Acquired Heart Disease

1. Acquired Heart Disease Mohammed Alghamdi, MD, FRCPC (peds), FRCPC (card), FAAP, FACC Assistant Professor and Consultant Pediatric Cardiology, Cardiac Science King Fahad Cardiac Centre King Saud University

2. Acquired Heart Disease • Rheumatic Fever • Kawasaki Disease • Infective Endocarditis • Pericarditis • Myocarditis • Cardiomyopathy • Cardiac Arrhythmias: Any case scenario of heart failure and a heart rate more than 200 is arrhythmia until proven other wise. The commonest arrhythmia in children is SVT.

3. Rheumatic Fever • RF: most common cause of acquired heart disease in developing countries. • 150 in 100,000 in developing countries (more common in developing) • 1 in 100,000 in developed countries. • Rheumatic heart disease (RHD) inflammatory changes to cardiac valves and myocardium. • RF is the commonest acquired heart disease in developing countries while Kawasaki is the commonest in developed countries.

4. Rheumatic Fever • Precipitated by Group A Streptococcal (GAS) pharyngitis (not other types of GAS infections) • Acquiring the disease depends on the host susceptibility which depends on genetic, environmental, socioeconomic and immunological status. • 2-4 weeks after untreated GAS Pharyngitis • T-cell and B-cell lymphocytes produce antibodies against some Streptococcal antigens that cross-react with antigens on myocytes or cardiac valve tissue. • RF no heart sequale unlike RHD. RHD will lead to rheumatic fever which will lead to well established disease.

5. It is not due to the infection itself rather due to cross-reactivity between myocytes and bacterial antigens. • Not from one infection but clinical + sub-clinical infection leading to acute rheumatic fever, and if it is not recognized rheumatic heart disease.

6. Jones criteria (1992) Rheumatic Fever Evidence of antecedent GAS infection 1. Positive throat culture or rapid antigen test for GAS 2. Raised or rising streptococcal antibody titre

7. Rheumatic Fever Jones criteria (1992) • Two majors or one major and two minors plus evidence of antecedent GAS infection. ASO titer may be –ve; repeat in 2-4 weeks if there is a high index of suspicion. • Exceptions: • Chorea & indolent carditis do NOT require evidence of antecedent GAS infection • Recurrent episode requires : • Only one major OR • Several minor manifestations • Plus evidence GAS infection

8. Rheumatic Fever • Arthritis: • Most common ARF presentation (75% of first attacks) • Migratory, Asymmetrical, Polyarthritis, of large joints • Typically: extremely painful • Highly responsive to salicylate and NSAID therapy • Within 3 days • Monoarthritisif history of early use of NSAID • Premature abortion the polyarthritis manifestation. • You can’t give aspirin if patient has already started on NSAID

9. Rheumatic Fever • Cardiatis: • Valvulitis: • Isolated MV is the commonest followed by MV plus AV then AV, and rarely affects the right side. • Early disease leads to valvular regurgitation, • Prolonged or recurrent attacks lead stenosis • Pericarditis and myocarditis may occur • RHD is less rare in children less than 5 ears because it requires recurrent infection.

10. Rheumatic Fever • Sydenham’s chorea • Jerky, uncoordinated movements esp. of hands, feet, tongue and face. • Disappear during sleep • More common in adolescence female • Occur after a prolonged latent period following GAS • 6 wks - 3 yrs following GAS infection • Strong association with carditis • In sydenhamchorea, it’s RHD until proven otherwise. Because it can happen 3 years post-GASwhere it’s hard to find any evidence of strep infection. Therefore, anyone with chorea should do echo. • Usually seen in people who have beautiful handwriting and suddenly drops.

