This document discusses cardiac disorders in pediatrics, including congenital and acquired disorders. The two major groups are congenital disorders present at birth, and acquired disorders that develop later in life such as bacterial endocarditis. Common congenital defects include atrial and ventricular septal defects, tetralogy of Fallot, transposition of the great arteries, and hypoplastic left heart syndrome. Management involves medications, oxygen management, nutrition, and surgery depending on the specific defects. Post-operative care focuses on pain management, cardiac monitoring, and family support.

1. It’s a worthwhile area to study! 

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.

8. Fetal circulation

9. Fetal to Neonatal Circulation

10. p.1262 10th ed.

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.

12. 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

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

18. Cath lab

19. Congestive heart failure
(Fig. 34-8 p. 1353, 9th ed. ; fig. 29-8 p. 1263, 10th ed. Hockeberry)

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

27. Obstructive Defects
 Coarctation of the
aorta, aortic
stenosis, pulmonic
stenosis
 Symptoms
dependent upon
area of obstruction

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!!!!!!!

29. Post-op Coarctation Care
Neuro checks
Urine output
Blood pressure
PAIN!!!!!!!

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!!!!!

35. Tetralogy Of Fallot

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

37. Knee-Chest Position

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.

52. QUESTIONS