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Mellss paeds yr3 rheumatic heart disease
- 1. Nur Amalina Aminuddin Baki 0820121000 67
- 2. Introduction Most common form of acquired HD Was leading cause of death among children
- 3. Epidemiology Predisposing factors Poor socioeconomy Unhygienic living condition Overcrowding Genetic Declining in improved development Age: 5- 15 years old Gender are equally affected Girls: Mitral valve disease, chorea Boys : Aortic valve disease
- 4. Etiology Unknown Strong association with beta hemolytic GAS More frequent: M type 1,3,5,6,18,24 Not associated : M type 4 However, streptococci never been isolated from rheumatic lesions Evidence that it is an antigen- antibody reaction
- 5. Pathogenesis Cytotoxic theory: Streptolysin O Immunologic theory : Cross reactivity between GAS components and mamallian tissues Antigenic determinants M protein – human tropomysin and myosin
- 6. Clinical features Streptococcal sore throat with fever Recurrence of fever with manifestation of acute rheumatic fever Revised Jones Criteria
- 7. Major criteria: oint Migratory polyarthritis of large joint Redness, warmth, swelling, pain , movement limitation Early manifestation ,30-50% Quick to appear, lasts 3- 7 days, subside and appear in other joint Respond to salicylates Inverse relationship between severity of arthritis and carditis Commoner in older patients
- 8. Major criteria: Carditis Early manifestation 90% (within 2 weeks of onset) Pancarditis Pericarditis : precordial pain, friction rub, ST and T changes Endocarditis: pansystolic murmur of MR w/wo AR murmur Others : cardiac enlargement, soft S1, S3 gallop, congestive cardiac failure, Carey Coombs’ murmur
- 9. Major criteria: odules Late manifestation 3- 20% ( 6 weeks after onset) Non- tender subcutaneous nodules on bony prominence Pinhead to almond size Lasts from days to weeks
- 10. Major criteria: rythema marginatum Early manifestation <3% Erythematous, serpiginous,non- pruritic, macular lesions with pale centre Increasing size, coalesce with adjacent spots serpiginous outline More over the trunk,extremities
- 11. Major criteria : ydenham’s Chorea Late manifestation 10-15% ( 3 months after onset) Semi- purposeful , jerky movements deranged speech, muscular incoordination, facial grimacing Exacerbated by stress and disappears with sleep More common in females Clinical maneuvers to elicit chorea: Demostrate milkmaid’s grip ‘spooning ’ appearance of extended ‘Bags of worms’ tongue when protruded Handwriting examination Self- limiting ( 2-6 weeks)
- 12. Minor criteria Supporting evidence of GAS infection Clinical features: • Arthalgia • Fever • Positive throat culture / rapid test Laboratory features: • Elevated acute phase reactants • Prolonged PR interval • Elevated ASO titre
- 13. WHO criteria for diagnosis Primary episode : 2 / 1+2 with supporting evidence Recurrent attack Without RHD:2 / 1+2 with supporting evidence With RHD:2 minors with supporting evidence Rheumatic chorea, insidious onset rheumatic carditis: not needed
- 14. Differential Diagnosis Arthritis Carditis Chorea • Rheumatoid arthritis • Viral myocarditis • Huntington chorea • Reactive arthritis • Viral pericarditis • Wilson disease • Serum sickness • IE • SLE • Sickle cell disease • Kawasaki disease • Cerebral palsy • Malignancy • Congenital heart disease • Tics • SLE • Mitral valve prolapse • Lyme disease • Gonococcal infection
- 15. Treatment
- 16. Symptomatic therapy Bed rest Recommended Continued for 1-3 months in carditis with CCF Diet Salt restriction if present with CCF and not responding well to treatment Penicillin Single injection of Benzathine penicillin Penicillin V ( 250mg qid X 10 days) Erythromycin ( 250mg qid X 10 days)
- 17. Anti-inflammatory therapy Guidelines: Carditis with CCF: steroids Carditis without CCF: steroids or aspirin No carditis: aspirin Aspirin (100mg/kg in 4 divided doses X 3-5 days, 75mg/kg in 4 divided doses X 4 weeks) Prednisolone ( 2mg/kg daily; max dose 60mg X 3 weeks , tapered in next 9 weeks)
