CHAPTER 11 – CARDIOVASCULAR SYSTEM

First Nations and Inuit Health Branch (FNIHB) Pediatric Clinical Practice Guidelines for Nurses in Primary Care.

The section on Rheumatic (Carditis) has been updated as of December 2017. The remaining content of this chapter was reviewed in September 2011.

Table of Contents

INTRODUCTION ...... 11–1 ASSESSMENT OF THE CARDIOVASCULAR SYSTEM ...... 11–1 In Infants ...... 11–1 In Children ...... 11–2 Medical History (Specific to Cardiovascular System) ...... 11–2 Physical Findings ...... 11–2 COMMON PROBLEMS OF THE CARDIOVASCULAR SYSTEM ...... 11–3 Murmurs ...... 11–3 Innocent ...... 11–4 EMERGENCY PROBLEMS OF THE CARDIOVASCULAR SYSTEM ...... 11–5 Cardiac Failure ...... 11–5 Cyanosis in the Newborn (Birth to 6 Weeks) ...... 11–6 (Carditis) ...... 11–8 Viral ...... 11–9 SOURCES ...... 11–12

Pediatric Clinical Practice Guidelines for Nurses in Primary Care 2011

Cardiovascular System 11–1

INTRODUCTION

Cardiovascular disease is uncommon in childhood. Symptoms of ventricular septal defect, including heart The major problems seen include congenital , usually occur at approximately 6 weeks of age. disease (usually abnormalities of the great vessels, For more information on the history and physical hypoplastic heart, pulmonary or aortic atresia and examination of the cardiovascular system in tetralogy of Fallot), cardiac failure, rheumatic fever older children and adolescents, see the chapter carditis and myocarditis. “Cardiovascular System” in the adult clinical Functional or innocent heart murmurs are common. practice guidelines. Congestive heart failure at birth is rare and usually suggests severe valvular deformities.

ASSESSMENT OF THE CARDIOVASCULAR SYSTEM

Symptoms of vary with the EXERCISE INTOLERANCE age of the child. –– Eats slowly or poorly Ask about: –– Tires easily during feeding and with poor weight gain –– Rapid or noisy breathing –– Cyanosis appears with feeding (exertional) –– Cough –– Often described by parents or caregiver as a –– Cyanosis “good baby”: always quiet, sleeps a lot, parents –– Sleeping patterns may find baby less energetic compared to siblings –– Exercise tolerance: indicated in a young child by at same age ability to feed and in an older child by ability to keep up with peers during play DIFFICULTY BREATHING –– Tachypnea IN INFANTS –– Chest retractions –– Nasal flaring CYANOSIS –– Anxious appearance –– An abnormality of oxygen transport related to –– Grunting heart, lungs or blood or inadequate oxygenation of blood due to mixing of venous and arterial EXCESSIVE PERSPIRATION blood. Transport problems include impairment of –– Infant’s head described as “always wet” the oxygen-carrying capacity of hemoglobin, as for example, in carbon monoxide poisoning, and –– Infant perspires freely and easily, especially hypoxemia secondary to ventilation/perfusion with excretion and feeding mismatches as for example in pneumonia SLOW GROWTH –– Causes bluish discolouration of mucous membranes, nail beds and skin, is a significant –– Child usually exhibits slow weight gain, relative clinical finding and is related to inadequate to height gain; difficulty in feeding may contribute oxygenation of arterial blood to this problem –– May be transient (related to increased oxygen –– Metabolic demands increased demand by tissues, for example, during feeding in infants or during play in toddlers) or permanent RESPIRATORY INFECTIONS from birth –– More common with congestive heart failure –– More severe with increased pulmonary flow

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IN CHILDREN INSPECTION –– Slow growth –– Respiratory distress –– Respiratory infections –– Cyanosis: central and peripheral –– Chest –– Hands and feet: cyanosis, clubbing –– –– Precordium: visible pulsations –– Dizzy spells or blackouts –– (hands, feet, sacrum) –– Exercise intolerance PALPATION –– Squatting with cyanotic episodes (“tetralogy spells”) –– Apical beat is located at fourth intercostal space, lateral to the mid-clavicular line in infants, and at fifth intercostal space, lateral to the mid-clavicular MEDICAL HISTORY (SPECIFIC TO line in older children CARDIOVASCULAR SYSTEM) –– Brief, localized apical tap is normal –– Prematurity (associated with a higher prevalence –– Apical beat may be laterally displaced, which of congenital cardiac malformation) indicates –– History of illnesses related to heart disease –– Thrills or heaves may be palpable through chest (for example, strep throat) wall; check supraclavicular area for thrills (in –– “Flu-like” illness children with a thin chest wall, normal heart movements can be easily palpated and should –– Joint or swelling not be confused with true thrills and heaves) –– Down syndrome (associated with a higher –– Hepatomegaly prevalence of congenital heart disease) –– Check for presence, rate, rhythm, amplitude and equivalence of peripheral , especially PHYSICAL FINDINGS femoral pulses (which are bounding in patent An examination of the cardiovascular system ductus arteriosus, absent in coarctation of aorta) involves more than just examining the heart. The –– Check for synchrony of radial and femoral pulses examination generally covers two systems: the central –– Capillary refill (normal < 3 seconds) cardiovascular system (head, neck and precordium –– Edema: pitting (rated 0 to 4) and level (how far up [anterior chest]) and the peripheral vascular system the feet and legs the edema extends); sacral edema (extremities). Examination of the cardiovascular –– Skin: temperature, turgor system must also include a full assessment of the lungs and neuromental status (for signs of confusion, irritability or stupor). –– S1 and S2 VITAL SIGNS –– Physiologic splitting of S2 heart sound –– Added heart sounds (S3 and S4): determine their –– Heart rate location and relation to respiration –– Respiratory rate –– Murmurs: determine location (where murmurs are –– Blood pressure (in both an upper and a lower best heard), radiation, their timing in cardiac cycle, limb, if possible) intensity, grade (see Table 1, “Characteristics of –– Temperature (may be elevated with myocarditis Heart Murmurs of Various Grades”) and quality or acute rheumatic fever) –– : may occur in carotid , abdominal –– Cardiovascular problems may present as failure aorta, renal arteries, iliac arteries, femoral arteries to thrive (weight and height below specified –– Crackles in lungs: may indicate heart failure percentiles for age) or as a sharp decline in the (in infants and children, this usually occurs as growth curve across a major percentile line, a late sign) therefore always document height and weight for all well baby and child examinations

