Congenital

Heart
Defects
Congenital Heart Disease
- a grouping of anatomic defects that results from abnormal
development of the heart and related structures that is present
although not always manifested at birth
Causes:
Genetic -Environmental
Classifications:
Acyanotic
Indicates the presence of a defect that permits the passage of blood
from the higher pressure, left sided cardiac chamber to a lower
pressure, right sided cardiac chamber. In other words, there is shunting
of oxygenated blood from the left side of the heart back to the right side
of the heart .
Signs of the left sided heart failure:
* Feeding difficulty
* Pallor
* Dyspnea
* Tachypnea
Cyanotic
Occurs when blood is shunted from the
venous to the arterial system as a result of
abnormal communication between the two
Signs of the right sided heart failure
Hepatomegaly;
abdominal pain
Anorexia
Dilated neck veins
Diaphoresis
Edema
1. Electrocardiogram
- Records the electrical activity of the cardiac muscle from different sites and in
different planes of the body to assess myocardial ischemia, or drainage chamber
enlargement or hypertrophy, electrolyte imbalance, or irregular rhythms.
2. Echocardiography
- Identifies and records, by means of reflected sound waves, intracardiac
structures and their motion, whether normal or abnormal; there are several types: 2
D, M mode, contrast, and Doppler.
3. Chest Radiograph
- Provide information regarding the cardiac size and the size of the specific
chambers of great vessels, the cardiac contour, the status of pulmonary blood flow,
and the status of the lungs and other non - cardiac tissues.
DIAGNOSTIC PROCEDURES
1. Electrocardiogram
- Records the electrical activity of the cardiac muscle from different sites and in
different planes of the body to assess myocardial ischemia, or drainage chamber
enlargement or hypertrophy, electrolyte imbalance, or irregular rhythms.
2. Echocardiography
- Identifies and records, by means of reflected sound waves, intracardiac
structures and their motion, whether normal or abnormal; there are several types: 2
D, M mode, contrast, and Doppler.
3. Chest Radiograph
- Provide information regarding the cardiac size and the size of the specific
chambers of great vessels, the cardiac contour, the status of pulmonary blood flow,
and the status of the lungs and other non - cardiac tissues.
6. Hematologic Testing
- Done to help in the work up and follow up of congenital heart disease. A
complete blood count or arterial blood gases may be ordered.
7. Cardiac Catheterization
- allows determination of oxygen saturation and pressure in the various cardiac
chambers and vessels; cardiac output and vascular resistance can be
calculated; the response of the heart to exercise and drugs can be evaluate
using this data.
4. Magnetic Resonance Imaging
- Imaging technique that uses strong magnetic field to cause movement of nuclei
that results in a resonant picture.
5. Transcutaneous Pulse Oximetry
- Noninvasive means of assessing arterial oxygen saturation. An effective
nonthreatening way of evaluating suspected cyanosis in infants and children at rest
and during rapidly changing circulatory states.
an abnormal opening in the wall
separating the right and left atria
abnormal communication between
the two atria
blood to shift from the left to the right
atrium
atrial septal defect
Asymptomatic
Subtle failure to thrive
decreased exercise tolerance
dyspnea
increased respiratory infections
harsh systolic murmur over the second or third interspace (the pulmonic area)
CHF
CLINICAL MANIFESTATIONS
Diagnostic Procedures
Small ASDs may occasionally close
spontaneously
Surgical correction 2 and 4 years of age
Through a median stenotomy and requires
cardiopulmonary bypass
Suturing the edges of the defect together,
or, with Dacron or Silastic patch
Therapeutic Management
Cardioseal Septal
Occluder
Gore Helix Septal
Occluder
Amplatzer occluder
Observe for arrhythmias
Difficulties on parental acceptance
of the child defect
Continued follow up by the
cardiologist
Subacute endocarditis (SBE)
prophylaxis prior to any dental or
surgical procedure
Nursing Interventions
VENTRICULAR SEPTAL DEFECT
opening in the ventricular septum that result
from imperfect ventricular division during early
fetal development
a communication between the right and left
ventricles
Incidence
most common congenital heart defect (CHD)
20-25% of all CHDs
about 3 in every 1,000 live births
systolic thrill may be palpated at the lower left sternal border
precordial bulge with very large VSDs
grade 2/6 to 5/6 regurgitant systolic murmur at the lower left sternal border
apical diastolic rumble by the increased blood flow returning from the lungs
ECG
left atrial enlargement and left
ventricular hypertrophy
Right ventricular hypertrophy may also be present
P waves may be notched or peaked
(M-mode)
Radiograph
cardiomegaly involving the left atrium,
left ventricle and possible the right
ventricle
Echocardiogram
large left atrium
2-d echo
size and location of the VSD
Phys. examination
reveal inadequate weight gain
cyanosis
clubbing of the digits
failure-to-thrive
Management
monitoring for signs of CHF
prompt initiation of therapy
CHF
diuretics digitalis
Infective endocarditis prophylaxis
for 6 months after surgery
Surgical management
direct closure of the VSD
Fortify formula to make 24-30 cal/oz formula
Large VSD
open-heart surgery
before 2 years of age pulmonary hypertension
Increase caloric intake:
Cardiopulmonary bypass
children weighing less than 7 kg
in deep hypothermia with circulatory arrest
edges of the opening are approximated and sutured
purse-string approach
a Silastic or Dacron patch is sutured into place
to occlude the space
If the defect is exceptionally large
approached through the right atrium and the
tricuspid valve
(includes 1- 5" x 3 " bladder patch,
2' ripstop tape, 1 1/2' white dacron
tape & 1 1/2' black dacron tape)
failure of the fetal artery connecting the aorta
& pulmonary artery to close within the first few
weeks of life
left-to-right shunting of blood
Incidence
PDA accounts for 5% to 10% of all CHDs
With a higher ratio in female patients (2:1)
PATENT DUCTUS
ARTERIOSUS
Higher incidence in preterm infants
PDA in Preterm Infants
Age of Gestation
Possible lung disease causing
hypoxia, which dilates the ductus
Prostaglandin Concentration
machinery-like murmur at the Left intraclavicular area
widened pulse pressure & bounding pulses from the runoff of blood
Small PDA
no hemodynamic compromise
asymptomatic
Large PDA
retardation of physical growth
easy fatigability
may cause
signs of CHF
respiratory difficulties
pallor
low diastolic pressure due to shunting of blood
Diagnostic Procedures
Color Flow 2-D Echo
Chest Radiograph
Echocardiogram
Small PDA: normal ECG
Electrocardiogram (ECG)
Large PDA: abnormal QRS
normal or moderately to markedly enlarged chambers
Doppler
PDA can be directly visualized
amount of blood flow across PDA can be estimated
LA:Ao Ratio
Management
Medical Management
Administration of Indomethacin (Indocin)
Surgical Management
Surgical division or ligation of the patent vessel
Nonsurgical Treatment
Insertion of Dacron-coated stainless-steel coils by
catheterization
COARCTATION OF THE AORTA
narrowing of the aorta between the upper-body artery
branches and the branches to the lower body
can occur anywhere in the aorta, but is most likely to happen
in the segment just after the aortic arch
The more severe the narrowing, the more symptomatic a
child will be, and the earlier the problem will be noticed.
Incidence
Accounts for about 6% of cases of congenital heart disease
Most common in boys than in girls and is the
leading cause of CHF in the first few months of life.
Weak or Absent femoral pulses
BP is higher on the upper extremities (20 mmHg differential)
Rapid and bounding pulse in the upper extremities
Headache and vertigo
Leg pains on exertion and cold feet
Failure to thrive symptoms
include:
respiratory distress,
poor wt. gain,
feeding problems
irritability
tachycardia
Normally a femoral pulse
occurs slightly before a
radial pulse, a radial
femoral delay occurs
when blood flow to the
descending Aorta is
dependent on collaterals
in w/c Case femoral pulse
is felt after the radial
pulse.

