rheumatic heart disease complete notes pptx

RHD

1. Acute Rheumatic Fever & RHEUMATIC HEART DISEASE Dr. KHHT

2. Acute rheumatic fever is a systemic disease of childhood, often recurrent that follows group A beta hemolytic streptococcal infection. It is a delayed non- suppurative sequelae to URTI with GABH streptococci. It is a diffuse inflammatory disease of connective tissue, primarily involving heart, blood vessels, joints, subcutaneous tissue and CNS.

3. Epidemiology Ages 5-15 yrs are most susceptible Rare <3 yrs Girls >boys Common in 3rd world countries Environmental factors-- over crowding, poor sanitation, poverty Incidence more during winter & early spring

4. Group A Beta Hemolytic Streptococcus Strains that produce rheumatic fever – M types 1, 3,5,6, 18 &24 Pharyngitis – produced by GABHS can lead to acute rheumatic fever, rheumatic heart disease and post streptococcal glomerulo nephritis. Skin infections –produced by GABHS leads to post streptococcal glomerulonephritis only. It will not result in rheumatic fever or carditis as skin lipid cholestrol inhibit antigenicity .

5. PATHOPHYSIOLOGY More support for an autoimmune phenomenon (Type II hypersensitivity reaction) During strep infection, antigen presenting cells present bacterial antigen to helper T cells. These helper T cells then activate B cells to induce production of antibodies against the Streptococcal cell wall. These antibodies can also interact with other cells in the body (for example, myocardium or joints, etc) producing the symptoms responsible with acute rheumatic fever.

6. After a latent period of 1-3 weeks, antibody induced immunological damage occur to heart valves, joints, subcutaneous tissue & basal ganglia of brain Most patient have elevated antibody titers to at least one streptococcal antibody Streptolysin O Hyaluronidase Streptokinase

7. Pathologic Lesions Fibrinoid degeneration of connective tissue, inflammatory edema, inflammatory cell infiltration & proliferation of specific cells resulting in formation of Ashcoff nodules , resulting in- - Pancarditis in the heart - Arthritis in the joints - Aschoff nodules in the subcutaneous tissue - Basal ganglia lesions resulting in chorea

8. Clinical Features 1.Arthritis Flitting & fleeting migratory polyarthritis , involving major joints Commonly involved joints- knee, ankle, elbow & wrist Occur in 80%, involved joints are exquisitely tender In children below 5 yrs arthritis usually mild but carditis more prominent Arthritis do not progress to chronic disease

9. 2.Carditis Manifest as pancarditis ( endocarditis , myocarditis and pericarditis ),occur in 40-50% of cases Carditis is the only manifestation of rheumatic fever that leaves a sequelae & permanent damage to the organ Valvulitis occur in acute phase Chronic phase- fibrosis, calcification & stenosis of heart valves ( fishmouth valves)

10. Rheumatic heart disease. Abnormal mitral valve. Thick, fused chordae

11. 3.Sydenham Chorea Occur in 5-10% of cases Mainly in girls of 1-15 yrs age May appear even 6/12 after the attack of rheumatic fever Clinically manifest as-clumsiness, deterioration of hand-writing, emotional lability or grimacing of face Chorea – purposeless, jerky, non-repetitive, involuntary movement of proximal parts of limb with facial grimacing. Lasts 2-4 months

12. 4.Erythema Marginatum Occur in <5%. Unique, transient, serpiginous -looking lesions of 1-2 inches in size Pale center with red irregular margin More on trunks & limbs & non-itchy Worsens with application of heat Often associated with chronic carditis May last from weeks to months

13. 5.Subcutaneous nodules Occur in 10% Firm, painless, pea-sized ( up to 2 cm )palpable nodules Mainly over extensor surfaces of joints, spine, scapulae & scalp Associated with strong seropositivity Always associated with severe carditis Usually persist for 1-2 weeks

14. Other features (Minor features) Fever- (up to 101˚ F) Arthralgia Pallor Anorexia Loss of weight

15. Rheumatic fever is mainly a clinical diagnosis No single diagnostic sign or specific laboratory test available for diagnosis Diagnosis based on MODIFIED JONES CRITERIA

