Pediatric Acquired Heart Disease Dr Sagui Gavri Pediatric Cardiology Hadassah Hebrew University...

Pediatric Acquired Pediatric Acquired Heart DiseaseHeart Disease

Dr Sagui GavriDr Sagui Gavri

Pediatric CardiologyPediatric Cardiology

Hadassah Hebrew University Hadassah Hebrew University HospitalHospital

Pediatric Acquired Heart Pediatric Acquired Heart DiseaseDisease


3 y/o healthy male3 y/o healthy male Looks illLooks ill Prolonged High Fever > 39.5 CProlonged High Fever > 39.5 C Red RushRed Rush Bilateral ConjunctivitisBilateral Conjunctivitis

Kawasaki Disease - Kawasaki Disease - Mucocutaneous Lymph Node Mucocutaneous Lymph Node

SyndromeSyndrome

Kawasaki Disease - Kawasaki Disease - EpidemiologyEpidemiology

9/100000 for the white American 9/100000 for the white American populationpopulation

Boys : Girls – 1.5:1Boys : Girls – 1.5:1 80% under 5y and over 1 year80% under 5y and over 1 year Increase risk for coronary aneurism Increase risk for coronary aneurism

under 1y/o and over 8y/ounder 1y/o and over 8y/o Clusters in winter and spring.Clusters in winter and spring.

Kawasaki – Clinical Kawasaki – Clinical CriteriaCriteria

Kawasaki Disease –Kawasaki Disease –Stages of Cardiovascular Stages of Cardiovascular

PathologyPathology Stage 1 (0–9 days)Stage 1 (0–9 days)

Microvascular angiitisMicrovascular angiitis Acute endoarteritis and perivasculitis of major coronary arteries Acute endoarteritis and perivasculitis of major coronary arteries Pericarditis, valvulitis, and endocarditis Pericarditis, valvulitis, and endocarditis Myocarditis including atrioventricular conduction system Myocarditis including atrioventricular conduction system Causes of death: heart failure and dysrhythmiaCauses of death: heart failure and dysrhythmiaStage 2 (12–25 days)Stage 2 (12–25 days) Panvasculitis of major coronary arteries with aneurysms and Panvasculitis of major coronary arteries with aneurysms and thrombusthrombus

formationformation Intimal proliferation of coronary arteries Intimal proliferation of coronary arteries Myocarditis, endocarditis, and pericarditis Myocarditis, endocarditis, and pericarditis Causes of death: same as in stage 1; also myocardial infarction, Causes of death: same as in stage 1; also myocardial infarction, aneurysm aneurysm

ruptureruptureStage 3 (28–31 days)Stage 3 (28–31 days) Granulation of coronary arteries Granulation of coronary arteries Marked intimal thickening Marked intimal thickening Disappearance of microvascular angiitis Disappearance of microvascular angiitis Cause of death: myocardial infarction Cause of death: myocardial infarctionStage 4 (40 days to 4 years)Stage 4 (40 days to 4 years) Scarring, stenosis, calcification, and recanalization of major coronary Scarring, stenosis, calcification, and recanalization of major coronary

arteriesarteries Fibrosis of myocardium and endocardium Fibrosis of myocardium and endocardium Cause of death: myocardial infarctionCause of death: myocardial infarction

Kawasaki – Coronary Kawasaki – Coronary PathologyPathology

Kawasaki - TreatmentKawasaki - Treatment

Acute phase – High dose IVIG with Acute phase – High dose IVIG with high dose Aspirin (50-100 mg/kg)high dose Aspirin (50-100 mg/kg)

Subsequent treatment – Antiplatelet Subsequent treatment – Antiplatelet dose of Aspirin 3-5 mg/kg.dose of Aspirin 3-5 mg/kg.

Steroids – only in IVIG resistant Steroids – only in IVIG resistant cases.cases.

Anticoagulation - Warfarin if Anticoagulation - Warfarin if aneurismatic changes occur. aneurismatic changes occur.


7 y/o male7 y/o male Fever up to 38.8 cFever up to 38.8 c Right ankle and later left knee Right ankle and later left knee

arthritis.arthritis. New systolic murmurNew systolic murmur s/p Partially treated sterp A s/p Partially treated sterp A

tonsilitis 1 month ago.tonsilitis 1 month ago.