epistaxis
The first and second heart sounds are usually normal.

A systoloic murmur is heard along the left sternal border
at the 3
rd
and 4
th
intercostal spaces
left or right ventricular hypertrophy is seen on ECG
in the infant with COA. The older child may have
left ventricular hypertrophy.
Diagnostic Procedures
Electrocardiogram (ECG)
increased heart size
and increased
pulmonary
vascularity.
The older child may have
a normal size heart but
display such
rib notching(10 yrs or
older) and prominent
descending aorta.
Chest Radiography
Presence of COA and degree of narrowing
as well as the presence of other cardiac
defects may be determined
Echocardiogram
To establish the diagnosis of COA and the
severity of the defect
Cardiac Catheterization
Management
Monitor blood pressure in both arms and legs
and pulse assessments
Manage CHF with the use of diuretics and digoxin
Provide adequate nutritional and fluid intake to
maintain the growth and devt. needs of the child.
Prepare the child and for diagnostic and treatment
procedures.
Relieve the respiratory distress associated with increased
pulmonary blood flow oxygen deprivation
Mechanical ventilation and inotropic support are
often necessary before surgery
Resection of coarcted portion with an end to end anastomosis
Subclavian flap aortoplasty
Patch aortoplasty
Cardiopulmonary bypass is not required and lateral
thoracotomy incision is used
Antihypertensives are used to treat post operative
hypertension
Balloon angioplasty
Prostaglandin E is given to reopen ductus and reestablish
lower extremity blood flow.
Elective surgery for COA is advised within the ages 2 to 4.
CVAs
Aneurysms
Rupture of the aorta
Calcification of the aorta
hypertension
Left ventricular
hypertrophy
Ventricular dysfunction
r/t increased pressure and
formation of
collateral circulation
If left untreated
TETRALOGY OF FALLOT
Four anomalies
Ventricular Septal Defect
Pulmonary Stenosis
Dextroposition of Aorta
Right Ventricular
Hypertrophy
cyanosis
Mild pulmonary
stenosis
Severe pulmonary
stenosis
Note: as infant grows the
stenosis increases
tet spells/blue
spells
Dyspnea and
hypoxia
Polycythemia



