VENTRICULAR SEPTAL DEFECT
 the most common type of congenital cardiac
disorder seen
 30% of all instances of congenital heart disease, or
about 2 in every 1000 live births
It is described, an opening is present in the septum between
the two ventricles
Pathology:
Because pressure in the left ventricle is greater than
that in the right ventricle, blood shunts from left to right
across the septum (an acyanotic disorder). 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
This leads to:
 right ventricular hypertrophy
 increased pressure in the pulmonary artery
Assessment:
1. At 4 to 8 weeks of age, as shunting begins, the infant
demonstrates easy fatigue
2. a loud, harsh pansystolic murmur ( along the left
sternal border at the third or fourth interspace)
3. A thrill (vibration) also may be palpable
Diagnostic Procedure/Diagnosis:
 VSD is based on examination by echocardiography
with color flow Doppler or MRI (which reveal right
ventricular hypertrophy)
 An ECG will also reveal right ventricular hypertrophy
Therapeutic Management:
 Up to 85% of VSDs are so small they close
spontaneously
 Closure is important because if the defect is left open,
cardiac failure from the artery hypertension can result.
The heart can become infected (endocarditis) because
of the recirculating blood flow
1. cardiac catheterization
2. Larger ones (over 3 mm) require open heart surgery.
 scheduled before 2 years of age to prevent
pulmonary artery hypertension
3. If the defect is exceptionally large, a Silastic or
Dacron patch can be sutured into place to occlude
the space
4. Postop Management includes:
 alert for arrhythmia (edema)
 prophylactic antibiotics to prevent bacterial
endocarditis for 6 months afterward
ATRAIL SEPTAL DEFECT
 is an abnormal communication between the two
atria, allowing blood to shift from the left to the right
atrium
 common in girls than boys
Pathology:
Blood flow is from left to right (oxygenated to
deoxygenated) 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 and generally results in ventricular
hypertrophy and increased pulmonary artery blood flow.
Two types of ASDs:
1. Ostium primum (ASD1) -where the opening is at the
lower end of the septum,
2. Ostium secundum (ASD2) - where the opening is
near the center of the septum.
 defects may be asymptomatic and not discovered
until infection from recirculating blood occurs
Assessment:
1. harsh systolic murmur is heard over the second or
third interspace (the pulmonic area
2. The second heart sound will be auscultated as split
(fixed splitting)
Note: Such a sound is almost always diagnostic of ASD
3. Echocardiography with color flow Doppler
Note: Reveals enlarged right side of the heart and the
increased pulmonary circulation.
4. Cardiac catheterization
Note: reveal the separation in the atrial septum and the
increased oxygen saturation in the right atrium
Complications:
1. Endocarditis
2. Heart failure
Therapeutic Management:
1. Surgery(done electively between 1 and 3 years of
age)
Note: particularly important that ASDs be repaired in girls,
because they can cause emboli during pregnancy.
2. Large defects may still require open heart surgery
and cardiopulmonary bypass
3. If very large a Silastic or Dacron patch may be
sutured into place to occlude the space
4. Postop Management includes:
 alert for arrhythmia (edema)
PATENT DUCTUS ARTERIOSUS
The ductus arteriosus is an accessory fetal structure
that connects the pulmonary artery to the aorta.
 closure begins with the first breath, and is usually
complete between 7 to 14 days of age, although full
closure may not occur until 3 months of age
 PDAs are twice as common in girls as boys
 occur at a higher incidence at higher altitudes
Pathology:
If it fails to close at birth blood will shunt from the
aorta (oxygenated blood) to the pulmonary artery
(deoxygenated blood) 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.
Results in:
 increased pressure in the pulmonary circulation
 right ventricle hypertrophy
 ineffective heart action
Assessment:
1. wide pulse pressure
2. Diastolic pressure is low
3. A typical continuous (systolic and diastolic)
“machinery” murmur can be heard at the upper left
sternal border or under the left clavicle in older
children
Diagnostic Test:
4. An ECG is generally normal, although it may show
ventricle enlargement if the shunt is large
5. Echocardiography provides good visualization of the
patent ductus.
6. Cardiac catheterization is generally not necessary
for diagnosis but may be performed to rule out
associated defect.
Complications:
1. Risk for HF
2. Endocarditis
Therapeutic Management:
1. An infant may be prescribed IV indomethacin
prostaglandin inhibitors.