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Table 1 – Characteristics of Heart Murmurs of Various Grades Grade Characteristics I Very quiet, barely audible II Quiet but audible III Easily heard IV Thrill can be felt, murmur is easily heard V Thrill can be felt and loud murmur can be heard with stethoscope placed lightly on chest VI Thrill can be felt and very loud murmur can be heard with stethoscope held off the chest wall

COMMON PROBLEMS OF THE CARDIOVASCULAR SYSTEM

HEART MURMURS –– An S3 sound may occur after the second heart sound. This may be found in healthy children. It is Most murmurs are innocent flow murmurs, which are a sign of heart failure in a symptomatic child. The present in up to 50% of children; see “Innocent Heart S3 sound is best heard when listening at the apex Murmur.” of the heart (in the fourth and fifth intercostal A heart murmur may signify congenital anatomic, spaces); a left side-lying position may accentuate infectious or inflammatory damage to valves and the sound. Use the bell part of the stethoscope outlets of the four chambers of the heart. –– Ejection “clicks” may be present in certain conditions; they are always abnormal PHYSICAL FINDINGS: AUSCULTATION If a murmur is present, several characteristics should Auscultation helps to distinguish significant murmurs be determined. from innocent murmurs. Timing within Cardiac Cycle Murmurs must be recognized in relation to other physiologic and pathologic sounds of the –– Systolic ejection murmurs occur after the first cardiac cycle. sound. They are caused by turbulence in the blood as it leaves the heart –– The S sound is caused by the closure of the 1 –– Pansystolic murmurs begin with the first heart mitral and tricuspid valves, which usually occurs sound and end with the second. They most often simultaneously. The S sound is best heard at the 1 occur in association with ventricular septal defects cardiac apex –– Diastolic murmurs begin with the second heart –– The S sound occurs with the closure of the aortic 2 sound. They are always abnormal and pulmonary valves. Because the closure of these two valves is somewhat asynchronous, what Shape or Contour is known as the S2 sound actually consists of two sounds. The separation of the two component –– Qualifies the intensity over time: murmurs can be sounds is often difficult to detect in young children, crescendo, decrescendo, or crescendo-decrescendo although it is more pronounced during inspiration.

Wide separation of the S2 sound is often a significant pathologic finding. The S2 sound is best heard in the second and third left intercostal spaces

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Location on the Thorax INNOCENT HEART MURMUR There are four general auscultatory areas: Heart murmur that occurs in the absence of anatomic –– Aortic: left ventricular outflow murmur or physiologic abnormalities of the heart and therefore (usually ejection) has no clinical significance. Such murmurs occur in –– Pulmonary: right ventricular outflow murmur, 50–80% of children. patent ductus arteriosus –– Tricuspid: tricuspid murmurs increase on TYPES OF INNOCENT HEART MURMURS inspiration; ventricular septal defects are heard Still’s murmur – vibratory, systolic ejection murmur best in this area (SEM), lower left sternal border (LLSB) or apex; ages –– Mitral: murmur at the cardiac apex 3 to 6 years.

Radiation Venous hum – infraclavicular hum, continuous, heard on right side more than left side; ages 3 to 6 years. Radiation of the murmur to the back, sides and neck should be carefully auscultated. Radiation of Peripheral pulmonic stenosis – pulmonic area, systolic, the murmur may give important diagnostic clues low pitched, radiates to axilla and back, seen in (for example, radiates to the neck). neonates; disappears usually by 3 to 6 months of age. Pulmonary ejection – soft, blowing, upper left Intensity of Murmur sternal border, systolic ejection murmur (SEM). –– Intensity expressed as a fraction of VI (for example, I/VI, II/VI), where a very loud murmur PATHOPHYSIOLOGY = V/VI or VI/VI, a loud murmur = III/VI or IV/ Most innocent heart murmurs are produced by the VI, and a soft murmur = I/VI or II/VI (see Table 1, forward flow of blood, which creates turbulence in “Characteristics of Heart Murmurs of Various the chambers of the heart or the great vessels. These Grades”) murmurs are often more pronounced in high-output –– Intensity (loudness) does not necessarily correlate states, such as during a fever. Because the intensity with the severity of the condition. Soft murmurs of the murmur parallels the ejection velocity of blood may be dangerous, whereas loud murmurs are not from the ventricles, innocent murmurs usually occur necessarily so. A murmur associated with a thrill during early to mid-systole, are short in duration, has an intensity of at least IV/VI have a crescendo-decrescendo contour (especially –– Intensity may also increase with increased blood an ejection murmur), are less than 3/6 in intensity flow, as with exercise and are never diastolic.

Pitch CLINICAL FEATURES –– Can be low, medium or high and is determined by Innocent heart murmurs are asymptomatic and are whether it can be auscultated best with the bell or usually found on routine physical examination. the diaphragm of a stethoscope DIAGNOSTIC TESTS Quality –– Electrocardiogram (ECG) –– Blowing –– Echocardiography (only as ordered by a physician) –– Harsh –– Musical MANAGEMENT –– Rumbling Reassure the parents or caregiver that no immediate –– Clanging treatment is necessary.

Referral Refer the asymptomatic child electively to a physician for assessment when a murmur is found.