Acute phase reactant – ESR, CRPAcute phase reactant – ESR, CRP Evidence of recent Strp A Evidence of recent Strp A

infection – ASLO, throat culture, infection – ASLO, throat culture, rapid antigen test, Anti DNAase b.rapid antigen test, Anti DNAase b.

ECG – prolong PR intervalECG – prolong PR interval Echocardiography – Valvulitis, Echocardiography – Valvulitis,

Myo/pericarditis, Functional heart Myo/pericarditis, Functional heart assessment. assessment.

Rheumatic Fever – 1Rheumatic Fever – 1stst Deg AVBDeg AVB

Acute Rheumatic FeverAcute Rheumatic Fever

Most Common acquired heart Most Common acquired heart disease in developing countries disease in developing countries 300-500/100000.300-500/100000.

Rate in the Developed world Rate in the Developed world dropped to nearly o at the 1980’s dropped to nearly o at the 1980’s with improved life quality and with improved life quality and penicillin treatment and came up penicillin treatment and came up to 0.5-3/100000.to 0.5-3/100000.

Acute Rheumatic FeverAcute Rheumatic Fever

Patients 5-14 years consist of Patients 5-14 years consist of 72% of the cases.72% of the cases.

Mortality dropped from 8-30% to Mortality dropped from 8-30% to zero.zero.

“ “ Acute Rheumatic Fever licks the Acute Rheumatic Fever licks the joint and bites the heartjoint and bites the heart”.”.

Acute Rheumatic Fever – Acute Rheumatic Fever – Diagnostic CriteriaDiagnostic Criteria

60-90%

70%

10-30%

0-5%

0-5%

Acute Rheumatic Fever – Acute Rheumatic Fever – CarditisCarditis

Found in 60%-90% of casesFound in 60%-90% of cases Mainly ValvulitisMainly Valvulitis 30-70% long term morbidity30-70% long term morbidity Mitral Valve most commonly Mitral Valve most commonly

affectedaffected Aortic Valve more specific for Aortic Valve more specific for

diagnosis.diagnosis. Acute heart damage is not Acute heart damage is not

influenced by the treatment.influenced by the treatment.

Acute Rheumatic Fever – Acute Rheumatic Fever – ArthritisArthritis

2-5 weeks latent period s/p group 2-5 weeks latent period s/p group A streptococcus infection.A streptococcus infection.

Large joint migratory polyarthritisLarge joint migratory polyarthritis Rapid response to anti Rapid response to anti

inflammatory treatment.inflammatory treatment. No long term morbidity.No long term morbidity.

Acute Rheumatic Fever – Acute Rheumatic Fever – Sydenham Chorea Sydenham Chorea (st. Vitus (st. Vitus

Dance)Dance)

Inflammation involving the basal Inflammation involving the basal ganglia, cerebral cortex and ganglia, cerebral cortex and cerebellum.cerebellum.

Diagnostic as single criteria.Diagnostic as single criteria. Self limited disease.Self limited disease.

Acute Rheumatic Fever – Acute Rheumatic Fever – Subcutaneous NodulesSubcutaneous Nodules

Not pathognomonic (could appear Not pathognomonic (could appear in SLE, RA)in SLE, RA)

Last 1-10 days, associated with Last 1-10 days, associated with carditis.carditis.

Acute Rheumatic Fever – Acute Rheumatic Fever – Erythema MarginatumErythema Marginatum

Will appear in less then 5% of Will appear in less then 5% of cases.cases.

Associated with carditisAssociated with carditis

Acute Rheumatic Fever – Acute Rheumatic Fever – Primary TreatmentPrimary Treatment

10 days penicillin to eradicate GAS.10 days penicillin to eradicate GAS. High dose Aspirin (50-100 High dose Aspirin (50-100

mg/kg/day) until clinical and mg/kg/day) until clinical and laboratory evidence of laboratory evidence of inflammation resolve.inflammation resolve.

