Pediatric Cardiology

Betsy Johnson, MSN, CPNP-PC

Aya Rahav, MS, RN, CPN
Objectives
• Describe fetal circulation and the changes that
occur in the transition to postnatal circulation;
• Compare and contrast the anatomy and physiology
of various congenital cardiac defects;
• Relate therapeutic management principles in the
care of a pediatric patient with heart failure;
• Describe rheumatic heart disease and Kawasaki
disease
• Relate the components of a comprehensive cardiac
history and physical exam; and
• Explain the nursing care of a pediatric patient
requiring cardiac catheterization.
In Utero
Embryologic cardiac development
First heart contraction: ~21 days after conception
Blood flow begins in the 4th week
Critical period: 20-50 days after conception
Fetal Circulation
 Fetal circulation
 Pressures
 Placenta = low pressure (low
systemic vascular resistance)
 Lungs = high pressure (high
pulmonary vascular resistance)
 R side of heart > L side of heart
 Shunts
 Foramen ovale (FO): connects R
and L atria
 Ductus arteriosus (DA):
connects main pulmonary artery
and aorta
 Ductus venosus (DV): connects
umbilical vein to inferior vena
cava
Transition to Postnatal Circulation
• Umbilical cord clamped and baby takes first
breath
▫ Pressures change
 Systemic vascular resistance > pulmonary vascular
resistance
▫ Shunts close
 Pressure changes  closure of FO
 Increased O2 & removal of Prostaglandin E
(PGE)  closure of DA
 Absence of umbilical venous flow  closure of DV
 6

Copyright © 2017,
Elsevier Inc. All
Rights Reserved.

Changes at Birth
Cardiovascular disorders
Congenital Cardiac Disorders Acquired Cardiac Disorders
• Present at birth  Occurs after birth
• Anatomic abnormality,  Disease process
abnormal function
 Anatomically normal or
• Due to:
abnormal
▫ Genetics
 Due to:
▫ Environment
 Infection
▫ Adverse maternal conditions
 Autoimmune responses
 Environment
 Genetics
Cardiac Assessment: History
• Prenatal history
▫ Alcohol use, drugs (Rx and street), infections, toxin exposure;
maternal health (e.g. DM)
• Patient medical history
▫ Chromosomal abnormalities, syndromes, congenital defects
• Family medical history
▫ Increased risk if first-degree relative affected (sibling) with
CHD increases risk threefold
• Review of systems
▫ Failure to thrive, poor weight gain, activity intolerance,
developmental delays
 Cardiac Assessment: Physical Exam
• Cardiovascular assessment
▫ Inspect color centrally and peripherally: maybe cyanotic, pale
▫ Auscultate heart sounds; determine apical rate
▫ Palpate chest for pulsations, heaves, vibrations. Locate PMI
▫ Palpate peripheral pulse rate, rhythm, quality. Compare bilaterally
and UE to LE
▫ Palpate capillary refill
▫ Assess for edema
▫ Take blood pressure and compare to expected values for age,
gender, height
• Respiratory assessment
▫ Inspect work of breathing (WOB); determine rate
▫ Auscultate breath sounds
Cardiac Assessment: Physical Exam Cont.
• Fluid status assessment
▫ Inspect for periorbital, facial, peripheral edema
▫ Assess fontanels
▫ Inspect and palpate for signs of dehydration
▫ Inspect and palpate abdomen for distention
▫ Palpate liver: blood can back up into liver and cause
hepatomegaly
• Activity assessment
▫ Assess feeding tolerance and growth trends.
▫ Observe activity tolerance and changes in activity level.
▫ Monitor for abrupt behavior changes, restlessness,
irritability, or changes in level of consciousness.
Physical Indicators of Cardiac Dysfunction