16. Diagnosis JONES CRITERIA Developed by Dr. T Duckett Jones in 1944 Need 2 major criteria or 1 major and 2 minor criteria in the presence of a prior strep infection to make the diagnosis Evidence of prior strep infection with positive throat culture or antigen test, elevated streptococcal antibody titer, or history of rheumatic fever/heart disease

17. Major Criteria Migratory Polyarthritis Carditis Subcutaneous Nodules Erythema Marginatum Sydenham’s Chorea (St. Vitus ’ Dance)

18. MINOR CRITERIA Fever Arthralgia : joint pain without inflammation Elevated CRP, ESR or leucocytosis EKG changes: primarily prolonged PR interval Evidence of Group A Streptococcal infection via elevated antistreptolysin O titer or DNAase Prior history of rheumatic fever or heart disease

19. Exceptions to Jones Criteria Chorea alone, if other causes have been excluded Insidious or late-onset carditis with no other explanation Patients with documented RHD or prior rheumatic fever, one major criterion or of fever, arthralgia or high CRP suggests recurrence.

20. Laboratory Findings High ESR Anemia, leucocytosis Elevated C-reactive protien ASO titre >200 Todd units.(Peak value attained at 3 weeks, then comes down to normal by 6 weeks) Anti- DNAse B test Throat culture-GABH streptococci

21. ECG - prolonged PR interval, 2nd or 3rd degree blocks, ST depression, T inversion 2D Echo cardiography - valve edema, mitral regurgitation, LA & LV dilatation, pericardial effusion, decreased contractility

22. Differential Diagnosis Juvenile rheumatiod arthritis Septic arthritis Sickle-cell arthropathy Kawasaki disease Myocarditis Scarlet fever Leukemia

23. Treatment Step I - primary prevention (eradication of streptococci) Step II - anti inflammatory treatment ( aspirin,steroids ) Step III - supportive management & management of complications Step IV - secondary prevention (prevention of recurrent attacks)

24. STEP I: Primary Prevention of Rheumatic Fever (Treatment of Streptococcal Tonsillopharyngitis ) Agent Dose Mode Duration Benzathine penicillin G 600 000 U for patients Intramuscular once 27 kg (60 lb) 1 200 000 U for patients >27 kg or Penicillin V Children : 250 mg 2-3 times daily Oral 10 d ( phenoxymethyl penicillin) Adolescents and adults: 500 mg 2-3 times daily For individuals allergic to penicillin Erythromycin: 20-40 mg/kg/d 2-4 times daily Oral 10 d Estolate (maximum 1 g/d) or Ethylsuccinate 40 mg/kg/d 2-4 times daily Oral 10 d ( maximum 1 g/d)

25. Step II: Anti inflammatory treatment For Arthritis only- Aspirin 75-100 mg/kg/day four divided doses for 6 weeks. For Carditis - Prednisolone – 2-2.5 mg/kg/day as two divided doses for 2 weeks, taper over 2 weeks and while tapering, add Aspirin 75mg/kg/day for 2 weeks, continue Aspirin alone 100 mg/ kg/day for another 4 weeks.

26. Step III: Supportive management & management of complications Bed rest Treatment of congestive cardiac failure: - digitalis, diuretics Treatment of chorea: - diazepam or haloperidol Rest to joints & supportive splinting

27. STEP IV : Secondary Prevention of Rheumatic Fever (Prevention of Recurrent Attacks) Agent Dose Mode Benzathine penicillin G 1 200 000 U every 4 weeks* Intramuscular or Penicillin V 250 mg twice daily Oral or Sulfadiazine 0.5 g once daily for patients 27 kg (60 lb Oral 1.0 g once daily for patients >27 kg (60 lb) For individuals allergic to penicillin and sulfadiazine Erythromycin 250 mg twice daily Oral *In high-risk situations, administration every 3 weeks is justified and recommended

28. Duration of Secondary Rheumatic Fever Prophylaxis Category Duration 1. Rheumatic fever with At least 10 yr since carditis and residual ht disease last episode and ( persistent valvular disease) at least age 40yr, sometimes life long prophylaxis may be needed 2. Rheumatic fever with 10 yr or well into adulthood carditis with no residual disease whichever is longer (no valvular disease) 3. Rheumatic fever without carditis 5 yr or until age 21 yr whichever is longer

