KAWASAKI DISEASE (MUCOCUTANEOUS LYMPH NODE SYNDROME) 10-15 per 100,000 children < 5 years in USA 150 per 100,000 children of Japanese descent ACUTE RHEUMATIC FEVER / RHEUMATIC HEART DISEASE 0.5-3 per 100,000 population in developed countries 200-300 per 100,000 in developing countries MYOCARDITIS / PERICARDITIS BACTERIAL ENDOCARDITIS CARADIOMYOPATHY CARDIAC TUMOR

1. PEDIATRIC ACQUIRED HEART DISEASES
• KAWASAKI DISEASE (MUCOCUTANEOUS LYMPH NODE
SYNDROME) 10-15 per 100,000 children < 5 years in USA
150 per 100,000 children of Japanese descent
• ACUTE RHEUMATIC FEVER / RHEUMATIC HEART DISEASE
0.5-3 per 100,000 population in developed
countries 200-300 per 100,000 in developing
countries
• MYOCARDITIS / PERICARDITIS
• BACTERIAL ENDOCARDITIS
• CARADIOMYOPATHY
• CARDIAC TUMOR
W. P. Rivindu H. Wickramanayake
Group no. 04a
6th Year 1st Semester – 2020 April
Tbilisi State Medical University, Georgia

2. Kawasaki Disease
– #1 cause of acquired heart disease in kids
– Systemic inflammatory process (vasculitis) with no
known etiology
– May be infectious etiology: cycles of 3 yrs; usually winter
and spring; usually younger ages (most < 4 yrs old)
• Diagnoses:
– Fever > 5 days
– At least 4 of the following:
• Changes in the extremities
– Erythema and edema of hands and feet
– Subsequent peeling of distal ends of digits
• Polymorphous rash  Desquamating rash
• Nonpurulent bilateral conjunctivitis
• Mucosal changes
– Strawberry tongue; red, cracked lips
• Cervical lymph node (1.5cm in diameter)

3. 1. Skin Rashes
2. Conjunctivitis
3. Stomatitis
4. Hand & Feet
Changes
5. Cervical
adenopathy
Nonpurulent
Conjunctivitis
Erythematous Induration
>/= 1.5 cm
Strawberry tongue

4. SKIN RASHES

5. DESQUAMATION
Second to fourth weeks

11. Newburger, J. W. et al. Pediatrics 2004;114:1708-1733
2D echocardiogram
 Coronary angiogram demonstrating giant
aneurysm of the LAD with obstruction and
giant aneurysm of the RCA with area of severe
narrowing in 6-year-old boy
RISK SCORES FOR
CORONARY ANEURYSM

12. 1. WBC > 12,000
2. Platelet < 350,000
3. CRP > 3+
4. Hct < 35
5. Albumin < 3.5 g/dL
6. Age </= 12 months
7. Male sex
Baseline WBC, Hb, Platelet
Temperature post IVIG within 1 day
 HARADA SCORE – is used to determine whether IVIG
treatment will be useful
 IVIG is given to children who fulfill 4 of the following
criteria assessed within 9 days of onset of illness.

15. • Prognosis
– 1/2 to 2/3 of aneurysms will “resolve” by 1-2
yrs post disease onset
• Positive factors for regression:
– Small (giant aneurysms (>8mm in diameter) have worst
prognosis)
– Fusiform (saccular and “beads on a string” have worse
prognosis)
– < 1 yr of age at time of disease onset
– Aneurysm in a distal coronary segment
– Myocardial dysfunction resolves post treatment
(unless ischemic damage)
• No correlation between severity of myocarditis and risk for
coronary aneurysms
– Peak mortality: 15-45 days post fever onset
• Myocardial infarction
– Recurrence rate: ~3% (Japan)

16. Acute Rheumatic Fever
–A pathological immune mediated
inflammatory disorder of the heart, brain,
joints, and skin after group A Strep throat
infection
–More common in underdeveloped countries
–How to diagnose:
• Evidence for group A Strep throat infection and
2 major, or 1 major and 2 minor, criteria

17. • Diagnostic criteria (major)
–Joints - severe polyarthritis, responds well to ASA
–O (Heart) - carditis; often MR, MR/AI
–Nodes - subcutaneous nodules (hard and
painless) on extensor surfaces
–Erythema marginatum – evanescent,
comes and goes, on trunk
–Sydenham’s chorea – neuropsychiatric
disorder
Jones, Davey; circa 1970

18. • Diagnostic criteria (minor)
– Arthralgia
– Fever
– Elevated acute phase reactants (ESR, CRP)
– Prolonged PR
ERYTHEMA MARGINATUM

19. SUBCUTANEOUS
NODULES

20. • Treatment:
– Benzathine PCN—knocks out strep
– ASA—relieves arthritis, helps mild to moderate
carditis
– Prednisone—only for severe carditis
– Support if CHF
– ABX prophylaxis (Penicillin Prophylaxis)