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EMERGENCY PROBLEMS OF THE CARDIOVASCULAR SYSTEM

CARDIAC FAILURE –– Crackles in lung fields –– Hepatomegaly The inability of the heart to pump blood –– Diminished peripheral pulses commensurate with the body’s needs. The symptoms and signs correlate with the degree of failure. DIFFERENTIAL DIAGNOSIS CAUSES –– Respiratory disease (for example, bronchiolitis or pneumonia) –– Congenital abnormality of cardiac structures –– Metabolic abnormality (for example, –– Inflammatory (for example, rheumatic fever) hypoglycemia; poisoning, as with salicylates) also –– Infectious (for example, viral , consider hyperglycemia with ketosis, head injuries subacute bacterial ) –– Sepsis including meningitis –– Severe anemia (that is, hemoglobin < 40 g/L) –– Other high-output states (for example, COMPLICATIONS thyrotoxicosis, arteriovenous malformation) –– Decreased cardiac output () –– Extracardiac disease (for example, chronic –– Death pulmonary disease, pulmonary hypertension) DIAGNOSTIC TESTS HISTORY –– oximetry The history varies according to the child’s age. –– Difficulty with feeding MANAGEMENT –– Shortness of breath Goals of Treatment –– Excessive sweating –– Poor weight gain –– Improve hemodynamic function –– Anxious appearance –– Prevent complications

PHYSICAL FINDINGS Appropriate Consultation –– Consult with a physician regarding emergency treatment. –– Tachypnea –– Blood pressure (assessed in both arms) usually Nonpharmacologic Interventions normal but may be reduced (if so, this is cause for concern, as it may indicate ) –– Nurse the child in head-elevated position (do not allow neck to become kinked) –– Temperature: if higher than normal, consider inflammatory or infectious cause –– Restrict oral fluids to no more than the quantity required to maintain hydration (see chapter –– Irritable “Fluid Management”) –– Anxious –– Fontanel full Adjuvant Therapy –– Nostrils flared –– Start IV therapy with normal saline to keep –– Cyanosis open (unless this would stress the child too much) –– Peripheral swelling (in older children) –– Give oxygen 6–10 L/min or more by non- –– Increased jugular venous distention rebreather mask.1 Titrate to keep oxygen –– Displaced, diffuse apical impulse (cardiomegaly) saturations > 97%2 –– Heave or thrill

–– (with extra S3 heart sound) –– Increased murmurs

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Pharmacologic Interventions Non-cardiac Causes Drugs used to treat heart failure in children are to be –– Pulmonary infection (for example, group B ordered by a physician. streptococcal infection) –– Diuretics to decrease volume: –– Intrauterine infection or systemic viral infection (for example, or Coxsackie B5) furosemide (Lasix), 1 mg/kg IV stat (may be given –– Aspiration of meconium PO if IV access not available) –– Pulmonary hypoplasia –– ACE inhibitors may be prescribed by a physician –– Respiratory distress syndrome (for example, for afterload reduction in premature infants) –– Digoxin may be used in some cases to increase contractility –– Hypoventilation (for example, neurologic depression) Monitoring and Follow-Up –– Persistent fetal circulation: seen in post-term infants with perinatal distress or pulmonary disease Acute Phase Monitor ABCs (airway, breathing and circulation), Clinical Features of Infants vital signs, pulse oximetry (if available), heart with Cyanotic Heart Disease and lung sounds, intake and output until child is The clinical features usually present in the first week transferred to hospital. of life but may present later: Over the Long Term –– Difficulty feeding; infant appears to tire easily Children with cardiac illness should be monitored –– Lethargy regularly within the community to ensure normal –– Cyanosis when feeding or active (for example, growth and development and to watch for while crying) complications. Frequency of follow-up depends –– Perspiration on face or forehead, especially when on the severity of the condition. feeding or active –– Rapid, noisy breathing Referral Medevac immediately. PHYSICAL FINDINGS –– Lethargy CYANOSIS IN THE NEWBORN –– Cyanosis, initially of the oral mucosa; in severe (BIRTH TO 6 WEEKS) cases, the cyanosis becomes generalized –– Reduced oxygen saturation Bluish discolouration of the skin and mucous –– Tachypnea membranes secondary to hypoxia. –– Poor perfusion (for example, pallor or gray, CAUSES ashen appearance; extremities cool; capillary refill diminished; peripheral pulses diminished) Congenital Heart Disease –– In coarctation of aorta, pulse quality and blood pressure may differ in different extremities Cardiac cyanosis is due to left-to-right shunting, so that systemic venous blood bypasses the pulmonary –– Heart sounds may be loud circulation and enters the arterial systemic circulation. –– Precordium may appear hyperdynamic (heaves or thrills may be present) Findings of increased risk for congenital heart disease: –– Heart murmur may be present –– Genetic syndromes (for example, Down syndrome) –– Hepatomegaly (if infant is in heart failure) –– Certain extracardiac anomalies (for example, omphalocele) DIFFERENTIAL DIAGNOSIS –– Maternal diabetes that is poorly controlled in –– Pulmonary causes as listed above the first trimester –– Sepsis –– Exposure to a cardiac teratogen (for example, lithium, isotretinoin [Accutane], alcohol) –– Family history of significant congenital heart disease

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COMPLICATIONS – Cardiac failure (see “Cardiac Failure”) – Failure to thrive (see “Failure to Thrive” in the pediatric chapter “Hematology, Endocrinology, Metabolism and Immunology”) – Death

DIAGNOSTIC TESTS – Pulse oximetry

MANAGEMENT

Appropriate Consultation Consult a physician immediately and prepare to medevac.

Adjuvant Therapy – Give oxygen 6–10 L/min (or more, if necessary) by non-rebreather mask. Titrate to keep oxygen saturations > 97%2 – Consider intravenous (IV) therapy with normal saline if infant is feeding poorly or is in significant clinical distress

Nonpharmacologic Interventions – Nurse in an upright position – Feed small amounts frequently

Monitoring and Follow-Up – Monitor level of consciousness, vital signs, heart and lung sounds, perfusion, pulse oximetry – Hydration status (intake and output) (see “Clinical Features of Dehydration” in the chapter “Fluid Management”) – Watch for signs of cardiac failure (see “Cardiac Failure”)

Referral – Medevac as soon as possible

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RHEUMATIC FEVER (CARDITIS)