If severe carditis – Steroid If severe carditis – Steroid (prednisone 2mg/kg/day for 2 (prednisone 2mg/kg/day for 2 weeks and taper down)weeks and taper down)

Acute Rheumatic Fever – Acute Rheumatic Fever – Secondary ProphylaxisSecondary Prophylaxis

Benzathine penicillin GBenzathine penicillin G 1.2 million units intramuscularly every 3–4 weeks 1.2 million units intramuscularly every 3–4 weeks

OrOr Phenoxymethylpenicillin (penicillin V)Phenoxymethylpenicillin (penicillin V)

250 mg orally BID 250 mg orally BIDOrOrSulfadiazine Or sulfisoxazoleSulfadiazine Or sulfisoxazole

0.5 g orally daily for patients ≤27 kg 0.5 g orally daily for patients ≤27 kg 1 g orally daily for patients >27 kg 1 g orally daily for patients >27 kg

Penicillin- and sulfa-allergic patientsPenicillin- and sulfa-allergic patientsErythromycinErythromycin 250 mg orally BID 250 mg orally BID

Category DurationCategory Duration RHD (clinical or echo) ≥10 y since last episode RHD (clinical or echo) ≥10 y since last episode

and at least until age 40 y; possibly lifelongand at least until age 40 y; possibly lifelong RF with carditis, but no RHD 10 y or well into RF with carditis, but no RHD 10 y or well into

adulthoodaadulthooda RF without carditis 5 y or until age 21 yRF without carditis 5 y or until age 21 y


12 y/o healthy female12 y/o healthy female Fever up to 38.8 cFever up to 38.8 c Pallor, Weakness, Red urinePallor, Weakness, Red urine Right ankle and later left knee Right ankle and later left knee

arthralgia.arthralgia. New systolic murmur.New systolic murmur. Known small restrictive VSD.Known small restrictive VSD.


3/6 Systolic Murmur over the 3/6 Systolic Murmur over the precordium, radiating to the precordium, radiating to the axilla. axilla.

Splinter hemorrhages are seen at Splinter hemorrhages are seen at the tip of the nails.the tip of the nails.


Laboratory testLaboratory test CBC – Leukocytosis, AnemiaCBC – Leukocytosis, Anemia ESR, CRP – ElevatedESR, CRP – Elevated Blood Cultures – At least 3 Blood Cultures – At least 3

different sets over 24hdifferent sets over 24h HematuriaHematuria


Roth spots

Infective Endocarditis - Infective Endocarditis - EpidemiologyEpidemiology

0.3/100000 children/year.0.3/100000 children/year. Mortality 11.6%Mortality 11.6% Increase in number of cases with Increase in number of cases with

previous congenital heart disease previous congenital heart disease in the developed countries. (VSD, in the developed countries. (VSD, TOF, PDA, AS are the major)TOF, PDA, AS are the major)

Infective Endocarditis – Infective Endocarditis – Diagnostic Criteria - DukeDiagnostic Criteria - Duke

Definite infective endocarditis (IE):Definite infective endocarditis (IE): Pathologic criteria: Pathologic criteria:

– Micro-organisms demonstrated by culture or histologic Micro-organisms demonstrated by culture or histologic examination of a vegetation, a vegetation that has embolized, or examination of a vegetation, a vegetation that has embolized, or an intracardiac abscess specimen; or an intracardiac abscess specimen; or

– Pathological lesions; vegetation or intracardiac abscess Pathological lesions; vegetation or intracardiac abscess confirmed by histologic examination showing active endocarditis confirmed by histologic examination showing active endocarditis

Clinical criteriaClinical criteria– 2 major criteria; or 2 major criteria; or – 1 major criterion and 3 minor criteria; or 1 major criterion and 3 minor criteria; or – 5 minor criteria5 minor criteria

Possible IE: Possible IE: 1 major criterion and 1 minor criterion; or 1 major criterion and 1 minor criterion; or 3 minor criteria3 minor criteria Rejected IE:Rejected IE:

Firm alternative diagnosis explaining evidence of IE; orFirm alternative diagnosis explaining evidence of IE; or Resolution of IE syndrome with antibiotic therapy for ≤4 days; or Resolution of IE syndrome with antibiotic therapy for ≤4 days; or No pathologic evidence of IE at surgery or autopsy, with antibiotic No pathologic evidence of IE at surgery or autopsy, with antibiotic

therapy for ≤4 therapy for ≤4 days; or does not meet criteria for possible IE as abovedays; or does not meet criteria for possible IE as above


