Rheumatic Heart Disease

1. Rheumatic Heart Disease 4 th BPT 15-16

2. Introduction • RHD complicates Rheumatic fever and predominantly manifests with valvular damage. • RF is an inflammatory reaction following a Group A streptococcus infection (sore throat) resulting in antibody cross reaction. A type II hypersensitivity.

3. Epidemiology • A common cause of “valvular heart failure” • Incidence falling in developed countries probably due to widespread antibiotics use to treat streptococcus infection • Primarily affects children ages 5-15 • Occurs usually 20days post streptococcal pharyngitis. • >3% of untreated infections progress to RF, BUT incidence of recurrence is up to 50%

4. Diagnosis • modified JONES criteria 2major or 1major + 2minor • Major- migrating polyathritis, carditis erythema marginatum , subcutaneous nodules, Sydenham's chorea( St. vitus’ dance) • Minor- fever, athralgia, raised ESR/CRP, leucocytosis, serology(anti DNAse B, antistretolysin O) hx of RF, ECG( Hrt block, prolonged PR

5. PATHOPHYSIOLOGY • Anti stretococcus antibodies cross reacting with cardiac myosin, glycogen, arterial smooth muscles, • Neutrophil &macrophage recruitment(aschoff giant cells) • ASCHOFF bodies • Acutely- pancarditis, serofibrinous bread & butter pericaditis • Endocarditis- fibrinoid necrosis, and McCallum plaques along the heart valves. • Chronic-valvular damage( thickening, fusion, cord shortening)

6. Treatment of RF • CULTURE • Antibiotics- penicillin, erythromycin • Maintenance low dose penicillin • Anti inflammatory- steroids, aspirin • CCF- diuretics, Digoxin, corticosteroids

7. Mitral stenosis • >90% of RHD involve the mitral valve. • >50% of cases involve only the mitral valve. • >50% of MS complicates a case of RF • OTHER causes: congenital, metastasingcarcinoid tumour, lutembacher synd.

8. features • Valve outline thickening • Cusp fusion • Calcification • Stenosis of valve opening • Progressive immobility of cusps

9. Pathophysiology • Normal valve orifice = 5cm2 • Moderate stenosis = 2cm2 • Severe stenosis = 1cm2 • Stenosis— LA pressure rises— LAhypertrophies and dilates— pulmvesselspressurerises(oedema)—Rt hrt pressure rises—RVH and dilatation —RHF

10. SYMPTOMS • ASSYMPTOMATIC IN EARLY DISEASE • With progression of stenosis and back pressure build up symptoms appear with worsening progression • Due pulm venous hypertension— • dyspnoea • cough + pink frothy sputum • Due to Rt Hrt Failure— • weakness, early satiety, tender hepatomegally, ascites and pedal oedema • Due to RA hypertrophy– • AF • Mural thrombi— CVD, mesenteric or renal infarction, PVD

11. SIGNS • Face: mitral facies (cheek flush) • Pulse: low volume, irregular (late as AF sets in) • JVP: raised, prominent a-wave • Praecordium—RVH, palpable mitral S1(M1) • AUSCULTATION: Pliable valve—loud M1(opening snap) mid diastolic murmur immobile valve—silence!

12. Investigation • CXR: small Hrt + large LA. calcification of MV. features of pulm oedema- kerley B lines, bat’s wing opacity, dilated prominent upper lobe vessels. • ECG: P-MITRALE( bifid p wave) due delayedLA activation. Rt axis deviation & tall R wave due to RVH • Echocardiography- valves, chambers • Cardiaccatheterization- measure pressure gradient

13. TREATMENT • .MILD stenosis– symptomatic relief and prevention of Cx • Prophylactic antibiotics • Tx of bronchitic attacks • Diuretics • Digoxin —AF • Anticoagulant— warfarin

14. Tx (contd) • Severe – surgery • Valvotomy- Trans septal balloon valvotomy closed open • Mitral valve replacement

15. Cx • ATRIAL FIBRILLATION • EMBOLIZATION • PULMONARY HYPERTETION • PULMONARY INFARCTION • CHEST INFECTIONS • INFECTIVE ENDOCARDITIS • RIGHT HEART FAILURE