OVERVIEW – Overcrowding(3) – Socioeconomic disadvantage(4) (4) Please refer to provincial/territorial guidelines − Lack of access to medical/health care for Acute Rheumatic Fever where available HISTORY OF PRESENT ILLNESS Acute rheumatic fever is a serious illness that Review risk factors and collect history of present may require medical evacuation. Consult with physician/nurse practitioner immediately when illness. there is suspicion of acute rheumatic fever. – Preceding GAS pharyngitis(2) – Joint pain, redness and swelling (migratory - arthritis, typically involving the large joints) (5) matory disease of the connective tissues involv- – of heart failure include: ing the heart, joints, skin, central nervous system • Exertional dyspnea and/or dyspnea at rest and subcutaneous tissue. ARF is the immunolog- • Orthopnea ic sequela to untreated Group A Streptococcal • /pressure and palpitations (GAS) infection. While acute rheumatic fever • Cough leaves no lasting damage to the brain, joints or • Fatigue and weakness skin, damage to heart valves may be permanent • Nocturia and oliguria which lead to a chronic heart condition called • Anorexia, weight loss, nausea rheumatic heart disease (RHD).(1) In the absence – Involuntary, uncoordinated muscular move- of secondary antibiotic prophylaxis, recurrence ments (Sydenham’s chorea) (typically pres- (6) of rheumatic fever is likely and each subsequent ents 2 to 6 months after initial infection) (6) episode may cause further cardiac damage to the – Emotional lability (1) valves.(1) – Non-pruritic, painless rash − Fever(7) CAUSES PHYSICAL FINDINGS Untreated GAS pharyngitis infection(2) Perform a physical examination using the IPPA ASSESSMENT approach.

Medication review: Review current medications, Major Manifestations including over-the-counter, complementary and Carditis alternative medicines, as well as any chemical or – Carditis may be clinical or subclinical.(1) substance intake that may impact management. – New or changing heart murmurs. For more information on heart murmurs, see Heart Allergy history: Screen for medication, latex, Murmurs in Appendix, Section A of this guide. environmental or other allergies and determine – Rubs may be audible with inspiration and approximately when and what type of reaction expiration if disease is associated with occurred. .(1) – (8) RISK FACTORS ). – Tachycardia at rest;(1) may be out of – Personal and family history of acute rheu- proportion to fever(8) matic fever; most common in those aged 5 to 15 years, but can occur in children as young as 3 years of age(2)

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– Tachypnea, orthopnea, jugular venous disten- the knees, elbows, and wrists; also seen in tion, crackles, hepatomegaly, a gallop rhythm, the occiput and along the spinous processes edema and swelling of the peripheral extrem- of the thoracic and lumbar vertebrae(7) ities if the client is in heart failure.(9) − Cardiomegaly, pulmonary congestion and Erythema Marginatum – may be observed on chest x-ray.(10) dark-skinned people)(1) – An evanescent, pink rash with a pale center Arthritis and rounded or serpiginous margins(7) – Large joints are usually affected, especially – The rash is usually present on the trunk and the knees and ankles.(1) proximal extremities; it is almost never on – the face(7) presence of 2 or more of the following:(1) – Blanches with pressure(7) • Limitation of movement – • Hot joint medication(1) • Pain in the joint and/or tenderness − Rarely seen as the sole major criterion for acute rheumatic fever and should be Sydenham’s Chorea accompanied by additional major criteria in – Jerky, uncoordinated movements of the order to make the diagnosis(1) extremities that disappear during sleep,(1) dysphonia and possible emotional lability(6) Minor Manifestations – Female predominance(7) – Fever(7) − Can be a standalone criterion for the – Arthralgia(7) diagnosis of acute rheumatic fever – Raised C-reactive protein (CRP) and eryth- without additional manifestations(1) rocyte sedimentation rate (ESR)(7) − Prolonged PR interval on electrocardio- Subcutaneous Nodules gram (ECG)(7) – Usually located over a bony prominence or near tendons(11) DIFFERENTIAL DIAGNOSIS − - sionally painful protuberances found on ex- Consult physician/nurse practitioner when practice is outside legislated scope and without authorized delegation.

TABLE 2 Differential Diagnoses of Arthritis, Carditis and Chorea ARTHRITIS CARDITIS CHOREA - Septic arthritis - Physiological mitral regurgitation - Drug intoxication - Connective tissue and other - prolapse - Tic disorder autoimmune diseases such as - Congenital valve disease - Intracranial tumor juvenile idiopathic arthritis - - Lyme disease - Lyme disease - Myocarditis, viral or idiopathic - Autoimmune: Systemic lupus - Infective endocarditis - Kawasaki disease erythematosus, systemic vasculitis

The diagnosis is based on a collection of signs known as Jones’ criteria; see Table 3, Revised Jones’ Criteria.

Pediatric Clinical Practice Guidelines for Nurses in Primary Care 2017 11–8c Cardiovascular System

TABLE 3 Revised Jones’ Criteria*(7) MAJOR CRITERIA MINOR CRITERIA Low-risk populations** High-risk populations Low-risk populations** High-risk populations - Carditis (clinical and/ - Carditis (clinical and/ - Polyarthralgia - Monoarthralgia or subclinical) or subclinical) - Fever (≥ 38.5°C) - Fever (≥ 38°C) - Polyarthritis - Arthritis (monoarthri- - - ESR ≥ 30 mm/h and/or - Chorea tis or polyarthritis, hour and/or CRP ≥ 30 mg/L - Erythema marginatum polyarthralgia) CRP ≥ 30 mg/L - Prolonged PR interval, - Subcutaneous nodules - Chorea - Prolonged PR interval, after accounting for - Erythema marginatum after accounting for age age variability (unless - Subcutaneous nodules variability (unless cardi- carditis is a major tis is a major criterion) criterion) *For all client populations with evidence of preceding group A Streptococcal pharyngitis infection: − Diagnosis of initial acute rheumatic fever is: • 2 major manifestations or • 1 major plus 2 minor manifestations − Diagnosis of recurrent acute rheumatic fever is: • 2 major or • 1 major and 2 minor or • 3 minor manifestations **Low-risk populations are those with acute rheumatic fever ≤ 2 per 100,000 school-aged children or all-age rheumatic heart disease prevalence of ≤ 1 per 1,000 population per year.

