Pediatric Cardiology Originally Developed by: Ryan Parnham, MSN, APN, CNP OSF Critical Care Service Revised Spring 2012 by Teresa D. Valerio, DNP, APN,

Pediatric Pediatric CardiologyCardiologyOriginally Developed by:Originally Developed by:

Ryan Parnham, MSN, APN, CNPRyan Parnham, MSN, APN, CNP

OSF Critical Care ServiceOSF Critical Care Service

Revised Spring 2012 Revised Spring 2012

by Teresa D. Valerio, DNP, APN, FNP-BCby Teresa D. Valerio, DNP, APN, FNP-BC

Today’s TopicsToday’s Topics

Cardiac Physical ExaminationCardiac Physical Examination Congenital Heart DiseaseCongenital Heart Disease Acquired Heart DiseaseAcquired Heart Disease

PurposePurpose

Enhance knowledge and Enhance knowledge and confidence with pediatric confidence with pediatric cardiology cardiology

Emphasize the importance of Emphasize the importance of recognizingrecognizing significant significant pediatric cardiovascular pediatric cardiovascular abnormalities as a primary care abnormalities as a primary care provider provider

Readings from TextbooksReadings from Textbooks Burns, C.E. et al. (2009). Burns, C.E. et al. (2009). Pediatric Pediatric Primary Care.Primary Care. – Chapter 30, pgs. 727-766.Chapter 30, pgs. 727-766.

Chiocca, E.M. (2011). Chiocca, E.M. (2011). Advanced Advanced Pediatric Assessment. Pediatric Assessment. – Chapter 18, pgs. 366-390.Chapter 18, pgs. 366-390.

Hay, W.H. et al. (2011). Hay, W.H. et al. (2011). Current Current Diagnosis & Treatment: Pediatrics.Diagnosis & Treatment: Pediatrics.– Chapter 19, pgs. 536-541, 547-566, Chapter 19, pgs. 536-541, 547-566, 571- 582, 593-594.571- 582, 593-594.

Other helpful resourcesOther helpful resources Westrol, M.S. & Raffi, K. (2012). Westrol, M.S. & Raffi, K. (2012). Pediatric ECG Cases: Benign Variants Pediatric ECG Cases: Benign Variants or Life-Threatening Abnormalities? or Life-Threatening Abnormalities? (PDF emailed)(PDF emailed)

Wierwille, L. (2011). Pediatric heart Wierwille, L. (2011). Pediatric heart murmurs: Evaluation and management in murmurs: Evaluation and management in primary care. primary care. The nurse practitionerThe nurse practitioner, , 36(3), 22-29. (PDF emailed)36(3), 22-29. (PDF emailed)

Rheumatic fever clinical guidelines at Rheumatic fever clinical guidelines at http://www.indianpediatrics.net/pdf/acute_rheumatic_fever.pdf

Elements of the Elements of the cardiac physical cardiac physical

examinationexamination

HistoryHistory

Pregnancy/Birth History: prenatal Pregnancy/Birth History: prenatal care, complicated prenatal course, care, complicated prenatal course, maternal risk factors (diabetes maternal risk factors (diabetes assoc. with hypertrophic assoc. with hypertrophic cardiomyopathy), prematurity, method cardiomyopathy), prematurity, method of delivery, prolonged hospital stay, of delivery, prolonged hospital stay, birth weight, APGARsbirth weight, APGARs

Family History: CHD, sudden death, Family History: CHD, sudden death, SIDS, cardiomyopathy (high incidence SIDS, cardiomyopathy (high incidence of inheritance, >20%), dysrhythmias, of inheritance, >20%), dysrhythmias, syndromes/genetic abnormalitiessyndromes/genetic abnormalities

Past Medical History Past Medical History

General HealthGeneral Health Concerns from parentsConcerns from parents Growth curve and weight gain (pay Growth curve and weight gain (pay attention to stature of parents)attention to stature of parents)

Chronic respiratory difficulties- Chronic respiratory difficulties- asthma, frequent URIs/pneumoniaasthma, frequent URIs/pneumonia

Other medical issues/surgeriesOther medical issues/surgeries Down Syndrome, Marfan Syndrome, Down Syndrome, Marfan Syndrome, etc.etc.

