Pediatric Acquired Heart Disease

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

2. Pediatric Acquired Heart Disease

3. Pediatric Acquired Heart Disease • 3 y/o healthy male • Looks ill • Prolonged High Fever > 39.5 C • Red Rush • Bilateral Conjunctivitis

4. Pediatric Acquired Heart Disease

5. Pediatric Acquired Heart Disease

6. Pediatric Acquired Heart Disease

7. Pediatric Acquired Heart Disease

8. Pediatric Acquired Heart Disease

9. Pediatric Acquired Heart Disease

10. Kawasaki Disease - Mucocutaneous Lymph Node Syndrome

11. Kawasaki Disease - Epidemiology • 9/100000 for the white American population • Boys : Girls – 1.5:1 • 80% under 5y and over 1 year • Increase risk for coronary aneurism under 1y/o and over 8y/o • Clusters in winter and spring.

12. Kawasaki – Clinical Criteria

13. Kawasaki Disease –Stages of Cardiovascular Pathology Stage 1 (0–9 days)Microvascular angiitis   Acute endoarteritis and perivasculitis of major coronary arteries   Pericarditis, valvulitis, and endocarditis   Myocarditis including atrioventricular conduction systemCauses of death: heart failure and dysrhythmiaStage 2 (12–25 days)Panvasculitis of major coronary arteries with aneurysms and thrombus formation   Intimal proliferation of coronary arteries   Myocarditis, endocarditis, and pericarditisCauses of death: same as in stage 1; also myocardial infarction, aneurysm ruptureStage 3 (28–31 days) Granulation of coronary arteries   Marked intimal thickening   Disappearance 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 endocardiumCause of death: myocardial infarction

14. Kawasaki – Coronary Pathology

15. 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.

16. 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.

17. Pediatric Acquired Heart Disease

18. Pediatric Acquired Heart Disease

19. 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.

20. Rheumatic Fever – 1st Deg AVB

21. 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 1980’s with improved life quality and penicillin treatment and came up to 0.5-3/100000.

22. 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”.

23. Acute Rheumatic Fever – Diagnostic Criteria 60-90% 70% 10-30% 0-5% 0-5%

24. 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.

25. 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.

26. Acute Rheumatic Fever – Sydenham Chorea (st. Vitus Dance) • Inflammation involving the basal ganglia, cerebral cortex and cerebellum. • Diagnostic as single criteria. • Self limited disease.

27. Acute Rheumatic Fever – Subcutaneous Nodules • Not pathognomonic (could appear in SLE, RA) • Last 1-10 days, associated with carditis.

28. Acute Rheumatic Fever – Erythema Marginatum • Will appear in less then 5% of cases. • Associated with carditis

29. 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)

30. Acute Rheumatic Fever – Secondary Prophylaxis Benzathine penicillin G   1.2 million units intramuscularly every 3–4 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 >27 kg Penicillin- and sulfa-allergic patientsErythromycin   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

31. 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.

32. Pediatric Acquired Heart Disease

33. 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.

34. Pediatric Acquired Heart Disease

35. Pediatric Acquired Heart Disease • Laboratory test • CBC – Leukocytosis, Anemia • ESR, CRP – Elevated • Blood Cultures – At least 3 different sets over 24h • Hematuria

36. Pediatric Acquired Heart Disease Roth spots

37. Pediatric Acquired Heart Disease

38. 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)

39. 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

40. 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 >12 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 anti–phase-1 IgG antibody titer >1:800 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)

41. Infective Endocarditis – Diagnostic Criteria - Duke Minor criteria • Predisposition, predisposing heart condition, or injection drug use • Fever, temperature >38°C • 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

42. Infective Endocarditis – Etiologic Agents Agent Frequency Streptococci • α-Hemolytic Most common • β-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

43. 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

44. Infective Endocarditis – Treatment • Start empiric treatment with wide range antibiotic. • Change antibiotic coverage by blood culture and sensitivity of the organism

45. 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 4–6 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 4–6 doses 2 sodium • Or Ceftriaxone sodium 100 mg/kg per 24 h IV/IM in 1 dose 2 Plus Gentamicinsulfatec 3 mg/kg per 24 h IV/IM in 3 doses 2 Vancomycinhydrochlorided 40 mg/kg per 24 h IV in 2–3 doses 4 Strains of viridans group streptococci and S. bovis relatively resistant to penicillin (MIC >0.12 to ≤0.5 µg/mL) • Regimen Dosagea Route Duration, weeks • Aqueous crystalline penicillin G 300,000 U/24 h IV in 4–6 doses 4 Sodium • Or • Ceftriaxone sodium 100 mg/kg per 24 h IV/IM in 1 dose 4 PlusGentamicinsulfatec 3 mg/kg per 24 h IV/IM in 3 doses 2 Vancomycinhydrochlorided 40 mg/kg 24 h IV in 2 or 3 doses 4

46. 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