29. WHO GUIDELINES At least 5 years of prophylaxis or if child until age 18 if not cardiac involvement 10 years prophylaxis or if child until age 25 if has mild mitral regurgitation Lifelong prophylaxis if has severe valve disease

30. Prognosis Rheumatic fever can recur whenever the individual experience new GABH streptococcal infection, if not on prophylactic medicines Good prognosis for older age group & if no carditis during the initial attack Bad prognosis for younger children & those with carditis with valvular lesion

31. Generally resolve over 12 weeks in 80% of patients Prognosis depends on severity of cardiac involvement during acute attack Primary Prevention: treatment of streptococcal infection (limitation is that 30% of streptococcal infections are subclinical) Secondary Prevention: prevention of recurrent attacks of Rheumatic Fever with prophylactic antibiotics.

32. Rheumatic heart disease Rheumatic Heart Disease is the permanent heart valve damage resulting from one or more attacks of ARF. It is thought that 40-60% of patients with ARF will go on to developing RHD. The commonest valves affecting are the mitral and aortic, in that order. However all four valves can be affected.

33. Differences between acute carditis and established valvular heart disease. Acute carditis 1. pericarditis with or without effusion chest pain pericardial rub ECG – ST elevation, flat or inverted T (V4 – V6). 2. myocarditis soft heart sounds with tachycardia ECG — prolonged PR to more than 0.18 s, flattened T waves prolonged QT interval can also occur   acute heart failure 3. endocarditis systolic murmur and Carey- Coomb’s murmur (in the mitral area)

34. Estabished valvular heart disease Mitral stenosis - RVH - loud 1st sound MDM +/- presystolic murmur in mitral area - loud P2 (if pulmonary hypertension is present) Mitral regurgitation - Systolic thrill, PSM in mitral area and radiation to the axilla - LVH Aortic incompetence - Collapsing pulse, wide pulse pressure - EDM in upper and middle left sternal border with radiation to the apex and to the aortic area - Left ventricular hypertrophy

35. The mitral valve is most commonly and severely affected (65-70% of patients), and the aortic valve is second in frequency (25%). The tricuspid valve is deformed in only 10% of patients and is almost always associated with mitral and aortic lesions. The pulmonary valve is rarely affected. Pericarditis , when present, rarely affects cardiac function or results in constrictive pericarditis .

36. Mitral Regurgitation Clinical Features no symptoms in mild cases fatigue, easily tired exertional dyspnoea , palpitation, paroxysmal nocturnal dyspnoea , orthopnoea , oedema heart enlargement heaving apex beat systolic thrill apical PSM radiating to axilla

37. Investigations CXR -Heart enlarged (LA & LV prominence), Perihilar congestion (pulmonary venous hypertension) ECG - LAH & RAH, LVH , LAD   Echocardiogram - LA enlargement & LVH, Severity of regurgitation

38. Mitral Stenosis Clinical Features no symptoms initially exercise intolerance & dyspnoea , orthopnoea , paroxysmal nocturnal dyspnoea . paroxysmal nocturnal dyspnoea . haemoptysis , hepatomegaly , ascites oedema moderate cardiomegaly , apical impulse is tapping left parasternal heave or epigastric pulsation (RVH) loud S1, opening snap MDM with presystolic accentuation at the apex loud pulmonary 2nd sound (if pulmonary hypertension is present)

39. Investigations CXR mitralization – straightening of left heart border left atrial enlargement (elevated left bronchus & double right border of the heart) prominent pulmonary artery and prominent right sided heart chambers Lungs - greater perfusion in upper lobes Kerley B lines (horizontal, at costophrenic angle) Bat’s wing hilum (alveolar oedema ) Pulmonary hypertension - prominent pulmonary conus , translucent peripheral lung fields

40. ECG LAH , RVH, RAD Echocardiogram LA enlargement & RVH, Severity of stenosis

41. Aortic Regurgitation Clinical Features no symptoms unless severe palpitations exertional dyspnoea orthopnoea collapsing pulse wide pulse pressure enlarged heart left ventricular apical heave EDM best over upper left sternal border with radiation to the apex and to aortic area.