• Tachycardia/ Bradycardia • Poor feeding

• Murmur
• Unequal pulses/ BPs
• Prolonged cap refill time
• Diaphoresis, fatigue
• Hepatomegaly
• Sternal lift
• Gross motor delays
• Poor growth
• Tachypnea, Dyspnea
• Crackles
• Cyanosis/ pallor
• Exercise intolerance
When obtaining a history from the parents of an
infant suspected to have altered cardiac
function, the nurse would expect to hear:
A. specific concerns related to
palpitation the infant is
having.
B. vague, nonspecific
complaints such as feeding
difficulties.
C. specific concerns about the
infant’s shortness of breath.
D. all the above.
 Diagnostic Tests
• In utero – ultrasound (@about 20 weeks GA)
• CCHD screen (4 extremity pulse oximetry)
• Chest X-ray: Heart size & pulmonary blood flow patterns
• EKG: Electrical activity of heart, dysrhythmias
• Arterial Blood Gas
• Echocardiography: Sound waves used to produce an image of
cardiac structures
• Cardiac catheterization:
▫ Measures O2 Sats in chambers, pressure gradients
▫ Identifies anatomy & blood flow patterns
▫ Biopsies may be obtained
▫ Interventional cardiac catheterization
Interventional Cardiac Catheter
Procedures in Children
• Examples:
▫ Transposition of the great vessels
▫ Some complex single-ventricle defects
▫ Atrial septal defect
▫ Pulmonary artery stenosis
Cardiac Catheter Pre-Procedure Care
• Assess and mark pulses • Baseline O2 sats, VS
• Height and weight • Nothing by mouth (NPO) prior
• History of Allergies (iodine) to the procedure (6-8hr)
• Use developmentally • Void prior to procedure
appropriate materials to • Lower extremity pulses, skin
explain the procedure to the color, temperature
child • IV fluids
• Sedation vs general anesthesia
 Cardiac Catheter Post-procedural care
• Take the vital signs every 15
minutes
• Check the pulse distal to the site
• Monitor the temperature, cap
refill, & color of extremities
• Bedrest/ pressure dressing 6-8
hours
• Keep leg straight for 6-8 hours
 sedation for younger children
who won’t lay flat
• Monitor the dressing for
bleeding or hematoma
Discharge Instructions:
• Limit activity for 3 days
• Monitor site: keep clean & dry
▫ Can take a shower; no bath
• Monitor for
infection/complications/bleeding
▫ Tired, fever  return ASAP
• Fluid Intake: ensure adequate
hydration and monitor for s/s
dehydration
Nursing care for the child prior to cardiac
catheterization includes which of the following.
Select all

A. Determining history of latex

allergy.
B. Obtaining base-line pedal
pulses and pulse oximetry.
C. Ensuring that the child eats
a meal just prior to the
procedure.
D. Reviewing laboratory
results including platelet
count.
E. Describing what to expect to
the child and family
Congenital Cardiac Defects
Congenital Heart Disease
• Incidence (5-8/1,000)
▫ Major cause of death in 1st year of life
▫ Most common is ventricular septal defect (VSD) 14-16%
▫ Gender trends
• Etiology
▫ Exact causes unknown
▫ Multifactorial inheritance (90%)
▫ Genetics (8%)
▫ Environmental teratogens (2%)
▫ In 28% of kids with CHD, another recognized anomaly
is also present
Overview of Congenital Cardiac Defects
Acyanotic Cyanotic

Increased Obstruction of Decreased Mixed blood

pulmonary blood flow from pulmonary flow
blood flow ventricles blood flow

• ASD • Coac. of aorta • TOF • TGA

• VSD • Aortic stenosis • Tricuspid • Total anomalous
• PDA • Pulm. stenosis atresia pulm. venous return
• AV canal • Truncus arteriosus
• HLHS
Acyanotic
Increased Pulmonary Blood Flow
• Due to:
▫ Septal defects
▫ Abnormal connections
between the great vessels
• Pressures:
▫ Left > Right
• Resultant shunt:
▫ Left to Right
• Volume changes:
▫ Right > Left
• Results in increased blood
flow to lungs
Increased Pulmonary Blood Flow -
Symptoms
• Increased HR/RR
• Diaphoresis
• Tire with feeds
• Poor weight gain
• Leads to CHF: dyspnea, tachypnea, retractions,
periorbital edema
• Increased respiratory infections
• Murmur: varying degrees
 Atrial Septal Defect (ASD)

