Pediatric Cardiac Disorders
Summer 2022
Review of the Heart

Normal cardiac anatomy and physiology
 Muscular pump divided into four chambers
 Upper chambers are atria
 Lower chambers are ventricles
 Atria are the filling chambers
 Ventricles are the pumping chambers
 Two atrioventricular (AV) valves (Tricuspid &
Mitral), two semilunar valves (Pulmonic & Aortic)
 Mechanical contraction of the heart begins with
electrical stimulation
Copyright © 2018 Elsevier Inc. All rights reserved.
2
Fetal Circulation
Intrauterine to Extrauterine
1. Infant takes first breath and the lungs inflate
__decreased___
pulmonary vascular resistance
Increased pulmonary blood flow
&
the pulmonary artery pressure increases.
2. Increased pressure in left atrium, decreases pressure in right
atrium
closure of foramen ovale
3. The ductus arteriosus and ductus venosus close
related to pressure changes and  blood oxygen
levels.
Fun Fetal Circulation Video

https://www.youtube.com/watch?v=3IkAnVZp
O5Y
Other helpful videos:
Fetal circulation:
https://www.youtube.com/watch?v=-IRkisEtzsk
Circulation right after birth
https://www.youtube.com/watch?v=jFn0dyU5wUw
RECAP: Transitional and Neonatal Circulation

Major changes in the circulatory system occur at
birth after the first breath.
 Gas exchange is transferred from the placenta
to the lungs.
 Fetal shunts close.
 Resistance to flow in the pulmonary system
decreases as systematic resistance increases.
 Pulmonary vascular resistance decreases.
 Marked increase in pulmonary blood flow
follows.
Copyright © 2018 Elsevier Inc. All rights reserved.
Oxygen Saturation

What is oxygen saturation?


Fraction of O2 saturated hemoglobin relative to total hemoglobin in
the blood
What is normal oxygen saturation levels?

95-100%

What values indicate hypoxemia?

Why is it important for the nurse to know the
oxygen saturation levels?
Congenital Heart Disease


Left-to-right shunting lesions
 Patent ductus arteriosus
 Atrial septal defect
 Ventricular septal defect
 Atrioventricular septal defect
Obstructive or stenotic lesions
 Pulmonary stenosis
 Aortic stenosis
 Coarctation of the aorta
Copyright © 2018 Elsevier Inc. All rights reserved.
Congenital Heart Disease (Cont.)


Cyanotic lesions with decreased pulmonary blood
flow
 Tetralogy of Fallot
 Tricuspid atresia
 Pulmonary atresia with intact ventricular
septum
Cyanotic lesions with increased pulmonary
blood flow
 Truncus arteriosus
 Hypoplastic left heart syndrome
 Transposition of the great arteries
Copyright © 2018 Elsevier Inc. All rights reserved.
Patent Ductus Arterious
Atrial septal defects
Ventricular septal defects
Patent Ductus Arteriosus
1. Blood shunts from
aorta (left) to the
pulmonary artery
(right)
2. Returns to the lungs
causing increase
pressure in the lung
3. Congestive heart
failure
PDA Treatment

Medical Management
 Medication


Indomethacin - given IV. Inhibits prostaglandins by
inhibiting cyclooxygenase.
(When levels of prostaglandins are decreased, the
ductus closes) Used mainly in preterm infants.
Surgery - video-assisted thoracoscopic
Ligate the
ductus
arteriosus
PDA Treatment

Cardiac Catheterization

Insert coil – tiny fibers
occlude the ductus
arteriosus when a
thrombus forms in
the mass of fabric and
wire
Atrial Septal Defect
1. Oxygenated blood is shunted
from left to right side of the
heart via defect
2. A larger volume of blood
than normal must be
handled by the right side of
the heart hypertrophy
3. Extra blood then passes
through the pulmonary
artery into the lungs,
causing higher pressure
than normal in the blood
vessels in the lungs 
congestive heart failure
ASD Treatment

