1. 1. Acquired Cardiovascular Disorders of Childhood

2. 2 Acute Rheumatic Fever ETIOLOGY:An immune reaction to untreated group A beta-hemolytic streptococcal infection, usually pharyngitis. It typically presents after a 2-6 week period following pharyngitis with one or more of the major or minor manifestations. It is a self-limiting disease, but may cause significant cardiac valve damage. PATHOLOGY: Rheumatic Fever is classified by the Jones Criteria who are divided into major and minor criteria:

3. 3 Jones Criteria: Major criteria Carditis – the most serious manifestation, diagnosed by changes on EKG, presence of a new murmur, especially Mitral regurgitation during systole or aortic regurgitation during diastole. Polyarthritis – common, caused by edema and swelling, inflammation and effusion in joint tissue, limited motion, tenderness, and erythema. Involves the large joints, knees, hips, elbows, wrists, and shoulders. It is a migratory type of arthritis, “migrates” to a different joint q1-2 days. It usually accompanies the acute febrile period in the first 1-2 weeks of the illness. Reversible. Erythema marginatum – uncommon, distinct pink macular rash with clear center usually on trunk and proximal extremities, transitory and non-pruritus, elicited by application of local heat. Will resolve.

4. 4 Jones Criteria: Subcutaneous nodules – uncommon, associated with repeated episodes and severe conditions; small (0.5-1 cm) nodules, firm, non-tender, present over bony prominences of elbows, knees, knuckles, ankles, scapulae, scalp and vertebrae. Persist indefinitely but will resolve. Chorea (Sydenham’s disease) “St. Vitus Dance” - uncommon, presents weeks to months after infection has resolved. Characterized by sudden, aimless, irregular movements of the extremities, involuntary facial grimacing, speech disturbances, emotional labiality, and profound muscle weakness. It is exaggerated by anxiety and attempts at fine, deliberative motor activity, relieved by rest, sleep. Reflects streptococcal infection of vascular tissue of brain and CNS. Increases in severity over first 2 weeks, reaches a plateau and begins to diminish and resolve over a 10 week period.

5. 5 Minor Criteria Fever (101-102) Arthralgia Previous RF Leucocytosis Elevated ESR and C reactive protein C-reactive protein – a protein normally found in serum with inflammatory conditions.  Before ESR (within 24-48hrs). Disappears when suppressed with salicylates, steroids. ESR- Erythrocyte sedimentation Rate Prolonged PR interval on EKG

6. 6 Criteria RF is diagnosed by the presence of one major + 2 minor, or 2 major + supporting evidence of recent GABHS infection (scarlet fever, + throat culture, elevated anti streptolysin O)

7. 7 Treatment and Nursing Care of RF: Bed rest during acute phase with fever to decrease workload of the heart. Provide child with quiet diversional activity in bed. NSAIDs (Motrin)for arthritic pain Steroids for severe, life-threatening Carditis only, since they do not reduce valular damage. Teach parents that steroids, ex. Prednisone cannot be discontinued suddenly, must be tapered down slowly. Penicillin to eradicate strep, erythromycin for penicillin allergy Penicillin prophylaxis is essential part of treatment: Bicillin 1.2 million unit’s IM q28 days, or monthly, for 10 years. Drastically reduces permanent damage to Mitral and aortic valves.

8. 8 Cultures/Titers RF can be prevented by doing throat cultures of all school-age children suspected of strep infection with GABHS. Anti-streptolysin-O titer – measure of concentration of antibodies against streptolysin-O, a toxin produced by GABHS.  7 days after infection and reaches maximum level in 4-6 weeks.

9. 9. KAWASAKI’S SYNDROME

10. 10 Kawasaki’s A multisystem disorder involving vasculitis (inflammation of the inner lining of arteries, veins and capillaries). Also called mucocutaneous lymph node syndrome. Characterized as an acute febrile illness associated with systemic vasculitis. Approx. 20% have cardiovascular complications.

11. 11 Kawasaki’s ETIOLOGY/PATHOPHYSIOLOGY: Unknown cause, generally affects young children 1-3 years, M>F, children under 1 year of age are most seriously affected by KD and are at the greatest risk for cardiac involvement. Asians > Blacks >Caucasians Increased incidence in winter to early spring Seasonal epidemics every 2-3 years Specific triggers - bacterial illness: strep viral: retrovirus

12. 12 Diagnostic Criteria The child must exhibit FIVE of the following six criteria, including fever. -Fever 5 or more days -Bilateral conjunctival injection (inflammation) without exudation -Changes in the oral mucosal membranes – dryness, erythema and fissuring of lips; “strawberry tongue”, oropharyngeal reddening

13. 13 Diagnostic Criteria -Changes in extremeties – peripheral edema, erythema of the palms and soles, and periungual desquamation (peeling) of the hands and feet -Polymorphous rash -Cervical lymphadenopathy (one lymph node >1.5cm)

14. 14 Kawasaki’s - Triphasal There are 3 phases to the disease: Acute phase: from onset of fever till resolution of fever (5-21days). Fever, high spiking 39.5- 40.5, does not respond to antibiotic and antipyretic therapy. Conjunctival exanthema: begins shortly after fever Swollen hands/feet Rash: begins 5 days after fever, fine, Erythematosus Enlarged cervical lymph nodes Child is VERY irritable ESR & platelet count are very elevated with potential for thrombosis occlusion of coronary arteries Cardiac: arrhythmias gallop rhythm, CHF with shock, pericarditis, pericardial effusion, cardiac tamponade, mitral or aortic valve regurgitation.

15. 15 Phases Subacute phase: from resolution of fever to disappearance of all clinical symptoms. Occurs 3-4 week after onset of fever. Lasts for 2-4 weeks. Peripheral extremity change: desquamation of fingers, toes palms of hands & feet. Changes in oral mucosa, dryness and fissuring of the lips, oropharyngeal redness, strawberry tongue. Cardiac: Coronary artery vasculitis, dilatation and or aneurysms of coronary arteries. Can first be detected at approx. 10 days. Peak occurrence for CA involvement is 3-4 weeks after onset Convalescent phase: ESR & platelet count returns too normal, usually lasts about 10-12 weeks.

16. 16 Neurologic Complications: Extreme irritability and lethargy Aseptic meningitis (25%) Hemiparesis or paralysis of extremities, ataxia Increased ICP Mild sensorineuronal hearing loss

17. 17 Treatment in acute phase: High dose IVIG (2g/kg),over 12hrs. Follow institutional policy on blood/blood product transfusions IVIG in conjunction with high dose ASA tx (80-100mg/kg/qd)-reduces fever and decreases probability of cardiac aneurysm formation. Once fever has subsided, ASA dose is decreased to antiplatelet dose (3-5mg/kg/qd). This low dose ASA is continued for 6-8 weeks.

18. 18 Nursing Considerations: Child needs both cardiology and infectious disease consult Continuous monitoring of cardiac status (EKG daily/echocardiogram) I&O Daily weight Assessment and prevention of dehydration (child is VERY prone to dehydration)

19. 19 Nursing Considerations: During IVIG infusion, follow protocol for blood transfusion Most nursing care focuses on symptomatic relief Skin care Mouth care. Parental support as child is extremely irritable and inconsolable. Follow institutional policy on blood product transfusions