Pediatric Acquired Heart Disease

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Pediatric Acquired Heart Disease

• Dr Sagui Gavri
• Pediatric Cardiology
• Hadassah Hebrew University Hospital

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Pediatric Acquired Heart Disease
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Pediatric Acquired Heart Disease

• 3 y/o healthy male
• Looks ill
• Prolonged High Fever gt 39.5 C
• Red Rush
• Bilateral Conjunctivitis

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Pediatric Acquired Heart Disease
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Pediatric Acquired Heart Disease
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Pediatric Acquired Heart Disease
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Pediatric Acquired Heart Disease
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Pediatric Acquired Heart Disease
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Pediatric Acquired Heart Disease
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Kawasaki Disease - Mucocutaneous Lymph Node
Syndrome
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Kawasaki Disease - Epidemiology

• 9/100000 for the white American population
• Boys Girls 1.51
• 80 under 5y and over 1 year
• Increase risk for coronary aneurism under 1y/o
  and over 8y/o
• Clusters in winter and spring.

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Kawasaki Clinical Criteria
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Kawasaki Disease Stages of Cardiovascular
Pathology

• Stage 1 (09 days)   Microvascular
  angiitis   Acute endoarteritis and
  perivasculitis of major coronary
  arteries   Pericarditis, valvulitis, and
  endocarditis   Myocarditis including
  atrioventricular conduction system   Causes of
  death heart failure and dysrhythmiaStage 2
  (1225 days)   Panvasculitis of major coronary
  arteries with aneurysms and thrombus
• formation   Intimal proliferation of
  coronary arteries   Myocarditis, endocarditis,
  and pericarditis   Causes of death same as in
  stage 1 also myocardial infarction, aneurysm
• ruptureStage 3 (2831
  days)   Granulation of coronary
  arteries   Marked intimal thickening   Disappear
  ance of microvascular angiitis   Cause of death
  myocardial infarctionStage 4 (40 days to 4
  years)   Scarring, stenosis, calcification, and
  recanalization of major coronary
• arteries   Fibrosis of myocardium and
  endocardium   Cause of death myocardial
  infarction

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Kawasaki Coronary Pathology
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Kawasaki - Treatment

• Acute phase High dose IVIG with high dose
  Aspirin (50-100 mg/kg)
• Subsequent treatment Antiplatelet dose of
  Aspirin 3-5 mg/kg.
• Steroids only in IVIG resistant cases.
• Anticoagulation - Warfarin if aneurismatic
  changes occur.

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Pediatric Acquired Heart Disease

• 7 y/o male
• Fever up to 38.8 c
• Right ankle and later left knee arthritis.
• New systolic murmur
• s/p Partially treated sterp A tonsilitis 1 month
  ago.

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Pediatric Acquired Heart Disease
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Pediatric Acquired Heart Disease
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Pediatric Acquired Heart Disease

• Acute phase reactant ESR, CRP
• Evidence of recent Strp A infection ASLO,
  throat culture, rapid antigen test, Anti DNAase
  b.
• ECG prolong PR interval
• Echocardiography Valvulitis, Myo/pericarditis,
  Functional heart assessment.

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Rheumatic Fever 1st Deg AVB
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Acute Rheumatic Fever

• Most Common acquired heart disease in developing
  countries 300-500/100000.
• Rate in the Developed world dropped to nearly o
  at the 1980s with improved life quality and
  penicillin treatment and came up to 0.5-3/100000.

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Acute Rheumatic Fever

• Patients 5-14 years consist of 72 of the cases.
• Mortality dropped from 8-30 to zero.
• Acute Rheumatic Fever licks the joint and bites
  the heart.

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Acute Rheumatic Fever Diagnostic Criteria
60-90
70
10-30
0-5
0-5
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Acute Rheumatic Fever Carditis

• Found in 60-90 of cases
• Mainly Valvulitis
• 30-70 long term morbidity
• Mitral Valve most commonly affected
• Aortic Valve more specific for diagnosis.
• Acute heart damage is not influenced by the
  treatment.