COMPLICATIONS DIAGNOSTIC TESTS – Rheumatic heart disease due to permanent damage to the heart valves, which leads Consult physician/nurse practitioner when to valvular disease, cardiac myopathy and practice is outside legislated scope and without sequelae such as:(1) authorized delegation. • Heart failure • Diagnostic test selection is based on client history, • Systemic embolism • Stroke test availability. Testing should be carried out as • Endocarditis per provincial/territorial policies and procedures. • Need for cardiac surgery The tests below are for consideration. Current- – Recurrent attack, which worsens the damage to the heart(12) the diagnosis of ARF.(1) ARF remains a clinical − Death(2) diagnosis and relies on health professionals being aware of its diagnostic features.(1)

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Laboratory Antipyretic/Analgesic – Rapid Antigen Detection Test (RADT) Acetaminophen(14) (if available) – Acetaminophen 10 to 15 mg/kg/dose PO – Throat culture and sensitivity (C+S) q4-6h PRN for group A Streptococcus(10) – [caution-hepatic, INR, renal] – WBC, ESR(13) − Maximum from all sources: acetaminophen 75 − Blood culture and sensitivity (C+S) if febrile(13) mg/kg/day or 4,000 mg/day, whichever is less

Other Diagnostic Tests Antibiotic Therapy – Chest x-ray(13) – Antibiotics should be initiated to eradicate − ECG to assess for prolonged PR interval(1) residual GAS infection in all cases while the diagnosis of ARF is being established.(1) MANAGEMENT – A full course of antibiotics should be given.(1) – Oral therapy such as penicillin, amoxicillin, Consult physician/nurse practitioner when cephalexin or clindamycin may be considered. practice is outside legislated scope and without FNIHB authorized delegation. Pediatric and Adolescent Care Clinical Practice Guidelines – Chapter 9 – Ears, Nose, Throat and Note: The diagnosis and treatment of rheumatic Mouth – Bacterial Pharyngotonsillitis – Antibiot- fever requires medical evacuation. Emergency ic Therapy. treatment of heart failure may be necessary; see MONITORING AND FOLLOW-UP FNIHB Pediatric and Adolescent Care Clinical Practice Guidelines – Chapter 11 – Cardiovascular System – Cardiac Failure. Consult physician/nurse practitioner when practice is outside legislated scope and GOALS OF TREATMENT without authorized delegation. – Provide symptomatic relief − Prevent complications MONITORING – Monitor vital signs as indicated by client’s NON-PHARMACOLOGIC INTERVENTIONS condition, including oxygen saturation. Interventions – Monitor intake and output. − Bed rest while awaiting medical evacuation – Monitor for signs of cardiac failure. If cli- ent is in cardiac failure, see FNIHB Pediat- PHARMACOLOGICAL INTERVENTIONS ric and Adolescent Care Clinical Practice Guidelines – Chapter 11 – Cardiovascular System – Cardiac Failure. In addition to consulting a physician/nurse − If administering an agent with risk of anaphy- practitioner, review the drug monograph, the laxis, monitor the client closely for 30 minutes. FNIHB Nursing Station Formulary or provincial/ territorial formulary before initiating treatment. FOLLOW-UP Note: With the exception of acetaminophen or Post-acute Phase medications to treat heart failure (if required), – All clients should receive regular fol- medications should not be started until the diagnosis has been clearly established. After low-up. The frequency and duration of consultation with receiving facility, antibiotics review is dependent on individual clinical may be started after throat swab(s) have been needs and should become more frequent in collected.(1) the event of symptom onset, symptomatic (1)

Pediatric Clinical Practice Guidelines for Nurses in Primary Care 2017 11–8e Cardiovascular System

– Discuss the importance of ongoing second- Approaches to Disease Prevention ary prophylaxis during every health profes- Effective control programs for acute rheumatic sional interaction with the client.(1) fever integrate features of primordial, primary – Because of the risk of recurrence, contin- and secondary prevention. ual antibiotic prophylaxis with benzathine penicillin G must be maintained. Primordial Prevention – The risk of recurrence is greatest in the − Primordial prevention refers to broad 5 years after the initial episode. social, economic and environmental ini- – Prophylaxis is initiated immediately after tiatives undertaken to prevent or limit the completion of a full therapeutic course of impact of GAS infection in a population. antibiotics, as initiated by a physician/nurse practitioner. Primary Prevention – The physician/nurse practitioner should − Primary prevention refers to medical in- also determine any discontinuation tervention using antibiotics to reduce GAS of prophylaxis. transmission, acquisition, colonization and – The duration of antibiotic prophylaxis carriage, or treating GAS infection effec- can range from 5 years to 30 years, as it tively to prevent the development of acute depends on a number of factors, including rheumatic fever.(13) age, clinical pattern, environment and time elapsed since the last episode of ARF.(1) Secondary Prevention − Clients may need to be on other cardiac and − Secondary prevention refers to medical anticoagulation therapies, depending on the intervention using long-term prophylactic severity of the heart damage due to ARF. antibiotics to reduce repeated acquisition of GAS that might induce recurrent episodes Referral of acute rheumatic fever in order to prevent − Arrange for medical evacuation. the development of rheumatic heart dis- ease, or for those clients who have estab- APPENDIX lished rheumatic heart disease in order to SECTION A: SUPPLEMENTAL CLINICAL prevent disease progression. MANAGEMENT INFORMATION BIBLIOGRAPHY Heart Murmurs – Those most commonly heard during acute The following references and other sources rheumatic fever are:(8) have informed the updating of this Clinical • Apical pansystolic murmur is a high- Practice Guideline. pitched, blowing-quality murmur of mitral regurgitation that radiates to the REFERENCES left axilla. The murmur is unaffected by 1. Heart Foundation of New Zealand. New respiration or position. Zealand guidelines for rheumatic fever: Diag- • Apical diastolic murmur (known as nosis, management and secondary prevention Carey-Coombs murmur) is heard with of acute rheumatic fever and rheumatic heart active carditis and accompanies severe disease: 2014 update. [Internet] Auckland (NZ): The National Heart Foundation of New Zealand; • Basal diastolic murmur is an early dia- 2014 [cited 2015 May 20]. Available from: http:// stolic murmur of aortic regurgitation and assets.heartfoundation.org.nz/shop/marketing/ is a high-pitched, blowing, decrescendo non-stock-resources/diagnosis-management-rheu- heard best along the right upper and mid- matic-fever-guideline.pdf left sternal border after deep expiration while the client is leaning forward.

