Pediatric Primary Care: Practice Guidelines for Nurses, 2nd Ed.


Cardiovascular Disorders

Karen M. Corlett


Asthma, 493.81

Musculoskeletal, 786.59

Asthma, exercise-induced, 493.9

Myocarditis, 429

Cardiac murmur, 782.2

Obesity, 278

Cardiomegaly, 429.3

Palpitations, 785.1

Cardiomyopathy, 425.4

Pericarditis, 423.9

Chest pain, 786.5

Pneumonia, 486

Chest pain, noncardiac, 786.59

Pneumothorax, 512.8

Congenital heart disease, 746.9

Presyncope, 780.2

Congestive heart failure, 428

Pulmonary, 786.52

Coronary artery anomalies, 746.9

Pulmonary embolus, 415.19

Coronary artery disease, 414.9

Rheumatic fever, 391.9

Dizziness, 780.4

Supraventricular tachycardia, 427.89

Dysrhythmias, 427.9

Syncope, 780.2

Enlarged liver, 789.1

Tachycardia, 785

Gastroesophageal refl ux, 530.81

Tachypnea, 786.06

Heart disease, acquired, 429.9

Turner syndrome, 758.6

Heart murmur, 785.2

Valvular defects, 424

Kawasaki disease, 446.1

Ventricular tachycardias, 427.1

Marfan syndrome, 759.82

Weak peripheral pulses, 785.9

A. Etiology.

1. Classes of chest pain.

a. Severe, acute, unremitting chest pain: Refer immediately to pediatrician or urgent/emergent care facility. Rare and most often due to cardiac, pulmonary, or gastrointestinal causes.

b. Chronic or recurrent chest pain: more likely.

• Typically patient has had several episodes of chest pain before medical attention is sought.

• Many times, physical exam may be normal.

• Patient history is crucial in elucidating etiology of chest pain although large percentage of chest pain in children will never have etiology determined.

• Chest pain is distressing to patients, families: chest pain in adult friends, relatives typically signifies cardiac event, most chest pain in childhood is noncardiac in origin.

• Thorough history and physical can rule out serious causes for chest pain.

2. Chest pain, cardiac in origin.

a. Coronary artery disease.

b. Congenital heart disease.

c. Typically have murmur.

d. Acquired heart disease.

e. Infectious etiologies.

f. Cardiomyopathy, pericarditis, Kawasaki disease, rheumatic fever.

g. Myocardial issues, myocarditis.

h. Cardiomyopathies.

i. Dysrhythmias.

j. Supraventricular or ventricular tachycardias.

3. Chest pain, noncardiac in origin: most common.

a. Musculoskeletal.

b. Pulmonary.

c. Gastrointestinal.

d. Psychogenic.

B. Occurrence.

1. Few studies of overall incidence of chest pain in pediatric population due to wide range of specialists to whom these patients are referred.

2. Incidence, particularly in adolescents, is significant.

C. Clinical manifestations.

1. Chronic or recurrent intermittent chest pain.

a. Most often occurs in adolescent population.

b. May or may not limit activities.

c. Usually chest pain has been long standing before treatment is sought.

d. Important to elucidate inciting factors and relieving factors.

• Relationship of pain to activity varies.

e. Associated symptoms.

• Syncope, presyncope, dizziness, palpitations.

f. Congenital heart disease or previous cardiac surgery is important factor in determining cardiac origin of chest pain; also increases likelihood of dysrhythmia as origin for chest pain.

D. Physical findings.

1. Most commonly patients will have no significant physical exam findings. Physical exam findings may be clues to origin of chest pain.

2. Stigmata of certain syndromes: Marfan, Turner.

3. Cardiac murmur.

4. Evidence of congestive heart failure.

a. Tachypnea, increased work of breathing, retractions, flaring, tachycardia, weak peripheral pulses, cool extremities, delayed capillary refill, rales, enlarged liver, easily fatigued, edema.

5. Obesity: increased incidence of gastroesophageal reflux (GER).

6. Reproducible pain with palpation.

7. Signs of trauma to chest wall.

8. Decreased breath sounds.

E. Diagnostic tests.

1. Refer to pediatrician/specialist if chest pain of cardiac origin is suspected; may conduct the following tests as part of evaluation.

2. Chest X-ray (CXR).

a. Evaluate for cardiomegaly. May also reveal rib fractures or pneumonia.

3. Electrocardiogram.

a. Clue to cardiac origins: hypertrophied atria or ventricles, long QT syndrome, congenital heart disease, infarcts, dysrhythmias.

4. Echocardiography.

a. Structural abnormalities.

b. Coronary artery anatomy.

c. Cardiac function.

5. Exercise testing.

a. ST segment response to exercise.

b. May provoke dysrhythmias.

c. May provoke exercise-induced asthma.