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Acute Rheumatic Fever Arthritis

• 2-5 weeks latent period s/p group A streptococcus
  infection.
• Large joint migratory polyarthritis
• Rapid response to anti inflammatory treatment.
• No long term morbidity.

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Acute Rheumatic Fever Sydenham Chorea (st.
Vitus Dance)

• Inflammation involving the basal ganglia,
  cerebral cortex and cerebellum.
• Diagnostic as single criteria.
• Self limited disease.

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Acute Rheumatic Fever Subcutaneous Nodules

• Not pathognomonic (could appear in SLE, RA)
• Last 1-10 days, associated with carditis.

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Acute Rheumatic Fever Erythema Marginatum

• Will appear in less then 5 of cases.
• Associated with carditis

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Acute Rheumatic Fever Primary Treatment

• 10 days penicillin to eradicate GAS.
• High dose Aspirin (50-100 mg/kg/day) until
  clinical and laboratory evidence of inflammation
  resolve.
• If severe carditis Steroid (prednisone
  2mg/kg/day for 2 weeks and taper down)

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Acute Rheumatic Fever Secondary Prophylaxis

• Benzathine penicillin G   1.2 million units
  intramuscularly every 34 weeks
• Or
• Phenoxymethylpenicillin (penicillin V)   250
  mg orally BID
• Or
• Sulfadiazine Or sulfisoxazole   0.5 g orally
  daily for patients 27 kg   1 g orally daily for
  patients gt27 kg
• Penicillin- and sulfa-allergic patientsErythromyc
  in   250 mg orally BID
• Category Duration
• RHD (clinical or echo) 10 y since
  last episode and at least until age 40 y
  possibly lifelong
• RF with carditis, but no RHD 10 y or well
  into adulthooda
• RF without carditis 5 y or
  until age 21 y

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Pediatric Acquired Heart Disease

• 12 y/o healthy female
• Fever up to 38.8 c
• Pallor, Weakness, Red urine
• Right ankle and later left knee arthralgia.
• New systolic murmur.
• Known small restrictive VSD.

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Pediatric Acquired Heart Disease
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Pediatric Acquired Heart Disease

• 3/6 Systolic Murmur over the precordium,
  radiating to the axilla.
• Splinter hemorrhages are seen at the tip of the
  nails.

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Pediatric Acquired Heart Disease
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Pediatric Acquired Heart Disease

• Laboratory test
• CBC Leukocytosis, Anemia
• ESR, CRP Elevated
• Blood Cultures At least 3 different sets over
  24h
• Hematuria

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Pediatric Acquired Heart Disease
Roth spots
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Pediatric Acquired Heart Disease
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Infective Endocarditis - Epidemiology

• 0.3/100000 children/year.
• Mortality 11.6
• Increase in number of cases with previous
  congenital heart disease in the developed
  countries. (VSD, TOF, PDA, AS are the major)

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Infective Endocarditis Diagnostic Criteria -
Duke

• Definite infective endocarditis (IE)
• Pathologic criteria
• Micro-organisms demonstrated by culture or
  histologic examination of a vegetation, a
  vegetation that has embolized, or an intracardiac
  abscess specimen or
• Pathological lesions vegetation or intracardiac
  abscess confirmed by histologic examination
  showing active endocarditis
• Clinical criteria
• 2 major criteria or
• 1 major criterion and 3 minor criteria or
• 5 minor criteria
• Possible IE
• 1 major criterion and 1 minor criterion or
• 3 minor criteria
• Rejected IE
• Firm alternative diagnosis explaining evidence of
  IE or
• Resolution of IE syndrome with antibiotic therapy
  for 4 days or
• No pathologic evidence of IE at surgery or
  autopsy, with antibiotic therapy for 4
• days or does not meet criteria for possible IE
  as above

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Infective Endocarditis Diagnostic Criteria -
Duke