16. MITRAL REGURGITATION • Pathophysiology- • chronic: prog LA dilatationm +little pressure rise • Acute: LA pressure rises + little dilatation • Pulm hypertension and oedema due to back pressure • Stroke vol reduces– rise in lt hrt workload— LHH

17. Symptoms • Pulm oedema- DYSPNOEA • Low CO- fatigue, lethargy, syncope • RHF— • Cardiac cachexia • Subacute I.E • Thromboembolism (rarer)

18. SIGNS • PULSE- rapid, low volume • Praecordium- hyper • Apex beat- displaced • Auscultation- S1 IS SOFT as valve is incompletely closed prior to diastole pan systolic murmur- loudest at apex, radiating over praecordium & to axilla. Prominent S3

19. Investigations • CXR : cardiomegally , LA, LV enlargement valve calcification • ECG: P-mitrale • LVH– tall R (1,V6), deep S waves (V1,V2) • ECHO: valves, chambers, regurgitant jet (Doppler, TOE) • Catheterization

20. Treatment • Mild MR with no symptoms— prophylactic antibiotics, serial echocardiography • Worsening state- MV replacement • Palliation- ACEI, diuretics, anticoagulants

21. Aortic stenosis • RHD – 40% in aortic valve damage & usually with MV • STENOSIS—CO falls—compensatory ventricular hypertrophy—(exercise induced angina, arrhythmias)—LHF

22. Symptoms • Early disease state is usually asymptomatic • Symptoms occur years later and death usually 2-3yrs thereafter. • Symptoms include- • exercise induced syncope, angina, • dyspnoea, orthopnoea, PND (LHF)

23. Signs • PULSE- sinus, low volume, slow rising carotid impulse • Bp– falls • Precordium—hyper active, systolic thrill, • Apex beat– not displaced • Auscultation– loudA2(ejection click) silent A2(immobile calcified cusps) MURMURS—Systolic. Radiates to carotid & praecordium,

24. Investigation • CXR—Increase CTR dilated ascending Aorta (post stenotic dilatation) calcification • ECG—pressure overload/ventricular strain pattern---ST depration, T-wave inversion • ECHO—valves, LVH, pressure gradient (doppler) • Catheterization • Coronary angiography

25. Treatment • Asymptomatic- Prophylactic antibiotics & echo reviews • Surgery- valvotomy, valvoplasty. aortic valve replacement.

26. AR • REFLUX blood from the aorta into the left ventricle through a damaged AV in diastole • reduction in CO( FALL IN DBP) • LVH– regurgitated volume, compensatory • Cardiac ischemia

27. Symptoms • Late usually after LVF • Pounding of heart • Exercise intolerance—weakness, syncope, • Angina pectoris. • Dyspnoea • arrhythmias

28. SIGNS • Due to hyper dynamic state , reflux and increase LV size • Pulse – bounding/collapsing • Quincke’s sign –capillary pulsation in nail bed • De mussetz’s sign—head nodding with pulsation • Duroziez’s sign—to &fro murmur heard when femoral A is auscultated + distal pressure • Pistol shot femoralis—sharp bang heard on auscultation over the femoral A with each heart beat

29. Signs • BP—low DBP • Precordium- hyperactive. • Apex- displaced, forceful • Auscultation- • Early diastolic murmur- high pitched, Lt sternal edge (4ICS). Leaning forward & holds breath in expiration • Austin flint’s mid-diastolic murmur– when the regurgitant blood impinge on the ant mitral cusp. • Ejection systolic murmur—due to volume overload

30. Investigation • CXR- LVH, dilated asending aorta, calcification • ECG— sinus rhythm, LVH( tall R, inverted T, deep S) • ECHO– vigorous cardiac contraction, LV dilatation, enlarged aortic root, diastolic mitral valve & septal fluttering(Austin Flint) • Doppler- regurgitant jet • Catheterization • Angiography

31. Treatment • Pre symptom surgery—valve replacement • Prophylactic Antibiotics– I.E

32. Conclusion