6. May reassure patient and parents if normal.

F.  Differential diagnosis.

Acute pancreatitis, 577

Peptic ulcer disease, 533.9

Asthma, 493.9

Pleural pain, 786.52

Asthma, exercise-induced, 493.81

Pleuritis, 511

Biliary colic, 574.2

Pneumonia, 486

Costochondritis, 733.6

Pneumothorax, 512.8

Esophagitis, 530.1

Precordial catch, 786.51

Gastroesophageal reflux, 530.81

Pulmonary embolus, 415.19

Muscular pain, 729.1

Slipping rib syndrome, 733.99

1. Musculoskeletal.

a. Costochondritis.

• Most often at 2nd to 5th costal cartilages.

• Often reproducible pain with pressure at costochondral junctions.

• Treated with nonsteroidal anti-inflammatory drugs (NSAIDs).

b. Slipping rib syndrome.

• Pain at lower costal margin, often associated with click.

• Reproducible at times with anterior motion of lower rib.

• Treated with avoidance of inciting movements.

c. Trauma (nonaccidental or accidental).

• Point tenderness at site of trauma.

• Pain is worse with movement of chest, self-limited.

d. Muscular pain.

• History of new physical activity, pain is worse if affected muscles are used.

e. Precordial catch.

• Sharp pain in anterior chest, often when child is bent over.

• Short, intermittent pain, relieved by shallow respirations.

f. Pleural pain.

• Infection affecting intercostal and upper abdominal muscles, intensified with coughing.

• Tender muscles lasting 3-7 days.

• Intense pain separated by pain-free intervals.

2. Pulmonary.

a. Asthma, particularly exercise-induced asthma.

b. Pneumonia.

c. Foreign body aspiration.

d. Pleuritis: often is remote occurrence to viral infection.

e. Diaphragmatic irritation.

f. Pulmonary embolus: associated with dyspnea, usually is acute episode.

g. Pneumothorax: associated with dyspnea, usually is acute episode.

3. Gastrointestinal: typically localized to substernal area.

a. GER.

b. Related to mealtimes. Exacerbated by supine positioning.

c. Esophagitis.

d. Biliary colic.

e. Acute pancreatitis: pain radiates to back.

f. Peptic ulcer disease.

4. Psychogenic causes: often have witnessed episodes of chest pain in family members.

G. Treatment.

1. Musculoskeletal.

a. Analgesics and anti-inflammatories.

b. Avoidance of inciting movements, rest.

2. Pulmonary.

a. Asthma: trial of bronchodilators, particularly if exercise-induced symptoms.

b. Immediate referral for concern of pulmonary embolus or pneumothorax.

3. Gastrointestinal.

a. GER: H2 blockers, dietary modifications, weight loss if obesity contributing to symptoms.

b. Referral to specialist if initial medical therapy does not relieve symptoms.

4. Psychogenic.

a. Frank discussion with patient, family about nonorganic cause of chest pain.

• Changes in patient's life leading to stress or depression?

• Assess for secondary gain that pain yields patient.

• Counseling may be indicated.

b. Refer to specialist.

5. Cardiac causes.

a. Refer to specialist.

b. Coronary artery anomalies, valvular defects: medical management, surgical repair.

c. Infectious etiologies.

• Cardiomyopathy/myocarditis.

• Treatment of inciting infection if elucidated.

• Supportive care until recovery of function.

• Consideration for transplantation if function does not recover.

d. Kawasaki disease (see later section).

e. Myocardial issues.

• Obstructive cardiomyopathy.

• Activity restriction.

• Consideration of medical or surgical therapy.

f. Dysrhythmias.

• Identification of dysrhythmia.

• Antidysrhythmic agents.

• Ablation of accessory pathways.

H. Follow up.

1. Follow up with pediatrician or specialist as indicated.

2. Support for chronic pain.

3. Assess for missed diagnosis if pain persists or worsens.

4. No cause of chest pain may have been identified. Patient, family may require ongoing support, education, particularly if pain continues.

I. Complications.

1. Misdiagnosis.

2. Sudden cardiac death rare.

J. Education.

1. Most often require assurance and education as to noncardiac nature of chest pain and resumption of normal activities.

2. If cause of chest pain found, education regarding cause and treatment plan.


Adrenal disorders, 255.9

Papilledema, 377

Anorexia, 783

Papilledema with increased intracranial

Diabetes, 250

pressure, 377.01

Epistaxis, 784.7

Pheochromocytoma, 194

Headache, 784

Renal vascular diseases, 593.9

Hypertension, 401.9

Seizure, 780.39

Hypertension, family history of, V17.4

Stroke, 436

Hypertension, secondary, 405.99

Systemic lupus erythematosus, 710

Hyperthyroidism, 242.9

Tiredness, 780.79

Irritability, 799.2

Tuberous sclerosis, 759.5

Myocardial infarction, 410

Turner syndrome, 758.6

Nausea, 787.02

Vascular lesions, 459.9

Obesity, 278

Vomiting, 787.03

A. Etiology.

1. May be early onset of essential hypertension.

2. Etiology of adult-onset essential hypertension is unclear and thought to be multifactorial with genetic, familial, environmental factors contributing to development of essential hypertension. Becoming more clear that elevations of blood pressure in childhood are beginnings of adult essential hypertension.