Muscle flaccidity
Localized systolic
murmur
Acidosis

Note: you can often see
them on a knee chest
position
Cardiac catheterization
To diagnose extent of damage in the heart
Detects:
Systolic ejection murmur
Ventricular tap
Systolic thrill
Chest radiograph
Normal or decrease form of the
heart with boot shaped contour
Diagnostic Procedures

Electrocardiogram
Right ventricular Hypertrophy

Echocardiogram
Uses to visualize the following
VSD
Overriding of aorta
Pulmonary stenosis

Laboratory test
Increased hematocrit, hemoglobin and red
blood cells
Palliative
Blalock Taussig Shunt
Potts
Waterston Cooley

Full repair
Brock procedure
Management
Medications
Propanolol (.15-.25 mg/kg/dose)

Morphine (.1-.02 mg/kg/dose)

Bicarbonate
TRANSPOSITION OF GREAT ARTERIES OR
VESSELS
The pulmonary artery leaves the left
ventricle and the aorta exists from
the right ventricle
No communication between the
systemic and pulmonary
circulations
Incidence Tends to occur on large newborns (9-10 lbs)
More often in boys than in girls
5% of congenital heart defects.
cyanotic (bluish color of skin, lips, and nail beds)
Opening of foramen ovale
Opening of ductus arteriousus
May not have murmurs
May not show enlarged heart
Clinical Manifestation
cyanotic (bluish color of skin, lips, and nail beds)
Opening of foramen ovale
Opening of ductus arteriousus
May not have murmurs
May not show enlarged heart
Clinical Manifestation
Decrease oxygen saturation
Balloon atrial septostomy
Surgical correction (1 week to 3 mos.)
Therapeutic Management
Two Major Types of Surgery
Intra-atrial baffle
(Mustard or Senning Procedure)
Therapeutic Management
The first creates a tunnel (a
baffle) between the atria. This
redirects the oxygen-rich blood
to the right ventricle and aorta
and the oxygen-poor blood to
the left ventricle and the
pulmonary artery. This
operation is called an atrial or
venous switch.
Two Major Types of Surgery
Atrial Switch
Therapeutic Management
The aorta and pulmonary artery are
switched back to their normal
positions. The aorta is connected
to the left ventricle, and the
pulmonary artery is connected to
the right ventricle.
The coronary arteries, which
carry the oxygen-rich blood that
nourishes the heart muscle,
also need to be re-attached to
the new aorta.
Surgical correction(1 week- 3 months)
Survival rate 95%
Therapeutic Management
Balloon arterial septal pull through operation
Surgical correction (1 week to 3 mos.)
Therapeutic Management

also known as lymph node
syndrome, mucocutaneous
node disease, infantile
polyarteritis and Kawasaki
syndrome
is a poorly understood self-
limited vasculitis that affects
many organs, including the skin
and mucous membranes, lymph
nodes, blood vessel walls, and
the heart
It does not seem to be
contagious. It was first described
in 1967 by Dr. Tomisaku
Kawasaki in Japan
Causes

The causative agent of
Kawasaki disease is still
unknown
However, current etiological
theories center primarily on
immunological causes for the
disease. Much research is being
performed to discover a
definitive toxin or antigenic
substance, possibly a
superantigen, that is the specific
cause of the disease.
Kawasaki disease often begins
with a high and persistent fever
that is not very responsive to
normal doses of paracetamol
(acetaminophen) or ibuprofen.
Affected children develop red
eyes, red mucous membranes
in the mouth, red cracked lips, a
"strawberry tongue"
swollen lymph nodes

Joint pain (arthralgia) and
swelling, frequently symmetrical

Irritability

Tachycardia (rapid heart beat)
Peeling (desquamation) palms
and soles (later in the illness);
peeling may begin around the
nails
Beau's lines(transverse grooves
on nails)
Signs and tests