- 18. Surgical replacement of valve Deteriorating patient despite aggressive decongestive measures Management of chorea Reassurence of parents Rest in a quiet room and medications Phenobarbitol (16-32mg/6-8h PO) Haloperidol or chlorpromazine
- 19. Primary Prevention Identify and treat streptococcal sore throat Educate community
- 20. Secondary Condition Duration of prophylaxis • Without carditis • 5 years or till 18 years old • Without carditis • 10 years or till 25 years old • Established RHD/ valve surgery • Lifelong Antibiotics Dose • Benzathine penicillin • IM 1.2 or 0.6 million units every 3-4 weeks • High prevalence area (every 3 or 2 weeks) • Penicillin V • 250mg BD oral • Erythromycin • 250mg BD oral
- 21. Prognosis Risk for residual heart disease increases with: Severity of initial episode Presence of recurrence
- 22. Sequelae
- 23. 1)Mitral Regurgitation Examination: Increased resting pulse rate with displaced, forcible apex and hyperkinetic precordium,systolic thrill, LVF Auscultation : Soft S1, splittted S2, S3, pansystolic murmur, delayed diastolic murmur ECG: normal axis with sinus tachycardia Roentrogenogram : cardiac enlargement ECHO: enlarged LA, LV Differential diagnosis: ASD ( primum), coarctation of aorta with congenital MR, Marfan and Hurler syndrome
- 24. Treatment Medical : digitalis, diuretics, systemic vasodilators ( ACEI, CCB) and penicillin prophylaxis Mitral valve surgery Persistent symptoms despite medications Evidence of ventricular dysfunction
- 25. 2) Mitral Stenosis More common in boys Symptoms: SOB, cough , hemoptysis , PND Examination: Small pulse volume, tachypnea, RVF, prominent ‘a’ wave, tapping apex beat, parasternal impulse, apical diastolic thrill Auscultation : prominent S1, splittted S2, opening snap, delayed diastolic murmur ECG: right axis deviation with RV hypertrophy Roentrogenogram : features of pulmonary hypertension ECHO: LA enlarged, narrow mitral opening Severity: increases as OS closer to S2, ECHO with Doppler
- 26. Differential diagnosis: Isolated congenital MS, cotriatriatum, left atrial myxoma Treatment Beta blocker or digoxin to improve diastolic filling Diuretics Percutaneous trans-septal mitral commmisurotomy or closed mitral valvulotomy
- 27. 3)Aortic Regurgitation More in boys Symptoms: palpitation Examination: wide pulse pressure, Corrigan sign, dancing peripheral arteries, waterhammer pulse , de Musset sign, Hill sign, pistol shot sounds,Duroziez sign, displaced apex Auscultation : soft S1, diastolic murmur, ejection systolic murmur ECG: deep S wave in V1, tall R wave in V6, diastolic overloading pattern ( deep Q wave, tall T wave) Roentrogenogram : LV enlargement and dilated ascending aorta ECHO:LV enlarged, dilated aorta, anterior mitral leaflet flutter
- 28. Differential diagnosis: Wide pulse pressure (PDA, anemia, thyrotoxicosis) Non rheumatic regurgitant diastolic murmur (pulmonary regurgitation, AR with VSD, congenital aortic valve disease) Marfan syndrome, Hurler syndrome, Takayasu arteritis Treatment Calcium channel blockers Aortic valve replacement
- 29. 4) Tricuspid Regurgitant Symptoms: right hypochondrium pain Examination: prominent ‘V’ wave, displaced apex beat, Auscultation : pansystolic murmur ECG: severe RV hypertrophy ECHO with Doppler : assess nature and severity Management Decongestive measures Mitral valvulotomy Tricuspid annuloplasty or repair
- 30. Reference Vinod K Paul, Arvind Bagga, Ghai Essential Pediatrics, 8th edition, 2014 Kliegman, Stanton, St.Geme, Behrmann, Nelson Textbook of Pediatrics, 19th edition, 2011.
Editor's Notes
- MC 50% of C adm 50/100000 Death: US at beg 20th cent, 100-200/ 100000500.5 0.3% after streptococcal throat infection 1-3% in epidemics streptococcal sore throat
- 66% have ho o URTI few weeks before Peak age n seasonal parallelto GAS Serological evidence of recent GAS infc Otbreaks of GAS outbreaks RF Anti microbial GAS prevent RF
- Hurdle: inability to establish animal model Cyto : x explain latent period btw phargitis n RF