3. May be a sign of underlying pathology (secondary hypertension). a. Renal, cardiac, endocrine diseases.

4. Related to systemic disease, chronic illness, or medication.

B. Occurrence.

1. By definition, 5% of children will have hypertension.

2. Secondary hypertension is more common in children.

C. Clinical manifestations.

1. Definition is systolic and/or diastolic blood pressure consistently above 95th percentile for age, sex, height taken on 3 separate occasions.

a. Stage 1 hypertension is 95th to 99th percentile +5 mmHg and should be repeated on 3 or more separate occasions to confirm.

b. Stage 2 hypertension is greater than the 99th percentile +5 mmHg.

• Prompt referral.

• Immediate referral if patient is symptomatic.

c. Prehypertension now defined as readings between the 90th and 95th percentile, and/or greater than or equal to 120/80 in adolescents.

d. Check blood pressure in left arm and one lower extremity if hypertension present.

2. Secondary hypertension.

a. Hypertension secondary to another cause.

b. Typically more severe elevation of blood pressure.

c. Younger age.

d. Severe elevation of blood pressure at any age should trigger an aggressive evaluation to look for an underlying cause.

3. Essential hypertension.

a. Typically milder elevation in blood pressure, but still > 95th percentile.

b. Often associated with obesity.

c. Typically, family history of essential hypertension.

d. Elevated heart rate is common.

e. Variable blood pressure measurements on repeated evaluation.

f. Often no additional findings on history or physical.

g. For mild elevation of blood pressure in asymptomatic adolescent, it is more likely to be essential hypertension, particularly if positive family history and/or presence of comorbid conditions.

D. Physical findings.

1. Essential hypertension: May be few abnormal physical findings but patient history is important adjunct to determine whether hypertension is essential or secondary.

a. Family history of hypertension in first- or second-degree relative, myocardial infarction, stroke, renal vascular diseases, diabetes, obesity.

b. Obesity in patient.

2. Secondary hypertension: May be physical findings or important clues in patient's history as to possible cause of secondary hypertension.

a. Neonatal history of invasive umbilical lines.

b. History of urinary tract infections.

c. Medication history: OTC, prescribed, and illicit drugs.

• Tobacco, alcohol, diet pills, anabolic steroids, oral contraceptive pills, pseudoephedrine, phenylpropanolamine.

d. Headaches.

e. Weight loss (pheochromocytoma, hyperthyroidism).

f. Overall slowing of growth parameters may indicate underlying chronic disease.

g. Webbed neck (Turner syndrome associated with coarctation).

h. Presence of skin lesions (tuberous sclerosis, systemic lupus erythematosus).

i. Retinal exam: presence of vascular lesions due to chronic hypertension, papilledema with increased intracranial pressure.

j. Dysmorphic features: Williams syndrome, Turner syndrome.

k. Adrenal disorders.

l. Anorexia, nausea.

m. Tiredness, irritability.

n. Epistaxis.

o. Neurologic symptoms.

• Headache, nausea, vomiting, anorexia, visual complaints, seizure, papilledema.

E. Diagnostic tests.

1. Errors in blood pressure measurement are common: use correct technique, appropriately sized equipment for repeated measurement.

a. All children 3 years of age or older: blood pressure measured at every pediatric visit.

b. Appropriate cuff size essential for accurate measurement.

c. Bladder width of cuff should be 40% of child's arm circumference, bladder should cover 80% or more of circumference of arm.

d. At least 3 separate measurements to diagnose hypertension.

e. Measurement should occur after 5 minutes of quiet; child should be seated with back supported and feet on floor.

f. Auscultation preferred method.

g. Right arm preferred for measurement.

h. Ambulatory blood pressure monitoring may be utilized by specialists to more accurately delineate blood pressure readings throughout the day and night.

2. For all identified as hypertensive:

a. Serum electrolytes, blood urea nitrogen, and creatinine.

b. Urinalysis and urine culture.

c. Complete blood count.

d. Renal ultrasound.

3. If overweight and prehypertensive and for all hypertensive:

a. Fasting lipid panel and fasting glucose.

4. Drug screen if concern for use of licit or illicit drugs.

5. Polysomnography if history elucidates snoring.

6. Evaluation for end-organ damage may be ordered by specialists.

a. Echocardiogram.

b. Retinal exam.

c. Plasma renin.

d. Renovascular imaging.

e. Plasma and urine steroid and catecholamine levels.