Medical Management


Cardiac Catheterizaton 

Medications – digoxin
Amplatzer septal occluder
Open-heart Surgery
ASD Treatment

Device Closure – Amplatzer septal
occluder
During cardiac catheterization the occluder is placed in the
Defect
Ventricular Septal Defect
1. Oxygenated blood is shunted
from left to right side of the
heart via defect
2. A larger volume of blood
than normal must be
handled by the right side of
the heart hypertrophy
3. Extra blood then passes
through the pulmonary
artery into the lungs,
causing higher pressure
than normal in the blood
vessels in the lungs 
congestive heart failure
VSD Treatment
Surgical repair with a patch inserted
Pulmonic Stenosis
Aortic Stenosis
Coarctation of Aorta
Pulmonic Stenosis


Narrowing of
entrance that
decreases blood
flow
Increases
preload causes
right ventricular
hypertrophy
Obstructive or Stenotic Lesions

Treatment:
 Medications – Prostaglandins to keep the
PDA open

Cardiac Catheterization
 Balloon Valvuloplasty

Surgery
 Valvotomy
Aortic Stenosis

The aortic valve is
thickened and rigid

Stenosis creates left
ventricular hypertrophy

Left ventricle may not be
large enough to eject a
normal cardiac output.
Aortic Stenosis

Symptoms
 Poor peripheral perfusion, feeding
difficulties, CHF

Treatment
 Balloon valvoplasty
 Surgery
Coarctation of the Aorta
1. Narrowing of Aorta
causing obstruction of
left ventricular blood
flow
2. Left ventricular
hypertrophy
Signs and Symptoms
1. What are B/P findings support the diagnosis?
Elevated B/P
2. What is different when assessing pulses?
Absent leg pulses (hallmark) and a difference in blood
pressure between the arms and legs. High in arms and
low to normal blood pressure in legs.
3. Why would the patient C/O leg pains?
Poor peripheral perfusion
4. What causes nose bleeds?
Elevated B/P
Treatment



Goals of management are to improve ventricular
function and restore blood flow to the lower body.
Medical management with Medication
 A continuous intravenous medication,
prostaglandin (PGE-1), is used to open the ductus
arteriosus allowing blood flow to areas beyond
the coarctation.
Baloon Valvoplasty
Surgery for Coarctation of Aorta
1. Resect
narrow
area
2. Anastomosis
Tetralogy of Fallot
Tetralogy of Fallot (TOF)
Four defects are:
1.
2.
3.
4.
TOF Signs and Symptoms
1.
Failure to thrive
2.
Squatting
3.
Lack of energy
4.
Infections
5.
Polycythemia
6.
Clubbing of fingers
7.
Cerebral abscess
8.
Cardiomegaly
Think Like a Nurse





What is the relationship between
polycythemia and a cardiac disorder?
What is the worst possible complication to
anticipate based on the polycythemia?
What nursing interventions are included
when planning care for this child?
When you see the RBC’s elevated – what
other lab work would you want to check?
What lab work would need to be trended?
Ask Yourself ?

Laboratory analysis on a child with Tetralogy
of Fallot indicates a high RBC count. The
polycythemia is a compensatory mechanism
for:
a. Tissue oxygen need
b. Low iron level
C. Low blood pressure
d. Cardiomegaly
Hypercyanotic Episode / “tet” spells

Cyanosis/ hypoxia suddenly worsens in
response to activity, such as crying, feeding, or
having a bowel movement.

Signs - The infant becomes very short of breath
with tachypnea and hyperpnea, and may lose
consciousness.