Important QuestionsImportant Questions Persistent tachypnea?Persistent tachypnea? Cyanosis?Cyanosis? Failure to thrive?Failure to thrive? Tiring and/or diaphoresis with Tiring and/or diaphoresis with feeding/minimal activity?feeding/minimal activity?

Palpitations?Palpitations? Chest Pain with exertion?Chest Pain with exertion? Syncope/Near-syncope? Syncope with Syncope/Near-syncope? Syncope with exertion? Palpitations with syncope?exertion? Palpitations with syncope?

Lethargy/Fatigue?Lethargy/Fatigue? Inability to keep up with peers?Inability to keep up with peers? Worsening activity tolerance?Worsening activity tolerance?

Vital SignsVital Signs

Respiratory Rate- should be counted Respiratory Rate- should be counted for a full minute in infants for a full minute in infants because of variabilitybecause of variability

Heart rate- resting if possibleHeart rate- resting if possible Blood pressure- bilateral upper and Blood pressure- bilateral upper and lower extremities (at least once in lower extremities (at least once in their life, unless more often if their life, unless more often if clinically indicated)- use correct clinically indicated)- use correct size cuff!size cuff!

Pulse ox- check/recheck multiple Pulse ox- check/recheck multiple extremities if abnormal readingextremities if abnormal reading

Overall AppearanceOverall Appearance

Anxious, distressed, diaphoretic, Anxious, distressed, diaphoretic, pale, tachypneic, retractions, pale, tachypneic, retractions, cyanotic, difficult to console, cyanotic, difficult to console, dysmorphic, clubbingdysmorphic, clubbing

A baby, child and adolescent can A baby, child and adolescent can have a significant cardiac lesion have a significant cardiac lesion and appear perfectly healthy with and appear perfectly healthy with apparently normal vitals signsapparently normal vitals signs

Cardiovascular Cardiovascular AssessmentAssessment

Is chest symmetrical? Evidence of Is chest symmetrical? Evidence of pectus excavatum, pectus carinatum.pectus excavatum, pectus carinatum.

Precordial activity quiet or Precordial activity quiet or hyperactive?hyperactive?

PMI displaced? (usually mid-PMI displaced? (usually mid-clavicular line)clavicular line)

Palpable thrill over chest? Thrill Palpable thrill over chest? Thrill at suprasternal notch?at suprasternal notch?

JVD?JVD?

AuscultationAuscultation

Listen at all landmarks Listen at all landmarks (aortic, pulmonic, 2(aortic, pulmonic, 2ndnd pulmonic, pulmonic, tricuspid, mitral) with bell tricuspid, mitral) with bell and diaphragmand diaphragm

Listen to heart soundsListen to heart sounds S1: closure of what valves?S1: closure of what valves? S2: closure of what valves?S2: closure of what valves?

S1S1

Closure of the atrioventricular Closure of the atrioventricular valves (mitral and tricuspid)valves (mitral and tricuspid)

Normally heard as one sound, Normally heard as one sound, although occasionally splitting although occasionally splitting may be heardmay be heard

S2S2

Closure of the semilunar valves Closure of the semilunar valves (pulmonary and aortic)(pulmonary and aortic)

Under normal circumstances, S2 is “split” Under normal circumstances, S2 is “split” during or near the end of inspiration during or near the end of inspiration (the right ventricle is filled more than (the right ventricle is filled more than the left with inspiration, causing a the left with inspiration, causing a short delay in closure of the PV)short delay in closure of the PV)

A fixed or widely split S2 could be A fixed or widely split S2 could be pathologic, as can a single S2 that does pathologic, as can a single S2 that does not split.not split.

www.med.ucla.edu/wilkes/inex.htm

MurmursMurmurs

What is a heart murmur?What is a heart murmur?

MurmursMurmurs Murmurs are audible sound waves caused Murmurs are audible sound waves caused by by turbulent blood flow-turbulent blood flow- a murmur is a murmur is not a hole, or a defective valve, etc.not a hole, or a defective valve, etc.