F. Differential diagnosis.

Cushing's syndrome, 255

Systemic lupus erythematosus, 710

Hypertension, secondary, 405.99

Tuberous sclerosis, 759.5

Hyperthyroidism, 244.9

Turner syndrome, 758.6

Renal vascular disease, 539.9


1. Secondary hypertension.

2. Renal vascular disease, renal parenchymal disease.

3. About 60-80% of secondary hypertension in childhood is due to renal causes.

4. Coarctation of aorta (Turner syndrome).

5. Endocrine and adrenal causes: hyperthyroidism, Cushing's syndrome.

6. Systemic diseases: systemic lupus erythematosus, tuberous sclerosis.

7. Pharmacologic effect: steroids, amphetamine or sympathomimetics, oral contraceptives, illicit drugs.

8. CNS manifestations: increased intracranial pressure, intracranial mass.

G. Treatment.

1. Treatment goal is to achieve blood pressure <95th percentile, or < 90th percentile if concurrent conditions present.

2. Nonpharmacologic treatment first-line therapy.

a. Weight loss if obese; prevention of obesity if normal weight.

b. Increase physical activity.

c. Dietary modifications.

d. Family-based interventions.

e. Tobacco cessation; avoid drugs of abuse, particularly cocaine.

f. Reevaluation of blood pressure.

3. Pharmacologic treatment indicated if symptoms present, target organ damage present, type 1 or 2 diabetes present, or failure of nonpharmacologic treatment with lifestyle modifications.

4. Single drug therapy preferred. Provider preference since no studies show absolute long-term benefit of any class or drug.

a. Angiotensin converting enzyme inhibitors (preferred if diabetes or proteinuria).

b. Angiotensin receptor blockers (preferred if diabetes or proteinuria).

c. Beta blockers (preferred if patient experiences migraine headaches).

d. Calcium channel blockers (preferred if patient experiences migraine headaches).

e. Diuretics.

5. Add second class of medication if maximum dose achieved or side effects experienced before blood pressure control achieved.

6. Consider gradual withdrawal of medication if target blood pressure achieved for long period of time.

H. Follow up.

1. Continued monitoring of blood pressure.

2. Encouragement of and follow up on lifestyle changes.

3. Refer back to pediatrician or specialist if treatment goals not achieved with lifestyle changes, current medication regime.

I. Complications.

1. End-organ dysfunction.

2. Hypertensive crisis.

J. Education.

1. Essential hypertension.

a. Chronicity of disease, likely long-term morbidity, end-organ damage if not controlled.

b. Potential improvements with dietary, lifestyle changes and medications if necessary.

c. Need for continuous follow up.

d. Adherence to medication regime if prescribed.

e. Encourage patients to return to prescriber if side effects unacceptable because many classes of medications are available for blood pressure control.

f. Goals of therapy.

2. Suspected or known secondary hypertension.

a. Thorough evaluation of cause, may be extensive testing.

b. Follow up with specialists.

c. Adherence to treatment or medication regimen.


Bruit, 785.9

Peripheral pulmonary arterial stenosis

Chromosomal abnormality, 758.89

  murmur, 747.3

Clubbing of digits, 781.5

Pulmonary flow murmur, 424.3

Cyanosis, 782.5

Shock, 785.5

Diaphoresis, 780.8

Splenomegaly, 789.2

Easily fatigued, 780.79

Still's murmur, 782.2

Edema, 376.33

Tachycardia, 785

Hepatomegaly, 789.1

Tachypnea, 786.09

Hypotension, 458.9

Weak pulses, 785.9

Innocent heart murmurs, 785.2


A. Etiology.

1. Innocent murmur is abnormal heart sound caused by turbulent blood flow not associated with structural heart disease, also called functional or nonorganic murmur.

B. Occurrence.

1. Cardiac murmurs are noted in 50-70% of children who are asymptomatic.

2. Vast majority of murmurs heard in infants and children are innocent in nature.

C. Clinical manifestations.

1. Innocent murmur is typically found on routine physical exam.

2. Presence of other clinical manifestations: concern for congenital, acquired heart disease.

3. Any murmur, innocent or organic, is typically louder with fever, anemia, other high cardiac output states.

D. Physical findings.

1. Absence of physical findings other than murmur should be expected if innocent murmur is suspected. Innocent murmurs often more prominent during high cardiac output states such as fever, anemia, pregnancy.

2. Physical exam findings of congenital or acquired heart disease that follow should specifically be evaluated.

a. Known or suspected syndrome or genetic or chromosomal abnormality.

b. Failure to grow.

c. Easily fatigued.

d. Tachypnea, increased work of breathing, retractions, flaring, grunting.

e. Diaphoresis particularly with exertion, feeding in the infant.

f. Cyanosis, central or peripheral.

g. Clubbing of digits.

h. Tachycardia.

i. Edema, particularly periorbital and facial in infant.

j. Active precordium.

k. Palpable thrill.

l. Weak pulses, delayed capillary refill, hypotension, or other signs of shock.

m. Differential between upper and lower extremity pulses or blood pressures.

n. Hepatomegaly or splenomegaly.

o. Murmur of grade IV (see Box 25-1) or higher in intensity.

p. Diastolic murmur.