Treatment – calming, knee-chest position,
oxygen, morphine , and beta-blockers
p. 1088
Treatment

Open-heart Surgical interventions
 Blalock – Taussig or Potts procedure –
increases blood flow to the lungs.
Something the Lord Made
View the Movie Trailer
About Blalock procedure to treat
Tetralogy of Fallot
https://www.youtube.com/watch?v=eUf
OvjNTM2M
•
•
Truncus Arteriosus
Transposition of Great Vessels
These present the greatest risk to survival
Truncus arteriosus
 A single arterial trunk
arises from both
ventricles that supplies
the systemic,
pulmonary, and
coronary circulations. A
vsd and a single,
defective, valve also
exist.
 Entire systemic
circulation supplied from
common trunk.
Transposition of Great Vessels
 Aorta arises from the right
ventricle, and the pulmonary
artery arises from the left
ventricle - which is not
compatible with survival
unless there is a large defect
present in ventricular or
atrial septum.
artery
aorta
Review of CHD

A 3-month-old has been diagnosed with a VSD.
The flow of blood through the heart with this
type of defect is:
A.
Right to left
B.
Equal between the two chambers
C.
Left to right
D.
Bypassing the defect
Acquired Heart Diseases







Infective endocarditis
Dysrhythmias
Rheumatic fever
Kawasaki disease
Hypertension
Cardiomyopathies
High cholesterol levels
Copyright © 2018 Elsevier Inc. All rights reserved.
A systemic inflammatory (collagen) disease of
connective tissue that usually follows a group A
beta-hemolytic streptococcus infection.
This disorder causes changes in the entire heart
(especially the valves), joints, brain, and skin
tissues.
Rheumatic Fever - Assessment

Major
 Carditis
 Arthritis
 Chorea
 Erythema
marginatum
 Subcutaneous
nodules
Jones Criteria

Minor



Arthralgia
Fever
Laboratory
Findings:
Erythrocyte
sedimentation
rate
C-reactive
protein
 Prolonged PR
interval
p. 1109
Fig 46.2
What is most common sign? Most serious?
What additional laboratory
tests help to confirm the
diagnosis of Rheumatic
Fever ?
C-Reactive Protein and
Erythrocyte Sedimentation
Rate (ESR)
Rheumatic Fever

Treatment






Antibiotic Therapy
Anti-inflammatory agents – steroids
Antipyretics – aspirin
Rest
Heat and cold to joints
Discharge Teaching

Antibiotic therapy - be sure to complete all
medication
Streptococcal Prophylaxis for the
Child with Rheumatic Fever

Damaged valves can become further damaged
with repeated infections

Streptococcal prophylaxis is lifelong if there is
actual valve involvement

Intramuscular penicillin, administered
monthly, is the drug of choice

Alternatives include oral penicillin twice daily
or oral sulfadiazine once a day
Multisystem vasculitis – inflammation of
blood vessels in the body, especially the
coronary arteries, with antigen-antibody
complexes
Kawasaki Disease

Mucocutaneous lymph node syndrome
 Acute, febrile, exanthematous
(maculopapular) illness
 Generalized vasculitis of unknown etiology
 Major cause of acquired heart disease
 Cause remains unknown
 Coronary artery aneurysms are seen in 20–
25% of children left untreated
Copyright © 2018 Elsevier Inc. All rights reserved.
Signs and Symptoms / Treatment

Three Phases of clinical manifestations:
 Acute
 Subacute
 Convalescent

What is one of the most common symptoms used
to diagnose Kawasaki disease?
•
•
•
•
Temp: > 101.3 F, lasts more than 3 days
Extremely red eyes with thick discharge
Rash on main part of body and in genital area
Red, dry, cracked lips and an extremely red, swollen
tongue (Strawberry Tongue)
Acute Phase – 1-10 days






Fever, often higher than 101.3 F, lasting one - two
weeks
Extremely red eyes (conjunctivitis)
without thick discharge
Red, dry, cracked lips and an extremely red, swollen
tongue ("strawberry" tongue)
Rash on the main part of the body (trunk)
and in the genital area
Swollen, erythema on the palms of the hands and the
soles of the feet
Swollen cervical lymph nodes
Kawasaki Disease Images
Erythematous rash of Kawasaki disease. (From Lookingbill, D.P., & Marks, J.G., Jr. [1992]. Principles of
dermatology [2nd ed., pp. 223]. Philadelphia: Saunders.)
Copyright © 2018 Elsevier Inc. All rights reserved.
Subacute Phase 11-25 days