42. Investigations CXR -left ventricle & aortic knuckle enlargement ECG - LVH Echocardiogram - LVH & LAH

43. Mortality/Morbidity Rheumatic heart disease is the major cause of morbidity from rheumatic fever and the major cause of mitral insufficiency and stenosis in the United States and the world. Variables that correlate with severity of valve disease include the number of previous attacks of rheumatic fever, the length of time between the onset of disease and start of therapy, and sex. The disease is more severe in females than in males. Insufficiency from acute rheumatic valve disease resolves in 60-80% of patients who adhere to antibiotic prophylaxis.

44. A diagnosis of rheumatic heart disease is made after confirming antecedent rheumatic fever. Physical findings in a patient with rheumatic heart disease include cardiac and non-cardiac manifestations of acute rheumatic fever. Some patients develop cardiac manifestations of chronic rheumatic heart disease.

45. CARDIAC MANIFESTATIONS Pancarditis is the most serious and second most common complication of rheumatic fever (50%). In advanced cases, patients may complain of dyspnea , mild-to-moderate chest discomfort, pleuritic chest pain, edema, cough, or orthopnea . Upon physical examination, carditis is most commonly detected by a new murmur and tachycardia out of proportion to fever. New or changing murmurs are considered necessary for a diagnosis of rheumatic valvulitis . Other cardiac manifestations include congestive heart failure and pericarditis .

46. The murmurs of acute rheumatic fever are typically due to valve insufficiency. The following murmurs are most commonly observed during acute rheumatic fever: Apical pansystolic murmur is a high-pitched, blowing-quality murmur of mitral regurgitation that radiates to the left axilla . Apical diastolic murmur (also known as a Carey-Coombs murmur) is heard with active carditis and accompanies severe mitral insufficiency.. Basal diastolic murmur is an early diastolic murmur of aortic regurgitation and is high-pitched, blowing, decrescendo, and heard best along the right upper and mid-left sternal border after deep expiration while the patient is leaning forward .

47. Congestive heart failure Heart failure may develop secondary to severe valve insufficiency or myocarditis . The physical findings associated with heart failure include tachypnea , orthopnea , jugular venous distention, rales , hepatomegaly , a gallop rhythm, edema, and swelling of the peripheral extremities.

48. NON CARDIAC MANIFESTATIONS Common noncardiac (and diagnostic) manifestations of acute rheumatic fever include polyarthritis , chorea, erythema marginatum , and subcutaneous nodules. Other clinical, non-cardiac manifestations include abdominal pain, arthralgias , epistaxis , fever, and rheumatic pneumonia.

49. CARDIAC MANIFESTATIONS OF CHRONIC RHD Valve deformities, thromboembolism , cardiac hemolytic anemia, and atrial arrhythmias are the most common cardiac manifestations of chronic rheumatic heart disease. Mitral stenosis occurs in 25% of patients with chronic rheumatic heart disease and in association with mitral insufficiency in another 40%. Progressive fibrosis ( ie , thickening and calcification of the valve) takes place over time, resulting in enlargement of the left atrium and formation of mural thrombi in that chamber.

50. IMAGING STUDIES Cardiomegaly , pulmonary congestion, and other findings consistent with heart failure may be seen on chest radiography.

51. Doppler-echocardiogram In acute rheumatic heart disease, Doppler-echocardiography identifies and quantitates valve insufficiency and ventricular dysfunction. With mild carditis , Doppler evidence of mitral regurgitation may be present during the acute phase of disease but resolves in weeks to months. The most important echocardiographic features of mitral regurgitation from acute rheumatic valvulitis are annular dilatation, elongation of the chordae to the anterior leaflet, and a posterolaterally directed mitral regurgitation jet. During acute rheumatic fever, the left ventricle is frequently dilated in association with a normal or increased fractional shortening. In chronic rheumatic heart disease, echocardiography may be used to track the progression of valve stenosis and may help determine the time for surgical intervention.

52. Heart catheterization In acute rheumatic heart disease, this procedure is not indicated. With chronic disease, heart catheterization has been performed to evaluate mitral and aortic valve disease and to balloon stenotic mitral valves. Post catheterization precautions include hemorrhage, pain, nausea and vomiting, and arterial or venous obstruction from thrombosis or spasm. Complications may include mitral insufficiency after balloon dilation of the mitral valve, tachyarrhythmias , bradyarrhythmias , and vascular occlusion

53. OTHER TESTS On ECG, sinus tachycardia most frequently accompanies acute rheumatic heart disease. Alternatively, some children develop sinus bradycardia from increased vagal tone. First-degree atrioventricular (AV) block (prolongation of the PR interval) is observed in some patients with rheumatic heart disease. First-degree AV block is a nonspecific finding and should not be used as a criterion for the diagnosis of rheumatic heart disease.