3. Careful consideration of murmur description.

a. Location.

• Where murmur is heard best, described by location over cardiac structures or location on chest.

Box 25-1 Grading of Cardiac Murmurs

Grade I/VI: soft, difficult to hear unless room quiet and child cooperative.

Grade II/VI: soft but heard immediately.

Grade III/VI: easily heard, moderately loud, no thrill associated.

Grade IV/VI: loud, can palpate the thrill of turbulent flow on chest wall.

Grade V/VI: loud, has thrill, able to hear murmur with stethoscope barely off chest wall.

Grade VI/VI: loud, has thrill, able to hear murmur with stethoscope off chest wall

• Most common areas to auscultate innocent murmurs are left upper sternal border and left lower sternal border.

b. Radiation.

• Descriptor of where else murmur can be heard.

• Innocent murmurs rarely radiate to distant parts of chest.

c. Timing: where in the cardiac cycle the murmur is heard.

• Systole: between Si and S2.

• Diastole: between S2 and S1.

• Continuous: starts in systole, continues into diastole, does not need to continue throughout cardiac cycle, but has same sound for duration of murmur.

• Innocent murmurs are typically systolic; venous hum is continuous murmur. Solely diastolic murmur is never innocent and should be referred.

• Children with innocent murmurs typically have normal first and second heart sounds (S1 and S2) that are audible in addition to murmur. Rapid heart rates of infants, particularly febrile infants, may make it difficult to distinguish between systole and diastole.

d. Pitch: sound frequency of murmur. Innocent murmurs typically low to medium in pitch.

e. Quality: musical or vibratory in quality.

f. Intensity: loudness of the murmur (Box 25-1).

• Innocent murmurs, typically Grade I-II/VI. Often change in intensity with change in position: sitting to lying, standing to sitting. May change in intensity from one visit to the next.

• No clicks or extra sounds if murmur is innocent.

4. Types of innocent murmurs.

a. Still's murmur.

• Most common innocent murmur in children; occurs typically in 2- to 6-year olds.

• Low to medium in pitch, buzzing or vibratory in nature.

• Short murmur occurring in early systole.

• Grade I-III/VI.

• Loudest in supine position, diminished by standing.

• Heard best at left lower sternal border.

b. Pulmonary flow murmur.

• Heard in children/adolescents.

• Harsh murmur, blowing, nonmusical.

• Systolic ejection murmur.

• Grade II-III/VI.

• Loudest in supine position, loudest on exhalation.

• Heard best in second to third intercostal space at left sternal border.

C. Peripheral pulmonary arterial stenosis murmur.

• Heard frequently in infants and newborns.

• Medium in pitch.

• Short systolic ejection murmur.

• Grade I-II/VI.

• Often heard best in axillae and over back.

• Turbulence is due to relative smallness of peripheral pulmonary arteries in newborn and angulation of takeoff of right- and left-branch pulmonary arteries from main pulmonary artery.

d. Supraclavicular or brachiocephalic systolic murmur or bruit.

• Heard in children, young adults.

• Low to medium in pitch, harsh.

• Is short, systolic murmur.

• Grade I-III/VI.

• Heard best above clavicles, radiates to neck.

• Heard best in supine, sitting positions; changes with change in neck position.

e. Venous hum.

• Also known as cervical venous hum.

• Continuous murmur.

• Heard over neck, immediately below clavicles.

• Intensity varies, loudness varies with position, activity; best heard in sitting position.

• May disappear when head is turned toward side of murmur.

• Murmur results from turbulence of flow as large veins converge.

