This is helpful
finding unusual patterns of mixed TAPVR.
Cardiac catheterization can also accurately determine
whether pulmonary veins are obstructed. It can see if
the atrial septal defect is “restrictive” (too small). If
the ASD is restrictive, a balloon dilation procedure can
be done to enlarge the defect
Treatment
Total anomalous pulmonary venous return is a defect
that needs surgery to fix. The timing of the surgical
repair varies depending on the type of TAPVR present.
The condition of the child is considered as well
Surgery is done right away for newborns with
obstructed TAPVR. Some of these children will need
extracorporeal life support (ECMO) prior to surgery
because of their blood flow instability.
Children with TAPVR without obstruction have surgery
days to weeks after the diagnosis is made.
ECMO stands for extracorporeal membrane
oxygenation. The ECMO machine is similar to the
heart-lung by-pass machine used in open-heart
surgery. It pumps and oxygenates a patient's blood
outside the body, allowing the heart and lungs to
rest
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Lesson 8: Care of Pediatric Client w/ Cardiovascular Disorder
Defects with Mixed Blood Flow: Truncus
Arteriosus

Truncus arteriosus is a rare defect of the heart in

which a single common blood vessel comes out of the
heart, instead of the usual two vessels (the main
pulmonary artery and aorta).

Treatment of Truncus Arteriosus

Initial treatment begins with stabilizing the infant.
Medications to control congestive heart failure are
often started.

Surgery is typically done in the first few weeks of life

after the infant is stabilized.

The surgical repair of truncus arteriosus uses the

heart-lung bypass machine for support. It involves
three major parts:
1. Separating the pulmonary arteries from the main
truncus
2. Closing the ventricular septal defect using a patch
3. Creating a connection between the right ventricle
and the pulmonary arteries using a conduit.

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NCM 109: Care of Mother & Child at Risk w/ Problems
Lesson 8: Care of Pediatric Client w/ Cardiovascular Disorder
Defects with Mixed Blood Flow: Hypoplastic
Left Heart Syndrome
Hypoplastic left heart syndrome (HLHS) is a birth
defect that affects normal blood flow through the
heart. As the baby develops during pregnancy, the left
side of the heart does not form correctly.
In a baby with hypoplastic left heart syndrome, the
heart’s left side is too small to pump enough blood to
the rest of the body. Their right ventricle or right
lower chamber of their heart then takes over to pump
blood to both their lungs and whole body. It does this
through a blood vessel called the ductus arteriosus.
The ductus arteriosus is a vessel that all fetuses have.
It typically gets smaller until it closes soon after birth.
With a closed ductus arteriosus, hypoplastic left heart
syndrome is usually fatal if babies don’t receive
treatment.
What causes hypoplastic left heart syndrome?
Most of the time, there is no known cause for
hypoplastic left heart syndrome. Sometimes, the
cause is genetic. Babies with mutations (changes) in
specific genes may have a higher risk of HLHS. These
genes are known as GJA1 or NKX2- 5.
Some babies with genetic disorders (such as Turner
syndrome or trisomy 18) may also have hypoplastic
left heart syndrome.
Tests for a hypoplastic left heart syndrome
diagnosis may include:
1. Chest X-ray: This shows the size and shape of
your baby’s heart and lungs.
2. Echocardiogram: This ultrasound shows
internal heart structures.
3. Electrocardiogram (EKG): This measures
electrical changes during a heartbeat.
4. Pulse oximetry screening: This tells how much
oxygen is in your baby’s bloodstream.
Is hypoplastic left heart syndrome treatable?
Yes. First, your baby will need a medication called
prostaglandin. This keeps their ductus arteriosus
open and functioning. Other medicines can help
your baby’s heart work more efficiently. Your
baby also may need help with breathing.
Then they’ll need a series of surgeries to direct
blood flow to their lungs and body. These
operations put their heart’s workload on their
right ventricle, which does all the pumping.
Hypoplastic left heart syndrome surgery stages
are the Norwood, Glenn and Fontan procedures.
Your baby’s provider will need to do the surgeries
in that order.
Norwood procedure: Babies with HLHS need
Norwood surgery within the first two weeks of
life. During the procedure, surgeons:
*Reconstruct your child’s underdeveloped aorta
to provide blood flow to their body.
*Place a shunt (tube) to reroute blood either from
their right ventricle or aorta to their pulmonary
arteries, which go to their lungs.
*Create a connection between the upper
chambers of their heart. This drains oxygen-rich
blood from their lungs to supply their body
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NCM 109: Care of Mother & Child at Risk w/ Problems
Lesson 8: Care of Pediatric Client w/ Cardiovascular Disorder

Bidirectional Glenn shunt operation: At 4 to 6

months of age, babies need a second operation.
During the Glenn procedure, surgeons:
*Remove the old shunt.
*Place a new shunt to attach your baby’s superior
vena cava to their pulmonary arteries. The
superior vena cava (SVC) is a large vein that
carries oxygen-poor blood from their upper body
to their heart.
*Use this shunt to reduce the strain on your Defects with Decreased Pulmonary
child’s right ventricle by letting blood flow right Blood Flow: Tricuspid Atresia
into their lungs. → Tricuspid atresia is a birth defect of the heart
where the valve that controls blood flow from
the right upper chamber of the heart to the right
lower chamber of the heart doesn't form at all.