Most murmurs are “innocent,” or Most murmurs are “innocent,” or Still’s murmurs, 32-80% of all Still’s murmurs, 32-80% of all children will have a murmur at some children will have a murmur at some point in their life, often withpoint in their life, often with fever fever

Structural or physiologic cardiac Structural or physiologic cardiac problems can also cause murmurs; 1% of problems can also cause murmurs; 1% of children have structural heart diseasechildren have structural heart disease

Grading of MurmursGrading of Murmurs

1: Heard only with intense concentration1: Heard only with intense concentration 2: Faint, but heard immediately2: Faint, but heard immediately 3: Easily heard, of intermediate 3: Easily heard, of intermediate intensityintensity

4: Easily heard and palpable thrill 4: Easily heard and palpable thrill presentpresent

5: Very loud, thrill present, and audible 5: Very loud, thrill present, and audible with edge of stethoscope on chest wallwith edge of stethoscope on chest wall

6: Audible with stethoscope off chest 6: Audible with stethoscope off chest wallwall

Innocent Vibratory Murmur Innocent Vibratory Murmur or Still’s Murmuror Still’s Murmur

Most common during childhoodMost common during childhood Low to medium pitch, early systoleLow to medium pitch, early systole Usually grade 2, but can range 1-3Usually grade 2, but can range 1-3 Maximal at LLSB w/ radiation to apexMaximal at LLSB w/ radiation to apex Usually loudest supine and fades Usually loudest supine and fades with sitting and standingwith sitting and standing

Vibratory, harmonic, musicalVibratory, harmonic, musical Often louder during febrile illness Often louder during febrile illness or times of increased cardiac outputor times of increased cardiac output

Usually disappear with time and can Usually disappear with time and can come and gocome and go

Innocent Pulmonary Flow Innocent Pulmonary Flow MurmurMurmur

A relatively soft systolic A relatively soft systolic murmur appreciated at the upper murmur appreciated at the upper left sternal borderleft sternal border

Often louder while lying supine Often louder while lying supine and fades/disappears with and fades/disappears with sittingsitting

Venous HumVenous Hum

Continuous murmur usually heard at the Continuous murmur usually heard at the infraclavicular area of the anterior infraclavicular area of the anterior chest, R>Lchest, R>L

Loudest while sitting (venous return Loudest while sitting (venous return from the jugular veins and subclavian from the jugular veins and subclavian veins entering the SVC ) and disappears veins entering the SVC ) and disappears supinesupine

Can be diminished or muted with gentle Can be diminished or muted with gentle compression of jugular veincompression of jugular vein

Commonly associated with vibratory Commonly associated with vibratory murmurmurmur

Pathological Systolic Pathological Systolic MurmursMurmurs

Upper Right Sternal Border: Upper Right Sternal Border: Aortic Stenosis, subaortic Aortic Stenosis, subaortic membrance, PPASmembrance, PPAS

Upper Left Sternal Border: Upper Left Sternal Border: Pulmonary Stenosis, PDA, PPASPulmonary Stenosis, PDA, PPAS

Mid to Lower Left Sternal Mid to Lower Left Sternal Border: VSD, TR, LVOT narrowing Border: VSD, TR, LVOT narrowing (HCM)/Subaortic stenosis(HCM)/Subaortic stenosis

Apex: MR, poss VSDApex: MR, poss VSD

Pathological Systolic Pathological Systolic MurmursMurmurs

Usually don’t change with Usually don’t change with position, except position, except HCM causing LVOT HCM causing LVOT obstruction becomesobstruction becomes louder with louder with standing.standing.

MR may be somewhat more pronounced MR may be somewhat more pronounced while lying on the left side.while lying on the left side.

Often times “harsh”, usually quite Often times “harsh”, usually quite different from an innocent murmur, different from an innocent murmur, but not alwaysbut not always

Diastolic MurmursDiastolic Murmurs

AlwaysAlways pathological- there are pathological- there are no “normal” or “innocent” no “normal” or “innocent” diastolic murmursdiastolic murmurs

What are some diastolic What are some diastolic murmurs?murmurs?

““Early” Diastolic Early” Diastolic MurmursMurmurs

Decrescendo in natureDecrescendo in nature Commonly Commonly aortic regurgitationaortic regurgitation or or pulmonary regurgitationpulmonary regurgitation (insufficiency)(insufficiency)

Mid to Late Diastolic Mid to Late Diastolic MurmursMurmurs

Tricuspid stenosisTricuspid stenosis or or mitral mitral stenosisstenosis

Continuous MurmursContinuous Murmurs

Heard through systole and Heard through systole and diastole- most common diastole- most common “pathological” continuous “pathological” continuous murmur is a murmur is a Patent Ductus Patent Ductus ArteriosusArteriosus

Cardiovascular Exam Cardiovascular Exam (cont’d)(cont’d)

Resp- Lungs clear? Equal? Congested? Resp- Lungs clear? Equal? Congested? Diminished? Tachypneic? Retractions?Diminished? Tachypneic? Retractions?