• Clinical manifestations
▫ Asymptomatic
▫ May develop heart failure (HF)
later

• Treatment
▫ May close spontaneously (if small
ASD)
▫ Surgically inserted patch
▫ Non-surgical catheter procedure
▫ Medical treatment of CHF
 Ventricular Septal Defect (VSD)
• Most common lesion- opening
between ventricles
• LR shunt (if pulmonary HTN
develops - R to L shunt with cyanosis)
• Clinical manifestations
▫ Size dependent: ranges from
asymptomatic to symptoms of HF
▫ Characteristic murmur
• Treatment
▫ May close spontaneously
▫ Surgical suture or patch
▫ Banding of pulmonary artery
▫ Medical treatment of CHF
 http://www.easyauscultation.com/cases?c
oursecaseorder=4&courseid=29
Persistence of Fetal Circulation
 Patent Ductus Arteriosus (PDA)
• Failure to close within 1st week of life
• More common in preterm infants
• LR shunt (if pulmonary HT develops R to
L shunt with cyanosis)
• Clinical manifestations
▫ Asymptomatic to symptoms of HF
▫ Machinery-like murmur
▫ Widened pulse pressure
▫ Bounding pulses
• Treatment
▫ Spontaneous closure (small PDA)
▫ Indomethacin
 Opposite of prostaglandin
 Strong NSAID that can close PDA
▫ Surgical ligation
▫ Non-surgical catheter procedures  http://www.easyauscultation.com/cases?
coursecaseorder=2&courseid=29
Obstructive Defects
• Obstruction of blood flow from the
heart due to stenosis (narrowing)
▫ Usually near valve
• Pressures:
▫ Area before obstruction > area after
obstruction
• Results in a pressure load on the
ventricle and decreased cardiac output
• Enlargement heart
• CHF, pulmonary edema
• Diminished pulses lower extremities,
delay cap refill
• Decreased UOP
• Arrhythmias
 Coarctation of the Aorta
• Aortic constriction where the ductus arteriosus
used to be
• Pressures
▫ Increased in head and upper extremities (UE)
▫ Decreased in body and lower extremities (LE)
• Clinical manifestations
▫ Increased BP in the upper extremities
▫ Decreased to absent pulses in LE
▫ Bounding pulses in UE
▫ 4 extremity BP: UE > LE by at least 20mmHg
▫ 4 extremity pule ox: : UE > LE by at least 3 points
▫ May be signs of HF in infants
▫ Older kids: dizziness, headaches, fainting, epistaxis
▫ Leg pain due to lack of oxygen
• Treatment
▫ Surgical repair
▫ Non-surgical dilation and/or stent placement
Aortic Stenosis
Narrowing or stricture of aortic valve
Leads to:
 LV outflow resistance
 Decreased cardiac output
 LV hypertrophy
 Pulmonary vascular congestion
Clinical manifestations
 Newborns: faint pulses, hypotension,
tachycardia, poor feeding, chest pain
 Older kids: exercise intolerance, chest pain,
dizziness and fainting with prolonged
standing
 Characteristic murmur
Treatment
 Surgical replacement or dilation
Pulmonic Stenosis
Narrowing at the entrance to the
pulmonary artery
Leads to:
 RV outflow resistance
 RV hypertrophy
 Decreased pulmonary blood flow
Clinical manifestations
 Dependent upon severity of lesion
 Range from asymptomatic to mild
cyanosis to HF
 Characteristic murmur
Treatment
 Surgical repair
 Non-surgical dilation
Cyanotic
Decreased pulmonary blood flow
• Due to:
▫ Obstruction of pulmonary blood
flow AND
▫ Anatomic defect between L & R
sides of heart
• Pressures:
▫ Right > Left
• Resultant shunt:
▫ Right  Left
• Results in:
▫ Hypoxemia
▫ Cyanosis (usually)
Decreased pulmonary blood flow –
Signs/Symptoms
• Cyanosis
• Hypercyanotic episodes
• Dyspnea/Fatigue - Increase w/exertion
• Polycythemia
• Clubbing
• FTT/Delayed milestones
• Murmur