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2. Gerber MA, Baltimore RS, Eaton CB, Gewitz 7. Gewitz MH, Baltimore RS, Tani LY, Sable M, Rowley AH, Shulman ST, et al. Prevention of CA, Shulman ST, Kaplan EL. Revision of the Rheumatic Fever and Diagnosis and Treatment Jones Criteria for the diagnosis of acute rheumat- ic fever in the era of doppler echocardiography: Statement From the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Association. Circulation. [Internet] Hagerstown, Disease Committee of the Council on Cardiovas- MD : Lippincott Williams & Wilkins; 2015 May cular Disease. Circulation. [Internet] Hagerstown, 19. doi:10.1161/CIR.0000000000000205. MD : Lippincott Williams & Wilkins; 2009. Available from: http://circ.ahajournals.org/con- 119(11), 1541–1551. doi:10.1161/CIRCULATIO- tent/early/2015/04/23/CIR.0000000000000205 NAHA.109.191959. Available from: http://www.circ.ahajournals.org/con- 8. Chin TK, Chin EM. Pediatric Rheumatic Heart tent/119/11/1541 Disease: Background, Pathophysiology, Epide- miology. [Internet] New York, NY: Medscape; 3. Gordon J, Kirlew M, Schreiber Y, Saginur R, 2014 [cited 2015 December 14]. Available from Bocking N, Blakelock B, et al. Acute rheumatic http://emedicine.medscape.com with authorized fever in First Nations communities in northwest- username and password. ern Ontario. Canadian Family Physician. Don Mills, Ont.: College of Family Physicians of 9. Colucci WS. Evaluation of the patient with Canada; 2015. 61(10), 881–886. Available from: suspected heart failure. [Internet] Waltham, MA http://www.cfp.ca/content/61/10/881 (US): UptoDate; 2016. [cited 2017 January 12]. Available from: http://www.uptodate.com with 4. Victoria State Government. Rheumatic heart authorized username and password. disease. Department of Health and Human Services. [Internet] Melbourne, Victoria (AU): Department of 10. Chin TK, Li D. Pediatric rheumatic fever: Health and Human Services, State Government of Background, pathophysiology, epidemiology. Victoria (AU); 2014 [cited 2015 May 6]. Available [Internet] New York, NY: Medscape; 2014 from: http://www.betterhealth.vic.gov.au/health/con- [cited 2015 December 7]. Available from: ditionsandtreatments/rheumatic-heart-disease. http://reference.medscape.com with authorized username and password. 5. Dumitru I, Baker MM. Heart Failure: Practice Essentials, Background, Pathophysiology. 11. Steer A, Gibofsky A. Acute rheumatic fever: [Internet] New York, NY: Medscape; 2015 Clinical manifestations and diagnosis. [Internet] [cited 2015 December 14]. Available from: Waltham, MA (US): UptoDate; 2015. Available http://emedicine.medscape.com with authorized from: https://www.uptodate.com with authorized username and password. username and password.

6. Madden S, Kelly L. Update on acute rheumatic 12. World Heart Federation. Rheumatic heart fever: It still exists in remote communities. Canadi- disease. [Internet] Geneva, Switzerland: World an Family Physician. [Internet] Don Mills, ON: Col- Heart Federation; 2012 [cited 2016 May 17]. lege of Family Physicians of Canada; 2009. 55(5), Available from: https://www.world-heart-federa- 475–478. Available from: http://www.ncbi.nlm.nih. tion.org/programmes/rheumatic-heart-disease/ gov/pmc/articles/PMC2682299/ with payment.

Pediatric Clinical Practice Guidelines for Nurses in Primary Care 2017 11–8g Cardiovascular System

13. RHD Australia (ARF/RHD writing group). Canadian Pharmacists Association. Amoxicillin The Australian guideline for prevention, diagno- (Amoxicillin). [Internet] Ottawa, ON: e-Thera- sis and management of acute rheumatic fever and peutics; 2015 [cited 2015 December 6]. Available rheumatic heart disease, 2nd ed. Quick reference from: http://www.e-therapeutics.ca with autho- guides. National Heart Foundation of Australia rized username and password. and the Cardiac Society of Australia and New Zealand. [Internet] Casuarina NT (AU): Men- Amoxicillin. [Internet] Hudson, OH: Lexi-Comp. zies School of Health Research; c2014 [cited Inc.; 2015 [cited 2015 December 6]. Available 2015 December 16]. Available from: http://www. from: http://online.lexi.com with authorized username and password. quick_reference_guides_0.pdf Canadian Pharmacists Association. Cephalexin. 14. Acetaminophen. [Internet] Hudson, OH: [Internet] Ottawa, ON: e-Therapeutics; 2009 Lexi-Comp. Inc.; 2015 [cited 2015 April 23]. [cited 2015 December 6]. Available from: http:// Available from: http://online.lexi.com with www.e-therapeutics.ca with authorized username authorized username and password. and password.

OTHER SOURCES Cephalexin. [Internet] Hudson, OH: Lexi-Comp. Health Canada. First Nations and Inuit Health Inc.; 2015 [cited 2015 December 6]. Available http://online.lexi.com Branch (FNIHB) Nursing Station Formulary and from: with authorized user- name and password.

Canadian Pharmacists Association. Acetamino- Canadian Pharmacists Association. Clindamycin phen (Tylenol). [Internet] Ottawa, ON: e-Thera- (Dalacin C). [Internet] Ottawa, ON: e-Therapeu- peutics; 2012 [cited 2015 December 13]. Avail- tics; 2014 [cited 2015 December 6]. Available http://www.e-therapeutics.ca able from: http://www.e-therapeutics.ca with from: with autho- authorized username and password. rized username and password.