Skin- Warm? Pink? Well-perfused?Skin- Warm? Pink? Well-perfused? Abdomen- soft? Liver enlarged? Abdomen- soft? Liver enlarged? Ascites? Situs solititus or Ascites? Situs solititus or inversus?inversus?

Pulses- Weak? Bounding? Normal? Pulses- Weak? Bounding? Normal? Brachial/radial-femoral delay? Brachial/radial-femoral delay?

Extremities: Capillary Refill Brisk? Extremities: Capillary Refill Brisk? Clubbing? Edema?Clubbing? Edema?

Exam (con’t)Exam (con’t)

If you want to truly perform a If you want to truly perform a quality cardiovascular quality cardiovascular examination, auscultate the examination, auscultate the patient lying, sitting, standing patient lying, sitting, standing and squatting, especially with and squatting, especially with athletes, patients with syncope athletes, patients with syncope with exertion, or family history with exertion, or family history of sudden death and/or of sudden death and/or hypertrophic cardiomyopathy!hypertrophic cardiomyopathy!

When Do You Refer for a When Do You Refer for a Murmur?Murmur?

Is this a new murmur?Is this a new murmur? What are the characteristics of What are the characteristics of the murmur? Systolic vs. the murmur? Systolic vs. diastolic.diastolic.

Is the child febrile?Is the child febrile? Is the child “symptomatic”?Is the child “symptomatic”? What testing has been done thus What testing has been done thus far- EKG, CXR, ECHOfar- EKG, CXR, ECHO

Family historyFamily history

When Do You Refer for a When Do You Refer for a Murmur?Murmur?

If no definite “red flags,” consider having If no definite “red flags,” consider having the child RTC to re-listen in a week or two the child RTC to re-listen in a week or two (especially if febrile!!!)(especially if febrile!!!)

If some concern, order an EKG and CXR- If some concern, order an EKG and CXR- relatively inexpensive and can provide useful relatively inexpensive and can provide useful infoinfo

If EKG and CXR normal, but still uncertain, ok If EKG and CXR normal, but still uncertain, ok to refer or consider ordering an echo to refer or consider ordering an echo (caution- expensive and may “over-diagnose”)(caution- expensive and may “over-diagnose”)

If child in obvious distress- refer If child in obvious distress- refer immediately/admit for evaluationimmediately/admit for evaluation

If the patient is in competitive athletics and If the patient is in competitive athletics and it is a new murmur, consider referral.it is a new murmur, consider referral.

At least order an EKG and CXR prior to At least order an EKG and CXR prior to referral- appreciated by the cardiology group!referral- appreciated by the cardiology group!

Congenital Heart Congenital Heart DiseaseDisease

Congenital Heart Congenital Heart DiseaseDisease

8-10/ 1,000 liveborn infants will have 8-10/ 1,000 liveborn infants will have a congenital cardiac malformation a congenital cardiac malformation (0.8-1%)(0.8-1%)

Risk of recurrence in families with Risk of recurrence in families with one parent or one sibling with CHD is one parent or one sibling with CHD is 1-4%1-4%

Some defects are associated with even Some defects are associated with even higher recurrence rates in families, higher recurrence rates in families, and geneticists are beginning to and geneticists are beginning to identify certain genes that may help identify certain genes that may help explain thisexplain this

What causes CHD?What causes CHD?

Genetic factors?Genetic factors? Environmental factors?Environmental factors? Genetic Genetic andand environmental environmental factors?factors?

Possible environmental factors: Possible environmental factors: maternal infection/illness, maternal infection/illness, medication use, substance abuse, medication use, substance abuse, chronic diseases such as chronic diseases such as diabetes, lupus, etc.diabetes, lupus, etc.