Nursing Consideration:
 assess for possible side effects, including reduced
glomerular filtration, impaired platelet
aggregation, and diminished gastrointestinal and
cerebral blood flow
2. Ibuprofen is becoming the drug of choice; it can
even be used as prophylaxis in preterm infant due to
too many side effects of indomethacin.
3. If medical management fails , the disorder can be
closed by insertion of Dacron-coated stainless-steel
coils by interventional cardiac catheterization
 Done when child is 6 months to 1 year of age
4. Exceptionally large defects can be closed surgically
by ductal ligation
TRANSPOSITION OF THE GREAT ARTERIES
 The aorta arises from the right ventricle instead of
the left, and the pulmonary artery arises from the
left ventricle instead of the right.
 Makes the entire heart one mixed circulatory
system
 It tends to occur in large newborns (9 to 10 lb)
 occurs more often in boys than in girls
 accounts for about 5% of congenital heart
anomalies
Associated with the presence:
1. Atrial septal defects
2. Ventricular Septal Defects
Pathology:
Blood enters the heart from the vena cava to the
right atrium, then flows to the right ventricle, and goes out
into the aorta to the body completely deoxygenated; it
returns again by the vena cava. A secondary source of blood
enters the heart from the pulmonary veins, goes to the left
atrium, left ventricle, and out the pulmonary artery to the
lungs to be oxygenated, and returns to the left atrium, a
second closed circulatory system.
Assessment
1. cyanotic from birth
2. no murmur, or there may be various murmurs,
depending on the shunting of blood
3. Echocardiography generally reveals an enlarged
heart.
4. ECG may or may not reveal heart changes.
5. Cardiac catheterization will reveal the low oxygen
saturation
Therapeutic Management
1. PGE1 (prostaglandin E1), a prostaglandin, will be
administered to keep the ductus arteriosus patent.
2. A balloon atrial septal pull-through operation to
enlarge the septal openings may also need to be
done in the infant’s first few days.
3. Surgical correction of transposition of the great
vessels, done at 1 week to 3 months of age.
TETRALOGY OF FALLOT
 one of the first types of congenital heart disease
described
 occurs in about 10% of children with congenital
cardiac disease
 children with this disorder show a deletion
abnormality of chromosome 22 (22q11.2)
 the disorder can result from a documented
chromosome disorder
It is called a tetralogy because four anomalies are present:
1. pulmonary stenosis
2. VSD (usually large)
3. Dextroposition (overriding) of the aorta
4. hypertrophy of the right ventricle
Pathology:
Because of the pulmonary stenosis, pressure builds
up in the right side of the heart. Blood then shunts from this
area of increased pressure into the left ventricle and the
overriding aorta. The extra effort involved to force blood
through the stenosed pulmonary artery causes the fourth
deformity, hypertrophy of the right ventricle.
Therapeutic Management:
1. may not exhibit a high degree of cyanosis
immediately after birth, becoming more active,
however, their skin acquires a bluish tint as cyanosis
begins
2. polycythemia
Complications associated with this:
 thrombophlebitis
 embolism
 cerebrovascular accident
3. If the condition is not corrected: development of
severe dyspnea, growth restriction, and clubbing of
the fingers
4. assume a squatting or a knee–chest position when
resting (provides physiologic relief, if not done the
heart can be overwhelmed)
5. Develop syncope (fainting) and hypercyanotic
episodes (sometimes called tet spells).
6. A loud, harsh, widely transmitted murmur or a soft,
scratchy localized systolic murmur in the left
second, third, or fourth parasternal interspace may
be present
Note: Follow prolonged crying or exertion. They can be so
extreme, if long term, that children develop cognitive
challenge
Diagnosis of the disease is based on:
 history
 physical symptom
 echocardiography
 ECG
 cardiac catheterization
 laboratory findings: polycythemia, increased Hgb,
Hct, and total RBC as well as reduced oxygen
saturation
Therapeutic Management
1. surgery to correct the heart defects
 done at 1 to 2 years of age
2. If a baby begins to have a hypoxic episode:
 administering oxygen
 placing the baby in a knee–chest position
3. Administering morphine sulfate generally reduces
symptoms.
4. If not, propranolol (Inderal, a beta-blocker) may be
given orally to aid pulmonary artery dilation.