Fontan procedure: Between 18 months and 4

years of age, babies need a final surgery. This final
procedure allows all blood returning from their
body to go straight to their lungs instead of mixing
in their heart. During a Fontan procedure,
surgeons will connect your baby’s inferior vena
cava (IVC) to their pulmonary arteries. Similar to
the SVC, the IVC is the large vein that carries
oxygenpoor blood from your child’s lower body to
their heart.

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NCM 109: Care of Mother & Child at Risk w/ Problems
Lesson 8: Care of Pediatric Client w/ Cardiovascular Disorder
Symptoms
Tricuspid atresia symptoms are usually seen soon
after birth. Symptoms of tricuspid atresia may
include:
 Blue or gray skin and lips due to low blood
oxygen levels
 Difficulty breathing
 Tiring easily, especially during feedings
 Slow growth and poor weight gain
Some people with tricuspid atresia also develop
symptoms of heart failure. Heart failure symptoms
include:
 Fatigue and weakness
 Shortness of breath
 Swelling in the legs, ankles and feet
 Swelling of the belly area, a condition called
ascites
 Sudden weight gain from a buildup of fluid

Defects with Decreased Pulmonary Blood

Tetralogy of Fallot Complications
Flow: Tatralogy of Fallot
 Abnormal heart rhythm (arrhythmia)
 Blood clots.
 Heart failure.
 Infection in the lining of the heart and heart
valves (bacterial endocarditis)
 Leakage (regurgitation) from the repaired
pulmonary valve.
 Death.
ACQUIRED HEART DISEASE: RHEUMATIC
FEVER
→ Rheumatic fever may develop if strep throat or
scarlet fever infections are not treated properly
or after strep skin infections (impetigo). Bacteria
Tetralogy of Fallot is a heart defect made up of four called group A Streptococcus (group A strep)
different heart problems: ventricular septal defect, cause these infections. It usually takes about 1 to
overriding aorta, pulmonary stenosis and right 5 weeks after one of these infections for
ventricular hypertrophy. rheumatic fever to develop.
→ Rheumatic heart disease is a condition in which
the heart valves have been permanently
damaged by rheumatic fever. The heart valve

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Lesson 8: Care of Pediatric Client w/ Cardiovascular Disorder
damage may start shortly after untreated or
under-treated streptococcal infection such as
strep throat or scarlet fever. An immune
response causes an inflammatory condition in
the body which can result in on-going valve
damage.

The C-reactive protein (CRP) test is a blood test that

Signs and symptoms: checks for inflammation in your body
 Swollen, tender and red joints, especially the
large joints such as the knees, ankles and
elbows
 Chest pain or abnormal heartbeat.
 Feeling overly tired all the time (fatigue).
 Fever, especially one over 100.4 degrees
Fahrenheit.
 Flat, red rash with a jagged edge.
 Unexplained or ongoing headaches, especially
if your child has never complained of head
pain before.
 Jerky movements you can’t control in your
hands, feet or other body parts “CHOREA”.
 Muscle aches or painful, tender joints.
 Small bumps under the skin.
 Swollen, red tonsils.

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NCM 109: Care of Mother & Child at Risk w/ Problems
Lesson 8: Care of Pediatric Client w/ Cardiovascular Disorder

ACQUIRED HEART DISEASE: Kawasaki

disease

→ Kawasaki disease causes swelling (inflammation)

in children in the walls of small to medium-sized
blood vessels that carry blood throughout the
body.

→ Kawasaki disease commonly leads to

inflammation of the coronary arteries, which
supply oxygen-rich blood to the heart. Kawasaki
disease was previously called mucocutaneous
lymph node syndrome because it also causes
swelling in glands (lymph nodes) and mucous
membranes inside the mouth, nose, eyes and
throat.

→ Children with Kawasaki disease might have high

fever, swollen hands and feet with skin peeling,
and red eyes and tongue. But Kawasaki disease is
usually treatable, and most children recover
without serious problems if they receive
treatment within 10 days of onset.

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NCM 109: Care of Mother & Child at Risk w/ Problems
Lesson 8: Care of Pediatric Client w/ Cardiovascular Disorder

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Lesson 8: Care of Pediatric Client w/ Cardiovascular Disorder
5. Administer antipyretics as ordered and as
needed.
ACQUIRED HEART DISEASE: BACTERIAL 6. Prepare the patient for anticipated surgical
ENDOCARDITIS intervention to repair or replace affected
valves.

Bacterial endocarditis, is an infection caused by

bacteria that enter the bloodstream and settle in the
heart lining, a heart valve or a blood vessel.

Nursing Management
1. Provide supplemental oxygen at 2 to 4 L/min
to maintain or improve oxygenation.
2. Minimize oxygen demand to decrease anxiety.
3. Maintain patient on bed rest if in acute heart
failure.
4. Administer multi-I.V. antibiotic regimen as
ordered.

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