Review of Fetal Review of Fetal CirculationCirculation

Congenital Cardiac Congenital Cardiac LesionsLesions

Acyanotic and CyanoticAcyanotic and Cyanotic

Acyanotic LesionsAcyanotic Lesions

Atrial Septal Defect (ASD)Atrial Septal Defect (ASD) Ventricular Septal Defect (VSD)Ventricular Septal Defect (VSD) Atrioventricular Septal Atrioventricular Septal Defects/AV CanalDefects/AV Canal

Patent Ductus Arteriosus (PDA)Patent Ductus Arteriosus (PDA) Aortic StenosisAortic Stenosis Pulmonary StenosisPulmonary Stenosis Coarctation of the AortaCoarctation of the Aorta

Cyanotic LesionsCyanotic Lesions

d-transposition of the great d-transposition of the great arteries (d-TGA)arteries (d-TGA)

Tetralogy of FallotTetralogy of Fallot Hypoplastic right heart syndromeHypoplastic right heart syndrome Hypoplastic left heart syndromeHypoplastic left heart syndrome Double outlet right ventricle (DORV)Double outlet right ventricle (DORV) Truncus arteriosusTruncus arteriosus Total anomalous pulmonary venous Total anomalous pulmonary venous return (TAPVR)return (TAPVR)

Commonly Seen Commonly Seen Acyanotic LesionsAcyanotic Lesions

Atrial Septal Defects Atrial Septal Defects (ASD)(ASD)

Common defectCommon defect May be a PFO/small secundum ASD- 20-25% May be a PFO/small secundum ASD- 20-25% of the population has thisof the population has this

May be larger, causing significant L to May be larger, causing significant L to R shuntingR shunting

Can go undetected for yearsCan go undetected for years Exam: wide, fixed splitting of S2, often Exam: wide, fixed splitting of S2, often a systolic murmur r/t increased pulm a systolic murmur r/t increased pulm flowflow

““Easily” fixed (surgery vs. Easily” fixed (surgery vs. transcatheter)transcatheter)

Ventricular Septal Ventricular Septal DefectDefect

Ventricular septum fails to “fill in” Ventricular septum fails to “fill in” completely during embryonic developmentcompletely during embryonic development

Various degrees of VSDs from tiny to largeVarious degrees of VSDs from tiny to large May be asymptomatic, mildly symptomatic, May be asymptomatic, mildly symptomatic, or in congestive heart failureor in congestive heart failure

May not present clinically until 1-2 May not present clinically until 1-2 months of lifemonths of life

Often associated with other lesionsOften associated with other lesions Isolated VSD’s typically have favorable Isolated VSD’s typically have favorable surgical outcomessurgical outcomes

Many small and even mod sized VSD’s can Many small and even mod sized VSD’s can close spontaneously for up to 4 years of close spontaneously for up to 4 years of ageage

Mitral Valve ProlapseMitral Valve Prolapse The most common valvular abnormality (2-6% The most common valvular abnormality (2-6% of the population)- more common in females of the population)- more common in females than males (2:1)than males (2:1)

Usually benign, but may also have mitral Usually benign, but may also have mitral regurgitationregurgitation

Can worsen with time, or improveCan worsen with time, or improve Exam: mid-late systolic click (often), Exam: mid-late systolic click (often), blowing, holosystolic murmur if significant blowing, holosystolic murmur if significant degree of MRdegree of MR

Diagnosis- echocardiogram (often wrongly Diagnosis- echocardiogram (often wrongly and over-diagnosed by exam only)and over-diagnosed by exam only)

May be associated with connective tissue May be associated with connective tissue disorders such as Marfan Syndromedisorders such as Marfan Syndrome

Coarctation of the Coarctation of the AortaAorta

Narrowed area of the aorta (usually Narrowed area of the aorta (usually descending or transverse arch)descending or transverse arch)

May cause hypertensionMay cause hypertension Often systolic murmur LUSB with Often systolic murmur LUSB with radiation to left backradiation to left back

Discrepancy between upper Discrepancy between upper and lower extremity blood and lower extremity blood pressure and pulsespressure and pulses

Usually an “easy” repairUsually an “easy” repair

Common Cyanotic Common Cyanotic LesionsLesions

Tetralogy of FallotTetralogy of Fallot

4 defining characteristics- 4 defining characteristics- Pulmonary stenosis, RVH, VSD, and Pulmonary stenosis, RVH, VSD, and aortic overrideaortic override

May be associated with a genetic May be associated with a genetic syndromesyndrome

Surgical repair in first year of Surgical repair in first year of life (often need a shunt placed life (often need a shunt placed soon after birth)soon after birth)

If PV is replaced, usually have If PV is replaced, usually have some degree of dysfunction that some degree of dysfunction that needs to be monitored over timeneeds to be monitored over time