E. Diagnostic tests.

1. When murmur is detected, refer to pediatrician or cardiologist to determine significance.

2. Many innocent murmurs do not require further diagnostic tests after thorough history, physical, auscultatory exam.

3. CXR, electrocardiogram, cardiac echocardiography may determine whether murmur is truly innocent.

4. Murmurs associated with structural abnormalities (organic murmurs) may require additional diagnostic evaluation.

F. Differential diagnosis.

Cardiomyopathy, 425.4

Kawasaki disease, 446.1

Congenital heart disease, 746.9

Rheumatic heart disease, 398.9

Cyanotic or acyanotic disease, 782.5


1. Congenital heart disease.

2. Cyanotic or acyanotic disease.

3. Acquired heart disease.

4. Cardiomyopathy.

5. Kawasaki disease.

6. Rheumatic heart disease.

G. Treatment.

1. Innocent heart murmurs have no organic cause, no structural abnormality, therefore, no treatment required.

2. Education of patient, family is most important treatment modality.

H. Follow up.

1. If innocent murmur confirmed, patients may return to usual health maintenance schedule for follow-up visits.

2. If innocent murmur suspected, but not yet confirmed as innocent, should return for reevaluation to physician.

I. Complications.

1. None, since no abnormality is present.

J. Education.

1. Crucial for patient, family.

2. Explain murmur as noise.

3. Noise is result of blood flow, no structural problem.

4. No heart disease or abnormality is present.

5. Murmur may change or disappear with time but if persists still no problem with heart.

6. No activity restrictions required.

7. No treatment required.


Abdominal pain, 789

Increased liver enzymes, 794.8

Bilateral conjunctivitis, 372.3

Irritability, 799.2

Cardiomegaly, 429.3

Jaundice, 782.4

Cervical lymphadenopathy, 785.6

Joint pain, 719.4

Coronary artery aneurysms, 414.11

Kawasaki disease, 446.1

Cough, 786.2

Murmur, gallop, 427.89

Diarrhea, 787.91

Peripheral arterial aneurysms, 442.

Distention of gallbladder, 575.8

Polymorphous exanthem, 782.1

ECG changes, 794.31

Proteinuria, 791

Elevated white blood cell count, 288.8

Reye's syndrome, 331.81

Erythema, unspecified, 695.9

Rhinorrhea, 478.1

Erythematous rash, 782.1

Seizure, 780.39

Fever, 780.6

Strawberry tongue, 529.3

Hypoalbuminemia, 273.8

Systemic vasculitis, acute, 447.6

Ileus, 560.1

Vomiting, 787.03

A. Etiology.

1. Syndrome of acute systemic vasculitis of unknown origin.

2. Affects mostly small- to medium-sized arteries, particularly coronary arteries.

3. Likely of infectious origin or infection-triggered immune disorder.

B. Occurrence.

1. Most prominent in Japan.

2. Children of Asian descent more susceptible than Caucasians.

3. 50% of cases in younger than 2 years of age, 80% of cases in younger than 5 years of age. Peak incidence at 1 year. Rare after 10 years of age.

4. Boys 1.5 times > girls.

5. 1-2% of siblings affected.

6. Recurrence rate: 1-3%.

7. More common in winter and early spring.

C. Clinical manifestations.

1. Vasculitis of small- to medium-sized arteries, especially coronary arteries. May progress to aneurysm formation in coronary arteries; can lead to thrombosis or scarring, increased risk for myocardial infarction, ischemic heart disease, and sudden death.

D. Physical findings.

1. Fever.

2. Reddened, edematous, indurated palms/soles progressing to desquamation. May be unwilling to walk/use hands to grasp objects.

3. Erythematous rash; most prominent on trunk, groin.

4. Bilateral conjunctival injection; particularly bulbar conjunctiva; nonexudative.

5. Red or peeling lips, strawberry tongue, injected oral, pharyngeal mucosa.

6. Cervical lymphadenopathy.

7. Irritability: particularly in infants.

8. Evidence of vasculitis in other systems.

a. Cardiovascular: murmur, gallop, ECG changes, cardiomegaly, peripheral arterial aneurysms.

b. Gastrointestinal: diarrhea, vomiting, abdominal pain, ileus, jaundice, increased liver enzymes, distention of gallbladder.

c. Hematologic: elevated WBC count, elevated platelet count.

d. Renal: proteinuria, hypoalbuminemia.

e. Respiratory: cough, rhinorrhea.

f. Joint: pain, swelling.

g. CNS: pleocytosis of cerebral spinal fluid (CSF), seizure.

E. Diagnostic tests.

1. No specific laboratory test to confirm diagnosis.

2. Diagnostic criteria to establish diagnosis.

3. Fever of greater than or equal to 5 days and 4 of the following 5 criteria:

a. Erythematous, edematous, indurated palms/soles progressing to desquamation later in course.

b. Polymorphous exanthem.

c. Bilateral conjunctival injection.

d. Red or peeling lips, strawberry tongue, injected oral, pharyngeal mucosa.

e. Cervical lymphadenopathy.

f. OR: Fever of greater than or equal to 5 days, less than 4 of the above 5 criteria, and coronary aneurysms documented by echocardiography or cardiac angiography.

4. Kawasaki should be considered in any young child with unexplained fever for 5 or more days, leukocytosis, elevated erythromycin sedimentation rate or C-reactive protein, and thrombocytosis.

a. “Incomplete” Kawasaki, with symptoms inadequate to meet classic criteria is more common in younger than 1 year of age and adolescents.

b. Echocardiography may show evidence of coronary arteritis.