Irritability

Anorexia

Desquamation of the skin on the hands and feet,
especially the tips of the fingers and toes, often in
large sheets

Arthritis and Arthralgia

Arrhythmias

Thrombocytosis -Coronary aneurysms
Convalescent Phase

Day 26 - until the erythrocyte sedimentation
rate returns to normal
Nursing Care

Give Medications



Promote comfort and hydration





Aspirin
Intravenous Immunoglobulin
Lubricate the lips
Cool compresses or sponge baths
Keep skin cool and dry
Small feedings of soft, non-acidic foods, and liquids
that are not too hot
Facilitate joint movement

Passive Range of Motion exercises
Kawasaki Disease

Which phase of Kawasaki is this child
exhibiting?
Inflamed, Cracked,
Peeling Lips
Strawberry tongue
Kawasaki Disease
Hint:
Heart Failure

What is wrong with the heart?
Unable to circulate blood sufficient to maintain
metabolic demands of the body
Heart Failure

Why does the pump fail?
Etiology and Pathophysiology
Heart Failure

What does the body do
to compensate for this
congestion and heart
failure?
See box on p. 1083
Heart failure

What is the effect on:
 Heart rate
Contractility
Afterload
Kidneys
Preload
Compensatory Mechanisms
Brain
O2
Renal blood flow
GFR
Increase heart rate
Stimulation of renin,
Angiotension system
Afterload
Na and H2O
Intravascular volume
vasoconstriction
Systemic vascular
resistance
Myocardium stretches
Increase contractility
Hypertrophy
Preload
Early Signs of CHF

The earliest signs are often subtle:
 Infants: tachypnea, difficulty feeding
 Children: Dyspnea, tachypnea, decreased
energy
 What are the most common signs that the
family or nurse would report?
1.
2.
3.
Clinical Manifestations









Signs of respiratory distress
Wheezing, rales and rhonchi
Tachycardia
Extremities cool, capillary refill >2 seconds
Diaphoretic, sweating, hypotension
Hepatomegaly
Edema with fluid weight gain
Failure to thrive
Exercise intolerance
Clinical Manifestations cont’d







Difficulty feeding, poor weight gain
Mild tachypnea, tachycardia
Cardiomegaly
Galloping rhythm
Poor perfusion, edema
Liver and spleen enlargement
Mottling, cyanosis, pallor
Copyright © 2018 Elsevier Inc. All rights reserved.
Goal of Treatment
Stroke volume (SV): Liters/min blood ejected from heart with each heartbeat.
Cardiac Output (CO): SV X HR = CO
Treatment of Heart Failure

Medication Therapy
 Digitalis – strengthens force contractility and
decreases heart rate.


Beta Blocker - increases contractility
ACE-inhibitors - blocks release of angiotensionaldosterone; arterial vasodilator / afterload
reducing agent

Diuretics - enhance renal secretion of sodium and
water by reducing circulating blood volume and
decreasing preload,  pulmonary congestion.
CHF is B(eta blockers) A(ce inhibitors) D(iuretics)
Digitalis

Digitalization






Obtain baseline ECG before initiating Digoxin
Given in divided doses over 12-18 hrs
Check serum electrolytes
Assess vital signs and quality of peripheral pulses
Maintenance

Given daily, usually in two divided doses

Take pulse for one full minute prior to each dose
Therapeutic vs. Toxicity


Therapeutic range – 0.8 to 2.0 ng/ml
Toxicity



**EKG changes – arrhythmia
Slow pulse- bradycardia
Vomiting – very rare in infants
Nursing Care
How would the nurse
recognize digitalis toxicity in
an infant or child?
Bradycardia, decreased appetite, N/V, diarrhea, dyspnea
What are the pulse rate
criteria in administration of
digitalis?
<90 bpm infant
<70 bpm child
Digitalis