D-transposition of the D-transposition of the Great ArteriesGreat Arteries

Aorta rises from the RV and Aorta rises from the RV and Pulmonary Artery rises from the Pulmonary Artery rises from the LV- complete separation of LV- complete separation of pulmonary and systemic pulmonary and systemic circulationscirculations

Arterial switch procedure Arterial switch procedure

There are many advances There are many advances underway in pediatric underway in pediatric

cardiac surgery and cardiac cardiac surgery and cardiac catheterization techniques catheterization techniques that are not only improving that are not only improving

surgical outcomes, but surgical outcomes, but resulting in shorter resulting in shorter

hospital stays and a better hospital stays and a better quality of life.quality of life.

Acquired Heart Acquired Heart DiseaseDisease

Kawasaki Disease (KD)Kawasaki Disease (KD)

An acute, self-limited vasculitisAn acute, self-limited vasculitis Unknown cause, but an infectious Unknown cause, but an infectious cause/virus is suspectedcause/virus is suspected

Leading cause of acquired heart Leading cause of acquired heart disease in US and Japandisease in US and Japan

Usually seen in children <5 y.o.Usually seen in children <5 y.o. First described by Dr. Kawasaki First described by Dr. Kawasaki in Japan, 1961in Japan, 1961

KD- Diagnostic CriteriaKD- Diagnostic Criteria Fever >/= 5 daysFever >/= 5 days Bilateral conjuctival injectionBilateral conjuctival injection Changes of mucous membranes- Changes of mucous membranes- injected pharynx, fissured lips, injected pharynx, fissured lips, strawberry tonguestrawberry tongue

Changes of peripheral extremities- Changes of peripheral extremities- peripheral edema, peripheral peripheral edema, peripheral erythema, desquamation of palmserythema, desquamation of palms

Polymorphous rashPolymorphous rash Cervical adenopathyCervical adenopathy Diagnosis is presence of fever and 4 Diagnosis is presence of fever and 4 of 5 remaining criteriaof 5 remaining criteria

KD- Cardiovascular KD- Cardiovascular concernsconcerns

Development of coronary artery Development of coronary artery aneurysms (usually around 2 wks after aneurysms (usually around 2 wks after onset of symptoms)onset of symptoms)

Evaluated by echocardiogramEvaluated by echocardiogram Half of patients with aneurysms will Half of patients with aneurysms will remodel vessel wall- never completely remodel vessel wall- never completely normal. Probably at higher risk for normal. Probably at higher risk for future coronary artery disease- long-future coronary artery disease- long-term antiplatelet therapy generally term antiplatelet therapy generally recommendedrecommended

Strawberry TongueStrawberry Tongue

TreatmentTreatment

Standard treatment is high-dose Standard treatment is high-dose IVIG (immunoglobulins) in IVIG (immunoglobulins) in combination with high doses of combination with high doses of aspirinaspirin

Screening/repeat echocardiogramsScreening/repeat echocardiograms Possible cardiac cath with Possible cardiac cath with intervention if coronary artery intervention if coronary artery aneurysms causing myocardial aneurysms causing myocardial dysfunction or for further dysfunction or for further evaluationevaluation

Long-term prognosis Long-term prognosis without coronary aneurysmwithout coronary aneurysm

No significant risk of increased No significant risk of increased mortalitymortality

Possible increased risk of premature Possible increased risk of premature atherosclerosis r/t vasculitis?atherosclerosis r/t vasculitis?

Little long-term data, although Little long-term data, although there have been a couple of studies there have been a couple of studies suggesting abnormal vasodilatory suggesting abnormal vasodilatory properties of coronary arteries in properties of coronary arteries in KD patients with seemingly KD patients with seemingly “unaffected” coronaries“unaffected” coronaries

Rheumatic FeverRheumatic Fever A systemic illness thought to occur A systemic illness thought to occur following group A beta hemolytic following group A beta hemolytic streptococcal pharyngitis (GABHS) in streptococcal pharyngitis (GABHS) in children, median age 10 years (can children, median age 10 years (can happen in adults though)happen in adults though)

Although uncommon in present day, Although uncommon in present day, accurate clinical diagnosis is vital accurate clinical diagnosis is vital for optimal long-term prognosis. for optimal long-term prognosis.