Canadian Pharmacists Association. Compendium Clindamycin. [Internet] Hudson, OH: Lexi-Comp. of Therapeutic Choices: Canada’s Trusted Refer- Inc.; 2015 [cited 2015 December 6]. Available http://online.lexi.com ence for Primary Care Therapeutics (CTC 7). from: with authorized Ottawa: Canadian Pharmacists Association; c2014. username and password.

Acetaminophen. [Internet] Hudson, OH: Lexi- Canadian Pharmacists Association. Penicillin Comp. Inc.; 2015 [cited 2015 December 13]. G. [Internet] Ottawa, ON: e-Therapeutics; 2009 http:// Available from: http://online.lexi.com with autho- [cited 2015 December 6]. Available from: www.e-therapeutics.ca rized username and password. with authorized username and password. Canadian Pharmacists Association. Penicillin V. [Internet] Ottawa, ON: e-Therapeutics; Penicillin G. [Internet] Hudson, OH: Lexi-Comp. 2009 [cited 2015 December 6]. Available from: Inc.; 2015 [cited 2015 December 6]. Available http://online.lexi.com http://www.e-therapeutics.ca with authorized from: with authorized username and password. username and password.

Penicillin V. [Internet] Hudson, OH: Lexi-Comp. Inc.; 2015 [cited 2015 December 6]. Available from: http://online.lexi.com with authorized user- name and password.

2017 Pediatric Clinical Practice Guidelines for Nurses in Primary Care Cardiovascular System 11–9

VIRAL MYOCARDITIS Risk Factors Myocarditis is an inflammatory disorder of the Younger patients, especially newborns and infants and myocardium with necrosis of the myocytes and immunocompromised individuals may have increased associated inflammatory infiltrate. susceptibility to myocarditis.

PATHOPHYSIOLOGY HISTORY Myocarditis generally results in a decrease in Clinical presentation varies widely. In mild myocardial function, with concomitant enlargement forms, few or no symptoms are noted. In severe of the heart and an increase in the end-diastolic cases, patients may present with acute cardiac volume caused by increased preload. Progressive decompensation and progress to death. increase in left ventricular end-diastolic volume increases left atrial, pulmonary venous and arterial pressures, resulting in increasing hydrostatic forces. These increased forces lead to both pulmonary edema and congestive heart failure.

CAUSES It is usually caused by a viral infection. Parvovirus B19 and human herpesvirus-6 are the most frequent pathogens in patients with acute myocarditis. Infecting organisms may include the following: – Parvovirus B19 – Herpesvirus – Coxsackievirus types A and B (especially type B) – Adenovirus (most commonly types 2 and 5) – Cytomegalovirus – Echovirus – Epstein-Barr virus – virus – Human immunodeficiency virus – Influenza and parainfluenza – – , associated with endocardial fibroelastosis (EFE) – Poliomyelitis virus – Rubella – Varicella

Pediatric Clinical Practice Guidelines for Nurses in Primary Care 2011 11–10 Cardiovascular System

In newborns and infants, symptoms may sometimes Older Children appear suddenly and may include: –– Low grade fever –– Irritability –– Tachycardia, weak pulse –– Failure to thrive –– Jugular venous distention and edema of the lower –– Feeding difficulties extremities may be present –– Fever and other symptoms of infection –– Heart sounds may be muffled, especially in the –– Lethargy presence of pericarditis –– Low urine output (a sign of decreasing kidney –– An S3 may be present function) –– Heart murmur caused by atrioventricular valve –– Pale hands and feet (a sign of poor circulation) regurgitation may be heard –– Rapid breathing –– Crackles may be heard in older children –– Rapid heart rate –– Hepatomegaly may be present in younger children –– Cool extremities Symptoms in children over age 2 may also include: –– Decreased capillary refill –– Belly area pain and nausea –– Pale or mottled skin may be present –– Cough –– Fatigue Adolescents –– Swelling (edema) in the legs, feet and face Presentation in adolescents is similar to that of –– Recent, nonspecific, flu-like illness children between 6 and 12 years old. However, –– Older children present with similar symptoms the following symptoms may be more prominent: as above and may experience lack of energy and –– Decreased exercise tolerance general malaise –– Lack of energy, malaise –– Chest pain: Although rare in young children, this –– Chest pain may be the initial presentation for older children –– Low-grade fever and adolescents and should be considered a serious symptom accordingly –– –– Cough PHYSICAL FINDINGS –– Low cardiac output

Neonates/Infants DIFFERENTIAL DIAGNOSIS –– Hypothermia or hyperthermia –– Myocarditis, nonviral –– Tachypnea –– Pericarditis, viral –– Tachycardia –– Aortic stenosis, valvular –– Cyanosis –– Enteroviral infections –– Cool extremities –– Cardiomyopathy, dilated –– Decreased capillary refill –– Glycogen-storage disease type I or type II –– Pale or mottled skin may be present –– Coarctation of the aorta –– Wheezing, and diaphoresis with feeding –– Coronary anomalies –– Irritability –– Somnolence DIAGNOSTIC TESTS –– Hypotonia Chest x-ray may show cardiomegaly and –– Seizures cardiac failure. –– Oliguria (ECG) –– End-organ damage (for example, renal failure may develop because of direct viral infestation or because of low cardiac output