Note: Prostaglandin E will keep ductus arteriosus

open in an emergency until surgery
 Tetralogy of Fallot (TOF)
• The most common complex heart
disease
• Four defects:
▫ Pulmonic stenosis
▫ RV hypertrophy
▫ Overriding aorta
▫ VSD: non-restrictive (pressure in R and L
ventricle are the same)
• Hemodynamics vary depending upon
severity of defects
• Clinical manifestations
▫ Varying degrees of cyanosis
▫ Systolic murmur bc of pulmonic stenosis
▫ Tet spells (blue or pink)
• Treatment  http://www.easyauscultation.com/ca
▫ Surgeries: palliative or corrective ses?coursecaseorder=5&courseid=
29
Hypercyanotic Spells (Tet Spells)
• Occurs due to reduction of pulmonary blood
flow leading to R to L shunt
• Precipitating events:
▫ Crying
▫ Eating
▫ Awakening from naps
 Change in position causes increase in pulmonary resistance
▫ Tachycardia
▫ Fever
▫ Dehydration
▫ Medications
Hypercyanotic Spells (Tet Spells) Cont.
• Presentation: • Nursing Management:
▫ Cyanosis ▫ Knee-to-chest position
▫ Increased RR/HR (squatting, or hold child
in this position)
▫ Diaphoresis
▫ Oxygen
▫ Irritability ▫ Morphine – decreases HR
▫ Seizures and oxygen demand
▫ LOC ▫ IVF – helps with
intracardiac mixing
▫ Calm the child – teach
parents to stay calm
TOF - Hypoxemia Compensation
• Squatting
(characteristic of TOF)
▫ Unconscious attempt to
relieve chronic hypoxia.
May occur several times a
day.
 Reduces venous blood
flow from lower
extremities (which is
desaturated)
 Increases systemic
vascular resistance
 Common!
Mixed blood flow
• Hemodynamics:
• O2’d systemic blood + de-O2’d pulmonary blood = relatively de-
O2’d systemic blood
• Pressures:
• aortic pressure > pulmonary artery pressure
• Resultant shunt: LR
• Leads to:
• Pulmonary congestion
• Signs of HF
• Cyanosis (not always visible)
Transposition of the Great Arteries (TGA)
• Reversal of great arteries
• Systemic and pulmonary
circulations are independent
parallel circuits
▫ Requires septal defect or PDA
to allow for mixing
• Clinical manifestations
▫ Dependent upon type and size
of associated defects
• Treatment
▫ PGE (vasodilator) to maintain
PDA
▫ Surgical correction
Truncus Arteriosus
• Single great artery arises from both
ventricles, overriding a VSD
• Clinical manifestations
▫ HF with bounding pulses and
widened pulse pressure
▫ Pulmonary hypertension
▫ Right-left shunt
▫ Poor growth
▫ Activity intolerance
▫ HF within weeks
• Treatment
▫ Surgical repair
▫ Close follow-up
Hypoplastic Left Heart Syndrome (HLHS)
• Underdeveloped left side of the
heart leads to pulmonary venous
congestion and edema
• PDA required for systemic blood
flow
• Clinical manifestations
▫ Progressive cyanosis, decreased
cardiac output, signs of HF
▫ Common cause of death in the 1st
week of life • Defects:
• Treatment • Hypoplastic LV
• ASD
▫ Stabilize with PGE
• Small aorta
▫ Surgical repair
• PDA
▫ Transplant if surgery doesn’t work
Hypoplastic Left Heart Syndrome
• Norwood procedure
▫ Within a week of life
▫ Enlarge ASD
▫ Close PDA
▫ Blalock-Taussig (BT) shunt is
placed between pulm. arteries
and aorta to supply blood to
the lungs
▫ Main pulm. artery and aorta
connected to create
functioning aorta
▫ Convert RV to main ventricle.
Blood is pumped both to
lungs and body
Hypoplastic Left Heart Syndrome
• Bi-directional Glenn
Procedure
▫ 4-6 months after Norwood
▫ Shunt placed in Norwood is
disconnected
▫ SVC connected to R PA
Hypoplastic Left Heart Syndrome
• Fontan Procedure
▫ Performed 18-36 months
after Glenn
▫ Connects IVC to L PA
Use of Oxygen Cardiac Defects
• Be cautious!
• Use of oxygen
blender – allows
for titration of O2%
• Can give flow
Closed Heart Procedures
• Examples:
▫ Shunt/conduit
placement
▫ ASD closure with
cardiac catheterization
and umbrella placement
▫ Radio ablation of
abnormal pathways
Surgical Interventions
• Examples:
▫ Open-heart surgery
▫ Staged procedures
• Prepare the child and
family for the procedure
Post-operative Nursing Considerations
• Main goals for the nurse to focus on:
▫ Maintaining a patent airway with adequate
oxygenation
▫ Maintain tissue perfusion/ suture integrity
▫ Alleviating pain
▫ Providing adequate hydration and nutrition
▫ Prevent complications
Post-Operative Nursing Care
• Assessment:
▫ VS
▫ Pulses: BL
▫ Surgical site: s/o infection
▫ Chest tubes
▫ I’s & O’s
 Daily weight: fluid status
 Slow progression of feeds
 Activity - monitor tolerance
▫ Pain control
▫ Sternal Precautions
Congestive Heart Failure
Heart Failure: Most Heart muscle Decreased Blood flow
common damaged to kidneys =
complication of CHD hormone response

