2. 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
3. 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
4. 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
5. 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
6. Comparison of CHD Classification Systems—
p.1276 10th ed. Hockenberry
7. 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.
11. 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.
13. Tests of cardiac function
Prenatal ultrasound
Chest x-ray
Electrocardiogram (ECG)
Echocardiogram
Cardiac catheterization
Stress test (dobutamine
or exercise)
Cardiac MRI
14. 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
15. 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
16. 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.
17. 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
20. Symptoms of CHF
Increased work of
breathing
Tachycardia
Decreased pulses
Decreased urinary output
Poor weight gain
Diaphoresis with activity
Hepatomegaly
Cold, cool extremities,
especially with stress or
activity
JVD?
Decreased BP is LATE
sign
21. Defects with Increased Pulmonary Blood Flow
Abnormal connection
between two sides of heart
leads to
Increased blood volume on
right side of heart
Increased pulmonary
blood flow
Decreased systemic blood
flow
PDA, ASD, VSD
Symptoms
Increased work of
breathing
Rales/rhonchi
and/or wheezing
Failure to thrive
22. Patent Ductus Arteriosus
Ductus doesn’t close
Common in preemies
“machinery” murmur
audio
Treatment
Indomethacin
Cath lab
Ligation
23. 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
24. 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
25. 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
26. 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
28. 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!!!!!!!
30. 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
31. 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.
32. 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
33. Effects of Hypoxemia
Main clinical manifestations:
Cyanosis
Polycythemia
Thicker blood
Clubbing
Clotting abnormalities
Delayed growth and development – can be associated
with any heart defect
34. 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!!!!!
36. 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
39. 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%!
40. 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
41. 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
42. 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
43. 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.
44. Post-operative Care
PAIN!!!!!!!!!!!!!!!!!!!
Cardiac monitoring
Heart rate
Blood pressure
Intracardiac pressures
Chest tube care
Quantity & quality of
output
Urine output
Minimum 1 ml/kg/hour
Neurological checks
Move all extremities
Back to baseline
Respiratory care
Deep breathing
IS
Rest & activity
Up next day
Ambulate
GI distress
Avoid vomiting
45. 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
46. 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
47. 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
48. 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’
49. 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/
50. Newburger, J. W. et al. Circulation 2004;110:2747-2771
From the
American Heart
Association
p. 1300
10th ed.
51. 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.