21. Myocarditis
• What is it?
–Inflammed myocardium
• Etiology?
–Viral
• Enteroviruses, esp. Coxsackie B
• Adenoviruses
• Others: Flu, HSV, Parvo, CMV, HCV, EBV,
Mumps, Rubella, Varicella, HIV, RSV…
–Bacterial, Rickettsial, Fungal, Parasitic
–Other Noninfectious Inflammatory Diseases
• SLE, Kawasaki, Rheumatic Fever…

22. • Presentation:
– Clinically, symptoms and signs similar to dilated
cardiomyopathy (CHF)
– ECG: low voltages, arrhythmias (any type), ST and T wave
changes, + wide Q waves
– CXR: cardiomegaly, pulmonary edema
– Echo: dilated and poorly functioning ventricles; often
occurs with pericardial effusion

23. • Treatment
–Support to relieve CHF and to maintain
good cardiac output
• Don’t aggressively load Digoxin in acute
inflammatory stage (hypersensitivity)
–Treat arrhythmias
• Lidocaine or amiodarone for ventricular
arrhythmias (may require cardioversion if
unstable)
• Complete heart block: pace
–Treat etiology, if known
–No proven benefit of immunosuppresives
–If irreversible damage to myocardium,
may need surgical intervention

24. Dilated Cardiomyopathy
• Large, thin-walled
chamber
• Poor systolic function
• Etiologies:
– Idiopathic (50%)
– Infectious (viral, esp.
HIV)
– Drugs (anthracyclines)
– Ischemic
– Poor nutrition (low
carnitine, selenium,
thiamine)

25. • What’s the big deal with DCMP?
– Some types are reversible (improve w/ time and/or Rx), but
most are progressive
– 5 yr survival as low as 20-80%
– Death due to intractable CHF, ventricular arrhythmias
• Common medical therapies
– Goal: improve ventricular function/efficacy and reduce
pulmonary venous congestion
– Digoxin: inotrope, improves systolic function and helps reduce end-
diastolic pressure
– Enalapril: ACE-I, reduces afterload
– Lasix(Furosemide) &/or Aldactone: diuretic, relieves pulmonary
congestion
– Carvedilol: beta-blocker, alpha-blocker, antioxidant, improves
survival
– Warfarin: anticoagulant, prevents thromboemboli
• Invasive therapies
– ICD: defibrillator, useful w/ ventricular arrhythmias
– Transplant: last option

26. Etiologies of Pericarditis
• Purulent
– #1 Staph aureus
– #2 H flu B
– Others: Neisseria,
Pseudomonas,
Salmonella, Listeria,
Pasteurella, E. coli,
Brucella, Yersinia,
Legionella,
Campylobacter
– ABX and drain
(surgical)
– 25-75% mortality
• Viral
– Coxsackie, ECHO,
Adeno, Flu, Mumps,
Varicella, EBV, HIV
– Many WBC’s in fluid
– Supportive care and
antiinflammatories
– Up to 15% relapse
UREMIA
MYXEDEMA

27. • Drug induced
– Lupus-like syndrome
• Hydralazine
• Isoniazid
• Procainamide
– 33% of those who
develop
+ANA will develop-
lupus like syndrome
– Treat w/
antiinflammatories
and stop drug
• Post-pericardiotmy
syndrome
– Unclear mechanism
– Less likely in kids <2y/o
– Typically:
• Fever, irritability,
chest pain,
malaise, poor
appetite
• Presents 1 wk post
surgery (can be
many months)
– Treat w/ ASA (or
other
antiinflammatory)

28. • TB
– Usually secondary to
direct spread or
hematogenous spread
– Fluid shows mostly
Lymphocytes
– Cx takes up to 6 wks
– 15-42% show acid-fast
bacilli in fluid
– Fluid adenosine
deaminase level >50 U/L
– Treat w/ INH,
Pyrazinamide, Rifampin,
Streptomycin +/- steroids
initially
– 35% may develop
constrictive pericarditis
• Connective tissue
diseases
– Up to 50% of kids w/
JRA
– Treat with NSAIDS
– Up to 50% of kids w/
SLE
– Fluid may show low
complement levels,
+ANA, or
+Rheumatoid factor

29. Clinical Presentation
• Dull chest pain, increases with lying
supine
• + rub
• Muffled heart sounds
• Pulsus paradoxus if tamponade
• ECG: low voltages, may have ST changes
• CXR: cardiomegaly, often no pulm
congestion
• ECHO: pericardial effusion

30. Pulsus Paradoxus
• SBP drop >10 mmHg with
inspiration
• Mechanism in tamponade:
– Inspiration increases venous
return to RA/RV
– Delayed venous return to LV
and leftward displacement of
IVS decreases LV filling
(preload)
– Diminished SV
– SBP drops
• Neurohormonal
compensation for lower CO:
– Increased sympathetic
tone and catechol
release
– Elevated HR, increased
contractility,
vasoconstriction