• Double pump & each • LR shunting • Angiotensin II 

side can fail but (volume overload) vasoconstriction
unusual to see one- • Decreased • Aldosterone  Na+
sided failure in contractility retention
children (ineffective pump) • ADH  water
• Due to pulmonary • High cardiac output conservation
congestion, systemic demands
venous congestion,
and impaired
myocardial function
Compensatory mechanisms
RAAS
CHF Effects
• Increased volume overload
• Heart cannot eject all of the blood from the ventricle
▫ Na+ & water retention
▫ increased blood volume
▫ increased preload
• Increased workload
▫ vasoconstriction
▫ increased resistance
▫ increased afterload
Clinical manifestations
▫ Tachypnea, dyspnea, retractions, crackles
▫ Activity intolerance (feeding)
▫ Wt gain/edema /hepatomegaly/vein distention due to
fluid retention
▫ Cardiomegaly, ventricular hypertrophy, tachycardia at
rest
▫ Decreased tissue perfusion
▫ Sweating
▫ Failure to thrive
Heart Failure: Therapeutic Management
Improve cardiac function
 Administer medications such as digoxin & ACE inhibitors
Remove accumulated fluid and sodium
 Administer diuretics (K+ replacement prn)
Decrease cardiac demands
 Minimize metabolic needs: cluster care, let them rest; normothermic;
treat infections; might sedate until surgery
Improve tissue oxygenation
 Supplemental O2 as needed
Immunoprophylaxis
 Respiratory Syncytial Virus (RSV) – Synergis ($$$)
CHF Nursing Interventions
• Assess frequently
• Assess labs (BMP, H&H)
• Provide rest - cluster cares
• Monitor cardiac/O2 saturation - add humidified
O2 if appropriate, elevate HOB
• Neutral thermal environment
• Small, frequent feedings
• Strict I/O
• Daily weight
Impaired Nutrition
• Less than body requirements r/t increased
metabolism & decreased intake (fatigue)
▫ Increased caloric density(24-30 kcal) or add
calories to pumped breast milk
▫ Feed q3hrs with rest periods during feeding
▫ Well rested before feeding
▫ Premie/slow-flow nipple
▫ Hold semi-upright during feeds
▫ NGT/GT
 Gavage feeding at night
Medications Used
Digoxin
 contractility &  afterload
 Monitor dig levels (0.5-2ng/ml)
 Monitor Apical pulse 1 minute
 Hold <90 for infant, <70 for child
 Monitor for dig toxicity: Greater risk if hypokalemic
 Anorexia
 Poor feeding
 Nausea/vomiting
 Bradycardia
 Dysrhythmias
Aspirin
• Platelet aggregation inhibitor
• MOA: changes the balance between prostacyclin
(inhibitor) and thromboxane (promotor)
• Outcomes:
▫ Prevent thromboembolic events
• Use:
▫ Prophylactically after certain corrective surgeries
• Reye’s syndrome
▫ In this case benefits outweigh risks of ASA
Angiotensin-Converting Enzyme (ACE)
Inhibitors
• Block conversion of • Side effects
angiotensin I to angiotensin II ▫ Hypotension
▫ Results in vasodilation ▫ Renal dysfunction
