Pediatric Cardiac Disorders

Cardiac Disorders in Peds

Two major groups of disorders:
◦ Congenital
◦ Aka “born with”
◦ Most structural defects
◦ Acquired
◦ Develop later in life
◦ Bacterial endocarditis
◦ Rheumatic fever
◦ Kawasaki disease
◦ Systemic HTN
 Incidence & Causes

5 to 8 in 1000 live births

Cause unknown
◦Multiple factors
◦ Genetics/family history
◦ Environment
◦ Toxins
◦ Viruses
◦Maternal chronic illness (diabetes, seizure
meds)
◦Chromosomal abnormalities
◦Down syndrome
◦Di George syndrome
◦Noonan or William syndrome
◦Trisomy 13 or 18
 Older Classifications of CHD
Acyanotic
◦ “pink”
◦ NO unoxygenated blood goes to the periphery
Cyanotic
◦“blue”
◦ Unoxygenated blood is shunted to the periphery
◦ May be pink
Newer Classifications of CHD
Hemodynamic characteristics
◦ Increased pulmonary blood flow
◦ Too much to lungs; “pink”; pulmonary edema
◦ Decreased pulmonary blood flow
◦ Too little to lungs; “blue”; cyanotic
◦ Obstruction of blood flow out of the heart
◦ Can’t get to lungs or body
◦ Mixed blood flow
◦ Most common
Comparison of CHD Classification Systems—
p.1276 10th ed. Hockenberry
Background info/Hemodynamics
 Review fetalth to neonatal circulation
th
(pp. 1342-1343
Hockenberry, 9 ed.; pp. 1252-1253, 10 ed.) See Khan Academy links on
Course Calendar. 
 Blood flows from area of high pressure to one of low
pressure (Fig. 34-7 p. 1351 Hockenberry, 9th ed.; Fig. 29-7 p. 1262, 10th
ed.)
 The greater the pressure gradient, the greater the rate
of flow.
 The greater the resistance, the lower the rate of flow
 In the NORMAL HEART, pressures on the R side are less
than the L side, and the resistance in the pulmonary
circulation is less than that in the systemic circulation.
Fetal circulation
Fetal to Neonatal Circulation
p.1262 10th ed.
Rahi, A., Grosse, SD, Ailes, EC, Oster, ME. Association of US State Implementation of
Newborn Screening Policies for Critical Congenital Heart Disease With Early Infant
Cardiac Deaths. JAMA. 2017;318(21):1-8.

Main Findings from this Study—Critical Congenital Heart Disease

Screening mandated in certain states.
A 33% decline in infant deaths from CCHD and a 21% decline in
infant deaths from other or unspecified cardiac causes were
observed in eight states with mandated and implemented
screening policies compared to states without mandated
screening policies.
Adoption of other screening policies (voluntary policies or
mandatory policies not yet implemented) was not associated with
reductions in infant deaths.
Mandatory screening policies applied nationwide are projected to
save about 120 babies each year.
The pulse-oximetry monitoring protocol based on results from the right hand (RH) and either
foot (F).

Kemper A R et al. Pediatrics 2011;128:e1259-e1267

©2011 by American Academy of Pediatrics

Tests of Cardiac Function

Prenatal ultrasound
Chest x-ray
Electrocardiogram (ECG)
Echocardiogram
Cardiac catheterization
Stress test (dobutamine or
exercise)
Cardiac MRI
 Cardiac Catheterization
Invasive routine diagnostic procedure
Benefits:
◦ Better visualization
◦ Actual pressures, sats, hemodynamic values
Risks:
◦ Hemorrhage
◦ Fever
◦ N/V
◦ loss of a pulse
◦ transient dysrhythmias
Nursing interventions for Cardiac Catheterization
(pp.1348-9 , 9th ed., pp. 1258-60, 10th ed. Hockenberry)

Pre-procedure:
◦ Complete a thorough hx & physical exam
◦ Check for allergies to iodine and shellfish
◦ Age appropriate teaching & preparation
◦ Don’t forget the parents
◦ NPO 4-6 hrs before procedure; sedation~ IV or po
◦ Monitor VS, SaO2, Hgb, Hct, coags, BMP
◦ Mark pedal pulses—before procedure to ensure correct
palpation afterwards.
◦ Determination the amount of sedation based on the
child’s age, condition & type of procedure
 Cardiac Catheterization
 Post-procedure:

 For bleeding at site of insertion of catheter in groin

 pulses esp. distal to site of insertion, temp & color
of extremities,
VS q 15
 Remember the 5 P’s (pain, pallor, pulse,
paresthesia, paralysis) OR CMTS—circulation,
mobility, temperature, sensation
 Heart rate for one full minute, for signs of
dysrhythmias or bradycardia
 Prevent bleeding by keeping leg immobilized for 4-
8 hrs
 I & O, especially O. Fluids may be offered po
starting with clear liquids.