54. Second-degree (intermittent) and third-degree (complete) AV block with progression to ventricular standstill have been described. When acute rheumatic fever is associated with pericarditis , ST segment elevation may be present and is marked most in lead II, III, aVF , and V 4 -V 6 . Patients with rheumatic heart disease also may develop atrial flutter, multifocal atrial tachycardia, or atrial fibrillation from chronic mitral valve disease and atrial dilation.

55. TREATMENT AND MANAGEMENT Medical therapy in rheumatic heart disease includes attempts to prevent rheumatic fever (and thus rheumatic heart disease). In patients who develop rheumatic heart disease, therapy is directed toward eliminating the group A streptococcal pharyngitis (if still present), suppressing inflammation from the autoimmune response, and providing supportive treatment for congestive heart failure. Following the resolution of the acute episode, subsequent therapy is directed towards preventing recurrent rheumatic heart disease in children and monitoring for the complications and sequelae of chronic rheumatic heart disease in adults.

56. Preventive and prophylactic therapy is indicated after rheumatic fever and acute rheumatic heart disease to prevent further damage to valves. Primary prophylaxis (initial course of antibiotics administered to eradicate the streptococcal infection) also serves as the first course of secondary prophylaxis (prevention of recurrent rheumatic fever and rheumatic heart disease).

57. Patients with rheumatic heart disease and valve damage require a single dose of antibiotics 1 hour before surgical and dental procedures to help prevent bacterial endocarditis . Patients who had rheumatic fever without valve damage do not need endocarditis prophylaxis.

58. If moderate to severe carditis is present as indicated by cardiomegaly , third-degree heart block or congestive heart failure, substitute PO prednisone for salicylate therapy. Continue prednisone for 2-6 weeks depending on the severity of the carditis , and taper prednisone during the final week(s) of therapy. Weaning prednisone therapy after a shorter period (2-4 weeks) while initiating and maintaining salicylates for several weeks can minimize adverse effects of the steroids while preventing rebound of the carditis .

59. digoxin and diuretics, afterload reduction, supplemental oxygen, bed rest, and sodium and fluid restriction are additional treatments for patients with acute rheumatic fever and heart failure. The diuretics most commonly used in conjunction with digoxin for children with heart failure include furosemide and spironolactone . Initiate digoxin only after checking electrolytes and correcting hypokalemia .

60. The total digitalizing dose is 20-30 mcg/kg PO, with 50% of the dose administered initially, followed by 25% of the dose 12 hours and 24 hours after the initial dose. Maintenance doses typically are 8-10 mcg/kg/d PO in 2 divided doses. For older children and adults, the total loading dose is 1.25-1.5 mg PO, and the maintenance dose is 0.25-0.5 mg PO every day. Therapeutic digoxin levels are present at trough levels of 1.5-2 ng / mL.

61. Afterload reduction ( i.e , using ACE inhibitor captopril ) may be effective in improving cardiac output, particularly in the presence of mitral and aortic insufficiency. Start these agents judiciously. Use a small, initial test dose (some patients have an abnormally large response to these agents), and administer only after correcting hypovolemia . When heart failure persists or progresses during an episode of acute rheumatic fever in spite of aggressive medical therapy, surgery is indicated and may be life-saving for severe mitral and/or aortic insufficiency.

62. Patients with rheumatic fever with carditis and valve disease should receive antibiotics for at least 10 years or until age 40 years.

63. Surgical Care When heart failure persists or worsens after aggressive medical therapy for acute rheumatic heart disease, surgery to decrease valve insufficiency may be life-saving. Forty percent of patients with acute rheumatic heart disease subsequently develop mitral stenosis as adults. In patients with critical stenosis , mitral valvulotomy , percutaneous balloon valvuloplasty , or mitral valve replacement m ay be indicated. Due to high rates of recurrent symptoms after annuloplasty or other repair procedures, valve replacement appears to be the preferred surgical option.

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