• Outcomes ▫ Cough
▫ Decreased pulmonary and • Monitor labs
systemic vascular resistance ▫ Protein, albumin, BUN,
▫ Decreased afterload creatinine, WBC, Urine output,
▫ Decreased atrial pressures urine SG & protein
• Decreases secretion of • Examples:
aldosterone ▫ Captopril
▫ Decrease in sodium and water ▫ Enalapril
reabsorption from the kidney
 Reduction in preload
Beta Blockers
• Blocks β-adrenergic • Examples
receptors ▫ Metoprolol
• Outcomes ▫ Carvedilol
▫ Decreased HR
▫ Decreased BP
▫ Vasodilation
• Side effects:
▫ Dizziness
▫ Headache
▫ Hypotension
Diuretics
• Decreases preload
• Monitor serum Potassium
• Potassium supplements/ foods
• Monitor for hypokalemia
▫ Muscle weakness
▫ Cramping
▫ Confusion
▫ Irritability
▫ Dysrhythmias/ inverted T-wave
Diuretics Cont.
• Loop
▫ Site: loop of Henle.
▫ Action: Powerful. Block
reabsorption of Na+, K+, Cl-
▫ May cause hypokalemia
▫ Decrease preload decreasing
heart size
▫ Example: Furosemide
• Potassium-sparing
▫ Site: distal tubule
▫ Action: blocks reabsorption of
Na+, Cl- and conserves K+
▫ Example: Spironolactone
• Thiazide
▫ Site: distal tubule
▫ Action: blocks reabsorption of
Na+, K+ and HCO3-
reabsorption
▫ Example: Hydrochlorothiazide
Parent Education
• Discharge teaching • Teach the parents how
starts on Admission to take care of the child
• Have a supportive at home:
relationship with the  S/S
parents and the  Medications
healthcare team.  Treatments
Make sure to have
 Nutrition
 Daily weights
good communication
 Developmental
milestones
 CPR
 Maintain discipline
Cardiac Red Flags
• Infant: cyanosis, poor growth and development, poor feeding,
symptoms of HF
• Older child: dyspnea, chest pain with exertion, shortness of breath,
abnormal vital signs, activity level
• Extra heart sounds (S3 or S4), murmurs
• Tachycardia
• Abnormal pulses
• Low BP (seen in hypoxemia)
• Hepatic enlargement
• Peripheral edema
• Clubbing
• Recurrent respiratory infections
• Diaphoresis
The nurse has admitted a child with an
increased pulmonary flow defect to the unit. An
appropriate nursing diagnosis for this child is:
A. Impaired Gas Exchange
Related to Pulmonary
Congestion Secondary to the
Increased Blood Flow to lungs.
B. Deficient Fluid Volume Related
to Hyperthermia Secondary to
the Congenital Heart Defect.
C. Acute Pain Related to the
Effects of a Congenital Heart
Defect.
D. Hypothermia Related to
Decreased Metabolic State
A newborn with transposition of the great
arteries is being admitted to the unit.
The nurse should anticipate an order to
administer:
A. Digoxin.
B. Indomethacin.
C. Furosemide.
D. Prostaglandin E.
A child has been admitted to the hospital unit
in congestive heart failure (CHF). What
symptoms would be expected related to this
admission diagnosis? Select all