F. Differential diagnosis.

Hypersensitivity reactions, 995.2

Stevens-Johnson pharyngitis, 695.1

Juvenile rheumatoid arthritis, 714.3

Streptococcal, 041

Measles, 055.9

Systemic lupus erythematosus, 710

Polyarteritis nodosa, 716.59

Toxic shock syndrome, 040.82

Rheumatic fever, 390

Viral exanthems, 057.9

Staphylococcus aureus, 041.11


1. Infectious illnesses, particularly streptococcal and staphylococcal infections.

2. Drug reactions or hypersensitivity reactions.

3. Stevens-Johnson syndrome: erythema, swelling, peeling all advance beyond hands, feet.

4. Other vasculitic diseases.

5. Polyarteritis nodosa, systemic lupus erythematosus.

6. Juvenile rheumatoid arthritis: smaller joints, morning stiffness, fingers involved, joint deformities.

7. Rheumatic fever: positive antistreptococcal antibody.

8. Viral exanthems: do not meet all criteria for Kawasaki disease.

9. Measles: rash more prominent on face and neck.

10. Toxic shock syndrome: sandpaper rash, more toxic-appearing child, often hypotension with evidence of shock.

G. Treatment.

1. Referral to pediatrician and specialists.

2. Hospitalization.

3. Aspirin.

a. Used for antipyretic, anti-inflammatory, and antiplatelet effects.

b. High-dose aspirin for at least 48 hours.

c. Low-dose aspirin to complete 6-8 weeks of therapy if no coronary artery abnormalities.

d. Aspirin continued indefinitely if coronary artery aneurysms, second antiplatelet agent may be added.

4. IV immunoglobulin.

a. Decreases incidence of coronary artery aneurysms.

b. Mechanism of protection unknown.

c. If fever persists > 36 hours after IVIG repeat IVIG dose.

H. Follow up.

1. Pediatric cardiology for echocardiographic evaluation of coronary artery aneurysms. Aneurysms may not develop until after acute stage of illness.

I. Complications.

Congestive heart failure, 428. 0

Myocarditis, 429.0

Coronary artery aneurysms, 414.11

Pericardial effusion, 423.9

Coronary artery thrombosis, 410.9

Pericarditis, 423.9

Intrahepatic vasculitis, 447.6

Valvular heart disease, 424.0

1. Coronary artery aneurysms: develop in < 20% of individuals, fewer if treated with immunoglobulin.

2. If coronary artery aneurysms develop, increased risk of coronary artery thrombosis, stenosis, and resultant myocardial infarction.

3. May also develop valvular heart disease; mitral valve is affected more frequently than aortic valve.

4. Pericarditis, myocarditis, pericardial effusion, congestive heart failure may occur.

5. Can have development of vascular aneurysms elsewhere in body.

6. Can have liver dysfunction from intrahepatic vasculitis.

J. Education.

1. Serious nature of illness.

2. Importance of specialty care and follow up.

3. Possibility of complications after initial phase of illness.

4. Importance of adherence to prescribed medication: aspirin.

5. Importance of not using aspirin to treat fever in other childhood illnesses: association with Reye's syndrome.

6. Consider influenza vaccine due to association of Reye's syndrome with flulike illnesses and aspirin.


Arthralgia, 719.4

Pericarditis, 423.9

Cardiomegaly, 429.3

Polyarthritis, 716.59

Carditis, 429.89

Pulmonary edema, 514

Chorea, 333.5

Rash, 782.1

Congestive heart failure, 428

Rheumatic fever, 390

Diffuse vasculitis, 447.6

Rheumatic heart disease, acute, 391.9

Erythema marginatum, 695

Streptococcal pharyngitis, 034

Erythrocyte sedimentation rate, 790.1

Subacute bacterial endocarditis, 421

Fever, 780.6

Subcutaneous nodules, 782.2

Group A streptococcal pharyngitis, 041.01

Tachycardia, 785

Migratory polyarthritis, 390

Valvular disease, 424.9

Myocarditis, 429

White blood cell count, 288.9

A. Etiology.

1. Complication of Group A beta-hemolytic streptococcal (GABHS) pharyngitis.

2. Thought to be unique host factors that make certain individuals more susceptible to rheumatic fever after GABHS pharyngitis.

3. Rheumatic fever is classified as collagen vascular disease or connective tissue disease resulting in diffuse vasculitis.

B. Occurrence.

1. Most common in underdeveloped countries but resurgence in United States.

2. Occurs in 2-3% of patients with untreated GABHS.

3. Rheumatic fever is rare in infants, toddlers. Most cases younger than 5 years of age.

C. Clinical manifestations.

1. Inflammation of joints and heart.

2. Rarely, inflammation of brain and skin.

D. Physical findings.

1. Migratory polyarthritis.

a. Occurs in 75% of initial episodes, 50% of recurrent episodes.

b. Arthritis is typically of larger joints (knees, elbows, wrists, ankles).

c. Each joint typically affected for < 1 week, often overlap so that more than 1 joint is affected at a time.

d. Joints are painful to touch, red, swollen; painful with movement.