Why are we so concerned
with the potassium levels
when the child is on
digitalis therapy?
Can increase
risk of
digitoxin
toxicity
p. 1083

Nursing implications with Other
Medications
Diuretics – furosemide (Lasix)





ACE inhibitors – Captopril, Enalopril



Action – enhance renal secretion of sodium and water decreasing preload and pulmonary congestion
Monitor vital signs
Intake and output
Electrolytes
Action – promotes vasculature relaxation / vasodilation;
increases renal blood flow thereby, decreases release of
aldosterone/angiotensin – decreasing preload. It also reduces
peripheral vascular resistance, reducing afterload.
Monitor vital signs – check for signs of hypotension
Beta Blockers – Inderal
 Slow the heart rate - assess vital signs
Treatment of Heart Failure

What is the type of Diet most commonly
ordered?

Nursing care:







Measure intake and output – weighing diapers, weigh daily
Observe for changes in peripheral edema and circulation
If ascites present – take serial abdominal measurements to
monitor changes.
Skin care
Turning schedule
Promote rest
Provide Oxygen
Feeding the child with HF

Feed the infant or child in a relaxed environment;
frequent, small feedings may be less tiring

Hold infant in upright position; may provide less
stomach compression and improve respiratory effort

If child unable to consume appropriate amount during
30-minute feeding q 3 h, consider nasogastric feeding

Monitor for increased tachypnea, diaphoresis, or
feeding intolerance (vomiting)

Concentrating formula to 30 kcal/oz may increase
caloric intake without increasing infant’s work
Educating Parents

Signs and symptoms of heart failure
 Increased cyanosis
 Dehydration
 Infection
 Dysrhythmias
 Decreased nutritional intake
Copyright © 2018 Elsevier Inc. All rights reserved.
Review

An infant with heart failure is receiving digoxin.
What should the nurse assess prior to
administrating the medication?
A.
Pulse oximetry
B.
Apical pulse rate
C.
Breath Sounds
D.
Liver function tests
Cardiac Catheterization
Invasive procedure that can be
1-Diagnostic
•
•
Measure oxygen saturations and pressure in the
cardiac chambers and great arteries
Evaluate cardiac output
2-Therapeutic
3-Interventional
Cardiac Catheterization

This process involves passing a
catheter through the femoral
vein or artery into the heart.

Performed to evaluate heart
valves, heart function and blood
supply, or heart abnormalities in
newborns.
Cardiac Catheterization

Pre-care:





History and Physical
Lab work – EKG, ECHO cardiogram,
CBC
NPO
Vital signs
Preprocedural teaching
Best Nursing Action
Situation: During post-procedure
assessment every 5-15 minutes, the
nurse notes bleeding at the insertion
site.

What should the nurse do first?


Apply pressure on the insertion site for 10-15
minutes
What additional interventions should be
implemented post-procedure?
Post Cardiac Catheterization Care
Post Cardiac Catheterization Care
Childs leg
stabilized
keeping it
extended
Cardiac Anomalies - Treatment
The Child Undergoing Cardiac Surgery


Preoperative preparation
Postoperative management
 Monitoring cardiac output
 Supporting respiratory function
 Monitoring fluid and electrolyte balance
 Promoting comfort
 Healing and recovery
Copyright © 2018 Elsevier Inc. All rights reserved.
Review

A 7-year-old child is recovering from a severe
sore throat. The caregiver states that the child
now has chest pain, in addition – the child is
noted to have swollen joints, nodules on the
fingers, and a rash on the chest. The likely
cause of this is_______________________.
Thank you!

Resources:
 McKinney (2018). Ch. 46, p. 1077-1104; 11081112.
 Please contact Dr. Vicki Johnson at
vicki.johnson@austincc.edu with any
questions regarding this information.