11. Rheumatic Fever • Erythemamarginatum • Rare & difficult to detect esp. in dark-skinned patients • Circular patterns of bright pink macules or papules that blanch under pressure • Trunk and proximal extremities • Almost never on face • Not itchy or painful vs eczema

12. Rheumatic Fever • Subcutaneous nodules • Rare < 2% of cases, • Highly specific manifestation of ARF but it is not sensitive. • Round, firm, mobile and painless nodules • Appear 1-2 weeks after onset of other symptoms • last 1-2 weeks (rarely > 1 month) • Strongly associated with carditis

13. Rheumatic Fever • Treatment: • Antibiotics to eradicate of residual GAS bacteria • Anti-inflammatory agents • High dose aspirin or NSAIDs. • Steroids for severe carditis • CHF therapy as indicated • Primary prevention by preventing the infection from happening, secondary prevention to prevent repeated infection (because RHD does it occur from the first attack it needs several episodes to occur. • Sydenham Chorea: • Haloperidol

14. Rheumatic Fever Secondary prophylaxis Secondary Prophylaxis

15. Rheumatic Fever Duration of Secondary Prophylaxis

16. Damage to the heart is permanent whereas the arthritis.

17. Kawasaki Disease • KD: inflammatory disease of unknown etiology causing acute, diffuse vasculitis of medium size blood vessels (mainly coronary arteries). • Most common cause of acquired heart diseases in developed countries. • More common in Japanese population • Higher in males than females • Typical age: 6 months - 5 years

18. Kawasaki Disease • Cardiac Involvement: • Coronary arteries( late): if left untreated the risk of CAD is 25%, this is reduced to 2% with treatment. • Dilation and aneurysm formation • Thrombus formation • Fibrosis and stenosis • Myocardial infarction. • May cause myocarditis and endocarditis • Other medium size arteries • Axiliary, femoral, iliac, and renal arteries.

19. Kawasaki Disease • Fever:at least 5 days duration • At least 4 of the following (to call it complete typical KD). • Conjunctivitis:bilateral, non-purulent • Skin Rash: polymorphous skin rash • Mucous membrane changes:red, dry, and cracked lips, strawberry tongue • Extremities changes:palms/soles erythema, hands/ feet edema, Skin peeling • Cervical lymphadenopathy:unilateral and >1.5 cm in diameter

20. Kawasaki Disease • DDX of KD: • Scarlet fever • EBV infection • Adenovirus infection • Staphylococcal scalded skin syndrome • Drug reactions • Stevens–Johnson syndrome.

21. Kawasaki Disease • Atypical (incomplete) KD: • Cases of KD that do not fulfill diagnostic criteria. • More common in infants • Children with • Fever greater than 5 days • Two or three classic symptoms • Supporting laboratory abnormalities or echo evidence of coronary involvements • Should be treated as KD

22. Kawasaki Disease • Labs abnormalities in KD: • Elevated ESR > 50 (70%) • Elevated C-reactive protein (50%) • CBC: • Neutropenia , leukocytosis (50%) • Nonspecific anemia • Thrombocytosis more than 500,000 is very pathagnomonic. • Marker of KD • Not seen until 2nd week of the disease • Elevated liver transaminases (40%) • Low serum albumin level • Sterile pyuria (33%) • Aseptic meningitis (up to 50%).

23. Kawasaki Disease • Treatments: • IVIG 2 g/kg • 2nd dose if persistent fever within 48 h of the initial dose • High dose of aspirin of 30–100 mg/kg/day. • Once afebrile: aspirin is decreased to 3–5 mg/kg/day • Aspirin acts as an anti-inflammatory agent if give in high doses for long time, analgesic if given in medium doses when needed and antiplatelet if given in small doses for long time. • Echo at base line • Repeat echocardiogram and inflammatory markers at 6–8 weeks • If normal coronary arteries and inflammatory markers : aspirin can be stopped • If coronary artery abnormalities: long-term Rx with aspirin • Other anticoagulants warfrin if giant aneurysm of coronary arteries

24. Infective Endocarditis • infection of the endocardial lining of the heart or cardiac vessels • Rare but with high mortality • Usually affect abnormal cardiac structure • Valvular disease • Septal defects • Presence of foreign material such as mechanical valves and patch material after surgical repair.

25. Infective Endocarditis • Etiology: • Gram- positive bacteria: • > 90 % 0f bacterial case • Streptococci Viridans : most common infectious agent • Staphylococcal species – especially prosthetic valves. • Enterococci : less common pediatric age group. • Gram-negative bacteria: • < 10% of bacterial cases • Example: HACEK group • Haemophilus, Actinobacillus, Cardiobacterium, Eikenella and Kingella • Fungal – uncommon • Neonate, immunocompromised patients, prolonged antibiotic • 10% IE case: organisms cannot be identified.

