Pediatric Cardiac

Disorders

Dr. Muthia Mutmainnah S.Kp.,M.Kep.,Sp.Mat

FAKULTAS KEDOKTERAN DAN ILMU KESEHATAN
UNIVERSITAS JAMBI
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
 DiGeorge 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 fetal to neonatal circulation (pp. 1342-1343 Hockenberry,
9th ed.; pp. 1252-1253, 10th 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,
Decreased pulses especially with stress or
Decreased urinary output activity
Poor weight gain JVD?
Diaphoresis with activity Decreased BP is LATE
sign
Defects with Increased Pulmonary Blood Flow

Abnormal connection PDA, ASD, VSD

between two sides of heart Symptoms
leads to
Increased blood volume on Increased work of
right side of heart breathing
Increased pulmonary Rales/rhonchi
blood flow and/or wheezing
Decreased systemic blood
Failure to thrive
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
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-
1358, 9th ed. P.1269-70, 10th ed.—
good info on meds
 Improves contractility of heart
 Review dig toxicity—pulse rates in
infants & children
 Diuretics—furosemide
 Watch for what ??
 Ace-inhibitors (angiotensin
converting inhibitors—the
PRIL’s)
 Reduce afterload on the heart
make heart pump more efficiently.
 Beta-blockers—cause
decreased heart rate, BP *
vasodilatation
Decrease cardiac workload
 Meds-as stated
 Decrease stimulation
 Cluster care
 Maintain neutral thermal
environment
 Sedation for irritable child
Remove accumulated fluid
& sodium
 Closely monitor I&O
 Restrict fluid in acute phase
 Weigh daily if stable
Continued management of CHF
Nutrition
 Smaller, more frequent
feeds
 High calorie formula
Decrease respiratory effort
 Rest
 Avoid colds, RSV
 Position with HOB
 Avoid crying and distress
Family support/education
 Keep them present, holding,
rocking, AMAP
Improve tissue
oxygenation
 Meds assist with this by
increasing efficiency of the
heart
 Oxygen may be added with
appropriate order,
especially if there is
pulmonary edema, or lower
respiratory infection.
Post-operative Care
Neurological checks
PAIN!  Move all extremities
 Back to baseline
Cardiac monitoring
 Heart rate Respiratory care
 Blood pressure  Deep breathing
 Intracardiac pressures  IS

Chest tube care Rest & activity

 Quantity & quality of  Up next day
output  Ambulate
Urine output GI distress
 Minimum 1 ml/kg/hour  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.
THE END