31. Pericarditis: typical ECG

32. ECG of Large Pericardial Effusion
Goldberger: Clinical Electrocardiography: A Simplified Approach, 6th ed., 1999

33. Radiography
• Cardiac silhouette
– May be normal if
acute
– Enlarged, “water-
bottle”, triangular
• Usually no pulm.
vasc.
congestion/edema
Yale, 2004 (http://info.med.yale.edu/intmed/cardio/imaging/
cases/pericardial_effusion/)

34. Echocardiography
• Presence and relative size of effusion
• Chamber dimensions and wall dynamics (compression)
– Early diastole: RV free wall compression
– Late diastole: RA compression
– LA compression—very specific for tamponade
– Swinging heart—large effusion
• May be limited by quality of imaging windows and location of
fluid (if loculated)

35. Assessing Hemodynamic
Significance
• Insignificant pericardial
effusion
– No JVD (JVP <7mmHg)
– Nml VS’s and good perfusion
– No compression on echo
• Significant, but compensated
– JVD/elevated JVP (8-12mmHg)
– Nml HR and BP, good
perfusion
– Mild pulsus paradoxus (<20
mmHg)
– Mild compression of RA &/or
RV
• Severe with maximum compensation
– JVD/elevated JVP (>15mmHg)
– Elevated HR, poor perfusion
(vasoconstriction)
– Nml BP
– Significant pulsus paradoxus (>20
mmHg)
– Significant chamber collapse
• Severe and decompensated
– JVD/elevated JVP (>20mmHg)
– Elevated HR, RR
– Poor perfusion
– Low BP, palpable pulsus paradoxus
– Chamber collapse/swinging heart
Modified from Goldstein, Current Prob Cardio, 2004; 29(9)

36. • Treatment
–Treat underlying disorder (uremia, etc.)
–If idiopathic or viral, Acetyl Salicylic Acid
–If tamponade, Pericardiocentesis
–If purulent, Surgical Drainage/Antibiotics

37. Infective Endocarditis
– Seeding of bacteria and inflamatory response
within the endocardial layer of the heart
– Occurs when
• 1) pt is bacteremic and
• 2) pt has intracardiac structural abnormality
• Clinical findings:
– MURMUR
– Fever
– Emboli (skin, eye, nails, lungs, brain, kidney, etc.)

38. Janeway lesion
Splinter hemorrhage,
Osler nodes

39. • Pathogens:
– Strep viridans (bad teeth)
– Staph aureus (post-operative cardiac pts)
– Enterococci (GU/GI procedures)
– HACEK (neonates, immunocompromised pts):
Haemophilus, Actinobacillus, Cardiobacterium,
Eikenella, Kingella)
– Fungal
– (Pseudomonas or serratia
(IV drug users))
• Treatments:
– PCN
– Oxacillin or Vanc
– Amp/PCN and Gent
– CTX(Ceftrixone)
– Ampho B

40. Questions
1. Kawasaki disease is more common in which season in northern hemisphere?
a. Winter
b. Summer
c. Rainy season
d. No season variation
2. Kawasaki Disease is considered resistant to IVIG when the fever does subside in _______ hours
after completion of IVIG
a. 24 hours post IVIG completion
b. 36 hours post IVIG completion
c. 48 hours post IVIG completion
d. 72 hours post IVIG completion
3. Which is the most common differential diagnosis of Kawasaki Disease?
a. Chikungunya fever
b. SLE
c. Leptospirosis
d. Measles
4. Which is the most common coronary artery involved in Kawasaki Disease?
a. Left Circumflex Artery
b. Distal Right Coronary Artery
c. Left Anterior Descending Artery
d. Distal Left Main Coronary Artery
5. Which part of heart is involved in KD?
a. Pericardium
b. Myocardium
c. Endocardium
d. All

41. 6. Rheumatic Fever usually follows an infection with:
a. Streptococcus group A
b. Staphylococcus aureus
c. Streptococcus viridans
d. Hemophelus influenza
7. To diagnose a patient with R.F, The patient should have at least:
a. One major, two minor
b. Two major , one minor
c. One major , one minor
d. Two major , two minor
8. A patient with R.F comes with dancing movement, how can you explain this movement:
a. Myopathy
b. Neuromuscular junction disease
c. Sydenham's chorea
d. Psychosis
9. Which of the following is one of Jone’s major criteria?
a. Pancarditis
b. Fever
c. Elevated ESR
d. Previous RF
10. Acute and sub-acute endocarditis are caused by which bacteria respectively?
a. S. Pyogenes, S. Aureus
b. S. Viridans, S Aureus
c. S. Aureus, S. viridans
d. S. Pyogenes, S. viridans