 Labs; infants are at risk for hypoglycemia—monitor

blood glucose as child may need IV with dextrose
 Encourage the child to void to promote excretion
of contrast medium.
 Cardiac Catheterization
(cont’d)
Potential cardiac catheterization complications:
◦ Nausea &/or vomiting
◦ Low-grade fever
◦ Loss of pulse in catheterized extremity
◦ Transient dysrhythmias
Acute hemorrhage from entry site
◦ apply direct continuous pressure at 2.5cm above the catherter
entry site to localize pressure over the location of the vessel
puncture.
◦ Keep child flat and notify the physician
◦ Prepare for possible administration of additional fluids prn
Cath lab
Congestive heart failure
(Fig. 34-8 p. 1353, 9th ed. ; fig. 29-8 p. 1263, 10th ed. Hockeberry)
 Symptoms of CHF
Increased work of breathing Hepatomegaly
Tachycardia Cold, cool extremities,
especially with stress or
Decreased pulses
activity
Decreased urinary output
Decreased BP is LATE
Poor weight gain sign
Diaphoresis with activity
Defects with Increased Pulmonary Blood
Flow
Abnormal connection
PDA, ASD, VSD
between two sides of heart
leads to Symptoms
◦Increased blood volume ◦ Increased work of
on right side of heart breathing
◦ Rales/rhonchi and/or
◦Increased pulmonary wheezing
blood flow
◦ Failure to thrive
◦Decreased systemic
blood flow
Patent Ductus Arteriosus
Ductus doesn’t close Common
in preemies “machinery”
murmur
audio
Treatment
◦ Indomethacin
◦ Cath lab
◦ Ligation
Atrial Septal Defect
Hole between two atria of
heart
Usually asymptomatic
If not treated, increased
risk of atrial dysrhythmia
or stroke
Usually close on own
Ventricular Septal Defect
Hole between two
ventricles of heart
Symptoms related to size
& location of VSD and
amount of pulmonary
blood flow
Fix by patching with
Goretex
Atrioventricular Canal Defect
ASD, VSD, and affected mitral
& tricuspid valves
Associated with Down
syndrome
Symptoms related to size of
holes, degree of valvular
involvement, & size of
ventricles
Often accompanied with
pulmonary hypertension
Nursing Management
 AVOID OXYGEN!!!!!!!!—use
judiciously
 Especially pre-op
 Diuretics—furosemide,
chlorothiazide, spironolactone
 Monitor VS, I & 0, daily wt.
 Encourage rest periods to
conserve energy
 Monitor labs: Hgb, Hct,
electrolytes
 Closely monitor feedings
 May need higher calorie feeds
OBSTRUCTIVE DEFECTS
Coarctation of the
aorta, aortic stenosis,
pulmonic stenosis
Symptoms dependent
upon area of
obstruction
COARCTATION OF AORTA
Narrowed aorta leads to
decreased systemic blood flow
May not present until early
childhood
Bounding upper extremity
pulses, weak to absent lower
extremity pulses
HYPERTENSION!!!!!!!
Post-Op Coarctation Care

◦Neuro checks
◦Urine output
◦Blood pressure
◦PAIN!!!!!!!
AORTIC STENOSIS
Obstructs blood flow to body
Leads to left ventricle
hypertrophy
Asymptomatic often
Chest pain with exercise
Sometimes see sudden death
Repair with ballooning, repair,
or replacement of valve
Pulmonary Stenosis and Catheter
Placement

Leads to right ventricular hypertrophy

which may lead to reopening of the
foramen ovale. If severe, my lead to
congestive heart failure.
Defects with Decreased Pulmonary Blood
Flow and Mixed Defects
May or may not be cyanotic (usually are)
Tetralogy of Fallot
Transposition of Great Arteries
Truncus Arteriosus
Hypoplastic Left Heart Syndrome (HLHS)
LOTS of other defects that are uncommon, book
discusses them
Effects of Hypoxemia
Main clinical manifestations:
◦ Cyanosis
◦ Polycythemia
◦Thicker blood
◦ Clubbing
◦ Clotting abnormalities
◦ Delayed growth and development – can be
associated with any heart defect
Hypoxemia Management
Prostaglandin E1 given if cyanosis shown
as newborn
Assess for and treat tet spells
Surgery
Corrective or palliative—often staged
Prevent dehydration
AVOID OXYGEN!!!!!
Tetralogy Of Fallot
Hypercyanotic “tet spells”
Acutely cyanotic
↓ pulm. blood flow & ↑ right to left shunting
Prompt tx to prevent brain damage &/or death
◦ Calm infant/child
◦ Place in knee chest position
◦ Toddler will get in “squatting” position to compensate for
hypoxia
◦ Give oxygen
◦ Morphine/fentanyl/versed given
Knee-Chest Position
Tet Repair
Complicated
Dependent on how big RV is,
how stenotic pulmonic valve is,
and how big the VSD is
Either fly or die
Palliative shunt: modified
Blalock-Taussig shunt (p.1364, Table
34-4, 9th ed.
Fig. 29-11, p.1274, 10th ed.)
Complete repair—operative
mortality <3%!
 Transposition of the Great Arteries