A. Weight gain.
B. Bradycardia.
C. Tachycardia.
D. Increased blood
pressure.
E. Tachypnea
Which of the following signs indicate a patient is
in congestive heart failure? (Choose all that apply)

A. Sweating
B. Decreased urinary
output
C. Tachypnea and
retractions
D. Crackles
E. Grade III/VI
holosystolic murmur
F. Hepatosplenomegaly
The nurse plans which of the following
interventions for the nursing diagnosis Impaired
nutrition (less than body requirements). (Choose
all that apply)
A. Nutrition consult
B. Lactation consult
C. Gradually increase caloric
content of pumped breast
milk by adding powdered
formula
D. Rigorous Q4hour feeding
schedule
E. Offer breaks during
feeding
F. Limit feeding to 15
minutes at a time
Question
Identify the order of events that lead to congestive
heart failure in an infant with a ventricular septal
defect (VSD).
▫ __4__ Right side of heart becomes hypertrophied
▫ __2__ Increased blood flows to the pulmonary artery
▫ __1__ Blood shunts from the left ventricle to the right
ventricle
▫ __3__ There is an increase in pulmonary pressure
Acquired Heart Disorders
 Acute Rheumatic Fever
 https://www.youtube.com/watch?v
=HOalYWvVLU8
• Inflammatory disease
• Group A B-hemolytic streptococci (GABHS)
pharyngitis  rheumatic fever
• Jones criteria for diagnosis
▫ Major clinical manifestations
 Carditis
 Polyarthritis
 Erythema marginatum
 Subcutaneous nodules
 Chorea: involuntary movements of face/extremities
▫ Minor manifestations
 Fever
 Arthralgia
 Prolonged PR interval on ECG
 Elevated ESR or CRP
• Test: Elevated ASO titer
 75

Treatment of Acute Rheumatic Fever

• Prevention of GABHS
▫ Culture sore throat, check family for carriers
• Treatment of streptococcal tonsillitis and pharyngitis
▫ Antibiotic therapy: penicillin (or if allergy erythromycin)
• Management of Inflammation and Fever
▫ Monitor VS
▫ ASA, Corticosteroids
 Initially use high doses of aspirin for anti-inflammatory
properties
▫ Joints in neutral position
• Support for Chorea
▫ Reassure that it will go away
Rheumatic Heart Disease (RHD)
• Carditis: >50% of Rheumatic Fever
develop RHD
• Clinical manifestations
▫ Reflect valvulitis, myocarditis, pericarditis…..Mitral
valve
▫ Apical systolic murmur
▫ Mitral regurgitation
▫ Rare cases - leads to heart failure
Nursing Care for Rheumatic Heart Disease
Prevent permanent Prevent recurrences/
cardiac damage further damage