Major criteria Major criteria Blood culture positive for infective endocarditis (IE)Blood culture positive for infective endocarditis (IE)

– Typical micro-organisms consistent with IE from 2 separate blood Typical micro-organisms consistent with IE from 2 separate blood cultures:cultures:

Viridans streptococci, Viridans streptococci, Streptococcus bovisStreptococcus bovis, HACEK group, , HACEK group, Staphylococcus aureusStaphylococcus aureus; or ; or

Community-acquired enterococci in the absence of a primary focus; orCommunity-acquired enterococci in the absence of a primary focus; or– Micro-organisms consistent with IE from persistently positive blood Micro-organisms consistent with IE from persistently positive blood

cultures defined as followscultures defined as follows: : At least 2 positive cultures of blood samples drawn >12 h apart; or At least 2 positive cultures of blood samples drawn >12 h apart; or All of 3 or a majority of ≥4 separate cultures of blood (with first and last All of 3 or a majority of ≥4 separate cultures of blood (with first and last

sample drawn ≥1 h apart)sample drawn ≥1 h apart)– Single positive blood culture for Single positive blood culture for Coxiella burnetiiCoxiella burnetii or anti–phase-1 or anti–phase-1

IgG antibody titer >1:800IgG antibody titer >1:800Evidence of endocardial involvementEvidence of endocardial involvement

– Echocardiogram positive for IEEchocardiogram positive for IE (TEE recommended for patients with (TEE recommended for patients with prosthetic valves, rated at least “possible IE” by clinical criteria, or prosthetic valves, rated at least “possible IE” by clinical criteria, or complicated IE [paravalvular abscess]; TTE as first test in other patientscomplicated IE [paravalvular abscess]; TTE as first test in other patients)) defined as follows: defined as follows:

Oscillating intracardiac mass on valve or supporting structures, in the Oscillating intracardiac mass on valve or supporting structures, in the path of regurgitant jets, or on implanted material in the absence of an path of regurgitant jets, or on implanted material in the absence of an alternative anatomic explanation; or alternative anatomic explanation; or

Abscess; or Abscess; or New partial dehiscence of prosthetic valveNew partial dehiscence of prosthetic valve

– New valvular regurgitationNew valvular regurgitation (worsening or changing or pre-existing (worsening or changing or pre-existing murmur not sufficient)murmur not sufficient)


Minor criteriaMinor criteria Predisposition, predisposing heart condition, or Predisposition, predisposing heart condition, or

injection drug use injection drug use Fever, temperature >38°C Fever, temperature >38°C Vascular phenomena, major arterial emboli, septic Vascular phenomena, major arterial emboli, septic

pulmonary infarcts, mycotic aneurysm, intracranial pulmonary infarcts, mycotic aneurysm, intracranial hemorrhage, conjunctival hemorrhages, and Janeway hemorrhage, conjunctival hemorrhages, and Janeway lesions lesions

Immunologic phenomena: glomerulonephritis, Osler Immunologic phenomena: glomerulonephritis, Osler nodes, Roth spots, and rheumatoid factor nodes, Roth spots, and rheumatoid factor

Microbiologic evidence: positive blood culture, but Microbiologic evidence: positive blood culture, but does not meet a major criterion as noted above or does not meet a major criterion as noted above or serologic evidence of active infection with organism serologic evidence of active infection with organism consistent with IE consistent with IE

Echocardiographic minor criteria eliminatedEchocardiographic minor criteria eliminated

Infective Endocarditis – Infective Endocarditis – Etiologic AgentsEtiologic Agents

Agent Frequency Agent Frequency Streptococci Streptococci α-Hemolytic Most common α-Hemolytic Most common β-Hemolytic Uncommon β-Hemolytic Uncommon Enterococci Rare Enterococci Rare Pneumococci Rare Pneumococci Rare Others Uncommon Others Uncommon Staphylococci Staphylococci S. aureus Second most common S. aureus Second most common Coagulase-negative Uncommon, but increasing Coagulase-negative Uncommon, but increasing Gram-negative agents Gram-negative agents Enterics Rare Enterics Rare Pseudomonas species Rare Pseudomonas species Rare HACEKa Rare HACEKa Rare Neisseria species Rare Neisseria species Rare Fungi Fungi Candida species Uncommon Candida species Uncommon Others Rare Others Rare