• Major criteria
• Blood culture positive for infective endocarditis
  (IE)
• Typical micro-organisms consistent with IE from 2
  separate blood cultures
• Viridans streptococci, Streptococcus bovis, HACEK
  group, Staphylococcus aureus or
• Community-acquired enterococci in the absence of
  a primary focus or
• Micro-organisms consistent with IE from
  persistently positive blood cultures defined as
  follows
• At least 2 positive cultures of blood samples
  drawn gt12 h apart or
• All of 3 or a majority of 4 separate cultures of
  blood (with first and last sample drawn 1 h
  apart)
• Single positive blood culture for Coxiella
  burnetii or antiphase-1 IgG antibody titer
  gt1800
• Evidence of endocardial involvement
• Echocardiogram positive for IE (TEE recommended
  for patients with prosthetic valves, rated at
  least possible IE by clinical criteria, or
  complicated IE paravalvular abscess TTE as
  first test in other patients) defined as follows
  
• Oscillating intracardiac mass on valve or
  supporting structures, in the path of regurgitant
  jets, or on implanted material in the absence of
  an alternative anatomic explanation or
• Abscess or
• New partial dehiscence of prosthetic valve
• New valvular regurgitation (worsening or changing
  or pre-existing murmur not sufficient)

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Infective Endocarditis Diagnostic Criteria -
Duke

• Minor
  criteria
• Predisposition, predisposing heart condition, or
  injection drug use
• Fever, temperature gt38C
• Vascular phenomena, major arterial emboli, septic
  pulmonary infarcts, mycotic aneurysm,
  intracranial hemorrhage, conjunctival
  hemorrhages, and Janeway lesions
• Immunologic phenomena glomerulonephritis, Osler
  nodes, Roth spots, and rheumatoid factor
• Microbiologic evidence positive blood culture,
  but does not meet a major criterion as noted
  above or serologic evidence of active infection
  with organism consistent with IE
• Echocardiographic minor criteria eliminated

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Infective Endocarditis Etiologic Agents

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

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Infective Endocarditis Treatment

• Prolong antibiotic treatment 4-6 w
• Bactericidal rather than bacteriostatic.
• Parenteral treatment.
• Consider surgical treatment for
• a. Significant embolic events
• b. Progressive heart failure
• c. Failure of antibiotic treatment

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Infective Endocarditis Treatment

• Start empiric treatment with wide range
  antibiotic.
• Change antibiotic coverage by blood culture and
  sensitivity of the organism

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Infective Endocarditis Treatment Native Valve -
Strep

• Highly penicillin-susceptible viridans group
  streptococci and Streptococcus bovis (MIC 0.12
  µg/mL)
• Regimen Dosagea
  Route Duration,
  weeks
• Aqueous crystalline penicillin G 200,000 U/kg
  per 24 h IV in 46 doses 4
• sodium
• Or
• 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 46 doses
  2
• sodium
• Or
• Ceftriaxone sodium 100
  mg/kg per 24 h IV/IM in 1 dose
  2
• Plus Gentamicin sulfatec 3
  mg/kg per 24 h IV/IM in 3 doses
  2
• Vancomycin hydrochlorided 40 mg/kg
  per 24 h IV in 23 doses 4
  
• Strains of viridans group streptococci and S.
  bovis relatively resistant to penicillin (MIC
  gt0.12 to 0.5 µg/mL)
• Regimen Dosagea
  Route Duration, weeks
• Aqueous crystalline penicillin G 300,000 U/24
  h IV in 46 doses 4
• Sodium
• Or
• Ceftriaxone sodium 100 mg/kg
  per 24 h IV/IM in 1 dose 4
• Plus Gentamicin sulfatec 3
  mg/kg per 24 h IV/IM in 3 doses
  2

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Pediatric Acquired Heart Disease - Summery

• Less Common then congenital heart disease.
• Variable clinical appearance
• High index of suspicion
• Early treatment can change the outcome.
• THANK YOU