• Bedrest with quiet activities • Long-term PCN: BID PO if

• Monitor with echocardiograms compliant, Q month IM if non-
• Teach about meds: PCN, ASA compliant
• Report s/s strep to PCP
immediately: want to prevent
further strep A
Kawasaki Disease (KD)
• Leading cause of acquired heart disease.
• Acute systemic vasculitis of unknown cause.
▫ 75% Children <5 years
▫ Most frequently in toddlers
▫ Increased in Japanese/Korean race
• Pathophysiology
▫ Extensive inflammation of the arterioles, venules, &
capillaries
▫ Most serious complication: coronary artery aneurysms 
MI
▫ https://www.youtube.com/watch?v=s-J-IIZubz8
 79

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Coronary Arteries Over Time
Lab Values
▫ Elevated WBC and platelets
▫ Elevated Bilirubin/ liver values if liver involved

Labs show signs of systemic inflammation

▫ ESR >40mm/hr
▫ C-reactive protein>3mg/L
Clinical Manifestations of KD
• Acute phase:1-2 weeks
▫ Abrupt onset high temp (>102) unresponsive to ABx or
antipyretics, lasting >5 days
▫ Excessive irritability
▫ Hyperemic conjunctiva without exudate (swollen eyes)
▫ Changes in oral mucous membranes
 Redness, dryness, fissures, strawberry tongue
▫ Erythematous, maculopapular rash
▫ Cervical lymphadenopathy
 Sore throat
▫ Swollen, red palms & soles
 Might peel after a few days, non-painful
▫ Hepatic dysfunction
Kawasaki Disease
Kawasaki Disease
• Subacute phase: 2-4 weeks
▫ Fever resolves, cracking lips, joint pain, persistent
irritability, Painless desquamation hands and feet
 Risk for cardiac disease/ coronary aneurysm and
thrombocytosis
• Convalescent phase: 6-8 weeks
▫ Clinical signs resolve; labs return to normal
• Long-term cardiology follow-up related to
degree of coronary artery involvement
Medical Treatment of Kawasaki Disease
• Single infusion high-dose IV gamma globulin (treat like blood
product)
▫  duration of fever and coronary artery damage if given in 1 st 10
days
▫ May repeat x1 if symptoms persist/ return
▫ May pre-medicate with diphenhydramine to reduce risk of allergic
reaction
• Salicylates
▫ Initially high anti-inflammatory dose: 80-100mg/kg/day
▫ Once fever down anti-platelet dose: 3-5 mg/kg/day until platelet
count normal
▫ If coronary artery abnormalities – remain on ASA (could be years)
▫ Monitor for aspirin toxicity/ serious SE’s
Nursing care
• Assessments: cardiac, pain • Range of motion for chorea
• I&O • Cluster care with rest periods
• VS (fevers) • Symptomatic relief
• Daily wt ▫ Minimize skin discomfort
• Maintain ▫ Mouth care
hydration/nutrition ▫ Cool compresses
• Monitor medication side ▫ Quiet environment
effects
Discharge Teaching for
Kawasaki Disease

Record temp until

Need for follow-up: Symptoms of cardiac
afebrile for several
PCP/ cardiology complications
days

Irritability may persist Defer live vaccines for

ASA-side effects
for 2 months 11-12 months

Arthritis may persist

for weeks
Peeling of hands &
•• Stiff
Stiff in
in AM
AM &
& cold
cold weather,
feet is painless after
weather,
after naps
naps
•• Passive
Passive ROM
ROM in
in warm
warm bath
bath
What care would the nurse plan to implement
on a child with Kawasaki Disease? Select all

A. Monitor for s/s

aspirin toxicity
B. Frequent temp
checks
C. Force fluids
D. Oral care q2-4 hrs
E. Monitor for skin
for breakdown
According to the Jones Criteria, which of the
following sets of symptoms would indicate a
positive diagnosis for Rheumatic fever? Select
all that apply
A. Carditis, fever, and an
elevated WBC count
B. Positive strep throat
culture, arthritis,
chorea
C. Recent scarlet fever,
carditis, fever,
arthralgia
D. Elevated C-reactive
protein, carditis, fever
Questions?
ayarahav@umaryland.edu