26. Infective Endocarditis • Clinical Features: • General: • Fever • Nonspecific manifestations • myalgias, arthralgias, headache, and general malaise • Cardiac: • New onset or worsening valvular regurgitation • Congestive heart failure • Heart block

27. Infective Endocarditis • Clinical Features: • Extracardiac - septic embloism: ischemic changes can occur anywhere even in the brain causing stroke. • CNS: infraction, brain abscess • Renal: proteinuria, hematuria, pyuria • PHx - Stigmata of SBE: • Janewaylesions:Non-tender, flat lesions on palms and soles. • Osler’s nodes: Tender, raised nodules on fingers and toes. • Roth’s spots: Exudative lesions of the retina. • Splinter hemorrhages: Non-blanching, linear, under nails. • Splenomegaly

28. Infective Endocarditis • Labs: • Blood cultures are the most important lab test • Three blood cultures collected over a 24-h period from different sites at different times. Because if only one of the cultures is positive it could be contamination. • CBC: • Anemia • Elevated ESR/CRP • Positive rheumatoid factor

29. Infective Endocarditis • Treatment: • Supportive medical therapy • Prolonged antibiotic therapy • Initially empiric broad spectrum Abx which then adjusted according to organism sensitivity. Admit for I.V Abx with a combination of two or three drugs. • 4-8 weeks course depending on the type of organism and resistance. • Removal of infected line • Surgical intervention: may be required

30. Infective Endocarditis • SBE prophylaxis: • Only indicated at dental procedures or respiratory procedure (not for gynecological procedures anymore) for high risk patients • No longer recommended for GI or GU procedures • Prophylaxis regimens: • Oral amoxicillin 50 mg/kg (up to 2 g) OR • IV/IM ampicillin 50 mg/kg • Patients allergic to penicillin • Cephalexin 50 mg/kg PO (up to 2 g) OR • Clindamycin 20 mg/kg (up to 600 mg) OR • Azithromycin (or clarithromycin) 15 mg/kg (up to 500 mg)

31. Infective Endocarditis

32. Myocarditis • Inflammatory infiltrate of the myocardium with necrosis/degeneration of the myocytes • Viral infections: most common causes in order: • Coxsackievirus type B • Adenovirus • Parvovirus B19 • Others: CMV, EBV, HIV, Hep C • Other infectious causes: bacteria, rickettsiae, protozoa. • Non-infectious causes: • Rheumatologic disease: SLE, rheumatoid arthritis • Drugs and Toxins: chemotherapy

33. Myocarditis • Variable initial presentation • Viral prodrome: fever, URTI or GI symptoms precede the S/S of HF by 3 weeks. • Hx: lethargy, poor feeding, irritability, respiratory distress, exercise intolerance • PHx: • Signs of CHF • Tachycardia • Tachypnia (increase work of breathing) • Gallop rhythm and murmur of mitral regurgitation • Hepatomegaly

34. Myocarditis • Investigation: • CXR: • Cardiomegaly • Pulmonary congestion • ECG: • Sinus tachycardia • Low voltage QRS • Non-specific T wave changes • Echo: • Dilated LV with reduced systolic function • MR • Pericardial effusion

35. Myocarditis • Investigation: • Myocardial biopsy: • Historically used to be the gold standard test • Currently not routinely performed • Invasive • low sensitivity of the procedure (3–63%) • patchy involvement of the myocardium • Treatment: • Cardio-respiratory Support: • Inotropes, Ventilation, ECMO • Anti-CHF therapy • IVIG and Steroid – not supporting evidence for their benefit • Antiviral agents and interferon – need further studies.

36. Cardiomyopathy • Cardiomyopathy: myocardial disease resulting in thickening of myocardial fibers or fibrosis. • Pediatric cardiomyopathy: almost exclusively non- ischemic • Types: • Dilated cardiomyopathy (58%) the commonest, must rule out arrhythmia. • Hypertrophic cardiomyopathy (30%) syncope while exercising might lead to sudden cardiac death in athletics because of ventricular tachycardia progressing to ventricular fibrillation. • Restrictive cardiomyopathy (5%) • Arrhythmogenic Right Ventricular Cardiomyopathy ARVC (5%)