Was the leading cause of death in Was the leading cause of death in patients aged 5-20 in the US about patients aged 5-20 in the US about 100 yrs ago.100 yrs ago.

Increased risk- untreated or delayed Increased risk- untreated or delayed treatment of a strep infectiontreatment of a strep infection

HistoryHistory

Sore throat 1-5 wks prior to onset of Sore throat 1-5 wks prior to onset of RF symptomsRF symptoms

Fever, rash, headache, wt loss, Fever, rash, headache, wt loss, fatigue, diaphoresis, chest fatigue, diaphoresis, chest pain/pounding, migratory joint pain, pain/pounding, migratory joint pain, skin nodules, motor dysfunction, skin nodules, motor dysfunction, previous RF (higher risk of recurrence)previous RF (higher risk of recurrence)

Can cause endocarditis, myocarditis, Can cause endocarditis, myocarditis, and pericarditis. Usually affecting and pericarditis. Usually affecting the mitral and aortic valves resulting the mitral and aortic valves resulting in regurgitationin regurgitation

Jones CriteriaJones CriteriaRequires presence of 2 major, Requires presence of 2 major,

or 1 major and 2 minor or 1 major and 2 minor criteria. Previous Group A criteria. Previous Group A strep is also necessarystrep is also necessary

Major CriteriaMajor Criteria Carditis (EKG, CXR, ECHO, Exam)- new murmur Carditis (EKG, CXR, ECHO, Exam)- new murmur and tachycardia. Wide pulse pressure if and tachycardia. Wide pulse pressure if severe aortic regurgitation. May also have severe aortic regurgitation. May also have CHF- JVD, hepatomegaly, gallop rhythm, CHF- JVD, hepatomegaly, gallop rhythm, friction rub, peripheral edemafriction rub, peripheral edema

PolyarthritisPolyarthritis Chorea- brief, irregular, unpredictable, Chorea- brief, irregular, unpredictable, purposeless movements that flow from one body purposeless movements that flow from one body part to another without a rhythmic pattern part to another without a rhythmic pattern

Erythema marginatum- 1 to 3 cm pink/red Erythema marginatum- 1 to 3 cm pink/red nonpruitic macules or papules on trunk and nonpruitic macules or papules on trunk and limbs (not on face)limbs (not on face)

Subcutaneous nodules- infrequent, but occur Subcutaneous nodules- infrequent, but occur on surfaces of elbows, knees, ankles, on surfaces of elbows, knees, ankles, knuckles, and other spinous processes- firm knuckles, and other spinous processes- firm and nontenderand nontender

Subcutaneous NodulesSubcutaneous Nodules

Erythema MarginatumErythema Marginatum

Minor CriteriaMinor Criteria

Fever- usually greater than 39 Fever- usually greater than 39 deg C, although may be low-grade, deg C, although may be low-grade, may last 2-3 weeksmay last 2-3 weeks

Arthralgia- cannot be considered Arthralgia- cannot be considered minor if arthritis is presentminor if arthritis is present

Prolonged PR interval in EKGProlonged PR interval in EKG Elevated erythrocyte Elevated erythrocyte sedimentation rate (ESR) and C-sedimentation rate (ESR) and C-Reactive Protein (CRP)Reactive Protein (CRP)

Evidence of GAS Evidence of GAS pharyngitis – one of the pharyngitis – one of the following MUST be presentfollowing MUST be present

Positive throat culture or Positive throat culture or rapid strep antigen testrapid strep antigen test

Elevated or rising Elevated or rising streptococcal antibody titer streptococcal antibody titer (such as antistreptolysin O (such as antistreptolysin O [ASO])[ASO])

Lab StudiesLab Studies

Throat cultureThroat culture Rapid antigen detection testRapid antigen detection test Antistreptococcal antibodies- Antistreptococcal antibodies- usually using antistreptolysin usually using antistreptolysin O (ASO)O (ASO)

C-RP, ESRC-RP, ESR

ImagingImaging

CXR- pulm cong/CHFCXR- pulm cong/CHF Echo- MR, AR, LV dilationEcho- MR, AR, LV dilation ECG- sinus tachy, 1ECG- sinus tachy, 1stst, 2, 2ndnd or 3 or 3rdrd degree AV block (myocarditis), degree AV block (myocarditis), ST segment elevation, atrial ST segment elevation, atrial arrhythmiasarrhythmias