5. A temporary or palliative surgical repair, called the
Blalock-Taussig procedure, can create a shunt
 between the aorta and the pulmonary artery
(creating a ductus arteriosus)
 This will allow blood to leave the aorta
and enter the pulmonary artery,
oxygenate in the lungs, and return to the
left side of the heart, the aorta, and the
body
 When a Blalock-Taussig procedure is
done, a child will not have a palpable
pulse in the right arm after this
procedure. For this reason, blood
pressure and venipuncture should be
avoided in the affected arm

6. Postop Management includes:
 alert for arrhythmia (which may result
from any ventricular septal repair, edema,
and conduction interference)
STATUS ASTHMATICUS
Under ordinary circumstances, an asthma attack
responds readily to the aerosol administration of a
bronchodilator such as albuterol, terbutaline, levalbuterol
(Xopenex), or salmeterol (Serevent).
 When children fail to respond and an attack
continues, they are in status asthmaticus.
 This is an extreme emergency because, if the attack
cannot be relieved, a child may die of heart failure
caused by the combination of:
 Exhaustion
 Atelectasis
 respiratory acidosis from bronchial
plugging
Assessment:
1. Acute respiratory distress
2. Both heart rate and respiratory rate are elevated.
Both oxygen saturation and PO2 are low
3. PCO2 is elevated because the bronchi are so
constricted the child cannot exhale, resulting in CO2
accumulation.
4. The rising PCO2 rapidly leads to acidosis.
5. In contrast to the loud wheezing initially heard in an
asthma attack, children with status asthmaticus may
have so little air able to pass in or out of their lungs
that breathe sounds are limited.
6. Pulse oximetry (will reveal the low oxygen saturation
level.)
Often initiated by a respiratory infection, which acts as the
triggering mechanism for the prolonged attack.
If this occurs Nursing interventions include:
1. obtain cultures from coughed sputum,
2. Be prepared to administer a broad-spectrum
antibiotic until the culture results are available.
3. Be certain the sputum obtained for culture was
coughed from deep in the respiratory tract and
not just from the back of the child’s throat.
Therapeutic Management
1. Continuous nebulization with an inhaled beta-2-
agonist and intravenous corticosteroids (to reduce
symptoms)
2. Oxygen is given by face mask or nasal prongs
 To maintain the PO2 at more than 90 mm
Hg. These methods supply good oxygen
concentrations and yet leave the child’s
face unobscured for easy observation.
3. To prevent drying of pulmonary secretions, always
give oxygen with humidification.
 It is best administered at a concentration of
30% to 40%, not 100%.
 If concentrations greater than 40% are
needed, a Venturi mask that allows for
rebreathing may be used.
Some children in severe status asthmaticus have such a
carbon dioxide buildup (because they cannot exhale properly)
that they develop carbon dioxide narcosis with no stimulation
for inhalation.
The child’s respiratory stimulus, therefore, is hypoxia, or lack
of oxygen.
1. If 100% oxygen were administered, the oxygen lack
would disappear, and respirations would cease.
2. The idea “if a little is good, a lot is better” does not
apply here.
3. After it has been ascertained that the child is not in
acidosis (from blood gas and pH studies), oxygen
levels may be increased, but for initial therapy,
unless prescribed otherwise, keep the level at 40%.
4. During the acute stage of status asthmaticus,
children need increased fluid
a. To combat dehydration and keep airway
secretions moist.
5. Drinking tends to aggravate coughing, so an
intravenous infusion such as 5% glucose in 0.45
saline is usually prescribed to supply fluid.
6. If a child can drink, do not offer cold fluids because
these tend to aggravate bronchospasm.
7. Monitor intake and output; measure the specific
gravity of urine.
8. Under stress, antidiuretic hormone is released, so
fluid retention and overhydration may occur.
An increasing PCO2 is a danger sign because it indicates the
degree of hypoventilation.
 In severe attacks, endotracheal intubation and
mechanical ventilation may be necessary to maintain
effective respiration
NEPHROBLASTOMA (WILM’S TUMOR)
 A malignant tumor that arises from the upper pole
of the kidney.
 accounts 20% of solid tumors in childhood
 may have deletion on chromosome 11
 discovered between 6 months to 5 years
 metastasize rapidly
Associated with:
1. aniridia (lack of color in the iris)
2. cryptorchidism
3. hypospadias
4. cystic kidneys
5. hemangioma
Clinical Manifestations:
1. firm, non-tender abdominal mass
2. low grade fever
3. hematuria
4. hypertension
 5. anemia
Diagnostic Test:
 Sonogram
 CT Scan
Important Nursing Responsibility:
Place a sign “NO ABDOMINAL PALPATION” over child’s crib
or head of the bed.