Infective Endocarditis – Infective Endocarditis – TreatmentTreatment

Prolong antibiotic treatment – 4-6 wProlong antibiotic treatment – 4-6 w Bactericidal rather than Bactericidal rather than

bacteriostatic.bacteriostatic. Parenteral treatment.Parenteral treatment. Consider surgical treatment for :Consider surgical treatment for :

a. Significant embolic eventsa. Significant embolic events

b. Progressive heart failureb. Progressive heart failure

c. Failure of antibiotic treatmentc. Failure of antibiotic treatment

Infective Endocarditis – Infective Endocarditis – TreatmentTreatment

Start empiric treatment with wide Start empiric treatment with wide range antibiotic.range antibiotic.

Change antibiotic coverage by Change antibiotic coverage by blood culture and sensitivity of blood culture and sensitivity of the organism the organism

Infective Endocarditis – Infective Endocarditis – Treatment Native Valve - Treatment Native Valve -

StrepStrepHighly penicillin-susceptible viridans group streptococci and Highly penicillin-susceptible viridans group streptococci and Streptococcus Streptococcus

bovisbovis (MIC ≤0.12 µg/mL) (MIC ≤0.12 µg/mL) Regimen Dosagea Route Duration, Regimen Dosagea Route Duration,

weeksweeks Aqueous crystalline penicillin G 200,000 U/kg per 24 h IV in 4–6 doses 4Aqueous crystalline penicillin G 200,000 U/kg per 24 h IV in 4–6 doses 4 sodiumsodium OrOr Ceftriaxone sodium 100 mg/kg per 24 h IV/IM in 1 dose 4 Ceftriaxone sodium 100 mg/kg per 24 h IV/IM in 1 dose 4 Aqueous crystalline penicillin G 200,000 U/kg per 24 h IV in 4–6 doses 2Aqueous crystalline penicillin G 200,000 U/kg per 24 h IV in 4–6 doses 2 sodiumsodium OrOr Ceftriaxone sodium 100 mg/kg per 24 h IV/IM in 1 dose 2Ceftriaxone sodium 100 mg/kg per 24 h IV/IM in 1 dose 2Plus Plus Gentamicin sulfatec 3 mg/kg per 24 h IV/IM in 3 doses 2Gentamicin sulfatec 3 mg/kg per 24 h IV/IM in 3 doses 2 Vancomycin hydrochlorided 40 mg/kg per 24 h IV in 2–3 doses 4 Vancomycin hydrochlorided 40 mg/kg per 24 h IV in 2–3 doses 4 Strains of viridans group streptococci and Strains of viridans group streptococci and S. bovisS. bovis relatively resistant to relatively resistant to

penicillin (MIC >0.12 to ≤0.5 µg/mLpenicillin (MIC >0.12 to ≤0.5 µg/mL)) Regimen Dosagea Route Duration, Regimen Dosagea Route Duration,

weeksweeks Aqueous crystalline penicillin G 300,000 U/24 h IV in 4–6 doses 4Aqueous crystalline penicillin G 300,000 U/24 h IV in 4–6 doses 4 SodiumSodium OrOr Ceftriaxone sodium 100 mg/kg per 24 h IV/IM in 1 dose 4Ceftriaxone sodium 100 mg/kg per 24 h IV/IM in 1 dose 4PlusPlus Gentamicin sulfatec 3 mg/kg per 24 h IV/IM in 3 doses 2Gentamicin sulfatec 3 mg/kg per 24 h IV/IM in 3 doses 2 Vancomycin hydrochlorided 40 mg/kg 24 h IV in 2 or 3 doses 4Vancomycin hydrochlorided 40 mg/kg 24 h IV in 2 or 3 doses 4

Pediatric Acquired Heart Pediatric Acquired Heart Disease - SummeryDisease - Summery

Less Common then congenital Less Common then congenital heart disease.heart disease.

Variable clinical appearanceVariable clinical appearance High index of suspicionHigh index of suspicion Early treatment can change the Early treatment can change the

outcome.outcome.

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