TreatmentTreatment Antibiotics- Oral penicillin remains drug of Antibiotics- Oral penicillin remains drug of choice- alternatives: IM Pen G, E-mycin, 1choice- alternatives: IM Pen G, E-mycin, 1stst gen Cephalosporin (do not use tetracyclines gen Cephalosporin (do not use tetracyclines or sulfonamides)or sulfonamides)

High dose aspirin and steroid to treat High dose aspirin and steroid to treat inflammation (cont to monitor C-RP and ESR inflammation (cont to monitor C-RP and ESR for effectiveness)for effectiveness)

Digoxin, diuretics, afterload reduction Digoxin, diuretics, afterload reduction depending on cardiac manifestationsdepending on cardiac manifestations

After acute phase symptoms have subsided, After acute phase symptoms have subsided, improved, may undergo cardiac surgery for improved, may undergo cardiac surgery for valve repair/replacement- if AV and/or MV valve repair/replacement- if AV and/or MV replaced with prosthetic, will require replaced with prosthetic, will require lifetime anticoagulation (Warfarin)lifetime anticoagulation (Warfarin)

What does this mean as What does this mean as FNP’sFNP’s

Recognizing and treating streptococcal Recognizing and treating streptococcal infections in a timely mannerinfections in a timely manner

Awareness of potential systemic symptoms Awareness of potential systemic symptoms related to untreated/delayed treatment of related to untreated/delayed treatment of a strep infection- these may not occur a strep infection- these may not occur until up to several weeks after the sore until up to several weeks after the sore throat has resolvedthroat has resolved

Only infections of the pharynx initiate Only infections of the pharynx initiate RF (in developed countries)RF (in developed countries)

Secondary prophylaxis of patients with Secondary prophylaxis of patients with history of RF- daily PCN or injections q history of RF- daily PCN or injections q 3-4 wks3-4 wks

SBE prophylaxis with antibiotics prior to SBE prophylaxis with antibiotics prior to dental and other proceduresdental and other procedures

Revised AHA guidelines for Revised AHA guidelines for Bacterial Endocarditis Bacterial Endocarditis

PreventionPrevention Previous guidelines recommended Previous guidelines recommended antibiotics prior to dental/surgical antibiotics prior to dental/surgical procedures for anyone with any type of procedures for anyone with any type of intracardiac abnormality (MVP, bicuspid intracardiac abnormality (MVP, bicuspid AV, etc)AV, etc)

Recent findings- bactremia is transient Recent findings- bactremia is transient and can occur with routine activities and can occur with routine activities (brushing teeth, flossing, chewing)(brushing teeth, flossing, chewing)

Only “high-risk” patients are Only “high-risk” patients are recommended to have antibiotic SBE recommended to have antibiotic SBE prophylaxisprophylaxis

““High risk” population High risk” population includes:includes:

Prosthetic heart valvesProsthetic heart valves Previous endocarditisPrevious endocarditis Unrepaired cyanotic defectUnrepaired cyanotic defect Palliative shunt/conduit presentPalliative shunt/conduit present Complete repair with prosthetic material for Complete repair with prosthetic material for first 6 months after procedurefirst 6 months after procedure

Heart transplantationHeart transplantation Recommended only for procedures which Recommended only for procedures which manipulates the tissue or oral mucosa manipulates the tissue or oral mucosa causing bleeding, etc. (not a routing causing bleeding, etc. (not a routing cleaning)cleaning)

Those not in “high risk” category are not at Those not in “high risk” category are not at zero risk- still slightly elevated compared zero risk- still slightly elevated compared to those without intracardiac abnormalitiesto those without intracardiac abnormalities

Other common complaintsOther common complaints

Chest pain- rarely attributed to any Chest pain- rarely attributed to any underlying cardiovascular disease. underlying cardiovascular disease. Coronary artery abnormalities in Coronary artery abnormalities in children rarely present with chest painchildren rarely present with chest pain

Syncope- very common in adolescents and Syncope- very common in adolescents and teenagers. Usually vasovagally teenagers. Usually vasovagally mediated and r/t hydration, eating mediated and r/t hydration, eating habits, etc. “Red flags”- syncope habits, etc. “Red flags”- syncope during strenuous activity, accompanied during strenuous activity, accompanied with palpitations, family history of with palpitations, family history of sudden death or arrhythmiassudden death or arrhythmias