2011 Pediatric Clinical Practice Guidelines for Nurses in Primary Care Cardiovascular System 11–11

In some patients with mild cardiac involvement, ECG Adjuvant Therapy changes may be the only abnormal findings suggestive –– Give supplemental oxygen as necessary via of myocarditis. non-rebreather mask. Titrate to keep oxygen –– Low-voltage QRS (< 5 mm throughout the limb saturations > 97% leads) is the classic pattern –– Start an intravenous line with normal saline. Run –– Pseudoinfarction patterns with pathologic Q waves at a rate sufficient to maintain hydration depending and poor progression of R waves in the precordial on oral intake of child. Do not overhydrate. Keep leads may also be present line open until consultation with an emergency –– T-wave flattening or inversion is a common finding physician. Always weigh infant before starting associated with small or absent Q waves in V5 any intravenous fluids as a measure of hydration and V6 Nonpharmacologic Interventions –– Left with strain may be present –– Bed rest is necessary during the acute phase –– Other nonspecific findings include a prolonged of the illness PR segment and prolonged QT interval –– Nurse in an upright position –– is the most common finding Pharmacologic Interventions –– Premature ventricular contractions and have been reported Consult a physician for medication orders. –– is common and may Medications may include the following, when worsen congestive heart failure indicated: see “Cardiac Failure”. –– Occasional second-degree and third-degree –– Diuretics to decrease volume: may be present furosemide (Lasix), 1 mg/kg IV stat (may be given –– is commonly associated PO if IV access not available) and may be the initial presentation –– ACE inhibitors may be prescribed by a physician COMPLICATIONS for afterload reduction –– Digoxin may be used in some cases to increase –– Arrhythmia contractility –– Cardiac failure (see “Cardiac Failure”) –– Antiarrhythmics –– Thromboembolism –– Anticoagulants –– Decrease in ventricular function –– Monitoring and Follow-Up

MANAGEMENT Acute Phase Monitor ABCs (airway, breathing and circulation), Goals of Treatment vital signs, pulse oximetry, heart and lung sounds, –– Stabilize cardiovascular function neuromental status, intake and output and medication –– Prevent complications response and adverse effects closely until child is transferred to hospital. Appropriate Consultation Over the Long Term Consult a physician urgently if you suspect this condition. Children with cardiac illness should be monitored regularly within the community to ensure normal growth and development and to watch for complications. Frequency of follow-up depends on the severity of the condition.

Referral Medevac to a facility with intensive and cardiology care.

Pediatric Clinical Practice Guidelines for Nurses in Primary Care 2011 11–12 Cardiovascular System

SOURCES

Internet addresses are valid as of February 2012. Chin TK, Chin EM, Siddiqui T, Sundell, A-K. (2008, October). Rheumatic Heart Disease: BOOKS AND MONOGRAPHS Treatment & Medication. Available at: http:// Bickley LS. Bates’ guide to physical examination and emedicine.medscape.com/article/891897-overview history taking. 7th ed. Baltimore, MD: Lippincott Hong W, Tang W. (2008, September). Myocarditis. Williams & Wilkins; 1999. Available at: http://emedicine.medscape.com/ Cheng A, et al. The Hospital for Sick Children article/156330-overview handbook of pediatrics. 10th ed. Elsevier; 2003. Poothirikovil V. (2008, November). Cardiomyopathy, Colman R, Somogyi R (Editors-in-chief). Toronto notes Dilated. Available at: http://emedicine.medscape.com/ – MCCQE 2008 review notes. 24th ed. Toronto, ON: article/895187-overview University of Toronto, Faculty of Medicine; 2008. Rodriguez-Cruz E, Ross RD. (2008, October). Gray J (Editor-in-chief). Therapeutic choices. 5th ed. Myocarditis, Viral. Available at: http://emedicine. Ottawa, ON: Canadian Pharmacists Association; 2007. medscape.com/article/890740-overview Health Canada. Canadian immunization guide. 7th ed. Satou GM, Herzberg G, Erickson LC. (2006, June; Ottawa, ON: Health Canada; 2006. updated 2009, March). Heart Failure, Congestive. Available at: http://emedicine.medscape.com/ Jensen B, Regier L (Editors). The Rx files. 7th ed. article/901307-overview Saskatoon, SK; 2008 Rudolph CD, et al. Rudolph’s pediatrics. 21st ed. END NOTES McGraw-Hill; 2003. 1 Emergency Nurses Association (ENA) (2007) Trauma Nursing Core Competencies Provider Uphold CR, Graham MV. Clinical guidelines in child Manual. 6th Edition. p. 70. health. 3rd ed. Gainesville, FL: Barmarrae Books; 2003. 2 Debbie M. Popovich, Nancy Richiuso, Gale Danek. Repchinsky C (Editor-in-chief). CPS Compendium Pediatric health care providers’ knowledge of of pharmaceuticals and specialties. Ottawa, ON: pulse oximetry Pediatric Nursing, Jan-Feb, Canadian Pharmacists Association; 2007. 2004; p. 2. Available at: http://findarticles.com/p/ articles/mi_m0FSZ/is_1_30/ai_n17206797/ JOURNAL ARTICLES pg_2/?tag=content;col1 Arnold JMO, Liu P, Demers C, et al. Canadian 3 UpToDate Online 18.1. 2010. Treatment and Cardiovascular Society consensus conference prevention of rheumatic fever. And UpToDate Online recommendations on heart failure 2006: Diagnosis 18.1. 2010. Antibiotic therapy of GAS pharyngitis, which is based on Gerber, MA, Baltimore, RS, and management. Can J Cardiol 2006;22(1):23-45. Eaton, CB, et al. Prevention of rheumatic fever Birdi N, Hosking M, Clulow MK, Duffy CM, et and diagnosis and treatment of acute streptococcal al. Acute rheumatic fever and poststreptococcal pharyngitis: A scientific statement from the American reactive arthritis: Diagnostic and treatment practices Heart Association Rheumatic Fever, Endocarditis, of pediatric subspecialists in Canada. J Rheumatol and Kawasaki Disease Committee of the Council 2001;28:1681-88. on Cardiovascular Disease in the Young, the Interdisciplinary Council on Functional Genomics INTERNET GUIDELINES / DOCUMENTS / and Translational Biology, and the Interdisciplinary OTHER PUBLICATIONS Council on Quality of Care and Outcomes Research. Circulation 2009;119(11):1541-51. Available by Cilliers A, Manyemba J, Saloojee HH. (2003). Anti- subscription: www.uptodate.com inflammatory treatment for carditis in acute rheumatic 4 Secondary prevention of rheumatic fever (prevention fever. Available at: http://summaries.cochrane.org/ of recurrent attacks). UpToDate Online 18.1. CD003176/corticosterids-andimmunotherapy-to- Available by subscription: www.uptodate.com prevent-heart-damage-as-a-resultof-rheumatic-fever

2011 Pediatric Clinical Practice Guidelines for Nurses in Primary Care