2. Carditis.

a. Up to 50% of patients with rheumatic fever have evidence of carditis.

b. May manifest as myocarditis with poor function, valvular disease with audible murmur, pericarditis with friction rub, or as general symptoms of tachycardia, cardiomegaly, pulmonary edema, or symptoms of congestive heart failure.

3. Subcutaneous nodule.

a. Small, hard nodules typically found on elbows, knees, wrists; also felt in occipital area, over vertebrae. Relatively rare finding in rheumatic fever.

4. Chorea.

a. Finding of abnormal, involuntary writhing, purposeless movements that extinguish with sleep.

b. Patients often have uncontrollable facial grimacing.

c. Often preceding emotional lability thought to be from CNS involvement.

d. Chorea is often late sign with latent period of 1-6 months.

5. Rash.

a. Erythema marginatum: typical rash of rheumatic fever; painless, does not itch. Spreads peripherally while central clearing, most common on trunk, limbs; face is usually spared.

6. Low-grade fever.

7. Painful joints without obvious joint inflammation.

E. Diagnostic tests.

1. Evidence of previous GABHS.

2. Modified Jones criteria to make diagnosis.

a. Major criteria:

• Carditis.

• Polyarthritis.

• Chorea.

• Erythema marginatum.

• Subcutaneous nodules.

b. Minor criteria:

• Arthralgia.

• Fever.

• Elevated acute-phase reactants (erythrocyte sedimentation rate, C-reactive protein, WBC count).

• Prolonged PR interval.

c. Diagnosis requires 2 major criteria, or 1 major and 2 minor criteria, and evidence of previous GABHS infection.

d. Electrocardiogram and echocardiogram for confirmed diagnoses.

F.  Differential diagnosis.

Congenital heart disease: 746.9

Kawasaki disease. 446.1

Infective carditis, 429.89

Myocarditis, 429

Juvenile rheumatoid arthritis, 714.3


1. Juvenile rheumatoid arthritis: small joints involved, swelling and deformities of joints, morning stiffness, no positive antistreptococcal antibody.

2. Kawasaki disease.

3. Congenital heart disease: murmur without associated symptoms of rheumatic fever.

4. Infective carditis or myocarditis: positive viral or bacterial cultures.

G. Treatment.

1. Prevention: appropriate diagnosis and treatment of streptococcal pharyngitis.

2. Referral to pediatrician or specialist if diagnosis suspected.

3. Antibiotics to eradicate streptococcal infection (primary prevention; see Table 25-1).

4. Bed rest until fever, symptoms resolve. With carditis, bed rest may be indicated for longer period of time.

5. Aspirin.

a. If no relief in arthritis with initiation of aspirin therapy, question diagnosis.

b. Steroids may be considered if no response to aspirin therapy and/or for those with moderate to severe carditis.

6. Secondary prevention (Table 25-1).

a. To prevent recurrence of rheumatic fever in susceptible individuals.

b. Antibiotics until 21 years of age and minimum of 5 years if no cardiac involvement.

c. Antibiotics until 21 years of age and minimum of 10 years if cardiac involvement but no residual cardiac disease.

d. Antibiotics until 40 years of age and minimum of 10 years if residual valve abnormalities; consider lifelong antibiotic prophylaxis.

H. Follow up.

1. With specialist.

2. Subacute bacterial endocarditis (SBE) prophylaxis if residual valve disease.

I. Complications.

Valvular heart disease, 424.9


Table 25-1 Primary and Secondary Antibiotic Prophylaxis for Rheumatic Heart Disease


Source: Adapted from: Gerber MA, Baltimore RS, Eaton CB, et al. Prevention of rheumatic fever and diagnosis and treatment of acute Streptococcal pharyngitis: a scientific statement from the American Heart Association Rheumatic Fever, Endocarditis and Kawasaki Committee of the Council on Cardiovascular Disease in the Young, the Interdisciplinary Council on Functional Genomics and Translational Biology and the Interdisciplinary Council on Quality of Care and Outcomes Research: endorsed by the American Academy of Pediatrics. Circulation.2009; 119(11):1541-1551.

1. Cardiac involvement.

a. Valvular heart disease.

2. Recurrence.

a. Increased risk of recurrence in susceptible hosts.

b. Cardiac damage is cumulative with each recurrence.

J. Education.

1. Nature of disease.

2. Recurrence risk.

3. Prompt diagnosis and treatment of Group A streptococcal pharyngitis.

4. Cumulative nature of cardiac damage with repeat episodes.

5. Secondary prevention.

a. Streptococcal infections do not have to be symptomatic to cause additional cardiac damage.

6. Importance of continued pediatric subspecialty follow up and transition to adult healthcare providers when appropriate.

7. SBE prophylaxis if indicated.


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