Blood tests
Complete blood count (CBC) may
reveal normocytic anemia and
eventually thrombocytosis
Erythrocyte sedimentation rate

(ESR) will be elevated

C-reactive protein (CRP) will be
elevated

Liver function tests may show
evidence of hepatic
inflammation and low serum
albumin
Treatment

Children with Kawasaki disease
should be hospitalized and
cared for by a physician who
has experience with this
disease.
Intravenous immunoglobulin
(IVIG) is the standard treatment
for Kawasaki disease, and is
administered in high doses with
marked improvement usually
noted within 24 hours
If the fever does not respond,
an additional dose may have to
be considered. IVIG by itself is
most useful within the first 7
days of onset of fever, in terms
of preventing coronary artery
aneurysm.
Salicylate therapy particularly
aspirin, remains an important
part of the treatment (though
questioned by some) but
salicylates alone are not as
effective as Intravenous
immunoglobulin.
Corticosteroids have also been
used especially when other
treatments fail or symptoms
recur, but in a randomized
controlled trial, the addition of
corticosteroid to immune globulin
and aspirin did not improve
outcome.
Prognosis

With early treatment, rapid
recovery from the acute
symptoms can be expected and
the risk of coronary artery
aneurysms greatly reduced.
Untreated, the acute symptoms
of Kawasaki disease are self-
limited (i.e. the patient will
recover eventually), but the risk
of coronary artery involvement
is much greater
Rheumatic fever
> is an autoimmune
inflammatory disease which may
develop two to three weeks after
a Group A streptococcal
infection (such as strep throat or
scarlet fever) and can involve
the heart, joints, skin, and brain
Acute rheumatic fever
commonly appears in children
ages 5 through 15, with only
20% of first time attacks
occurring in adults
It gets its name for its similarity
in presentation to rheumatism
Rheumatic fever is common
worldwide and responsible for
many cases of damaged heart
valves
Diagnosis: Modified Jones
Criteria (MJC)
Two major criteria, or one
major and two minor criteria,
when there is also evidence of a
previous strep infection, support
the diagnosis of rheumatic fever
Major criteria

The mnemonic JONES is often
used to recall the Major Criteria.
Joints (Migratory polyarthritis):

a temporary migrating
inflammation of the large joints,
usually starting in the legs and
migrating upwards.
O [imagine heart-shaped O]
(carditis): inflammation of the
heart muscle which can
manifest as congestive heart
failure with shortness of breath,
pericarditis with a rub, or a new
heart murmur.
Nodules (subcutaneous nodules
- a form of Aschoff bodies):
painless, firm collections of
collagen fibers on the back of
the wrist, the outside elbow, and
the front of the knees. These
now occur infrequently.
Erythema marginatum: a long
lasting rash that begins on the
trunk or arms as macules and
spreads outward to form a
snakelike ring while clearing in
the middle. This rash never
starts on the face and is made
worse with heat.
Sydenham's chorea (St. Vitus'
dance): a characteristic series of
rapid movements without
purpose of the face and arms.
This can occur very late in the
disease
an additional way to remember
the major criteria is by the
mnemonic: C.A.N.C.ER
C: Carditis
A: Arthritis
N: Nodules (sub cutaneous)
C: Chorea
ER: ERythema Marginatum

Minor criteria

Fever: temperature elevation
Arthralgia: Joint pain without
swelling
Laboratory abnormalities:
increased Erythrocyte
sedimentation rate, increased C
reactive protein, leukocytosis

Evidence of Group A Strep
infection: elevated or rising
Antistreptolysin O titre, or
DNAase, though by the time
clinical illness begins, cultures
for the streptococci bacterium
will be negative.
Previous rheumatic fever or
inactive heart disease
Treatment

The management of acute
rheumatic fever is geared
toward the reduction of
inflammation with anti-
inflammatory medications such
as aspirin or corticosteroids
Individuals with positive cultures
for strep throat should also be
treated with antibiotics
Aspirin is the Drug of choice
and should be given at high
doses of 100mg/kg/day.
Infection

Patients with positive cultures for
streptococcus pyogenes should
be treated with penicillin as long
as allergy is not present. This
treatment will not alter the
course of the acute disease.
Inflammation

Patients with significant
symptoms may require
corticosteroids. Salicylates are
useful for pain.

Heart failure

Some patients develop
significant carditis which
manifests as congestive heart
failure.
This requires the usual
treatment for heart failure:
diuretics and digoxin. Unlike
normal heart failure, rheumatic
heart failure responds well to
corticosteroids.
Prevention

Prevention of recurrence is
achieved by eradicating the
acute infection and prophylaxis
with antibiotics
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