 NOT GOOD!
 Cath lab initially
 Prostaglandins
 Surgery at 6-7 days old—arterial
switch of pulmonary artery and
aorta, but also coronary arteries
are switched and re-
anastomosed.
 Long term prognosis very good
Hypoplastic Left Heart Syndrome

 VERY VERY VERY BAD!! However--Survival rates have changed

dramatically in the last 15 years. Can be has high as 95%
 Can not correct easily—parents must choose…
 3 staged surgeries: Norwood, Mod Blalock Taussig, & Glenn procedure
vs. transplant
 Long-term data not in yet, will probably need transplant
Management of Children with Mixed Defects
 Medications
 Digoxin—KNOW!! pp.1354-  Decrease cardiac workload
1358, 9th ed. P.1269-70, 10th ed.—
good info on meds  Meds-as stated
 Improves contractility of heart  Decrease stimulation
 Review dig toxicity—pulse rates in
infants & children  Cluster care
 Diuretics—furosemide  Maintain neutral
 Watch for what ?? thermal environment
 Ace-inhibitors (angiotensin  Sedation for irritable
converting inhibitors—the
PRIL’s) child
 Reduce afterload on the heart make  Remove accumulated fluid
heart pump more efficiently.
 Beta-blockers—cause & sodium
decreased heart rate, BP *  Closely monitor I&O
vasodilatation  Restrict fluid in acute phase
 Weigh daily if stable
Continued management of CHF
 Nutrition
 Smaller, more frequent
 Improve tissue oxygenation
feeds
 High calorie formula  Meds assist with this by
increasing efficiency of the
 Decrease respiratory effort heart
 Rest  Oxygen may be added with
 Avoid colds, RSV appropriate order, especially if
 Position with HOB there is pulmonary edema, or
 Avoid crying and distress lower respiratory infection.
 Family support/education
 Keep them present, holding,
rocking, AMAP
 Post-operative Care
PAIN!!!!!!!!!!!!!!!!!!! Neurological checks
◦ Move all extremities
Cardiac monitoring
◦ Heart rate ◦ Back to baseline
◦ Blood pressure Respiratory care
◦ Intracardiac pressures ◦ Deep breathing
Chest tube care Rest & activity
◦ Quantity & quality of output ◦ Up next day
◦ Ambulate
Urine output
◦ Minimum 1 ml/kg/hour GI distress
◦ Avoid vomiting
Care of the Family and Child with
Congenital Heart Disease

 Help family adjust to the disorder

 May be grieving loss of normal child
 Educate family
 Help family cope with effects of the disorder
 Prepare child and family for surgery
 Remember developmental level of child
 Pain, scars, IS, activity
 Refer to support group with families who have
already been through the experience
 TOUCH is the IL Assoc. This link opens a broad
site, then click on IL.
 Congenital Heart Information Network: lots of
links for families and persons with CHD
 Website: From Cincinnati Children’s Hospital
 Kawasaki Disease
Multisystem disorder involving vasculitis & may
progress to coronary arteries causing aneurysm
formation
Leading cause of acquired heart dz in US
Etiology still unknown
3 phases:
◦ acute
◦ subacute
◦ convalescent
Criteria for KD (must meet 5 out of 6)

Box 34-10, p. 1388, Hockenberry (9th ed); Box 29-9,

p. 1299 (10th ed.)
◦ fever > 5 days
◦ conjunctival infection without exudate
◦ oral changes: erythema, “strawberry tongue,
fissured lips
◦ extremities changes: peripheral edema, erythema of
palms and soles, peeling of hands & feet
◦ erythematous rash
◦ cervical lymphadenopathy
 Other manifestations
Symptoms of inflammation
◦  C reactive protein level
◦  ESR
Cardiac symptoms
◦  L ventricular function as seen on Echocardiogram
◦ Children do NOT generally have sx of CHF
Other lab changes
◦ Anemia
◦ Leukocytosis with ‘L shift’
Kawasaki continued
 Tx best within first 7- 10 days. :
 ASA 80-100mg/kg/day initially.
This is one dx that requires use of high doses of
aspirin even in children. Dose is decreased to 3-5
mg/kg/day once afebrile 48-72 hrs.
 IVIG 2 g/kg over 8-12 hr
 Here is a website with some good information
on the diagnosis and management of this
disease:
 http://www.kdfoundation.org/
 From the
American Heart
p. 1300 Association
10th ed.
Newburger, J. W. et al. Circulation 2004;110:2747-2771
Education of Parents
Teach parents common signs of Aspirin toxicity while on
high doses of ASA
◦ Tinnitus
◦ Headache
◦ Dizziness
◦ Confusion
Teach parents to report recurrence of fever
Teach parents CPR
Inform parents that final cardiac sequelae may not be
known for some time.
QUESTIONS