Therapeutic Management:
1. Nephrectomy
2. Radiation Therapy
3. Chemotherapy
Note: 90% of children with no metastasis survive for at least 5
years
NEPHROTIC SYNDROME/ NEPHROSIS
 Altered glomerular permeability due to fusion of
the glomeruli membrane surfaces, causes abnormal
loss of protein in urine.
Risk Factors:
 highest incidence is at 3 years of age, and it occurs
more often in boys than in girls
 progression of glomerulonephritis
 sickle cell anemia or systemic lupus erythematous
(SLE)
Etiology:
1. Hypersensitivity to an antigen-antibody reactions
2. Autoimmune process
3. Congenital
Nephrotic syndrome in children occurs in three forms:
1. Congenital (an autosomal recessive disorder)
2. Secondary to (Glomerulonephritis, SLE or Sickle cell
anemia)
3. Idiopathic (is most common in children)
Nephrosis can be further classified according to the amount
of membrane destruction:
1. Minimal change nephrotic syndrome (MCNS) - is the
type most often seen in children (80%).
 Little scarring of glomeruli occurs. Children
with this degree of scarring respond well to
therapy
2. Focal glomerulosclerosis (FGS)
3. Membranoproliferative glomerulonephritis (MPGN)
 scarring of glomeruli, and these children will
have a poorer response to therapy
Assessment:
Four characteristic symptoms of nephrotic syndrome are:
1. proteinuria
 occurs because increased glomerular
permeability leads to protein loss in the
urine and, subsequently, hypoalbuminemia
2. Edema
 low level of protein in the bloodstream,
osmotic pressure causes fluid to shift
 blood volume decreases, the kidneys begin
to conserve sodium and water, adding to
the potential for edema.
3. Hypoalbuminemia
4. Hyperlipidemia
 hyperlipidemia occurs because the liver
increases production of lipoproteins to try
to compensate for protein loss
 too large to be lost in urine, so they rise to
high levels in the blood serum
Symptoms:
1. edema around the eyes (periorbital edema)
 morning from a head-dependent position
2. clothing no longer fits a child around the waist,
 edematous fluid is beginning to collect in
the abdominal cavity (ascites)
3. As edema progresses skin becomes pale, stretched,
and taut
4. scrotal edema becomes extremely marked
5. Anorexia or vomiting.
6. Children may have diarrhea caused by intestinal
edema and poor absorption by the edematous
membrane.
7. growth may stop
 poor nutrition
8. The child may become malnourished but yet appear
deceptively obese because of the extensive edema
9. ascites becomes even more extensive, children may
have difficulty breathing
10. children are irritable and fussy, probably from the
feeling of abdominal fullness and generalized edema
Laboratory Findings:
 Reveal marked proteinuria. A single test
will show a 1 to 4 protein
 24-hour total urine test will show up to 15
g protein (normally urine contains no
protein.
 Hematuria
 The erythrocyte sedimentation rate
(demonstrating the inflammation of the
glomeruli membrane) is elevated
Note: protein loss with nephrotic syndrome is almost entirely
albumin, differentiating it from the proteinuria of
glomerulonephritis
Diagnostic Procedure:
 A renal biopsy may be done to determine whether
there is scarring of the glomerular membrane and
document the type of nephrotic syndrome present
Therapeutic Management:
1. Corticosteroid therapy – directed towards reducing
proteinuria and edema.
o IV methylprednisolone or oral prednisone
NDitursing Considerations:
1. DO NOT stop abruptly
2. Avoid infections
3. Inform parent of the physical changes.
2. Diuretic- Furosemide (Lasix)
o Administered if patient is POORLY
RESPONSIVE to corticosteroids
o Increase potassium diet
o ADMINISTRED AFTER albumin
administration
3. IV albumin- to replace albumin
o Draws fluid from the interstitial space into
the blood stream
4. Cytotoxic Agent (immunosuppressant) –
cyclophosphamide (Cytoxan)
o Administer IF PT. IS NOT REPONSIVE TO
CORTICOSTEROIDS
Nursing considerations:
1. Increase fluid intake because this drug is
bladder irritant
5. Diet: adequate protein, increase potassium,
limit/restrict Sodium and Fluid
6. Weight daily (same clothing, same weighting scale
and same time of the day)
7. Monitor I&O
8. Monitor PR and BP
9. Skin Integrity: edematous area is prone to skin
breakdown
a) Frequent position changes
b) Changes in frequently
c) Avoid constrictive clothing
10. Semi Fowlers positions- to reduce periorbital edema.